Prenatal Shone’s complex; unusual presentation with premature foramen ovale closure

Prenatal Shone’s complex; unusual presentation with premature foramen ovale closure | Authorea try { document.documentElement.classList.add('js'); } catch (e) { } var _gaq = _gaq || []; _gaq.push(['_setAccount', 'G-8VDV14Y67G']); _gaq.push(['_trackPageview']); (function() { var ga = document.createElement('script'); ga.type = 'text/javascript'; ga.async = true; ga.src = ('https:' == document.location.protocol ? 'https://ssl' : 'http://www') + '.google-analytics.com/ga.js'; var s = document.getElementsByTagName('script')[0]; s.parentNode.insertBefore(ga, s); })(); Skip to main content Preprints Collections Wiley Open Research IET Open Research Ecological Society of Japan All Collections About About Authorea FAQs Contact Us Quick Search anywhere Search for preprint articles, keywords, etc. Search Search ADVANCED SEARCH SCROLL This is a preprint and has not been peer reviewed. Data may be preliminary. 2 August 2025 V1 Latest version Share on Prenatal Shone’s complex; unusual presentation with premature foramen ovale closure Authors : Sameh Aborizk 0000-0002-0651-2504 [email protected] and Rania El Kaffas Authors Info & Affiliations https://doi.org/10.22541/au.175412184.44603323/v1 225 views 85 downloads Contents Abstract Information & Authors Metrics & Citations View Options References Figures Tables Media Share Abstract Here in, we present an unusual case of a severe form of fetal shone complex complicated by premature closure of foramen ovale and subsequent severe pulmonary hypertension and right heart compression with restrictive filling and consequent right side diastolic failure and fetal hydrops. Prenatal Shone’s complex; unusual presentation with premature foramen ovale closure Here in, we present an unusual case of a severe form of fetal shone complex complicated by premature closure of foramen ovale and subsequent severe pulmonary hypertension and right heart compression with restrictive filling and consequent right side diastolic failure and fetal hydrops. Authors: Sameh Abdullatif Aborizk, MD Rania El Kaffas, MD Affiliations: 1 radiology department, Kasr Alainy Faculty of medicine, Cairo university. 1 Fetal cardiology clinic, Alnas Hospital 2 Pediatric cardiology department, Kasr Alainy Faculty of medicine, Cairo university. 2 Fetal cardiology clinic, Alnas Hospital A written informed consent was obtained from the patient for publication of this case report and accompanying echocardiographic images. Conflicts of Interest The authors declare no conflicts of interest. A 32-year-old female patient presented at 33 weeks of gestation with fetal cardiomegaly and hydrops, following a prior diagnosis of critical aortic valve stenosis and dilated left ventricle. Findings Our study revealed cardiomegaly with discrete ventricular disproportion. The left ventricle (LV) was dilated, exhibiting severely impaired systolic function and endocardial fibroelastosis, while the right ventricle (RV) appeared compressed with a marked restrictive filling pattern despite preserved systolic function ( image 1 panel A and B ). The mitral valve apparatus was severely dysplastic, featuring echogenic hypoplastic papillary muscles, thickened tendinous cords, and markedly thickened dysplastic leaflets. This resulted in restrictive opening, near-complete systolic non-coaptation, and severe mitral regurgitation ( image 1 panel D, E and H, image 2 image short axis stack ). Consequently, the left atrium (LA) was dilated, with deviation of the foramen ovale flap to the point of near-complete intrauterine closure, effectively abolishing interatrial shunting. The combination of severe mitral stenosis/regurgitation and premature foramen ovale closure led to LA hypertension, marked pulmonary vein attenuation, and severe pulmonary hypertension (image 1 panel A and B) . The right atrium (RA) was severely compressed, mirroring the RV’s restrictive filling, resulting in systemic venous congestion and fetal hydrops (notably ascites) (image 1 panel C and G) . Outflow Tract and Great Vessel Evaluation The left ventricular outflow tract (LVOT) was severely narrowed (∼2.5 mm), with a small aortic valve annulus (∼2.5 mm) and aortic root followed by mild post-stenotic dilation of the ascending aorta. The aortic valve leaflets had normal thickness and mobility, ruling out severe valvular stenosis ( image 1 panel F and I ). The distal aortic arch was hypoplastic (∼2.6 mm) with complete flow reversal, indicating duct-dependent systemic circulation ( image 3 panel A and B ). The ductus arteriosus was small (∼3 mm) due to low RV output secondary to compression and restrictive filling, despite being the sole supply for systemic circulation (supra- and infra-diaphragmatic). A final diagnosis of Severe Shone complex was reached based upon the constellation of these findings: Pathophysiology Elevated LA pressures stemmed from increased LV diastolic pressures and severe mitral regurgitation, exacerbated by premature foramen ovale closure. This led to severe pulmonary venous hypertension. As well, marked RV and RA compression resulted in systemic venous congestion, hydrops, and critically reduced RV output (evidenced by the diminutive ductus arteriosus). Although the LV showed some reversibility criteria with preserved mitral annular size and LV length despite poor contraction and fibroelastosis (e.g., potential for balloon valvuloplasty in isolated critical aortic stenosis), multi-level left-sided obstructions (severe LVOTO, hypoplastic aortic annulus, aortic arch hypoplasia and severe mitral valve dysplasia with associated premature intrauterine foramen oval closure) precluded postnatal intervention. Postnatal Course After counseling regarding the poor prognosis, parents opted for intervention. The infant was delivered via urgent cesarean section, requiring immediate mechanical ventilation. Despite maximal support, systemic perfusion was very poor, with oxygen saturation declining from 48% to 20% over few hours. The infant died 12 hours post-delivery. Discussion: This case presents a complete form of Shone’s complex complicated by premature intrauterine closure of the foramen ovale and severe pulmonary hypertension. Notably, the condition was detected early in the prenatal course, when the left ventricle (LV) was subjected to severe pressure overload. This led to LV dilation with dysfunction and endocardial fibroelastosis, Right heart compression and restrictive filling, Fetal hydrops and severe pulmonary hypertension (due to premature foramen ovale closure). Had this sequence occurred earlier in gestation, the presentation might have resembled classic hypoplastic left heart syndrome (HLHS), with right ventricular (RV) decompression and resolution of hydrops. However, in this case, the severe LV outflow tract obstruction (LVOTO) and aortic annular hypoplasia together with left atrial hypertension and consequent severe pulmonary hypertension and poor fetal general condition due to poor perfusion and prematurity resulted in a poor prognosis, limiting postnatal catheter-based or surgical options. This variant of HLHS underscores the challenges in management due to advanced fetal hemodynamic compromise and complex multiple left sided obstructive lesions. Image1: Panel A and B systolic and diastolic phases of the 4-chamber anatomy showing the dilated LV, dilated LA, closed foramen ovale and compressed RA and RV. Panel C and G show free ascites and A wave reversal in the ductus venosus denoting elevated right atrial pressures. Panel D, E and H show severe mitral regurgitation. Panel F and I show systolic and diastolic phases of the LVOT obstruction and the small aortic annulus and aortic root. Image2: Short axis stack of images from mid ventricle to basal levels showing the rudimentary hypoplastic papillary muscles, thickened shortened tendinous cords and thick dysplastic mitral valve leaflets with almost systolic non coaptation. Image3: Panel A shows systolic still preserved limited antegrade flow through the LVOT and aortic root while panel B shows the retrograde flow within the smallish aortic arch representing duct dependent systemic circulation. Video 1: Long and short axis 2D cine clips showing the dilated poorly contractile left ventricle, the dilated left atrium, dysplastic mitral valve apparatus, the closed foramen ovale, severe left ventricular outflow tract obstruction and retrograde flow in the aortic arch as a duct dependent systemic circulation. Video 2: Long axis 2D and color doppler cine clips showing severe mitral regurgitation and 3 chamber views showing the severe LVOT obstruction and severe hydropic changes due to marked compression upon right heart structures. Information & Authors Information Version history V1 Version 1 02 August 2025 Copyright This work is licensed under a Non Exclusive No Reuse License. Keywords foramen ovale closure lvoto severe mitral regurgitation shone's complex Authors Affiliations Sameh Aborizk 0000-0002-0651-2504 [email protected] Alnas Hospital View all articles by this author Rania El Kaffas Alnas Hospital View all articles by this author Metrics & Citations Metrics Article Usage 225 views 85 downloads .FvxKWukQNSOunydq8rnd { width: 100px; } Citations Download citation Sameh Aborizk, Rania El Kaffas. Prenatal Shone’s complex; unusual presentation with premature foramen ovale closure. Authorea . 02 August 2025. DOI: https://doi.org/10.22541/au.175412184.44603323/v1 If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Simply select your manager software from the list below and click Download. For more information or tips please see 'Downloading to a citation manager' in the Help menu . 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