Concordant Ventriculoarterial Connections With Abnormally Related Arterial Trunks (Anatomically Corrected Malposition) Associated With Right Ventricular Outflow Tract Obstruction by Accessory Valvar Tissue

Anatomically corrected malposition of the great arteries is a rare conotruncal anomaly that is often confused with congenitally corrected transposition of the great arteries. This case report describes the successful surgical management of an adolescent with severe right ventricular outflow obstruction caused by accessory tricuspid valve tissue in the presence of situs inversus with atrioventricular and ventriculoarterial concordance with malposed great arteries (anatomically corrected malposition) {I,L,D}.

Staged Surgical Management of Anatomically Corrected Malposition of Great Arteries

A nine-month-old girl diagnosed with anatomically corrected malposition (atrioventricular discordance, ventriculoarterial concordance, and malposed great arteries) complicated by multiple ventricular septal defects (VSD) and multifactorial left ventricular outflow tract obstruction (LVOTO) presented for management after pulmonary artery banding. She underwent interim palliation in the form of bilateral cavopulmonary connections, a modified Damus-Kaye-Stansel-type anastomosis, and subsequent staged one-and-a-half ventricle repair (1.5 repair) at the age of three years in the form of VSD closure, hemi-Mustard, and LVOTO resection.

Unusual Ventriculo-Arterial Alignments and Connections

In this issue of World Journal for Pediatric and Congenital Heart Surgery, Kari and colleagues present a case of anatomically corrected malposition {S, L, L} with ventricular septal defect and complex subaortic stenosis managed by staged biventricular repair. This rare case with interesting management strategy provides an opportunity to clarify this and related unusual ventriculo-arterial alignments.