Pseudo Fallot: Septal prolapse with uninvited shunt

Pseudo Fallot: Septal prolapse with uninvited shunt | 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 Search Search ADVANCED SEARCH SCROLL This is a preprint and has not been peer reviewed.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. 8 September 2025 V1 Latest version Share on Pseudo Fallot: Septal prolapse with uninvited shunt Authors : Ashutosh Yadav and SOURABH AGSTAM 0000-0002-3060-5794 [email protected] Authors Info & Affiliations https://doi.org/10.22541/au.175736023.31501098/v1 132 views 83 downloads Contents Abstract Information & Authors Metrics & Citations View Options References Figures Tables Media Share Abstract A case of ventricular septal aneurysm with perimembranous ventricular septal defect, prolapsing into the right ventricular outflow tract causing dynamic obstruction and intermittent right to left shunt in a young adult. Title: Pseudo Fallot: Septal prolapse with uninvited shunt Names of authors: Dr. Ashutosh Yadav 1 Dr. Shivam Goel 2 Dr. Sourabh Agstam 2 Cardiology Fellow, Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Kerala All India Institute of Medical Sciences, New Delhi Address for correspondence: Dr Sourabh Agstam Assistant Professor Department of Cardiology Cardiothoracic Sciences Centre All India Institute of Medical Sciences Ansari Nagar, New Delhi -110029, India Email: [email protected] Total word count (excluding Title Page, References, and Figures Legends): 510 Conflict of Interest for all authors: None Funding: None Keywords: ventricular septal aneurysm, RVOT obstruction, VSD Case presentation: A 24 year old gentleman presented with exertional dyspnea(NYHA III severity) for 1 year, central cyanosis and pan digital clubbing(Grade III) for 3 years. On auscultation, a harsh ejection systolic murmur(Grade 4/6), loudest along the left upper sternal border was heard and was associated with a systolic thrill on palpation. He was afebrile with blood pressure 108/60 mm Hg, heart rate of 68/min and a saturation of 82% on ambient air. Transthoracic echocardiography revealed a non-restrictive perimembranous ventricular septal defect(VSD) with intermittent systolic right to left shunt due to dynamic prolapse of a thin walled structure into the right ventricular outflow tract(RVOT) upto the pulmonary valve(PV) with turbulent flow and elevated gradient across the RVOT. There was no malalignment of the interventricular septum, aortic override or presence of hypertrophic muscle bundles in the RVOT. There was no significant right ventricular hypertrophy and normal biventricular function was noted. Aortic and pulmonary valve morphology was normal with no regurgitation. Transesophageal echocardiography(TEE) revealed a large aneurysm of the membranous interventricular septum prolapsing anteriorly into the RVOT and across the PV during systole causing dynamic obstruction with intermittent right to left shunt across the VSD(Video 1A,1B, 2). Cardiac magnetic resonance imaging(CMR) corroborated the echocardiographic findings, ruled out thrombus and showed no focal late gadolinium enhancement(Figure 1). Right heart catheterisation showed significant right to left shunt(Qp:Qs0.8) across the VSD with mildly elevated pulmonary artery pressures(mean pulmonary arterial pressure 22 mm Hg, indexed pulmonary vascular resistance 6.6 WU/m2). Significantly elevated RV systolic pressure(114 mm Hg) was noted with a gradient of 72 mm Hg across the RVOT-PV. Right ventricular angiogram demonstrated the dynamic RVOT obstruction, confluent pulmonary arteries and no additional septum defects(Video 3). These findings were concordant with and complementary to echocardiographic evaluation. He underwent surgical excision of the aneurysmal membranous septum with VSD patch closure . He had an uneventful perioperative course. On follow up his functional status improved(NYHA I) and remained healthy at 18 months follow up. Discussion: Aneurysmal transformation of the membranous interventricular septum is rare and has been detected in 2 of 16000 autopsies[1]. It has been described in 20% of patients with a perimembranous ventricular septal defect[2]. The aneurysmal tissue could arise from the ventricular septum itself(true septal aneurysm) or from the tricuspid leaflets(tricuspid pouch). Once formed, it can extend into the membranous septum inlet, membranous septum outlet or into the RVOT. The size of the aneurysm sac is not consistent with the size of the associated ventricular septal defect nor the magnitude of shunt [3] . While it reduces the size of the defect, it it has been described to be associated with RVOT obstruction, aortic valve prolapse, tricuspid valve insufficiency, arrhythmias and endocarditis. In our patient, the dynamic RVOT obstruction due to prolapse of the aneurysmal membranous septum caused intermittent(systolic) right to left shunt leading to new onset systemic cyanosis, clubbing and patient symptoms. Multimodality evaluation was instrumental in delineating the anatomy, pathophysiology and hemodynamic significance of this unique presentation which improves our understanding of ventricular septal aneurysms in patients with perimembranous VSD. In patients with perimembranous VSD and ventricular septal aneurysm, surgical excision of the aneurysmal segment with patch closure of the ventricular septal defect has been described to avoid enlargement of the aneurysm and complications, irrespective of the symptoms[2]. In view of clinically and hemodynamically significant consequences, our patient underwent the same with a good outcome. References: 1. Steinberg I. Diagnosis of congenital aneurysm of the ventricular septum during life. Br Heart J 1957;19(1):8-12. 2. Yilmaz AT, zal E, Arslan M et al. Aneurysm of the membranous septum in adult patients with perimembranous ventricular septal defect. Eur J Cardiothorac Surg. 1997;11(12), 307-311. 3. Miyake T, Inoue T, Mushiake S. Right ventricular outflow tract obstruction by an aneurysm of the ventricular membranous septum: A systematic review of case reports. World J Pediatric and Congenital Heart Surg, 15(3), 380-388, 2024. Legend: Video 1(A and B): TEE modified Aortic long axis view showing perimembranous VSD with prolapse of aneurysmal septum into the RVOT and across the pulmonary valve showing intermittent right to left shunt. Video 2: TEE RV inflow outflow view showing perimembranous VSD with prolapse of aneurysmal membranous septum into the RVOT and across the pulmonary valve. Video 3: RV angiogram in lateral projection showing dynamic RVOT and pulmonary valve obstruction with confluent pulmonary arteries. Figure 1: CMRI demonstrating the RVOT obstruction due to prolapse of aneurysmal membranous septum. Information & Authors Information Version history V1 Version 1 08 September 2025 Copyright This work is licensed under a Non Exclusive No Reuse License. Keywords perimembranous ventricular septal defect right to left shunt right ventricular outflow tract obstruction ventricular septal aneurysm Authors Affiliations Ashutosh Yadav View all articles by this author SOURABH AGSTAM 0000-0002-3060-5794 [email protected] All India Institute of Medical Sciences New Delhi View all articles by this author Metrics & Citations Metrics Article Usage 132 views 83 downloads .FvxKWukQNSOunydq8rnd { width: 100px; } Citations Download citation Ashutosh Yadav, SOURABH AGSTAM. Pseudo Fallot: Septal prolapse with uninvited shunt. Authorea . 08 September 2025. DOI: https://doi.org/10.22541/au.175736023.31501098/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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