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Case report: morphological challenges to surgical repair in complete atrioventricular septal defect with isolated ventricular component

Published online by Cambridge University Press:  02 April 2025

Martina Larsson
Affiliation:
Department of Paediatric Cardiology, Great Ormond Street Hospital, London, UK
Mats Synnergren
Affiliation:
Department of Cardiothoracic Surgery, Great Ormond Street Hospital, London, UK
Thushara Rodrigo*
Affiliation:
Department of Paediatric Cardiology, Great Ormond Street Hospital, London, UK
*
Corresponding author: Thushara Rodrigo; Email: [email protected]

Abstract

We describe a trisomy 21 patient with postnatal diagnosis of atrioventricular septal defect with isolated ventricular component who had a complicated post-surgical course following complete repair. Clinical outcomes included moderate-severe residual atrioventricular valve regurgitation needing re-operation, complete heart block leading to pacemaker insertion, seizures secondary to subdural haemorrhages, and chylothorax. We describe the surgical considerations specific to this cardiac morphology.

Type
Case Report
Copyright
© The Author(s), 2025. Published by Cambridge University Press

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References

Lopez, L, Colan, S, Stylianou, M, et al. Relationship of echocardiographic Z scores adjusted for body surface area to age, sex, race, and ethnicity. Circ Cardiovasc Imaging 2017; 10: doi: 10.1161/CIRCIMAGING.117.006979.CrossRefGoogle ScholarPubMed
Dimopoulos, K, Constantine, A, Clift, P, et al. Cardiovascular Complications of Down Syndrome: Scoping Review and Expert Consensus. Circulation 2023; 147: 425441. doi: 10.1161/CIRCULATIONAHA.122.059706.CrossRefGoogle ScholarPubMed
Kaski, JP, Wolfenden, J, Josen, M, et al. Can atrioventricular septal defects exist with intact septal structures? Heart 2005; 92: 832835. doi: 10.1136/hrt.2005.069278.CrossRefGoogle ScholarPubMed
Suzuki, M, Kobayashi, M, Fukae, K. Atrioventricular septal defect with an absent or tiny ostium primum defect: a case series of three surgical cases. In J Thorac Cardiovasc Surg 2022; 38: 666669. doi: 10.1007/s12055-022-01404-4.Google ScholarPubMed
Kwon, MH, Schultz, AH, Lee, M, et al. Complete atrioventricular septal defect with absent or diminutive primum component: incidence, anatomic characteristics, and outcomes. J Thorac Cardiovasc Surg 2022; 163: 11561162. doi: 10.1016/j.jtcvs.2021.06.041.Google ScholarPubMed
Adachi, I, Uemura, H, McCarthy, KP, et al. Morphologic features of atrioventricular septal defect with only ventricular component: further observations pertinent to surgical repair. J Thorac Cardiovasc Surg 2009; 137: 132138.e2. doi: 10.1016/j.jtcvs.2008.03.032.Google ScholarPubMed
Khawaja, ZR, Cambronero, GE, Aboutabl, YM, et al. Superior transseptal versus left atriotomy approaches in isolated mitral valve surgery. JTCVS Open 2024; 22: 208213. doi: 10.1016/j.xjon.2024.08.019.Google ScholarPubMed
Guiraudon, GM, Ofiesh, JG, Kaushik, R. Extended vertical transatrial septal approach to the mitral valve. Ann Thorac Surg 1991; 52: 10581062. doi: 10.1016/0003-4975(91)91281-Y.CrossRefGoogle ScholarPubMed
Kan, C-D, Wang, J-N, Wu, J-M, et al. Isolated chylopericardium after intrapericardial procedures: possible role of inadvertent right efferent lymphatic trunk injury. Tex Heart Inst J 2007; 34: 8287. http://www.ncbi.nlm.nih.gov/pubmed/17420799.Google ScholarPubMed
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