Profound systemic hypoperfusion in a patient with ventricular inversion temporised with transcatheter ductal closure

Isolated atrioventricular discordance with ventriculoarterial concordance, or ventricular inversion, is a rare congenital cardiac anomaly that produces transposition-like physiology. We report a case of prenatally diagnosed ventricular inversion presenting with profound systemic hypoperfusion secondary to a large patent ductus arteriosus (PDA). Initial management included prostaglandin E1 to maintain ductal patency and balloon atrial septostomy to promote intracardiac mixing. Despite these measures, the patient developed significant pulmonary overcirculation and systemic steal, necessitating urgent transcatheter PDA closure. This intervention resulted in immediate hemodynamic improvement and stabilization, allowing for subsequent definitive repair with VSD closure and Senning atrial switch. The case underscores the importance of individualized hemodynamic assessment and the potential for transcatheter ductal closure to temporize systemic perfusion in select patients with ventricular inversion prior to anatomic correction.

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Publication Details

Journal
Cardiology in the Young
Published
2025-10-16
DOI
https://doi.org/10.1017/s1047951125109232
Primary Topic
Congenital Heart Disease Studies
Type
article
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article

Profound systemic hypoperfusion in a patient with ventricular inversion temporised with transcatheter ductal closure

J. Ryan Shea, Kieran Leong, M. Leong, Sally Hunt
Cardiology in the Young
Congenital Heart Disease Studies
article

Profound systemic hypoperfusion in a patient with ventricular inversion temporised with transcatheter ductal closure

J. Ryan Shea, Kieran Leong, M. Leong, Sally Hunt
article en

Abstract

Isolated atrioventricular discordance with ventriculoarterial concordance, or ventricular inversion, is a rare congenital cardiac anomaly that produces transposition-like physiology. We report a case of prenatally diagnosed ventricular inversion presenting with profound systemic hypoperfusion secondary to a large patent ductus arteriosus (PDA). Initial management included prostaglandin E1 to maintain ductal patency and balloon atrial septostomy to promote intracardiac mixing. Despite these measures, the patient developed significant pulmonary overcirculation and systemic steal, necessitating urgent transcatheter PDA closure. This intervention resulted in immediate hemodynamic improvement and stabilization, allowing for subsequent definitive repair with VSD closure and Senning atrial switch. The case underscores the importance of individualized hemodynamic assessment and the potential for transcatheter ductal closure to temporize systemic perfusion in select patients with ventricular inversion prior to anatomic correction.

Cardiology in the YoungVol. 35(11)
University of North Carolina at Chapel Hill (US)
Openalex Percentile: Top 61%
Congenital Heart Disease Studies
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