Palliative Operations for Cyanotic Congenital Heart Disease with Severely Asymmetrical Pulmonary Arteries

Abstract

Surgically managing patients with complex congenital heart disease and severely asymmetrical pulmonary arteries is challenging. Here, we report our experience using combined palliative procedures. The medical records of 28 patients with complex congenital heart disease and severely asymmetrical pulmonary arteries who underwent combined palliative procedures between January 2004 and April 2013 were retrospectively reviewed until January 2018. The patients were divided into three groups according to shunt procedure timing: in group A (n = 15), cavopulmonary and systemic-pulmonary shunting were performed simultaneously; in group B (n = 11), systemic-pulmonary shunting was performed first; and in group C (n = 2), cavopulmonary shunt was performed first. Patients were followed for a mean ± standard deviation of 4.18 ± 2.22 years. No operative deaths occurred. There were no postoperative complications in groups B or C, but there was one case of pulmonary effusion and one of chylothorax in group A. The superior vena cava pressures were higher in patients in groups A and B than in those in group C. The ventilatory support duration and intensive care unit stays were longer in group A than in groups B and C (p < 0.01). Hypoplastic pulmonary artery development significantly improved after the use of three systemic-pulmonary shunts (p < 0.05), while the peripheral oxygen saturation increased from 67%±17% preoperatively to 85%±8% postoperatively (p < 0.001). Haemoglobin concentration decreased from 190 ± 34 g/L preoperatively to 136 ± 26 g/L postoperatively (p < 0.001). Two patients underwent double ventricle correction. Two patients underwent Fontan procedure. One patient underwent one and a half ventricle correction. One patient underwent collateral occlusion. Combined palliative procedures can achieve acceptable arterial oxygen saturation without extra volume loading and rescue the hypoplastic pulmonary artery.