Staged Single Ventricle Palliation with Pulmonary Artery Rehabilitation for Unguarded Tricuspid Orifice and Hypoplastic Left Pulmonary Artery

TL;DR

This paper describes a complex surgical approach for a rare heart defect involving staged procedures to improve pulmonary artery function and manage right ventricular issues.

Contribution

The paper presents a novel staged surgical strategy for managing unguarded tricuspid orifice with pulmonary artery hypoplasia.

Findings

Staged single-ventricle palliation was successfully implemented for a patient with UGTO and left pulmonary artery hypoplasia.

Left pulmonary artery augmentation and bidirectional cavopulmonary shunt improved pulmonary blood flow.

The total cavopulmonary connection was ultimately achieved despite initial stenosis and required additional interventions.

Abstract

The patient was diagnosed with unguarded tricuspid orifice (UGTO), functional pulmonary atresia, and left pulmonary artery hypoplasia. In view of the severe right ventricular dysfunction, the staged single ventricular palliation procedure was selected. The surgical procedure on Starnes operation and left pulmonary artery augmentation was performed one month after birth. As left pulmonary artery stenosis was diagnosed, secondly, the bidirectional cavopulmonary shunt with additional systemic to pulmonary shunt and intrapulmonary patch septation and left pulmonary artery augmentation with in-situ pericardium were performed. Despite the necessity for additional balloon dilation and surgical blunt dilation, the total cavopulmonary connection operation was ultimately performed, resulting in the successful implementation of staged single-ventricle palliation in conjunction with left pulmonary artery rehabilitation.

Unguarded tricuspid orifice (UGTO) constitutes a rare congenital cardiac malformation, characterized by the absence of tricuspid valve (TV) leaflets and a normal annulus.

Figures