Clinical profile of adult patients referred to congenital heart surgery in Egypt, a single center experience.

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Amir Anwar Samaan, Mostafa M. Abdrabou, Amir Mostafa, Osama AbdElAziz, and 4 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-3860952/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background A major challenge facing adult congenital heart disease (ACHD) service in developing countries is the lack of adequate data on the disease burden and specific needs of this group. We aimed to describe ACHD patients in Egypt who are in need for further surgical correction, their various presentations, and the types of surgeries required. Methods The study included ACHD patients considered for further surgical correction after a heart team discussion at a tertiary cardiac center in Egypt. Data about age, gender, presentation, functional capacity, resting oxygen saturation, diagnosis, previous intervention and its timing, and the type of surgery decided were collected. Results Over one year, 103 cases (49.5% males, mean age 23 years) were referred to surgery out of 134 cases discussed at the heart team committee. One-third of the patients (34%) were cyanotic and the majority presented in NYHA functional class II (44.7%) and III (35.95%). More than half of the patients (56.3%) had a previous surgical or trans-catheter intervention with a median age of 4 years at the time of the first procedure. The most frequently encountered diagnoses were: Tetralogy of Fallot (ToF) (23.3%), atrial septal defect (18.4%), double outlet right ventricle (DORV) (11.6%), and transposition of great arteries (TGA) (7.8%). Surgical procedures that were required most were: Fontan procedure (20.3%), surgical ASD closure (18.4%), surgical pulmonary valve replacement (14.5%), total ToF repair (9.7%), subaortic membrane resection (6.7%) and tricuspid valve surgery (5.8%). Conclusions The group of ACHD patients in Egypt who require further surgical correction is heterogeneous with various presentations and needs. The required surgeries vary from simple to complex procedures. An efficient ACHD program necessitates the collaboration of dedicated cardiologists, radiologists, and cardiothoracic surgeons who are familiar with the unique demands and difficulties of this patient group. Adult congenital heart disease surgery Egypt Introduction Over the past decades, the population of adult patients with congenital heart disease (CHD) has remarkably grown worldwide. This is mainly related to the advances seen in congenital heart surgery and pediatric cardiac care. Some reports postulated that the adult congenital heart disease (ACHD) population is currently outnumbering the pediatric CHD group in Europe and the United States.[ 1 , 2 ] Data from the developing world are scarce, but some reports show similar trends.[ 3 , 4 ] Despite significant advances in the field of congenital heart surgery in recent years, significant challenges remain, particularly for surgeons operating on adult patients with congenital defects. The majority of cardiac surgical repair procedures during childhood are not curative,[ 1 ] and many of the remaining cardiac lesions may progress or begin to affect cardiac structure and function over time. Tissue valves and conduits frequently require repeated interventions when they degenerate. Redo surgeries entail higher risk sternotomy, which may injure previous conduits or dilated aortas adherent to the sternum.[ 5 ] In developing countries, where facilities for congenital cardiac surgery are not widely available, patients with CHD who survive till adulthood have a different profile than those in developed countries.[ 4 ] Many ACHD patients have either mild lesions that have been missed in childhood[ 3 ] or severe CHD that was operated on in infancy and childhood, while some patients have either survived despite complex unrepaired defects or had some form of palliative or incomplete repair. One of the major challenges facing ACHD service in low- and middle-income countries is the lack of adequate data on the disease burden and specific needs of this peculiar group.[ 4 , 6 ] Clearly, data on the various presentations and types of required surgeries are insufficient. Methods Our retrospective study included all CHD patients above the age of 14 years who sought medical advice at an Egyptian tertiary cardiac center and required surgical repair. It was conducted over one year. At the outpatient clinic, all referred patients were evaluated by one of the ACHD team cardiologists. Candidates for surgical intervention were presented in a heart team meeting that included a congenital cardiac surgeon, a congenital structural interventionist, a radiologist, and a cardiologist after all non-invasive testing and hemodynamic studies were completed. Accepted cases by the committee were included in the study. Age, gender, presentation, functional capacity, resting oxygen saturation, diagnosis, previous intervention and its timing, surgery type, need for cardiac magnetic resonance imaging (CMR) or computed tomography (CT), and need for pre-surgery catheter hemodynamic assessment were all collected. Approval was obtained from the ethics committee of Al Nas Hospital. The procedures used in this study adhere to the tenets of the Declaration of Helsinki. Results Between March 2022 and February 2023, 220 patients attended the ACHD clinic; the heart team committee discussed 134 patients and decided on surgery in 103 cases, while the remaining 86 patients were either given medical treatment/follow-up decisions or scheduled for trans-catheter intervention. Half of the patients were males (49.5%), the mean age was 23 years, 34% were cyanotic and the majority presented in NYHA functional class II (44.7%) and III (35.95). Fifty-eight patients (56.3%) had a form of previous surgical or trans-catheter intervention at a median age of 4 years (IQR 2.1–11.8 years) at the time of the first procedure: 15.5% of them had two procedures, while 7% had three or more separate surgical or catheter interventions. The types of these surgeries are summarized in table (1). Table (1). Types of previous surgeries/ catheter interventions. Type of previous surgeries/interventions Number of patients Total Fallot repair 14 BT (Blalock- Taussig) shunts 15 Glenn 14 PA banding 7 ASD closure 2 VSD closure 4 Surgical aortic coarctation repair 2 Balloon pulmonary valvuloplasty 2 Subaortic membrane resection 2 Rastelli 1 Kawashima 1 Common AV canal repair 2 Surgical pulmonary valvotomy 1 LPA stent 1 TV repair 2 TVR 1 AVR 2 (one with Kono procedure) BT shunt : Blalock Taussig shunt, PA : pulmonary artery, ASD : atrial septal defect, VSD : ventricular septal defect, common AV canal : common atrioventricular canal, LPA : left pulmonary artery, TV : tricuspid valve, TVR : tricuspid valve replacement, AVR : aortic valve replacement. Tetralogy of Fallot (TOF) was the most frequent diagnosis among our cohort (24 patients, 23.3%): 8 did not have any form of previous intervention, 2 had BT shunts during childhood and 14 had a previous total repair. One of the uncorrected patients presented with a large ascending aortic aneurysm and ruptured sinus of Valsalva. Nineteen patients (18.4%) were referred for surgical ASD closure: 11 had large secundum ASDs not amenable for trans-catheter closure, 7 had sinus venosus ASDs (6 of them had associated partial anomalous pulmonary venous drainage) and one patient had primum ASD (partial AV canal). Twelve patients (11.6%) had double outlet right ventricle (DORV); 9 with a previous palliative shunt with/without pulmonary artery banding while one patient had total repair (Rastelli’s operation). Eight patients (7.8%) had transposition of great arteries (TGA); two of them had congenitally corrected (CC) TGA with severe incompetence of the tricuspid (systemic) valve. The other 6 patients had D-TGA with protected pulmonary circulation in addition to either ASD or VSD; 3 of them had previous Glenn procedures and the remaining 3 had previous BT shunts. Five patients (4.8%) had a common atrioventricular (AV) canal; four with previous pulmonary artery banding (one of them had severe pulmonary stenosis), and 2 had previous total repair. Five patients (4.8%) had subaortic membranes with significant left ventricular outflow tract obstruction. Six patients (5.8%) had Ebstein anomaly; one had a previous repair and another had a tricuspid valve replacement. Four patients (3.8%) had ventricular septal defects; two were residual defects post-surgical closure associated with significant aortic regurgitation due to cusp prolapse, one was a restrictive defect with left ventricular dilatation and the last one had previous surgical closure with iatrogenic severe tricuspid incompetence. Three patients (2.9%) had tricuspid atresia; all had previous Glenn procedures. Three patients (2.9%) had severe pulmonary regurgitation post previous surgical or catheter pulmonary valvotomy. Two patients (1.9%) had double-chambered right ventricle. Two patients (1.9%) had partial anomalous pulmonary venous drainage. Two patients had unrepaired Shone complex (1.9%). Two patients (1.9%) had double inlet left ventricle. Single patients had the following diagnoses: supravalvular aortic stenosis, branch pulmonary stenosis, double outlet left ventricle, aortic paravalvular leak, pulmonary stenosis, and right atrial appendage aneurysm. Types of surgeries decided for the patients after the heart team discussion are listed in Table 2. Table (2). Types of required surgeries for ACHD patients. Type of surgery Number of patients (percentage) Total cavo-pulmonary anastomosis (Fontan surgery) 21 patients (20.3%); Completion Fontan surgery (after a previous Glenn shunt) in 16 patients (15.3%) and without previous surgery in 5 patients (4.8%) Surgical ASD closure 19 patients (18.4%) (with PAPVD repair in 6 patients and pulmonary valvotomy in 1 patient) Surgical pulmonary valve replacement 15 (14.5%) patients (with RVOT reconstruction in 1 patient, VSD closure in 1 patient, TV repair in 2 patients, and repair of LPA origin stenosis in one patient) Total ToF repair 10 patients (9.7%) (with MAPCAs surgical closure in 2 patients and after RVOT stenting in 2 patients) Tricuspid valve surgery 6 patients (5.8%); - TV repair in 5 patients (4.8%) in addition to ASD closure in one patient and Glenn surgery in another patient. - TV replacement in one patient who had a common AV canal, PA banding in infancy, and two previous attempts for repair, and presented with massive TR Subaortic membrane resection 7 patients (6.7%); catheter coiling of a saccular aortic aneurysm was required in one patient, additional mitral valve repair in 2 patients (Shone complex patients), aortic valve replacement in one patient Surgical VSD closure 3 patients (2.9%); with aortic valve repair in 2 patients. - Total repair of DORV - Tricuspid (Systemic) valve replacement - Double-chambered RV repair - Repair of PAPVD - Repair of bilateral branch pulmonary artery stenosis 2 patients (1.9%) each - Aortic valve repair - Hepatic incorporation surgery - Mitral valve replacement - Partial AV canal repair - RAA aneurysm excision - Redo AVR with relief of RVOT obstruction - Redo degenerated Tricuspid valve replacement in addition to mitral valve replacement - Repair of aortic aneurysm and ruptured sinus of Valsalva in a post-TOF repair patient - Replacement of RV-PA conduit - Surgical correction of CAVC and PS, - Surgical correction of RVOT obstruction in a previously repaired ToF patient, - Surgical correction of supravalvular AS One patient (0.9%) each ASD : atrial septal defect, AVR : aortic valve replacement, AS : aortic stenosis, CAVC : common atrioventricular canal, DORV : double outlet right ventricle, LPA : left pulmonary artery stenosis, MAPCAs : Major aortopulmonary collaterals, PAPVD : partial anomalous pulmonary venous drainage, RVOT : right ventricular outflow tract, TV: tricuspid valve, RV : right ventricle, RV-PA : right ventricle to pulmonary artery, RAA : right atrial appendage aneurysm, ToF : Tetralogy of Fallot Other than echocardiography, 65% of the patients required further non-invasive testing: 39 (37.8%) had cardiac magnetic resonance imaging, 23 (22.3%) had a CT scan and 5 (4.8%) had both tests. Pre-operative cardiac catheterization was performed in 26 patients (25.2%). Discussion The care of ACHD patients in developing countries constitutes a major challenge. In a previous report, the number of ACHD centers per 10 million inhabitants was estimated to be 0.1 in Africa in comparison to a substantially higher number in Europe (3.6) and North America (1.7).[ 7 ] According to the same report, the African research output in the field of ACHD was also extremely lower than in Europe and the United States. Because of this lack of scientific output, ACHD patients in those developing countries are poorly understood and ill-defined, making future planning more challenging. In this manuscript, we describe the surgical needs of a cohort of ACHD patients in Egypt. Determining the clinical profile, different presentations, and types of required surgical interventions should help initiate a service for this underserved and poorly defined group of patients. In this study, more than half of the patients had a form of previous palliative or total repair. Patients who have never had any previous intervention were divided into a group with simple defects including atrial septal defects, ventricular septal defects, and subaortic membrane (25% of all cases), and another group with more complex neglected cases like those with single ventricle physiology and unrepaired tetralogy of Fallot. In contrast, over 90% of ACHD in developed countries had a previous intervention in infancy or childhood.[ 3 ] On the other hand, smaller numbers of ACHD patients in developing countries had received a form of correction previously (35% in Ghana and only 19% in Egypt).[ 3 , 4 , 8 ] Reviewing our patient cohort, we can recognize that the surgical teams dealing with ACHD patients in Egypt are facing the complexity of both unrepaired naturally surviving adults, and those with previous repair presenting with residual defects or delayed second-stage single ventricle repair. Out of all patients requiring surgery, 13.6% had a previous Glenn shunt during childhood and presented with progressive effort intolerance and desaturation. There is insufficient evidence in Egypt about the frequency of single ventricle palliative surgeries, the age at which these surgeries take place, and the percentage of patients who undergo Fontan procedures after the first stage of superior cavo-pulmonary anastomosis. Many ACHD patients in Egypt require Fontan surgery for a variety of reasons, including inadequate resources, a lack of supplies and proper conduits, and insufficient expertise at some hospitals to deal with patients requiring univentricular repair in childhood. Furthermore, the debate about the optimal timing for Fontan completion renders them a lesser priority in many pediatric cardiac facilities. Delaying the Fontan procedure has been proposed by believers in the “ticking clock theory”: any Fontan circulation will eventually fail, and should thus be postponed to the last feasible stage.[ 9 ] However, it should be emphasized that contemporary Fontan surgical procedures have improved long-term prognosis[ 9 ], and several reports have shown that earlier Fontan surgery is associated with better outcomes.[ 10 , 11 ] Fontan completion in adulthood should therefore be considered the exception and can pose a challenge in treating ACHD patients in developing countries. Nonetheless, few reports have shown acceptable short and midterm outcomes.[ 12 , 13 ] Patients with tetralogy of Fallot (ToF), whether native or previously repaired, represented almost one-fourth of all ACHD patients in this study. Many previous reports have shown that patients with repaired ToF have a good mid-term survival, approaching 90% at 20 years post-repair.[ 14 , 15 ] This survival has led to increasing numbers of adult patients with repaired ToF presenting to ACHD centers worldwide. These patients frequently present with significant morbidities including severe pulmonary regurgitation and right ventricular dilatation requiring re-intervention, arrhythmias, residual shunts, and progressive effort intolerance.[ 16 , 17 ] In developing countries, naturally surviving adults with ToF are among those seeking medical help. In our cohort, unrepaired ToF, with or without previous palliative shunts, represented more than one-third of all ToF patients. Few previous studies investigated the outcome of late repair of ToF in adulthood with controversial results.[ 18 – 20 ] Management of unrepaired adult patients with ToF is usually considered a real challenge as these patients are subjected to chronic hypoxemia increasing the risk of myocardial dysfunction, arrhythmias, and other complications like cerebral insults caused by right to left shunting.[ 19 ] For our patients with repaired TOF, surgical pulmonary valve replacement was frequently indicated for severe pulmonary incompetence and/or stenosis. In general, it is considered safe with low short and mid-term mortality.[ 21 ] However, increased risk of mortality was reported in redo surgeries[ 22 ], older patients, and those with concomitant surgeries.[ 23 ] In recent years, percutaneous pulmonary valve replacement has become a less invasive and effective alternative to address native pulmonary valve regurgitation/stenosis and dysfunctional conduits. The learning curve and cost of the procedure are its main obstacles in developing countries making it largely unavailable.[ 24 , 25 ] Multimodality cardiovascular imaging is becoming an integral part of the evaluation of ACHD patients.[ 26 – 28 ] In the present study, the final decision about the indication and type of surgical procedure required either cardiac MRI or CT scan in two-thirds of the patients and cardiac catheterization with hemodynamic assessment and/or angiography in one-fourth of the patients. The choice of the imaging modality was usually based on the clinical question, in addition to the strengths and limitations of each modality. Performing and interpreting these tests requires trained personnel with sufficient knowledge and understanding of congenital heart anatomy, pathophysiology, and surgical / trans-catheter interventions. Certainly, building an efficient ACHD service requires a well-organized partnership between ACHD-specialized cardiologists, congenital cardiac surgeons, and cardiac imagers with this kind of training and expertise. Study limitations The present study has some limitations. It described only ACHD patients who needed further surgical correction, in a single center. Thus, this cohort does not represent the whole population of ACHD patients in Egypt. The outcome of surgeries was not described as it was out of the scope of the study. However, we believe it will be highly beneficial to report the results, complications, and challenges faced by the surgical team operating on this group of patients. Conclusion The group of ACHD patients in Egypt who require further surgical correction is heterogeneous with various presentations and needs. Most of them previously received a form of intervention. The required surgeries vary from simple to complex procedures. Creating an efficient ACHD program necessitates the collaboration of dedicated cardiologists, radiologists, and cardiothoracic surgeons who are familiar with the unique demands and difficulties of this group of patients. Declarations The authors did not receive support from any organization for the submitted work. The authors have no relevant financial or non-financial interests to disclose. Author Contribution All authors contributed to this manuscript's writing, revision, and finalization. Conceptualization, A.A.S, A.M, M.E., M.H., and A.A.E; methodology, M.M.A., A.M., S.L., M.E. and O.A; validation, A.A.S., M.H. and A.A.E; writing—original draft preparation, A.A.S., M.M., and A.A.E.; writing—review and editing, A.S., M.M. and A.A.E.; supervision, A.A.E. All authors have read and agreed to the published version of the manuscript. References Warnes CA (2017) Adult congenital heart disease: The challenges of a lifetime. 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J Thorac Cardiovasc Surg 148:1450–1453 Lee C, Lee C-H, Kwak JG (2016) Outcomes of redo pulmonary valve replacement for bioprosthetic pulmonary valve failure in 61 patients with congenital heart disease. Eur J Cardio-Thoracic Surg 50:470–475 Jain A, Oster M, Kilgo P et al (2012) Risk Factors Associated With Morbidity and Mortality After Pulmonary Valve Replacement in Adult Patients With Previously Corrected Tetralogy of Fallot. Pediatr Cardiol 33:601–606. https://doi.org/10.1007/s00246-012-0185-z Kothari S (2021) Percutaneous pulmonary valve implantation in India: Quo Vadis? Ann Pediatr Cardiol 14:310 Lurz P, Coats L, Khambadkone S et al (2008) Percutaneous Pulmonary Valve Implantation. Circulation 117:1964–1972. https://doi.org/10.1161/CIRCULATIONAHA.107.735779 Babu-Narayan SV, Giannakoulas G, Valente AM et al (2016) Imaging of congenital heart disease in adults. Eur Heart J 37:1182–1195 Di Salvo G, Miller O, Babu Narayan S et al (2018) Imaging the adult with congenital heart disease: a multimodality imaging approach—position paper from the EACVI. Eur Hear J - Cardiovasc Imaging 19:1077–1098 Burchill LJ, Huang J, Tretter JT et al (2017) Noninvasive Imaging in Adult Congenital Heart Disease. Circ Res 120:995–1014 Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted 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. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-3860952","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":267125348,"identity":"766f626e-2b10-4a3e-8057-0bfaecf74a3e","order_by":0,"name":"Amir Anwar Samaan","email":"","orcid":"","institution":"Cairo University","correspondingAuthor":false,"prefix":"","firstName":"Amir","middleName":"Anwar","lastName":"Samaan","suffix":""},{"id":267125349,"identity":"a5cf1e03-8ec1-44a0-a2d0-a1848b42f21b","order_by":1,"name":"Mostafa M. Abdrabou","email":"","orcid":"","institution":"Cairo University","correspondingAuthor":false,"prefix":"","firstName":"Mostafa","middleName":"M.","lastName":"Abdrabou","suffix":""},{"id":267125350,"identity":"29fa3038-0ae1-4eb5-98d9-d4fafb91a91f","order_by":2,"name":"Amir Mostafa","email":"","orcid":"","institution":"Cairo University","correspondingAuthor":false,"prefix":"","firstName":"Amir","middleName":"","lastName":"Mostafa","suffix":""},{"id":267125351,"identity":"ae67d583-574f-4ea0-a162-b79893d6576f","order_by":3,"name":"Osama AbdElAziz","email":"","orcid":"","institution":"Cairo University","correspondingAuthor":false,"prefix":"","firstName":"Osama","middleName":"","lastName":"AbdElAziz","suffix":""},{"id":267125352,"identity":"ed5358e5-54a2-4e99-8548-9d9d21ba2360","order_by":4,"name":"Mahmoud El Shazly","email":"","orcid":"","institution":"Al Nas Hospital","correspondingAuthor":false,"prefix":"","firstName":"Mahmoud","middleName":"El","lastName":"Shazly","suffix":""},{"id":267125353,"identity":"d530a60f-dc78-4658-99f0-d2eff0dae6a2","order_by":5,"name":"Sherif Lotfy","email":"","orcid":"","institution":"Al Nas Hospital","correspondingAuthor":false,"prefix":"","firstName":"Sherif","middleName":"","lastName":"Lotfy","suffix":""},{"id":267125354,"identity":"19e8e446-b857-4f9b-8aaf-dce93c5a371d","order_by":6,"name":"Mohamed Hassan","email":"","orcid":"","institution":"Cairo University","correspondingAuthor":false,"prefix":"","firstName":"Mohamed","middleName":"","lastName":"Hassan","suffix":""},{"id":267125355,"identity":"1bd5b3f2-4dd5-43bb-8ffa-5fd214b91bde","order_by":7,"name":"Ahmed A. Elamragy","email":"data:image/png;base64,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","orcid":"","institution":"Cairo University","correspondingAuthor":true,"prefix":"","firstName":"Ahmed","middleName":"A.","lastName":"Elamragy","suffix":""}],"badges":[],"createdAt":"2024-01-13 18:29:07","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-3860952/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-3860952/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":49713629,"identity":"7cd83318-363d-422d-983c-ea7ee396ea51","added_by":"auto","created_at":"2024-01-16 20:42:36","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":264571,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-3860952/v1/38bc9954-5a19-4b40-ada4-6ff95f860af5.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Clinical profile of adult patients referred to congenital heart surgery in Egypt, a single center experience.","fulltext":[{"header":"Introduction","content":"\u003cp\u003eOver the past decades, the population of adult patients with congenital heart disease (CHD) has remarkably grown worldwide. This is mainly related to the advances seen in congenital heart surgery and pediatric cardiac care. Some reports postulated that the adult congenital heart disease (ACHD) population is currently outnumbering the pediatric CHD group in Europe and the United States.[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e] Data from the developing world are scarce, but some reports show similar trends.[\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eDespite significant advances in the field of congenital heart surgery in recent years, significant challenges remain, particularly for surgeons operating on adult patients with congenital defects. The majority of cardiac surgical repair procedures during childhood are not curative,[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e] and many of the remaining cardiac lesions may progress or begin to affect cardiac structure and function over time. Tissue valves and conduits frequently require repeated interventions when they degenerate. Redo surgeries entail higher risk sternotomy, which may injure previous conduits or dilated aortas adherent to the sternum.[\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eIn developing countries, where facilities for congenital cardiac surgery are not widely available, patients with CHD who survive till adulthood have a different profile than those in developed countries.[\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e] Many ACHD patients have either mild lesions that have been missed in childhood[\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e] or severe CHD that was operated on in infancy and childhood, while some patients have either survived despite complex unrepaired defects or had some form of palliative or incomplete repair.\u003c/p\u003e \u003cp\u003eOne of the major challenges facing ACHD service in low- and middle-income countries is the lack of adequate data on the disease burden and specific needs of this peculiar group.[\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e, \u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e] Clearly, data on the various presentations and types of required surgeries are insufficient.\u003c/p\u003e \u003cp\u003eMethods\u003c/p\u003e \u003cp\u003eOur retrospective study included all CHD patients above the age of 14 years who sought medical advice at an Egyptian tertiary cardiac center and required surgical repair. It was conducted over one year. At the outpatient clinic, all referred patients were evaluated by one of the ACHD team cardiologists. Candidates for surgical intervention were presented in a heart team meeting that included a congenital cardiac surgeon, a congenital structural interventionist, a radiologist, and a cardiologist after all non-invasive testing and hemodynamic studies were completed. Accepted cases by the committee were included in the study. Age, gender, presentation, functional capacity, resting oxygen saturation, diagnosis, previous intervention and its timing, surgery type, need for cardiac magnetic resonance imaging (CMR) or computed tomography (CT), and need for pre-surgery catheter hemodynamic assessment were all collected. Approval was obtained from the ethics committee of Al Nas Hospital. The procedures used in this study adhere to the tenets of the Declaration of Helsinki.\u003c/p\u003e"},{"header":"Results","content":"\u003cp\u003eBetween March 2022 and February 2023, 220 patients attended the ACHD clinic; the heart team committee discussed 134 patients and decided on surgery in 103 cases, while the remaining 86 patients were either given medical treatment/follow-up decisions or scheduled for trans-catheter intervention.\u003c/p\u003e \u003cp\u003eHalf of the patients were males (49.5%), the mean age was 23 years, 34% were cyanotic and the majority presented in NYHA functional class II (44.7%) and III (35.95). Fifty-eight patients (56.3%) had a form of previous surgical or trans-catheter intervention at a median age of 4 years (IQR 2.1\u0026ndash;11.8 years) at the time of the first procedure: 15.5% of them had two procedures, while 7% had three or more separate surgical or catheter interventions. The types of these surgeries are summarized in table (1).\u003c/p\u003e \u003cp\u003e \u003cb\u003eTable\u0026nbsp;(1).\u003c/b\u003e Types of previous surgeries/ catheter interventions.\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"No\" id=\"Taba\" border=\"1\"\u003e \u003ccolgroup cols=\"2\"\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 \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eType of previous surgeries/interventions\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eNumber of patients\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTotal Fallot repair\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e14\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eBT (Blalock- Taussig) shunts\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e15\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eGlenn\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e14\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePA banding\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e7\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eASD closure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eVSD closure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSurgical aortic coarctation repair\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eBalloon pulmonary valvuloplasty\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSubaortic membrane resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eRastelli\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eKawashima\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCommon AV canal repair\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSurgical pulmonary valvotomy\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eLPA stent\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTV repair\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTVR\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAVR\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 (one with Kono procedure)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003e \u003cb\u003eBT shunt\u003c/b\u003e: Blalock Taussig shunt, \u003cb\u003ePA\u003c/b\u003e: pulmonary artery, \u003cb\u003eASD\u003c/b\u003e: atrial septal defect, \u003cb\u003eVSD\u003c/b\u003e: ventricular septal defect, \u003cb\u003ecommon AV canal\u003c/b\u003e: common atrioventricular canal, \u003cb\u003eLPA\u003c/b\u003e: left pulmonary artery, \u003cb\u003eTV\u003c/b\u003e: tricuspid valve, \u003cb\u003eTVR\u003c/b\u003e: tricuspid valve replacement, \u003cb\u003eAVR\u003c/b\u003e: aortic valve replacement.\u003c/p\u003e \u003cp\u003eTetralogy of Fallot (TOF) was the most frequent diagnosis among our cohort (24 patients, 23.3%): 8 did not have any form of previous intervention, 2 had BT shunts during childhood and 14 had a previous total repair. One of the uncorrected patients presented with a large ascending aortic aneurysm and ruptured sinus of Valsalva. Nineteen patients (18.4%) were referred for surgical ASD closure: 11 had large secundum ASDs not amenable for trans-catheter closure, 7 had sinus venosus ASDs (6 of them had associated partial anomalous pulmonary venous drainage) and one patient had primum ASD (partial AV canal). Twelve patients (11.6%) had double outlet right ventricle (DORV); 9 with a previous palliative shunt with/without pulmonary artery banding while one patient had total repair (Rastelli\u0026rsquo;s operation). Eight patients (7.8%) had transposition of great arteries (TGA); two of them had congenitally corrected (CC) TGA with severe incompetence of the tricuspid (systemic) valve. The other 6 patients had D-TGA with protected pulmonary circulation in addition to either ASD or VSD; 3 of them had previous Glenn procedures and the remaining 3 had previous BT shunts. Five patients (4.8%) had a common atrioventricular (AV) canal; four with previous pulmonary artery banding (one of them had severe pulmonary stenosis), and 2 had previous total repair. Five patients (4.8%) had subaortic membranes with significant left ventricular outflow tract obstruction. Six patients (5.8%) had Ebstein anomaly; one had a previous repair and another had a tricuspid valve replacement. Four patients (3.8%) had ventricular septal defects; two were residual defects post-surgical closure associated with significant aortic regurgitation due to cusp prolapse, one was a restrictive defect with left ventricular dilatation and the last one had previous surgical closure with iatrogenic severe tricuspid incompetence. Three patients (2.9%) had tricuspid atresia; all had previous Glenn procedures. Three patients (2.9%) had severe pulmonary regurgitation post previous surgical or catheter pulmonary valvotomy. Two patients (1.9%) had double-chambered right ventricle. Two patients (1.9%) had partial anomalous pulmonary venous drainage. Two patients had unrepaired Shone complex (1.9%). Two patients (1.9%) had double inlet left ventricle. Single patients had the following diagnoses: supravalvular aortic stenosis, branch pulmonary stenosis, double outlet left ventricle, aortic paravalvular leak, pulmonary stenosis, and right atrial appendage aneurysm.\u003c/p\u003e \u003cp\u003eTypes of surgeries decided for the patients after the heart team discussion are listed in Table\u0026nbsp;2.\u003c/p\u003e \u003cp\u003e \u003cb\u003eTable\u0026nbsp;(2).\u003c/b\u003e Types of required surgeries for ACHD patients.\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"No\" id=\"Tabb\" border=\"1\"\u003e \u003ccolgroup cols=\"2\"\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 \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eType of surgery\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eNumber of patients (percentage)\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTotal cavo-pulmonary anastomosis (Fontan surgery)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e21 patients (20.3%); Completion Fontan surgery (after a previous Glenn shunt) in 16 patients (15.3%) and without previous surgery in 5 patients (4.8%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSurgical ASD closure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e19 patients (18.4%) (with PAPVD repair in 6 patients and pulmonary valvotomy in 1 patient)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSurgical pulmonary valve replacement\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e15 (14.5%) patients (with RVOT reconstruction in 1 patient, VSD closure in 1 patient, TV repair in 2 patients, and repair of LPA origin stenosis in one patient)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTotal ToF repair\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e10 patients (9.7%) (with MAPCAs surgical closure in 2 patients and after RVOT stenting in 2 patients)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTricuspid valve surgery\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e6 patients (5.8%);\u003c/p\u003e \u003cp\u003e- TV repair in 5 patients (4.8%) in addition to ASD closure in one patient and Glenn surgery in another patient.\u003c/p\u003e \u003cp\u003e- TV replacement in one patient who had a common AV canal, PA banding in infancy, and two previous attempts for repair, and presented with massive TR\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSubaortic membrane resection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e7 patients (6.7%); catheter coiling of a saccular aortic aneurysm was required in one patient, additional mitral valve repair in 2 patients (Shone complex patients), aortic valve replacement in one patient\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eSurgical VSD closure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3 patients (2.9%); with aortic valve repair in 2 patients.\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e- Total repair of DORV\u003c/p\u003e \u003cp\u003e- Tricuspid (Systemic) valve replacement\u003c/p\u003e \u003cp\u003e- Double-chambered RV repair\u003c/p\u003e \u003cp\u003e- Repair of PAPVD\u003c/p\u003e \u003cp\u003e- Repair of bilateral branch pulmonary artery stenosis\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 patients (1.9%) each\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e- Aortic valve repair\u003c/p\u003e \u003cp\u003e- Hepatic incorporation surgery\u003c/p\u003e \u003cp\u003e- Mitral valve replacement\u003c/p\u003e \u003cp\u003e- Partial AV canal repair\u003c/p\u003e \u003cp\u003e- RAA aneurysm excision\u003c/p\u003e \u003cp\u003e- Redo AVR with relief of RVOT obstruction\u003c/p\u003e \u003cp\u003e- Redo degenerated Tricuspid valve replacement in addition to mitral valve replacement\u003c/p\u003e \u003cp\u003e- Repair of aortic aneurysm and ruptured sinus of Valsalva in a post-TOF repair patient\u003c/p\u003e \u003cp\u003e- Replacement of RV-PA conduit\u003c/p\u003e \u003cp\u003e- Surgical correction of CAVC and PS,\u003c/p\u003e \u003cp\u003e- Surgical correction of RVOT obstruction in a previously repaired ToF patient,\u003c/p\u003e \u003cp\u003e- Surgical correction of supravalvular AS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eOne patient (0.9%) each\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"2\"\u003e\u003cb\u003eASD\u003c/b\u003e: atrial septal defect, \u003cb\u003eAVR\u003c/b\u003e: aortic valve replacement, \u003cb\u003eAS\u003c/b\u003e: aortic stenosis, \u003cb\u003eCAVC\u003c/b\u003e: common atrioventricular canal, \u003cb\u003eDORV\u003c/b\u003e: double outlet right ventricle, \u003cb\u003eLPA\u003c/b\u003e: left pulmonary artery stenosis, \u003cb\u003eMAPCAs\u003c/b\u003e: Major aortopulmonary collaterals, \u003cb\u003ePAPVD\u003c/b\u003e: partial anomalous pulmonary venous drainage, \u003cb\u003eRVOT\u003c/b\u003e: right ventricular outflow tract, TV: tricuspid valve, \u003cb\u003eRV\u003c/b\u003e: right ventricle, \u003cb\u003eRV-PA\u003c/b\u003e: right ventricle to pulmonary artery, \u003cb\u003eRAA\u003c/b\u003e: right atrial appendage aneurysm, \u003cb\u003eToF\u003c/b\u003e: Tetralogy of Fallot\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eOther than echocardiography, 65% of the patients required further non-invasive testing: 39 (37.8%) had cardiac magnetic resonance imaging, 23 (22.3%) had a CT scan and 5 (4.8%) had both tests. Pre-operative cardiac catheterization was performed in 26 patients (25.2%).\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eThe care of ACHD patients in developing countries constitutes a major challenge. In a previous report, the number of ACHD centers per 10\u0026nbsp;million inhabitants was estimated to be 0.1 in Africa in comparison to a substantially higher number in Europe (3.6) and North America (1.7).[\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e] According to the same report, the African research output in the field of ACHD was also extremely lower than in Europe and the United States. Because of this lack of scientific output, ACHD patients in those developing countries are poorly understood and ill-defined, making future planning more challenging.\u003c/p\u003e \u003cp\u003eIn this manuscript, we describe the surgical needs of a cohort of ACHD patients in Egypt. Determining the clinical profile, different presentations, and types of required surgical interventions should help initiate a service for this underserved and poorly defined group of patients.\u003c/p\u003e \u003cp\u003eIn this study, more than half of the patients had a form of previous palliative or total repair. Patients who have never had any previous intervention were divided into a group with simple defects including atrial septal defects, ventricular septal defects, and subaortic membrane (25% of all cases), and another group with more complex neglected cases like those with single ventricle physiology and unrepaired tetralogy of Fallot. In contrast, over 90% of ACHD in developed countries had a previous intervention in infancy or childhood.[\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e] On the other hand, smaller numbers of ACHD patients in developing countries had received a form of correction previously (35% in Ghana and only 19% in Egypt).[\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e, \u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e] Reviewing our patient cohort, we can recognize that the surgical teams dealing with ACHD patients in Egypt are facing the complexity of both unrepaired naturally surviving adults, and those with previous repair presenting with residual defects or delayed second-stage single ventricle repair.\u003c/p\u003e \u003cp\u003eOut of all patients requiring surgery, 13.6% had a previous Glenn shunt during childhood and presented with progressive effort intolerance and desaturation. There is insufficient evidence in Egypt about the frequency of single ventricle palliative surgeries, the age at which these surgeries take place, and the percentage of patients who undergo Fontan procedures after the first stage of superior cavo-pulmonary anastomosis. Many ACHD patients in Egypt require Fontan surgery for a variety of reasons, including inadequate resources, a lack of supplies and proper conduits, and insufficient expertise at some hospitals to deal with patients requiring univentricular repair in childhood. Furthermore, the debate about the optimal timing for Fontan completion renders them a lesser priority in many pediatric cardiac facilities. Delaying the Fontan procedure has been proposed by believers in the \u0026ldquo;ticking clock theory\u0026rdquo;: any Fontan circulation will eventually fail, and should thus be postponed to the last feasible stage.[\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e] However, it should be emphasized that contemporary Fontan surgical procedures have improved long-term prognosis[\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e], and several reports have shown that earlier Fontan surgery is associated with better outcomes.[\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e] Fontan completion in adulthood should therefore be considered the exception and can pose a challenge in treating ACHD patients in developing countries. Nonetheless, few reports have shown acceptable short and midterm outcomes.[\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e, \u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]\u003c/p\u003e \u003cp\u003ePatients with tetralogy of Fallot (ToF), whether native or previously repaired, represented almost one-fourth of all ACHD patients in this study. Many previous reports have shown that patients with repaired ToF have a good mid-term survival, approaching 90% at 20 years post-repair.[\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e, \u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e] This survival has led to increasing numbers of adult patients with repaired ToF presenting to ACHD centers worldwide. These patients frequently present with significant morbidities including severe pulmonary regurgitation and right ventricular dilatation requiring re-intervention, arrhythmias, residual shunts, and progressive effort intolerance.[\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e, \u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e] In developing countries, naturally surviving adults with ToF are among those seeking medical help. In our cohort, unrepaired ToF, with or without previous palliative shunts, represented more than one-third of all ToF patients. Few previous studies investigated the outcome of late repair of ToF in adulthood with controversial results.[\u003cspan additionalcitationids=\"CR19\" citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e] Management of unrepaired adult patients with ToF is usually considered a real challenge as these patients are subjected to chronic hypoxemia increasing the risk of myocardial dysfunction, arrhythmias, and other complications like cerebral insults caused by right to left shunting.[\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eFor our patients with repaired TOF, surgical pulmonary valve replacement was frequently indicated for severe pulmonary incompetence and/or stenosis. In general, it is considered safe with low short and mid-term mortality.[\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e] However, increased risk of mortality was reported in redo surgeries[\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e], older patients, and those with concomitant surgeries.[\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e] In recent years, percutaneous pulmonary valve replacement has become a less invasive and effective alternative to address native pulmonary valve regurgitation/stenosis and dysfunctional conduits. The learning curve and cost of the procedure are its main obstacles in developing countries making it largely unavailable.[\u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e, \u003cspan citationid=\"CR25\" class=\"CitationRef\"\u003e25\u003c/span\u003e]\u003c/p\u003e \u003cp\u003eMultimodality cardiovascular imaging is becoming an integral part of the evaluation of ACHD patients.[\u003cspan additionalcitationids=\"CR27\" citationid=\"CR26\" class=\"CitationRef\"\u003e26\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR28\" class=\"CitationRef\"\u003e28\u003c/span\u003e] In the present study, the final decision about the indication and type of surgical procedure required either cardiac MRI or CT scan in two-thirds of the patients and cardiac catheterization with hemodynamic assessment and/or angiography in one-fourth of the patients. The choice of the imaging modality was usually based on the clinical question, in addition to the strengths and limitations of each modality. Performing and interpreting these tests requires trained personnel with sufficient knowledge and understanding of congenital heart anatomy, pathophysiology, and surgical / trans-catheter interventions. Certainly, building an efficient ACHD service requires a well-organized partnership between ACHD-specialized cardiologists, congenital cardiac surgeons, and cardiac imagers with this kind of training and expertise.\u003c/p\u003e \u003cp\u003eStudy limitations\u003c/p\u003e \u003cp\u003eThe present study has some limitations. It described only ACHD patients who needed further surgical correction, in a single center. Thus, this cohort does not represent the whole population of ACHD patients in Egypt. The outcome of surgeries was not described as it was out of the scope of the study. However, we believe it will be highly beneficial to report the results, complications, and challenges faced by the surgical team operating on this group of patients.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eThe group of ACHD patients in Egypt who require further surgical correction is heterogeneous with various presentations and needs. Most of them previously received a form of intervention. The required surgeries vary from simple to complex procedures. Creating an efficient ACHD program necessitates the collaboration of dedicated cardiologists, radiologists, and cardiothoracic surgeons who are familiar with the unique demands and difficulties of this group of patients.\u003c/p\u003e"},{"header":"Declarations","content":" \u003cp\u003eThe authors did not receive support from any organization for the submitted work.\u003c/p\u003e \u003cp\u003eThe authors have no relevant financial or non-financial interests to disclose.\u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eAll authors contributed to this manuscript's writing, revision, and finalization. Conceptualization, A.A.S, A.M, M.E., M.H., and A.A.E; methodology, M.M.A., A.M., S.L., M.E. and O.A; validation, A.A.S., M.H. and A.A.E; writing\u0026mdash;original draft preparation, A.A.S., M.M., and A.A.E.; writing\u0026mdash;review and editing, A.S., M.M. and A.A.E.; supervision, A.A.E. 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Circulation 117:1964\u0026ndash;1972. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1161/CIRCULATIONAHA.107.735779\u003c/span\u003e\u003cspan address=\"10.1161/CIRCULATIONAHA.107.735779\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eBabu-Narayan SV, Giannakoulas G, Valente AM et al (2016) Imaging of congenital heart disease in adults. Eur Heart J 37:1182\u0026ndash;1195\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eDi Salvo G, Miller O, Babu Narayan S et al (2018) Imaging the adult with congenital heart disease: a multimodality imaging approach\u0026mdash;position paper from the EACVI. Eur Hear J - Cardiovasc Imaging 19:1077\u0026ndash;1098\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eBurchill LJ, Huang J, Tretter JT et al (2017) Noninvasive Imaging in Adult Congenital Heart Disease. Circ Res 120:995\u0026ndash;1014\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Adult congenital heart disease, surgery, Egypt","lastPublishedDoi":"10.21203/rs.3.rs-3860952/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-3860952/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eA major challenge facing adult congenital heart disease (ACHD) service in developing countries is the lack of adequate data on the disease burden and specific needs of this group. We aimed to describe ACHD patients in Egypt who are in need for further surgical correction, their various presentations, and the types of surgeries required.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eMethods\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe study included ACHD patients considered for further surgical correction after a heart team discussion at a tertiary cardiac center in Egypt. Data about age, gender, presentation, functional capacity, resting oxygen saturation, diagnosis, previous intervention and its timing, and the type of surgery decided were collected.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eResults\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eOver one year, 103 cases (49.5% males, mean age 23 years) were referred to surgery out of 134 cases discussed at the heart team committee. One-third of the patients (34%) were cyanotic and the majority presented in NYHA functional class II (44.7%) and III (35.95%). More than half of the patients (56.3%) had a previous surgical or trans-catheter intervention with a median age of 4 years at the time of the first procedure. The most frequently encountered diagnoses were: Tetralogy of Fallot (ToF) (23.3%), atrial septal defect (18.4%), double outlet right ventricle (DORV) (11.6%), and transposition of great arteries (TGA) (7.8%). Surgical procedures that were required most were: Fontan procedure (20.3%), surgical ASD closure (18.4%), surgical pulmonary valve replacement (14.5%), total ToF repair (9.7%), subaortic membrane resection (6.7%) and tricuspid valve surgery (5.8%).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe group of ACHD patients in Egypt who require further surgical correction is heterogeneous with various presentations and needs. The required surgeries vary from simple to complex procedures. An efficient ACHD program necessitates the collaboration of dedicated cardiologists, radiologists, and cardiothoracic surgeons who are familiar with the unique demands and difficulties of this patient group.\u003c/p\u003e","manuscriptTitle":"Clinical profile of adult patients referred to congenital heart surgery in Egypt, a single center experience.","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-01-16 10:48:04","doi":"10.21203/rs.3.rs-3860952/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"d31107a2-8ede-43f5-828a-059ca0fdf40a","owner":[],"postedDate":"January 16th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2024-01-16T20:34:29+00:00","versionOfRecord":[],"versionCreatedAt":"2024-01-16 10:48:04","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-3860952","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-3860952","identity":"rs-3860952","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}