Objective: Moderate restrictive foramen ovale in neonates with hypoplastic left heart syndrome (HLHS) is considered to be favourable, reducing pulmonary overcirculation, before modified Norwood operation. However, some newborns with severe restriction of interatrial communication will have pulmonary vascular disease at birth, which correlates with increased perioperative mortality. This article studies the post-Norwood hemodynamic patterns and outcome for the particular group of HLHS newborns with restrictive left atrial outflow compared to other patients.
Methods: Restrictive left atrial outflow is defined as mitral and/or aortic atresia with intact ventricular septum, and restrictive foramen ovale, with 3 mm diameter or less with mean interatrial pressure gradient more than 5 mmHg at preoperative echo-Doppler. Four neonates fulfilled these criteriae among 18 consecutive patients, who underwent Norwood procedure from October 2002 to December 2003. Mean arterial pressure, heart rate, mean common atrial pressure, urinary output, central venous and arterial oximetry data, serum lactate levels, and dosages of milrinone, phentolamine and norepinephrine were collected at 0, 6, 12, 18 and 24 h after operation. Data were summarized as mean+/-SEM. For univariate comparison of different variables, Student's t-test was used.
Results: The postoperative hemodynamic pattern of patients with restrictive left atrial outflow was characterized by hypoxemia and low cardiac output. Arterial (66+/-3.0% vs 76+/-1.0%, P=0.01) and central venous (37+/-1.2 vs 52+/-1.1%, P=0.001) oxygen saturations were much lower than in patients without restriction. Arterio-venous oxygen saturation difference was wider (29+/-2.4% vs 23+/-0.9%, P=0.02) and serum lactate levels were higher (10.8+/-3.0 vs 2.8+/-0.2 mmol/l, P=0.03). Common atrial pressures were more elevated (12+/-0.8 vs 8+/-0.3 mmHg, P<0.001) and higher norepinephrine doses were needed (0.44+/-0.15 vs 0.06+/-0.01 microg/kg/min, P=0.03). The difference for the mean arterial pressures did not reach the significance level (48+/-2.0 vs 51+/-2.0 mmHg, P=0.2). Operative mortality was higher 75% (3/4) compared to 14.3% (2/14, P=0.04) for the other patients.
Conclusions: Restrictive left atrial outflow adversely affects outcome after modified Norwood procedure. Abnormal pulmonary vasculature leading to insufficient pulmonary perfusion is incriminated. To improve outcome, implantation of larger size modified Blalock-Taussig or right ventricle-to-pulmonary artery shunts and routine use of postoperative mechanical assist device should be considered.