Hydrops and Congenital Diaphragmatic Hernia: Reported incidence and postnatal outcomes. Analysis of the CDHSG Registry

Hydrops and Congenital Diaphragmatic Hernia: Reported incidence and postnatal outcomes. 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Analysis of the CDHSG Registry Carmen Mesas Burgos, Ashley Ebanks, Anna Löf-Granström, Kylie I Holden, and 3 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-3753580/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 30 May, 2024 Read the published version in Journal of Perinatology → Version 1 posted 10 You are reading this latest preprint version Abstract Obejctive: Congenital Diaphragmatic Hernia (CDH) associated with hydrops is rare. The aim of this study was to describe the incidence of this combination of anomalies and the postnatal outcomes from a large database for CDH. Study design: Data from the multicenter, multinational database on infants with prenatally diagnosed CDH (CDHSG Registry) born from 2015 to 2021 were analyzed. Results: A total of 3985 patients were entered in the registry during the study period, 3156 were prenatally diagnosed and 88 were reported to have associated fluid in at least 1 compartment, representing 2.8 % of all prenatally diagnosed CDH cases in the registry. The overall survival to discharge for CDH patients with hydrops was 43%. The hydropic CDH group had lower birth weight and gestational age at birth, and increased incidence of right sided CDH (55%) and rate of non-repair (45%). However, the survival rate for hydropic infants with CDH undergoing surgical repair was 80%. Other associated anomalies were more common in hydropic CDH (50% vs 37%, p=0.001). Conclusion: Hydropic CDH is rare, only 2.8% of all prenatally diagnosed cases, and more commonly occurring in right sided CDH. Survival rates are low, with higher rates of non-repair. However, early decision-making regarding goals of care and an aggressive surgical approach may result in survival rates comparable to non-hydropic cases. Health sciences/Risk factors Health sciences/Medical research/Outcomes research congenital diaphragmatic hernia hydrops prenatal prognosis outcome Figures Figure 1 Figure 2 Introduction Congenital diaphragmatic hernia (CDH) is a complex and life-threatening malformation, with an incidence of 2–3/10 000 live births (1). Overall survival rates are still low, ranging from 65–85% (1, 2, 3, 4). The prenatal detection rate with obstetrical ultrasound for CDH will vary depending on the setting but in average is approximately 60–80% (4, 5). Due to the heterogeneity of this malformation, it is difficult to accurately predict outcomes in individual cases (6, 7). Risk stratification according to several severity features allows for more adequate counseling. Prenatal factors associated with worse outcome include decreased gestational age or weight at diagnosis, decreased lung volume (observed to expected lung to head ratio (O/E LHR, US) or total fetal lung volume (TFLV, MRI), thoracic liver location and percent herniation, thoracic stomach position, and the presence of associated structural or chromosomal anomalies (8, 9, 10, 11, 12, 13, 14, 15). Hydrops is a prenatal, clinical entity characterized by the presence of fluid collection in two or more body compartments, including skin or scalp edema, ascites, hydrothorax or pericardial effusion (16, 17, 18). Non-immune hydrops fetalis may be caused by various genetic syndromes or a consequence of hyperdynamic circulation in the fetus that leads to high-output cardiac failure (16, 18). In the case of fetal thoracic anomalies such as CDH, hydrops may result from increased systemic venous pressure caused by obstruction of venous return to the heart. The combination of hydrops and CDH is rare, with only a few series and case reports published, and is generally associated with a worse outcome (19, 20, 21). The objective of the current study was to increase knowledge of CDH with associated hydrops, describe the incidence of this association, and investigate postnatal outcomes using a large, multi-institutional cohort of CDH patients. Material and Method Data from the Congenital Diaphragmatic Hernia Study Group (CDHSG) Registry on infants with CDH born at or transferred to any of the participating centers reporting to the registry were collected. During the specified study period, there were 83 participating centers that submitted data to the registry (specified in the Appendix 1 ), which includes prospectively collected information on newborns with CDH until death or discharge. The registry started in 1995. The variables collected have been updated several times and the data sheet is currently in version 5 of data collection. In 2007, when the third version was introduced, the CDH Study Group Staging System for diaphragm defect size was introduced (22) ( Appendix 2 ), and with the fourth version in 2015, prenatal information including fetal ultrasound, magnetic resonance imaging (MRI), echocardiography, and genetic information were added. Thus, in this study we focused on all infants reported to the CDHSG with a prenatal diagnosis between January 2015 and December 2021. Data from patients with prenatally diagnosed CDH entered in the registry with reported fluid/edema in at least 1 compartment were analyzed and compared to the group of prenatally diagnosed CDH without compartmental fluid/edema. We further performed a sub-analysis of the different compartment/compartments with fluid. True hydrops was defined as fluid, edema, and/or effusions in two or more fetal compartments. Since in CDH there is a communication between the thorax and abdomen through the diaphragmatic defect, inherent to the condition, these two compartments were considered a single compartment and thus non-true hydrops. Associated variables evaluated included perinatal characteristics, number of compartments with excess of fluid, prenatal intervention, survival rates, defect size and side, associated malformations, use of extra-corporeal life support (ECLS), timing of surgical repair and rates of non-repair, length of hospital stay (LOS) and need for oxygen at 30 days. Data are presented as absolute values (n), percentages (%), odds ratio (OR) and 95% confidence interval (CI). Survival to discharge, need for ECLS, risk for death within the first 7 days of life, rates of non-repair and need of oxygen at 30 days were used as end points in the analysis. For categorical data, Fisher’s test was performed to investigate differences between groups. For continuous variables, t-test or Mann-Whitney test were used. Logistic regression was used when appropriated to quantify the strengths of the association. Significance was defined as p ≤ 0.05. Analyses were performed using the R software version 2.38. The CDHSG registry has been approved for use by the Committee for the Protection of Human Subjects (CPHS) / Institutional Review Board (IRB) of the McGovern Medical School at UT Health in Houston (protocol number: HSC-MS-03-223). Results Fluid in at least one compartment A total of 3985 patients were entered in the registry during the study period, 3156 of those were prenatally diagnosed and 88 cases were reported to also have fluid in at least one compartment, representing 2.8% of all CDH cases, with an overall survival to discharge of 43% compared to 70% for CDH cases lacking fluid in any compartment, p = 0.001 (Table 1 , Fig. 2). Table 1 Background data for the cohort of prenatally diagnosed CDH, comparing patients with reported fluid in at least 1 compartment to non-reported-hydropic Fluid in compartment/’s Non-hydrops P value Prenatally diagnosed (n = 3156) 88 3068 Sex female (%) 42.0 44.3 0.772 EGA (median [IQR]) 37.00 [34.00, 38.00] 38.00 [37.00, 39.00] < 0.001 BirthWeigth (median [IQR]) 2.83 [2.32, 3.22] 2.93 [2.50, 3.28] 0.103 Side (%) Left 42.0 86.1 < 0.001 Right 55.7 12.9 Central 1.1 0.0 Bilateral 1.1 0.8 Defect Size (%) A 2.2 8.1 0.680 B 37.5 34.0 C 45.8 40.1 D 14.6 17.8 O/E LHR (median [IQR]) 44.95 [32.00, 52.62] 40.77 [30.84, 53.00] 0.552 Non-Repairs (%) 45.5 14.7 < 0.001 Type of Repair (%) Patch 66.7 63.5 < 0.001 ECLS (%) 34.1 30.2 0.480 Assoc anomalies (%) 50.0 37.3 0.01 Type of Assoc anomaly (%) Cardiac 26.1 24.3 0.018 Chromosomal 11.4 9.6 Other 27.3 15.7 Several 18.2 11.0 Isolated 50.0 62.7 O2 at 30D (%) 44.3 45.6 < 0.001 Survival to discharge (%) 43.2 70.3 < 0.001 DOL at surgery (median [IQR]) 4.50 [2.00, 11.00] 4.00 [2.00, 7.00] 0.136 DOL at Death (median [IQR]) 1.00 [0.00, 10.75] 10.00 [1.00, 32.00] < 0.001 LOS (median [IQR]) 51.00 [24.50, 93.00] 48.50 [29.00, 86.00] 0.694 DOL: Day of life, DC: Discharge, IQR: Interquartile range, O/E LHR: observed/expected lung to head ratio, ECLS: Extracorporeal Life Support *Significance P < 0.05 There was no significant difference in median birth weight or gestational age (GA) at birth between the groups. However, prematurity (born < 34 wks EGA) was associated with higher mortality in the CDH cohort with fluid in at least 1 compartment (p = 0.027, OR 4.75, CI 1.24–20.2). The CDH group with fluid in at least one compartment had an increased incidence of right sided CDH (55% vs 12.9%, p < 0.001) and higher rates of non-repairs (45.5% vs 14.7%, p < 0.001) (Table 1 ). However, when undergoing surgical repair, survival rates for prenatally diagnosed CDH with fluid in at least 1 compartment was 80% (Table 2 ). The utilization of ECLS was similar in both groups (34% vs 30%) (Table 1 ), with a 36.8% survival to discharge (Table 2 ). The length of hospital stay was similar for CDH with fluid in at least 1 compartment compared to prenatally diagnosed CDH, as was the need for O 2 at 30 days. Other associated anomalies were more common in prenatally diagnosed CDH with fluid in at least 1 compartment (50% vs 37%, p = 0.05). (Table 1 ) Table 2 Patient characteristics survival vs demised Overall Discharged alive Demised p value n 88 38 50 Hydrops with fluid at least in 2 compartments n (%) (“true hydrops”) 14 (15.9) 3 (21.4) 11 (78.6) 0.08 Hydrops with fluid in only 1 compartment or combined ascites + pleural effusion (%) 74 (84.1) 35 (47.3) 39 (52.7) 0.048 Fluid Distribution in different compartments, (%) : Ascites 10 (13.0) 4 6 Ascites + Pericardial effusion 1 (1.3) 0 1 Ascites + Pericardial effusion + Pleural effusion 2 ( 2.6) 0 2 Ascites + Pericardial effusion + Pleural effusion + Skin edema 1 ( 1.3) 0 1 Ascites + Pleural effusion 11 (14.3) 8 3 Ascites + Pleural effusion + Skin edema 5 ( 6.5) 0 5 Ascites + Skin edema 1 ( 1.3) 1 0 Pericardial effusion 6 ( 7.8) 3 3 Pericardial effusion + Pleural effusion 2 ( 2.6) 2 0 Pleural effusion 32 (41.6) 18 14 Pleural effusion + Skin edema 2 (2.6) 0 2 Skin edema 4 (5.2) 1 3 Associated anomalies n (%) 44 (50.0) 16 (42.1) 28 (56.0) 0.282 Type of associated anomaly n (%) Cardiac 12 (13.6) 7 (18.4) 5 (10.0) Chromosomal 4 (4.5) 2 (5.3) 2 (4.0) Dysmorphic features 1 (1.1) 0 (0.0) 1 (2.0) Other 11 (12.5) 1 (2.6) 10 (20.0) Several 16 (18.2) 6 (15.8) 10 (20.0) Isolated 44 (50.0) 22 (57.9) 22 (44.0) Side n (%) Left 37 (42.0) 9 ( 24.3 ) 28 (75.7) 0.003 Right 49 ( 55.7 ) 27 (55.1) 22 (44.9) Central 1 (1.1) 1 (2.6) 0 (0.0) Bilateral 1 ( 1.1) 1 (100) 0 Non-repair (%) 40 (45.5) 0 40 ( 80.0 ) < 0.001 Surgical repair (%) 48 (54.5) 38 (80.0) 10 (20.0) < 0–001 Survival to DC (%) 38 (43.2) 38 0 ECLS (%) 30 ( 34.1 ) 14 ( 36.8 ) 16 ( 32.0 ) 0.656 DC: Discharge, ECLS: Extracorporeal Life Support There was a higher rate of associated anomalies among CDH patients with fluids in at least 1 compartment who died before discharge (56 vs 42%)(Table 2 ). True hydrops The distribution of fluid collection in the different compartments is summarized in Table 3 and Fig. 1 . Only 14 patients had fluid collections in more than one compartment (excluding the combination pleural effusion and ascites for reasons noted above), representing the “true” hydrops cohort with an incidence of only 0.35%, and having a high mortality rate of 79%. Table 3 Sub-analysis of true vs non-true hydrops Fluid in > 1 compartment Fluid in 1 compartment (or combined ascites + pleural effusion) P value n 14 74 Side (%) Left 3 ( 21.4) 34 (45.9) < 0.001 Right 11 ( 78.6 ) 38 (51.4) Central 0 ( 0.0) 1 ( 1.4) Bilateral 0 ( 0.0) 1 ( 1.4) Repair Done (%) 7 ( 50.0) 41 (55.4) 0.775 Patch Repair (%) 5 ( 71.4) 27 (65.9) 1.000 ECLS (%) 6 ( 42.9) 24 (32.4) 0.542 Associated anomalies (%) 4 ( 28.6) 40 ( 54.1 ) 0.143 Type of associated anomaly (%) Cardiac 1 ( 7.1) 11 (14.9) 0.489 Chromosomal 0 ( 0.0) 4 ( 5.4) Dysmorphic features 0 ( 0.0) 1 ( 1.4) Other 0 ( 0.0) 11 (14.9) More than 1 3 ( 21.4) 13 (17.6) Isolated 10 ( 71.4) 34 ( 45.9 ) O2 requirement at 30 DOL (%) 5 ( 100.0 ) 34 (72.3) 0.314 Survival to discharge (%) 3 (21.4 ) 35 ( 47.3 ) 0.086 DOL at surgery (median [IQR]) 4.00 [2.00, 8.50] 5.00 [3.00, 11.00] 0.617 DOL at Death (median [IQR]) 1.00 [0.00, 23.00] 1.00 [0.50, 8.50] 0.895 DOL at DC (median [IQR]) 67.00 [46.50, 221.00] 50.00 [22.00, 93.00] 0.433 DOL: Day of life, DC: Discharge, IQR: Interquartile range, ECLS: Extracorporeal Life Support *P < 0.05 In the group of true hydrops (fluid in more than one compartment) there was a high rate of right sided CDH (78.6%), skin edema was present in 64.3%, surgical repair was done in 50% of the cases with a survival to discharge in the surgically treated group of 43%. Only 28.7% of true hydropic CDH had associated anomalies compared to the group of “non true hydrops” (54.1%) (Table 3 ). In the event of isolated skin edema, there is a higher mortality risk before discharge (OR 5.08, 95% CI 1.25–34.3). Side Left sided CDH with fluid in at least one compartment had lower survival rates than right sided CDH with fluid in at least 1 compartment ( 24 vs 55% , OR = 3.82, p = 0.005), and higher rates of associated anomalies (67.6 vs 34.7, p = 0.004) (Table 4 ). Table 4 Sub-analysis of side (left to right side only) l Left Right p-value Hydrops n = 88 37 49 EGA (median [IQR]) 37.00 [34.00, 38.00] 37.00 [34.00, 38.00] 0.654 FETO n(%) 1 (2.7) 4 (8.2) 0.3 Non-repairs (%) 75.7 24.5 < 0.001 Patch Repair (%) 66.7 64.9 1.000 ECLS (%) 16.2 44.9 0.006 Associated anomalies (%) 67.6 34.7 0.004 Type of Associated anomaly (%) Cardiac 10.8 14.3 0.014 Chromosomal 8.1 2.0 Other 18.9 8.2 Several 27.0 10.2 Isolated 32.4 65.3 O2 att 30DOL (%) 53.8 81.1 0.073 Survival to dscharge (%) 24.3 55.1 0.005 DOL at surgery (median [IQR]) 7.00 [4.00, 11.00] 4.00 [2.00, 9.00] 0.387 DOL at Death (median [IQR]) 1.00 [0.00, 3.25] 3.00 [1.00, 34.50] 0.043 LOS (median [IQR]) 31.00 [3.25, 57.25] 59.00 [30.00, 95.00] 0.071 DC: Discharge, ECLS: Extracorporeal Life Support, DOL: Day of life, IQR: Interquartile range, FETO: Fetoscopic endotracheal occlusion The rates of non-repairs were higher in the left-sided CDH with fluids in at least one compartment compared to right-sided (75% vs 24.5%, p < 0.0001) (Table 2 ). Discussion In this prospective multicenter study, we conclude that hydropic CDH is rare, only 2.8% of all prenatally diagnosed cases, and more commonly occurring in right sided CDH. Survival rates are low, with higher rates of non-repair. Hydrops is a clinical entity characterized by the presence of skin and scalp edema or fluid in two or more body compartments. In cases of fetal thoracic anomalies, such as CDH, it is thought to be caused by obstruction of venous return to the heart leading to elevated systemic venous pressures. When using the term ‘hydrops’ in CDH, effusions in the thoracic cavity and abdominal cavity concomitant to the side of the defect, should be considered as one compartment for obvious reasons, avoiding confusion. Thus, true hydrops in CDH should refer to patients with excess fluid in at least two non-concomitant compartments. However, the presence of excess of fluid in only one compartment is associated with worse outcome. Even if the association is rare, nearly 3% of all prenatally diagnosed CDH will present with fluid in 1 compartment, the survival rates are low (47.3%, Table 3 ). True hydropic CDH is even more rare, only identified in 0.35% of the prenatally diagnosed CDH cases, and more commonly occurring in right sided CDH. Survival rates for true hydropic CDH are very low: 78% of patients with true hydrops will not survive to discharge. Malformations are present in half of the CDH with fluid / edema, true hydrops (fluid in at least two compartments) or non-true hydrops (fluid in 1 compartment), and more common among those who died before discharge (56% vs 42%, Table 2 ). Skin edema seems to be an independent risk factor for worse outcome, with higher mortality. However, even though the pathophysiological mechanisms underlying this event remain unknown, an increased central venous pressure and/or obstructed lymphatic drainage may be the most plausible explanations. [refs] Although the pathogenesis of hydrops in fetuses with CDH is poorly understood, right-sided lesions seem to be more likely to cause hydrops in CDH. Most of the cases presented here were right sided and/or had a major portion of the liver in the chest, thus the pathophysiology may be similar to other intrathoracic lesions, with mediastinal obstruction from solid organ herniation obstructing blood return to the heart. Since there is a confined space within the thorax, the herniation of a solid organ such as the liver, leaving a smaller portion intraabdominally, may lead to kinking and obstruction of the inferior vena cava, portal or hepatic vein. Despite hydrops being more commonly associated with right sided CDH, in as many as half of the patients with hydrops have a right sided defect, left sided CDH has a poorer outcome, with higher rates of associated anomalies, high rates of non-repairs and is overrepresented among children who died before discharge (75% of the left sided CDH with hydrops will demise, probably due to the complexity of the CDH). Even if the rates of non-repairs are high, a more aggressive surgical approach leads to acceptable survival rates: 80% of the patients being repair will survive to discharge. ECLS support is offered to the same extent to patients who survive and those hydrops patients who died before discharge. In conclusion, the association of CDH and hydrops in the fetus is rare and the sole presence of excess fluid or edema in 1 compartment is associated with poorer outcome. The combination is associated with the presence of other associated anomalies and right-sided defects with liver involvement. Special attention should be given to the presence of skin edema resulting in higher mortality rates. An initially aggressive surgical approach may result in acceptable survival rates comparable to non-hydropic cases. Early decision-making regarding the aims of care clearly influence outcome, since survival varies depending on the severity of the CDH and the presence of other associated anomalies. Declarations Author contributions: Carmen Mesas Burgos – Study conception, analysis and interpretation of data, drafting of manuscript, critical revision of manuscript Ashley H Ebanks – Acquisition of data, analysis and interpretation of data, critical revision of manuscript Anna Löf-Granström- critical revision of manuscript Kylie I. Holden- critical revision of manuscript Anthony Johnson- critical revision of manuscript Peter Conner- critical revision of manuscript Matthew T. Harting - analysis and interpretation of data, critical revision of manuscript All authors have consent for publication All data is available upon request Conflict of interests There are no conflict of interests. Level of evidence : I for a prognosis study – This is a high-quality, prospective cohort study with 99% of patients followed to the study end point (death or discharge) References Burgos CM, Frenckner B. Addressing the hidden mortality in CDH: A population-based study. J Pediatr Surg. 2017;52(4):522-5. Lally P. Report from the Congenital Diaphragmatic Hernia Study Group Database. CDHSG [Webbsite / Database] 2008 2/4/2008 [cited 2010 6/1/2010]; C]. Available from: http://cdhsg.net/ 2008 [ Mesas Burgos C, Hammarqvist-Vejde J, Frenckner B, Conner P. Differences in Outcomes in Prenatally Diagnosed Congenital Diaphragmatic Hernia Compared to Postnatal Detection: A Single-Center Experience. Fetal Diagn Ther. 2015. Burgos CM, Frenckner B, Luco M, Harting MT, Lally PA, Lally KP, et al. Prenatally versus postnatally diagnosed congenital diaphragmatic hernia - Side, stage, and outcome. J Pediatr Surg. 2018. Monier I, Lelong N, Benachi A, Jouannic JM, Khoshnood B, Zeitlin J. Postnatal diagnosis of congenital anomalies despite active systematic prenatal screening policies: a population-based registry study. Am J Obstet Gynecol MFM. 2023;5(11):101170. Haroon J, Chamberlain RS. An evidence-based review of the current treatment of congenital diaphragmatic hernia. Clin Pediatr (Phila). 2013;52(2):115-24. Danzer E, Hedrick HL. Controversies in the management of severe congenital diaphragmatic hernia. Semin Fetal Neonatal Med. 2014;19(6):376-84. Akinkuotu AC, Cruz SM, Abbas PI, Lee TC, Welty SE, Olutoye OO, et al. Risk-stratification of severity for infants with CDH: Prenatal versus postnatal predictors of outcome. J Pediatr Surg. 2016;51(1):44-8. Jani J, Keller RL, Benachi A, Nicolaides KH, Favre R, Gratacos E, et al. Prenatal prediction of survival in isolated left-sided diaphragmatic hernia. Ultrasound Obstet Gynecol. 2006;27(1):18-22. Hedrick HL, Danzer E, Merchant A, Bebbington MW, Zhao H, Flake AW, et al. Liver position and lung-to-head ratio for prediction of extracorporeal membrane oxygenation and survival in isolated left congenital diaphragmatic hernia. Am J Obstet Gynecol. 2007;197(4):422 e1-4. Cannie M, Jani J, Meersschaert J, Allegaert K, Done E, Marchal G, et al. Prenatal prediction of survival in isolated diaphragmatic hernia using observed to expected total fetal lung volume determined by magnetic resonance imaging based on either gestational age or fetal body volume. Ultrasound Obstet Gynecol. 2008;32(5):633-9. Gucciardo L, Deprest J, Done E, Van Mieghem T, Van de Velde M, Gratacos E, et al. Prediction of outcome in isolated congenital diaphragmatic hernia and its consequences for fetal therapy. Best Pract Res Clin Obstet Gynaecol. 2008;22(1):123-38. Jani JC, Benachi A, Nicolaides KH, Allegaert K, Gratacos E, Mazkereth R, et al. Prenatal prediction of neonatal morbidity in survivors with congenital diaphragmatic hernia: a multicenter study. Ultrasound Obstet Gynecol. 2009;33(1):64-9. Cordier AG, Jani JC, Cannie MM, Rodo C, Fabietti I, Persico N, et al. Stomach position in the prediction of survival in left-sided congenital diaphragmatic hernia with or without fetoscopic endoluminal tracheal occlusion. Ultrasound Obstet Gynecol. 2014. Lee JY, Jun JK, Lee J. Prenatal prediction of neonatal survival in cases diagnosed with congenital diaphragmatic hernia using abdomen-to-thorax ratio determined by ultrasonography. J Obstet Gynaecol Res. 2014;40(9):2037-43. Neveling S, Knippel AJ, Kozlowski P. Isolated non-immune hydrops fetalis: an observational study on complete spontaneous resolution, perinatal outcome, and long-term follow-up. Arch Gynecol Obstet. 2023;308(2):487-97. Vanaparthy R, Mahdy H. Hydrops Fetalis. StatPearls. Treasure Island (FL)2023. Wei X, Yang Y, Zhou J, Zhou X, Xiong S, Chen J, et al. An Investigation of the Etiologies of Non-Immune Hydrops Fetalis in the Era of Next-Generation Sequence-A Single Center Experience. Genes. 2022;13(12). Ibrahim M, Hunter M, Gugasyan L, Chan Y, Malhotra A, Sehgal A, et al. Interstitial deletion of chromosome 1 (1p21.1p12) in an infant with congenital diaphragmatic hernia, hydrops fetalis, and interrupted aortic arch. Clin Case Rep. 2017;5(2):164-9. Rossi A, Delabaere A, Delmas-Laurichesse H, Beaufrere AM, Lemery D, Gallot D. The challenge of prenatal identification of congenital diaphragmatic hernia in the context of hydrops. Eur J Obstet Gynecol Reprod Biol. 2014;182:238-9. Sydorak RM, Goldstein R, Hirose S, Tsao K, Farmer DL, Lee H, et al. Congenital diaphragmatic hernia and hydrops: a lethal association? J Pediatr Surg. 2002;37(12):1678-80. Lally KP, Lasky RE, Lally PA, Bagolan P, Davis CF, Frenckner BP, et al. Standardized reporting for congenital diaphragmatic hernia--an international consensus. J Pediatr Surg. 2013;48(12):2408-15. Additional Declarations There is NO conflict of interest to disclose. Supplementary Files AppendixCenters.docx Appendix 1 Appendix2CDHSGStaging.pdf Appendix 2 CDHBurgos05052022.xls Data Set 1 Cite Share Download PDF Status: Published Journal Publication published 30 May, 2024 Read the published version in Journal of Perinatology → Version 1 posted Editorial decision: revise 12 Mar, 2024 Review # 2 received at journal 01 Mar, 2024 Reviewer # 2 agreed at journal 05 Feb, 2024 Review # 1 received at journal 03 Feb, 2024 Reviewer # 1 agreed at journal 21 Jan, 2024 Reviewers invited by journal 26 Dec, 2023 Submission checks completed at journal 18 Dec, 2023 First submitted to journal 16 Dec, 2023 Unknown event 15 Dec, 2023 Editor assigned by journal 14 Dec, 2023 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. 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Huston","correspondingAuthor":false,"prefix":"","firstName":"Anthony","middleName":"","lastName":"Johnson","suffix":""},{"id":263548621,"identity":"a2192393-197e-4683-8419-83dcbe4e558a","order_by":5,"name":"Peter Conner","email":"","orcid":"","institution":"Karolinska University Hospital","correspondingAuthor":false,"prefix":"","firstName":"Peter","middleName":"","lastName":"Conner","suffix":""},{"id":263548622,"identity":"f50c8afe-d694-47f1-befd-2735ec113edc","order_by":6,"name":"Matthew Harting","email":"","orcid":"https://orcid.org/0000-0002-8929-8311","institution":"McGovern Medical School, University of Texas, Houston","correspondingAuthor":false,"prefix":"","firstName":"Matthew","middleName":"","lastName":"Harting","suffix":""}],"badges":[],"createdAt":"2023-12-14 12:51:34","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-3753580/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-3753580/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1038/s41372-024-02010-5","type":"published","date":"2024-05-30T04:00:00+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":49080113,"identity":"b1be7581-858a-4562-b30b-27c35e8231c1","added_by":"auto","created_at":"2024-01-02 19:54:13","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":817555,"visible":true,"origin":"","legend":"\u003cp\u003eFluid distribution in the different compartments.\u003c/p\u003e","description":"","filename":"Figure1hydrops.png","url":"https://assets-eu.researchsquare.com/files/rs-3753580/v1/dac0a5b144a205cfdab997cf.png"},{"id":49081430,"identity":"90f7f842-df7f-4b39-8aa7-e41a6ecc25b7","added_by":"auto","created_at":"2024-01-02 20:02:13","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":5692,"visible":true,"origin":"","legend":"\u003cp\u003eThis image is not available with this version.\u003c/p\u003e","description":"","filename":"placeholderimage.png","url":"https://assets-eu.researchsquare.com/files/rs-3753580/v1/638bdf2ac5622cb17fc7993d.png"},{"id":57709512,"identity":"43a2f88f-da31-4b6e-95bb-89182d088d4c","added_by":"auto","created_at":"2024-06-04 15:34:38","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":2105606,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-3753580/v1/b230e591-40bd-47a0-866c-fa06d5ee5f56.pdf"},{"id":49080116,"identity":"f715efa2-964b-4281-8e17-435a1d29234a","added_by":"auto","created_at":"2024-01-02 19:54:13","extension":"docx","order_by":1,"title":"","display":"","copyAsset":false,"role":"supplement","size":21318,"visible":true,"origin":"","legend":"\u003cp\u003eAppendix 1\u003c/p\u003e","description":"","filename":"AppendixCenters.docx","url":"https://assets-eu.researchsquare.com/files/rs-3753580/v1/f6da59a33d97a77048d7fc72.docx"},{"id":49080115,"identity":"089f3e07-ba8e-4f08-8006-9117a8d907bf","added_by":"auto","created_at":"2024-01-02 19:54:13","extension":"pdf","order_by":2,"title":"","display":"","copyAsset":false,"role":"supplement","size":216816,"visible":true,"origin":"","legend":"\u003cp\u003eAppendix 2\u003c/p\u003e","description":"","filename":"Appendix2CDHSGStaging.pdf","url":"https://assets-eu.researchsquare.com/files/rs-3753580/v1/1dbb9757c05b1b224ad8e639.pdf"},{"id":49080117,"identity":"ffc47ff8-d07c-4876-a838-d16dfed309f7","added_by":"auto","created_at":"2024-01-02 19:54:13","extension":"xls","order_by":3,"title":"","display":"","copyAsset":false,"role":"supplement","size":5598720,"visible":true,"origin":"","legend":"Data Set 1","description":"","filename":"CDHBurgos05052022.xls","url":"https://assets-eu.researchsquare.com/files/rs-3753580/v1/4cee9aed1c935d69684c953f.xls"}],"financialInterests":"There is \u003cb\u003eNO\u003c/b\u003e conflict of interest to disclose.","formattedTitle":"Hydrops and Congenital Diaphragmatic Hernia: Reported incidence and postnatal outcomes. Analysis of the CDHSG Registry","fulltext":[{"header":"Introduction","content":"\u003cp\u003eCongenital diaphragmatic hernia (CDH) is a complex and life-threatening malformation, with an incidence of 2\u0026ndash;3/10 000 live births (1). Overall survival rates are still low, ranging from 65\u0026ndash;85% (1, 2, 3, 4). The prenatal detection rate with obstetrical ultrasound for CDH will vary depending on the setting but in average is approximately 60\u0026ndash;80% (4, 5). Due to the heterogeneity of this malformation, it is difficult to accurately predict outcomes in individual cases (6, 7). Risk stratification according to several severity features allows for more adequate counseling. Prenatal factors associated with worse outcome include decreased gestational age or weight at diagnosis, decreased lung volume (observed to expected lung to head ratio (O/E LHR, US) or total fetal lung volume (TFLV, MRI), thoracic liver location and percent herniation, thoracic stomach position, and the presence of associated structural or chromosomal anomalies (8, 9, 10, 11, 12, 13, 14, 15).\u003c/p\u003e \u003cp\u003eHydrops is a prenatal, clinical entity characterized by the presence of fluid collection in two or more body compartments, including skin or scalp edema, ascites, hydrothorax or pericardial effusion (16, 17, 18). Non-immune hydrops fetalis may be caused by various genetic syndromes or a consequence of hyperdynamic circulation in the fetus that leads to high-output cardiac failure (16, 18). In the case of fetal thoracic anomalies such as CDH, hydrops may result from increased systemic venous pressure caused by obstruction of venous return to the heart. The combination of hydrops and CDH is rare, with only a few series and case reports published, and is generally associated with a worse outcome (19, 20, 21).\u003c/p\u003e \u003cp\u003eThe objective of the current study was to increase knowledge of CDH with associated hydrops, describe the incidence of this association, and investigate postnatal outcomes using a large, multi-institutional cohort of CDH patients.\u003c/p\u003e"},{"header":"Material and Method","content":"\u003cp\u003eData from the Congenital Diaphragmatic Hernia Study Group (CDHSG) Registry on infants with CDH born at or transferred to any of the participating centers reporting to the registry were collected. During the specified study period, there were 83 participating centers that submitted data to the registry (specified in the \u003cb\u003eAppendix 1\u003c/b\u003e), which includes prospectively collected information on newborns with CDH until death or discharge. The registry started in 1995. The variables collected have been updated several times and the data sheet is currently in version 5 of data collection. In 2007, when the third version was introduced, the CDH Study Group Staging System for diaphragm defect size was introduced (22) (\u003cb\u003eAppendix 2\u003c/b\u003e), and with the fourth version in 2015, prenatal information including fetal ultrasound, magnetic resonance imaging (MRI), echocardiography, and genetic information were added. Thus, in this study we focused on all infants reported to the CDHSG with a prenatal diagnosis between January 2015 and December 2021.\u003c/p\u003e \u003cp\u003eData from patients with prenatally diagnosed CDH entered in the registry with reported fluid/edema in at least 1 compartment were analyzed and compared to the group of prenatally diagnosed CDH without compartmental fluid/edema. We further performed a sub-analysis of the different compartment/compartments with fluid. True hydrops was defined as fluid, edema, and/or effusions in two or more fetal compartments. Since in CDH there is a communication between the thorax and abdomen through the diaphragmatic defect, inherent to the condition, these two compartments were considered a single compartment and thus non-true hydrops. Associated variables evaluated included perinatal characteristics, number of compartments with excess of fluid, prenatal intervention, survival rates, defect size and side, associated malformations, use of extra-corporeal life support (ECLS), timing of surgical repair and rates of non-repair, length of hospital stay (LOS) and need for oxygen at 30 days.\u003c/p\u003e \u003cp\u003eData are presented as absolute values (n), percentages (%), odds ratio (OR) and 95% confidence interval (CI). Survival to discharge, need for ECLS, risk for death within the first 7 days of life, rates of non-repair and need of oxygen at 30 days were used as end points in the analysis. For categorical data, Fisher\u0026rsquo;s test was performed to investigate differences between groups. For continuous variables, t-test or Mann-Whitney test were used. Logistic regression was used when appropriated to quantify the strengths of the association. Significance was defined as p\u0026thinsp;\u0026le;\u0026thinsp;0.05. Analyses were performed using the R software version 2.38.\u003c/p\u003e \u003cp\u003eThe CDHSG registry has been approved for use by the Committee for the Protection of Human Subjects (CPHS) / Institutional Review Board (IRB) of the McGovern Medical School at UT Health in Houston (protocol number: HSC-MS-03-223).\u003c/p\u003e"},{"header":"Results","content":"\u003cdiv id=\"Sec4\" class=\"Section2\"\u003e \u003ch2\u003eFluid in at least one compartment\u003c/h2\u003e \u003cp\u003eA total of 3985 patients were entered in the registry during the study period, 3156 of those were prenatally diagnosed and 88 cases were reported to also have fluid in at least one compartment, representing 2.8% of all CDH cases, with an overall survival to discharge of 43% compared to 70% for CDH cases lacking fluid in any compartment, p\u0026thinsp;=\u0026thinsp;0.001 (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e, Fig.\u0026nbsp;2).\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eBackground data for the cohort of prenatally diagnosed CDH, comparing patients with reported fluid in at least 1 compartment to non-reported-hydropic\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"5\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eFluid in compartment/\u0026rsquo;s\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eNon-hydrops\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003eP value\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePrenatally diagnosed (n\u0026thinsp;=\u0026thinsp;3156)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e88\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e3068\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSex female (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e42.0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e44.3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.772\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eEGA (median [IQR])\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e37.00 [34.00, 38.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e38.00 [37.00, 39.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e\u0026lt;\u0026thinsp;0.001\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eBirthWeigth (median [IQR])\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2.83 [2.32, 3.22]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e2.93 [2.50, 3.28]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.103\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSide (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eLeft\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e42.0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e86.1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e\u0026lt;\u0026thinsp;0.001\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eRight\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e55.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e12.9\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eCentral\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1.1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0.0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eBilateral\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1.1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0.8\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eDefect Size (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2.2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e8.1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.680\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eB\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e37.5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e34.0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eC\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e45.8\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e40.1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eD\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e14.6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e17.8\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eO/E LHR (median [IQR])\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e44.95 [32.00, 52.62]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e40.77 [30.84, 53.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.552\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNon-Repairs (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e45.5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e14.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e\u0026lt;\u0026thinsp;0.001\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eType of Repair (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003ePatch\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e66.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e63.5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e\u0026lt;\u0026thinsp;0.001\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eECLS (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e34.1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e30.2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.480\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAssoc anomalies (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e50.0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e37.3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.01\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eType of Assoc anomaly (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eCardiac\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e26.1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e24.3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.018\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eChromosomal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e11.4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e9.6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eOther\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e27.3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e15.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eSeveral\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e18.2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e11.0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eIsolated\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e50.0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e62.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eO2 at 30D (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e44.3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e45.6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e\u0026lt;\u0026thinsp;0.001\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSurvival to discharge (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e43.2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e70.3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e\u0026lt;\u0026thinsp;0.001\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eDOL at surgery (median [IQR])\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e4.50 [2.00, 11.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4.00 [2.00, 7.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.136\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eDOL at Death (median [IQR])\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1.00 [0.00, 10.75]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e10.00 [1.00, 32.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e\u0026lt;\u0026thinsp;0.001\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eLOS (median [IQR])\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e51.00 [24.50, 93.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e48.50 [29.00, 86.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.694\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"5\"\u003eDOL: Day of life, DC: Discharge, IQR: Interquartile range, O/E LHR: observed/expected lung to head ratio, ECLS: Extracorporeal Life Support\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"5\"\u003e*Significance P\u0026thinsp;\u0026lt;\u0026thinsp;0.05\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eThere was no significant difference in median birth weight or gestational age (GA) at birth between the groups. However, prematurity (born\u0026thinsp;\u0026lt;\u0026thinsp;34 wks EGA) was associated with higher mortality in the CDH cohort with fluid in at least 1 compartment (p\u0026thinsp;=\u0026thinsp;0.027, OR 4.75, CI 1.24\u0026ndash;20.2). The CDH group with fluid in at least one compartment had an increased incidence of right sided CDH (55% vs 12.9%, p\u0026thinsp;\u0026lt;\u0026thinsp;0.001) and higher rates of non-repairs (45.5% vs 14.7%, p\u0026thinsp;\u0026lt;\u0026thinsp;0.001) (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eHowever, when undergoing surgical repair, survival rates for prenatally diagnosed CDH with fluid in at least 1 compartment was 80% (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). The utilization of ECLS was similar in both groups (34% vs 30%) (Table \u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e), with a 36.8% survival to discharge (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). The length of hospital stay was similar for CDH with fluid in at least 1 compartment compared to prenatally diagnosed CDH, as was the need for O\u003csub\u003e2\u003c/sub\u003e at 30 days. Other associated anomalies were more common in prenatally diagnosed CDH with fluid in at least 1 compartment (50% vs 37%, p\u0026thinsp;=\u0026thinsp;0.05). (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e)\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab2\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 2\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003ePatient characteristics survival vs demised\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"5\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eOverall\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eDischarged alive\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eDemised\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003ep value\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003en\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e88\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e38\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e50\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eHydrops with fluid at least in 2 compartments n (%)\u003c/b\u003e \u003cb\u003e(\u0026ldquo;true hydrops\u0026rdquo;)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e14 (15.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e3 (21.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e11 (78.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0.08\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eHydrops with fluid in only 1 compartment or combined ascites\u0026thinsp;+\u0026thinsp;pleural effusion (%)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e74 (84.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e35 (47.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e39 (52.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0.048\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eFluid Distribution in different compartments, (%)\u003c/b\u003e:\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAscites\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e10 (13.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e\u003cb\u003e6\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cem\u003eAscites\u0026thinsp;+\u0026thinsp;Pericardial effusion\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 (1.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cem\u003eAscites\u0026thinsp;+\u0026thinsp;Pericardial effusion\u0026thinsp;+\u0026thinsp;Pleural effusion\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 ( 2.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cem\u003eAscites\u0026thinsp;+\u0026thinsp;Pericardial effusion\u0026thinsp;+\u0026thinsp;Pleural effusion\u0026thinsp;+\u0026thinsp;Skin edema\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 ( 1.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAscites\u0026thinsp;+\u0026thinsp;Pleural effusion\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e11 (14.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e8\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cem\u003eAscites\u0026thinsp;+\u0026thinsp;Pleural effusion\u0026thinsp;+\u0026thinsp;Skin edema\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e5 ( 6.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e\u003cb\u003e5\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cem\u003eAscites\u0026thinsp;+\u0026thinsp;Skin edema\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 ( 1.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePericardial effusion\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e6 ( 7.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e\u003cb\u003e3\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cem\u003ePericardial effusion\u0026thinsp;+\u0026thinsp;Pleural effusion\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 ( 2.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePleural effusion\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e32 (41.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e18\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e14\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cem\u003ePleural effusion\u0026thinsp;+\u0026thinsp;Skin edema\u003c/em\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 (2.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e\u003cb\u003e2\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSkin edema\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (5.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e\u003cb\u003e3\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eAssociated anomalies n (%)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e44 (50.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e16 (42.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e28 (56.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0.282\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eType of associated anomaly n (%)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCardiac\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e12 (13.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e7 (18.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e5 (10.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eChromosomal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (4.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2 (5.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e2 (4.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eDysmorphic features\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 (1.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 (2.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eOther\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e11 (12.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 (2.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e10 (20.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSeveral\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e16 (18.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e6 (15.8)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e10 (20.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eIsolated\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e44 (50.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e22 (57.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e22 (44.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eSide n (%)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eLeft\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e37 (42.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e9 (\u003cb\u003e24.3\u003c/b\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e28 (75.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0.003\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eRight\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e49 (\u003cb\u003e55.7\u003c/b\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e27 (55.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e22 (44.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCentral\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 (1.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 (2.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0 (0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eBilateral\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 ( 1.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 (100)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eNon-repair (%)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e40 \u003cb\u003e(45.5)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e40 (\u003cb\u003e80.0\u003c/b\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e\u0026lt;\u0026thinsp;0.001\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eSurgical repair (%)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e48 (54.5)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e\u003cb\u003e38 (80.0)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e10 (20.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e\u0026lt;\u0026thinsp;0\u0026ndash;001\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eSurvival to DC (%)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e38 (43.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e\u003cb\u003e38\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eECLS (%)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e30 (\u003cb\u003e34.1\u003c/b\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e14 (\u003cb\u003e36.8\u003c/b\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e16 (\u003cb\u003e32.0\u003c/b\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0.656\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"5\"\u003eDC: Discharge, ECLS: Extracorporeal Life Support\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eThere was a higher rate of associated anomalies among CDH patients with fluids in at least 1 compartment who died before discharge (56 vs 42%)(Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec5\" class=\"Section2\"\u003e \u003ch2\u003eTrue hydrops\u003c/h2\u003e \u003cp\u003eThe distribution of fluid collection in the different compartments is summarized in Table\u0026nbsp;\u003cspan refid=\"Tab3\" class=\"InternalRef\"\u003e3\u003c/span\u003e \u003cb\u003eand Fig.\u0026nbsp;1\u003c/b\u003e. Only 14 patients had fluid collections in more than one compartment (excluding the combination pleural effusion and ascites for reasons noted above), representing the \u0026ldquo;true\u0026rdquo; hydrops cohort with an incidence of only 0.35%, and having a high mortality rate of 79%.\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab3\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 3\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eSub-analysis of true vs non-true hydrops\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"5\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eFluid in \u0026gt;\u0026thinsp;1 compartment\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eFluid in 1 compartment (or combined ascites\u0026thinsp;+\u0026thinsp;pleural effusion)\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003eP value\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003en\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e14\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e74\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSide (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eLeft\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e3 ( 21.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e34 (45.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e\u0026lt;\u0026thinsp;0.001\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eRight\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e11 ( \u003cb\u003e78.6\u003c/b\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e38 (51.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eCentral\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 ( 0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 ( 1.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eBilateral\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 ( 0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 ( 1.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eRepair Done (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e7 ( 50.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e41 (55.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.775\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatch Repair (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e5 ( 71.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e27 (65.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e1.000\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eECLS (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e6 ( 42.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e24 (32.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.542\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAssociated anomalies (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e4 ( 28.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e40 (\u003cb\u003e54.1\u003c/b\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.143\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eType of associated anomaly (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eCardiac\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 ( 7.1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e11 (14.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.489\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eChromosomal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 ( 0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 ( 5.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eDysmorphic features\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 ( 0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1 ( 1.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eOther\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0 ( 0.0)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e11 (14.9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eMore than 1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e3 ( 21.4)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e13 (17.6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eIsolated\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e10 ( \u003cb\u003e71.4)\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e34 (\u003cb\u003e45.9\u003c/b\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eO2 requirement at 30 DOL (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e5 (\u003cb\u003e100.0\u003c/b\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e34 (72.3)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.314\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSurvival to discharge (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e3 \u003cb\u003e(21.4\u003c/b\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e35 (\u003cb\u003e47.3\u003c/b\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.086\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eDOL at surgery (median [IQR])\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e4.00 [2.00, 8.50]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e5.00 [3.00, 11.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.617\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eDOL at Death (median [IQR])\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1.00 [0.00, 23.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e1.00 [0.50, 8.50]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.895\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eDOL at DC (median [IQR])\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e\u003cb\u003e67.00\u003c/b\u003e [46.50, 221.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e50.00 [22.00, 93.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.433\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"5\"\u003eDOL: Day of life, DC: Discharge, IQR: Interquartile range, ECLS: Extracorporeal Life Support\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"5\"\u003e*P\u0026thinsp;\u0026lt;\u0026thinsp;0.05\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eIn the group of true hydrops (fluid in more than one compartment) there was a high rate of right sided CDH (78.6%), skin edema was present in 64.3%, surgical repair was done in 50% of the cases with a survival to discharge in the surgically treated group of 43%. Only 28.7% of true hydropic CDH had associated anomalies compared to the group of \u0026ldquo;non true hydrops\u0026rdquo; (54.1%) (Table\u0026nbsp;\u003cspan refid=\"Tab3\" class=\"InternalRef\"\u003e3\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eIn the event of isolated skin edema, there is a higher mortality risk before discharge (OR 5.08, 95% CI 1.25\u0026ndash;34.3).\u003c/p\u003e \u003c/div\u003e\n\u003ch3\u003eSide\u003c/h3\u003e\n\u003cp\u003eLeft sided CDH with fluid in at least one compartment had lower survival rates than right sided CDH with fluid in at least 1 compartment (\u003cb\u003e24 vs 55%\u003c/b\u003e, OR\u0026thinsp;=\u0026thinsp;3.82, p\u0026thinsp;=\u0026thinsp;0.005), and higher rates of associated anomalies (67.6 vs 34.7, p\u0026thinsp;=\u0026thinsp;0.004) (Table\u0026nbsp;\u003cspan refid=\"Tab4\" class=\"InternalRef\"\u003e4\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab4\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 4\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eSub-analysis of side (left to right side only)\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"5\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003el\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eLeft\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eRight\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003ep-value\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eHydrops n\u0026thinsp;=\u0026thinsp;88\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e37\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e49\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eEGA (median [IQR])\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e37.00 [34.00, 38.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e37.00 [34.00, 38.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.654\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eFETO n(%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1 (2.7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4 (8.2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.3\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNon-repairs (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e75.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e24.5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e\u0026lt;\u0026thinsp;0.001\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatch Repair (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e66.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e64.9\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e1.000\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eECLS (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e16.2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e44.9\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.006\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAssociated anomalies (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e67.6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e34.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.004\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eType of Associated anomaly (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eCardiac\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e10.8\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e14.3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.014\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eChromosomal\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e8.1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e2.0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eOther\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e18.9\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e8.2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eSeveral\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e27.0\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e10.2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eIsolated\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e32.4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e65.3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eO2 att 30DOL (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e53.8\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e81.1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.073\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSurvival to dscharge (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e24.3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e55.1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.005\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eDOL at surgery (median [IQR])\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e7.00 [4.00, 11.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e4.00 [2.00, 9.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.387\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eDOL at Death (median [IQR])\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1.00 [0.00, 3.25]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e3.00 [1.00, 34.50]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.043\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eLOS (median [IQR])\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e31.00 [3.25, 57.25]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e59.00 [30.00, 95.00]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e \u003cp\u003e0.071\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"5\"\u003eDC: Discharge, ECLS: Extracorporeal Life Support, DOL: Day of life, IQR: Interquartile range, FETO: Fetoscopic endotracheal occlusion\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eThe rates of non-repairs were higher in the left-sided CDH with fluids in at least one compartment compared to right-sided (75% vs 24.5%, p\u0026thinsp;\u0026lt;\u0026thinsp;0.0001) (Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eIn this prospective multicenter study, we conclude that hydropic CDH is rare, only 2.8% of all prenatally diagnosed cases, and more commonly occurring in right sided CDH. Survival rates are low, with higher rates of non-repair. Hydrops is a clinical entity characterized by the presence of skin and scalp edema or fluid in two or more body compartments. In cases of fetal thoracic anomalies, such as CDH, it is thought to be caused by obstruction of venous return to the heart leading to elevated systemic venous pressures.\u003c/p\u003e \u003cp\u003eWhen using the term \u0026lsquo;hydrops\u0026rsquo; in CDH, effusions in the thoracic cavity and abdominal cavity concomitant to the side of the defect, should be considered as one compartment for obvious reasons, avoiding confusion. Thus, true hydrops in CDH should refer to patients with excess fluid in at least two non-concomitant compartments.\u003c/p\u003e \u003cp\u003eHowever, the presence of excess of fluid in only one compartment is associated with worse outcome. Even if the association is rare, nearly 3% of all prenatally diagnosed CDH will present with fluid in 1 compartment, the survival rates are low (47.3%, Table \u003cspan refid=\"Tab3\" class=\"InternalRef\"\u003e3\u003c/span\u003e). True hydropic CDH is even more rare, only identified in 0.35% of the prenatally diagnosed CDH cases, and more commonly occurring in right sided CDH. Survival rates for true hydropic CDH are very low: 78% of patients with true hydrops will not survive to discharge.\u003c/p\u003e \u003cp\u003eMalformations are present in half of the CDH with fluid / edema, true hydrops (fluid in at least two compartments) or non-true hydrops (fluid in 1 compartment), and more common among those who died before discharge (56% vs 42%, Table \u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eSkin edema seems to be an independent risk factor for worse outcome, with higher mortality. However, even though the pathophysiological mechanisms underlying this event remain unknown, an increased central venous pressure and/or obstructed lymphatic drainage may be the most plausible explanations. [refs]\u003c/p\u003e \u003cp\u003eAlthough the pathogenesis of hydrops in fetuses with CDH is poorly understood, right-sided lesions seem to be more likely to cause hydrops in CDH. Most of the cases presented here were right sided and/or had a major portion of the liver in the chest, thus the pathophysiology may be similar to other intrathoracic lesions, with mediastinal obstruction from solid organ herniation obstructing blood return to the heart. Since there is a confined space within the thorax, the herniation of a solid organ such as the liver, leaving a smaller portion intraabdominally, may lead to kinking and obstruction of the inferior vena cava, portal or hepatic vein.\u003c/p\u003e \u003cp\u003eDespite hydrops being more commonly associated with right sided CDH, in as many as half of the patients with hydrops have a right sided defect, left sided CDH has a poorer outcome, with higher rates of associated anomalies, high rates of non-repairs and is overrepresented among children who died before discharge (75% of the left sided CDH with hydrops will demise, probably due to the complexity of the CDH).\u003c/p\u003e \u003cp\u003eEven if the rates of non-repairs are high, a more aggressive surgical approach leads to acceptable survival rates: 80% of the patients being repair will survive to discharge. ECLS support is offered to the same extent to patients who survive and those hydrops patients who died before discharge.\u003c/p\u003e \u003cp\u003eIn conclusion, the association of CDH and hydrops in the fetus is rare and the sole presence of excess fluid or edema in 1 compartment is associated with poorer outcome. The combination is associated with the presence of other associated anomalies and right-sided defects with liver involvement. Special attention should be given to the presence of skin edema resulting in higher mortality rates. An initially aggressive surgical approach may result in acceptable survival rates comparable to non-hydropic cases. Early decision-making regarding the aims of care clearly influence outcome, since survival varies depending on the severity of the CDH and the presence of other associated anomalies.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eAuthor contributions:\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eCarmen Mesas Burgos \u0026ndash; Study conception, analysis and interpretation of data, drafting of manuscript, critical revision of manuscript\u003c/p\u003e\n\u003cp\u003eAshley H Ebanks \u0026ndash; Acquisition of data, analysis and interpretation of data, critical revision of manuscript\u003c/p\u003e\n\u003cp\u003eAnna L\u0026ouml;f-Granstr\u0026ouml;m-\u0026nbsp;critical revision of manuscript\u003c/p\u003e\n\u003cp\u003eKylie I. Holden-\u0026nbsp;critical revision of manuscript\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;Anthony Johnson-\u0026nbsp;critical revision of manuscript\u003c/p\u003e\n\u003cp\u003ePeter Conner- critical revision of manuscript\u003c/p\u003e\n\u003cp\u003eMatthew T. Harting - analysis and interpretation of data, critical revision of manuscript\u003c/p\u003e\n\u003cp\u003eAll authors have consent for publication\u003c/p\u003e\n\u003cp\u003eAll data is available upon request\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConflict of interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThere are no conflict of interests.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eLevel of evidence\u003c/strong\u003e:\u003c/p\u003e\n\u003cp\u003eI for a prognosis study \u0026ndash; This is a high-quality, prospective cohort study with 99% of patients followed to the study end point (death or discharge)\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eBurgos CM, Frenckner B. Addressing the hidden mortality in CDH: A population-based study. J Pediatr Surg. 2017;52(4):522-5.\u003c/li\u003e\n\u003cli\u003eLally P. Report from the Congenital Diaphragmatic Hernia Study Group Database. CDHSG [Webbsite / Database] 2008 2/4/2008 [cited 2010 6/1/2010]; C]. Available from: http://cdhsg.net/ 2008 [\u003c/li\u003e\n\u003cli\u003eMesas Burgos C, Hammarqvist-Vejde J, Frenckner B, Conner P. Differences in Outcomes in Prenatally Diagnosed Congenital Diaphragmatic Hernia Compared to Postnatal Detection: A Single-Center Experience. Fetal Diagn Ther. 2015.\u003c/li\u003e\n\u003cli\u003eBurgos CM, Frenckner B, Luco M, Harting MT, Lally PA, Lally KP, et al. Prenatally versus postnatally diagnosed congenital diaphragmatic hernia - Side, stage, and outcome. J Pediatr Surg. 2018.\u003c/li\u003e\n\u003cli\u003eMonier I, Lelong N, Benachi A, Jouannic JM, Khoshnood B, Zeitlin J. Postnatal diagnosis of congenital anomalies despite active systematic prenatal screening policies: a population-based registry study. Am J Obstet Gynecol MFM. 2023;5(11):101170.\u003c/li\u003e\n\u003cli\u003eHaroon J, Chamberlain RS. An evidence-based review of the current treatment of congenital diaphragmatic hernia. Clin Pediatr (Phila). 2013;52(2):115-24.\u003c/li\u003e\n\u003cli\u003eDanzer E, Hedrick HL. Controversies in the management of severe congenital diaphragmatic hernia. Semin Fetal Neonatal Med. 2014;19(6):376-84.\u003c/li\u003e\n\u003cli\u003eAkinkuotu AC, Cruz SM, Abbas PI, Lee TC, Welty SE, Olutoye OO, et al. Risk-stratification of severity for infants with CDH: Prenatal versus postnatal predictors of outcome. J Pediatr Surg. 2016;51(1):44-8.\u003c/li\u003e\n\u003cli\u003eJani J, Keller RL, Benachi A, Nicolaides KH, Favre R, Gratacos E, et al. Prenatal prediction of survival in isolated left-sided diaphragmatic hernia. Ultrasound Obstet Gynecol. 2006;27(1):18-22.\u003c/li\u003e\n\u003cli\u003eHedrick HL, Danzer E, Merchant A, Bebbington MW, Zhao H, Flake AW, et al. Liver position and lung-to-head ratio for prediction of extracorporeal membrane oxygenation and survival in isolated left congenital diaphragmatic hernia. Am J Obstet Gynecol. 2007;197(4):422 e1-4.\u003c/li\u003e\n\u003cli\u003eCannie M, Jani J, Meersschaert J, Allegaert K, Done E, Marchal G, et al. Prenatal prediction of survival in isolated diaphragmatic hernia using observed to expected total fetal lung volume determined by magnetic resonance imaging based on either gestational age or fetal body volume. Ultrasound Obstet Gynecol. 2008;32(5):633-9.\u003c/li\u003e\n\u003cli\u003eGucciardo L, Deprest J, Done E, Van Mieghem T, Van de Velde M, Gratacos E, et al. Prediction of outcome in isolated congenital diaphragmatic hernia and its consequences for fetal therapy. Best Pract Res Clin Obstet Gynaecol. 2008;22(1):123-38.\u003c/li\u003e\n\u003cli\u003eJani JC, Benachi A, Nicolaides KH, Allegaert K, Gratacos E, Mazkereth R, et al. Prenatal prediction of neonatal morbidity in survivors with congenital diaphragmatic hernia: a multicenter study. Ultrasound Obstet Gynecol. 2009;33(1):64-9.\u003c/li\u003e\n\u003cli\u003eCordier AG, Jani JC, Cannie MM, Rodo C, Fabietti I, Persico N, et al. Stomach position in the prediction of survival in left-sided congenital diaphragmatic hernia with or without fetoscopic endoluminal tracheal occlusion. Ultrasound Obstet Gynecol. 2014.\u003c/li\u003e\n\u003cli\u003eLee JY, Jun JK, Lee J. Prenatal prediction of neonatal survival in cases diagnosed with congenital diaphragmatic hernia using abdomen-to-thorax ratio determined by ultrasonography. J Obstet Gynaecol Res. 2014;40(9):2037-43.\u003c/li\u003e\n\u003cli\u003eNeveling S, Knippel AJ, Kozlowski P. Isolated non-immune hydrops fetalis: an observational study on complete spontaneous resolution, perinatal outcome, and long-term follow-up. Arch Gynecol Obstet. 2023;308(2):487-97.\u003c/li\u003e\n\u003cli\u003eVanaparthy R, Mahdy H. Hydrops Fetalis. StatPearls. Treasure Island (FL)2023.\u003c/li\u003e\n\u003cli\u003eWei X, Yang Y, Zhou J, Zhou X, Xiong S, Chen J, et al. An Investigation of the Etiologies of Non-Immune Hydrops Fetalis in the Era of Next-Generation Sequence-A Single Center Experience. Genes. 2022;13(12).\u003c/li\u003e\n\u003cli\u003eIbrahim M, Hunter M, Gugasyan L, Chan Y, Malhotra A, Sehgal A, et al. Interstitial deletion of chromosome 1 (1p21.1p12) in an infant with congenital diaphragmatic hernia, hydrops fetalis, and interrupted aortic arch. Clin Case Rep. 2017;5(2):164-9.\u003c/li\u003e\n\u003cli\u003eRossi A, Delabaere A, Delmas-Laurichesse H, Beaufrere AM, Lemery D, Gallot D. The challenge of prenatal identification of congenital diaphragmatic hernia in the context of hydrops. Eur J Obstet Gynecol Reprod Biol. 2014;182:238-9.\u003c/li\u003e\n\u003cli\u003eSydorak RM, Goldstein R, Hirose S, Tsao K, Farmer DL, Lee H, et al. Congenital diaphragmatic hernia and hydrops: a lethal association? J Pediatr Surg. 2002;37(12):1678-80.\u003c/li\u003e\n\u003cli\u003eLally KP, Lasky RE, Lally PA, Bagolan P, Davis CF, Frenckner BP, et al. Standardized reporting for congenital diaphragmatic hernia--an international consensus. J Pediatr Surg. 2013;48(12):2408-15.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"journal-of-perinatology","isNatureJournal":false,"hasQc":false,"allowDirectSubmit":false,"externalIdentity":"jp","sideBox":"Learn more about [Journal of Perinatology](http://www.nature.com/jp/)","snPcode":"41372","submissionUrl":"https://mts-jper.nature.com/cgi-bin/main.plex","title":"Journal of Perinatology","twitterHandle":"@jperinatology","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"ejp","reportingPortfolio":"Nature AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":false},"keywords":"congenital diaphragmatic hernia, hydrops, prenatal, prognosis, outcome","lastPublishedDoi":"10.21203/rs.3.rs-3753580/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-3753580/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eObejctive: \u003c/strong\u003e\u0026nbsp;Congenital Diaphragmatic Hernia (CDH) associated with hydrops is rare. The aim of this study was to describe the incidence of this combination of anomalies and the postnatal outcomes from a large database for CDH.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eStudy design:\u003c/strong\u003e Data from the multicenter, multinational database on infants with prenatally diagnosed CDH (CDHSG Registry) born from 2015 to 2021 were analyzed.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eResults: \u003c/strong\u003eA total of 3985 patients were entered in the registry during the study period, 3156 were prenatally diagnosed and 88 were reported to have associated fluid in at least 1 compartment, representing 2.8 % of all prenatally diagnosed CDH cases in the registry. The overall survival to discharge for CDH patients with hydrops was 43%.\u003c/p\u003e\n\u003cp\u003eThe hydropic CDH group had lower birth weight and gestational age at birth, and increased incidence of right sided CDH (55%) and rate of non-repair (45%). However, the survival rate for hydropic infants with CDH undergoing surgical repair was 80%. Other associated anomalies were more common in hydropic CDH (50% vs 37%, p=0.001).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusion: \u003c/strong\u003eHydropic CDH is rare, only 2.8% of all prenatally diagnosed cases, and more commonly occurring in right sided CDH. Survival rates are low, with higher rates of non-repair. However, early decision-making regarding goals of care and an aggressive surgical approach may result in survival rates comparable to non-hydropic cases.\u003c/p\u003e","manuscriptTitle":"Hydrops and Congenital Diaphragmatic Hernia: Reported incidence and postnatal outcomes. 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