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Rheology of right ventricular outflow tract obstruction: sub-pulmonary membrane developing months after primary intervention to treat pulmonary atresia with intact interventricular septum

Published online by Cambridge University Press:  31 May 2021

Baher M. Hanna*
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, Cairo University, Cairo, Egypt
Wesam E. El-Mozy
Affiliation:
Department of Radiology, Cairo University, Cairo, Egypt
Sonia A. El-Saiedi
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, Cairo University, Cairo, Egypt
*
Author for correspondence: Baher Hanna, MD, Tel: +201222441732.E-mail: baherhanna@gmail.com

Abstract

Isolated sub-pulmonary membrane is a rare condition, the origin of which has been debatable. Transcatheter treatment of pulmonary valve atresia with intact interventricular septum by radiofrequency perforation and balloon dilatation to restore biventricular circulation is gaining more popularity, with improving results over time. We report in our experience of 79 cases in 10 years the development of a sub-pulmonary membrane in 4 cases: causing significant obstruction requiring surgical excision in one case that revealed a fibrous membrane on pathology; causing mild right ventricular outflow tract obstruction in another and not yet causing obstruction in 2. On cardiac MRI, the right ventricular outflow tract and the right ventricular outflow tract/pulmonary atresia angle showed no morphological abnormalities.

Type
Original Article
Copyright
© The Author(s), 2021. Published by Cambridge University Press

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References

Duggal, B, Bajaj, M, Bansal, NO Subpulmonic membrane associated with a supracristal VSD: a rare cause of right ventricular outflow obstruction. Echocardiography 2011; 28: E89E90.CrossRefGoogle ScholarPubMed
Raff, GW, Gaynor, JW, Weinberg, PM, Spray, TL, Gleason, M Membranous subpulmonic stenosis associated with ventricular septal defect and aortic insufficiency. J Am Soc Echocardiogr 2000; 13: 5860.CrossRefGoogle ScholarPubMed
Tefera, E, Bermudez-Canete, R, Rubio, L Discrete subpulmonic membrane in association with isolated severe pulmonary valvar stenosis. BMC Cardiovasc Disord 2013; 13: 43.CrossRefGoogle ScholarPubMed
Tomar, M, Radhakrishnan, S, Sharma, R Isolated subpulmonary membrane causing critical neonatal pulmonary stenosis with concordant atrioventricular and ventriculoarterial connections. Ped Cardiol 2009; 11: 59.Google ScholarPubMed
Anderson, RH, Jacobs, ML The anatomy of tetralogy of Fallot with pulmonary stenosis. Cardiol Young 2008; 18 (Suppl 3): 1221.CrossRefGoogle ScholarPubMed
Vasec, Jl, Goetz, KK Balloon valvuloplasty of a subpulmonic membrane. Am Heart J 1990; 119: 14191421.Google Scholar
Spicer, D, Bridgeman, J, Brown, N, Mohun, T, Anderson, R The anatomy and development of the cardiac valves. Cardiol Young 2014; 24: 10081022.CrossRefGoogle ScholarPubMed
Foker, JE Outcomes and questions about discrete subaortic stenosis. Circulation 2013; 127: 14471450.CrossRefGoogle ScholarPubMed
Shar, JA, Brown, KN, Keswani, SG, Grande-Allen, J, Sucosky, P Impact of aortoseptal angle abnormalities and discrete subaortic stenosis on left-ventricular outflow tract hemodynamics: Preliminary computational assessment. Front Bioeng Biotechnol 2020; 8: 114.CrossRefGoogle ScholarPubMed
Hickey, EJ, Caldarone, CA, McCrindle, BW Left Ventricular Hypoplasia, a spectrum of disease involving the left ventricular outflow tract, aortic valve, and aorta. JACC 2012; 59: S43S54.CrossRefGoogle ScholarPubMed
Gewillig, M, Daenen, W, Dumoulin, M, et al. Rheologic genesis of discrete subvalvular aortic stenosis: a Doppler echocardiographic study. J Am Coll Cardiol 1992; 19: 818824.CrossRefGoogle ScholarPubMed
Mohan, JC, Shukla, M, Mohan, V, Sethi, A Acquired discrete subaortic stenosis late after mitral valve replacement. Indian Heart J 2016; 68: 105109.CrossRefGoogle ScholarPubMed
Silbiger, JJ, Lee, S, Christia, P, Perk, G Mechanisms, pathophysiology, and diagnostic imaging of left ventricular outflow tract obstruction following mitral valve surgery and transcatheter mitral valve replacement. Echocardiography 2019; 36: 11651172.CrossRefGoogle ScholarPubMed
Gale, AW, Cartmill, TB, Bernstein, L Familial subaortic membranous stenosis. Aust N Z J Med 1974; 4: 576581.CrossRefGoogle ScholarPubMed
Schmidt, MA, Ensing, GJ, Michels, VV, Carter, GA, Hagler, DJ, Feldt, RH Autosomal dominant supravalvular aortic stenosis: large three-generation family. Am J Med Genet 1989; 32: 384389.CrossRefGoogle ScholarPubMed
Richardson, ME, Menahem, S, Wilkinson, JL Familial fixed subaortic stenosis. Int J Cardiol 1991; 30: 351353.CrossRefGoogle ScholarPubMed
Chiu, JJ, Chien, S Effects of disturbed flow on vascular endothelium: pathophysiological basis and clinical perspectives. Physiol Rev 2011; 91: 173.CrossRefGoogle ScholarPubMed
Butany, J, Vaideeswar, P, David, TE Discrete subaortic membranes in adults- a clinicopathological analysis. Cardiovasc Pathol 2009; 18: 236242.CrossRefGoogle ScholarPubMed