A Case of Univentricular Heart of Left Ventricular Type with Atresia of Left Atrioventricular Valve and Coarctation of Thoracic Aorta / 日本心臓血管外科学会雑誌

ABSTRACT

A 3-month-old girl of univentricular heart of left ventricular type with atresia of left atrioventricular valve (LAVV) and coarctation of the aorta (Co/AO) is presented. UCG and angiography revealed concordant AV connection with straddling RAVV with transposed great arteries [SDDT]. The following pressures (in mmHg) were noted on catheterization RA mean 1 (a=3, v=1), LA mean 12 (a=17, v=14), LV 84/0/8, Ao 81/41, and PA 74/39. Patent foramen ovale (PFO) was restrictive and balloon atrioseptostomy was not feasible. Blalock-Hanlon atrial septectomy (8×6mm), subclavian flap aortoplasy (SFA) and pulmonary arterial banding were performed simultaneously under bilateral thoracotomy. Acute renal failure occurred after surgery and the girl required peritoneal dialysis for 5 days. At 6 months after surgery, girl is doing well. There will be a predictable fall in pulmonary vascular resistance after atrial septectomy and SFA with a ligation of PDA may result transient increase in systemic resistance. Therefore, atrial septectomy and SFA in conjunction with pulmonary arterial banding should be done simultaneously.