Surgical outcome of staged univentricular-type repairs for patients with univentricular physiology and pulmonary hypertension.

ABSTRACT

BACKGROUND: There is no consensus about the most appropriate limits of pulmonary artery pressure and vascular resistance in case of patients undergoing univentricular or one and one-half ventricular repair. This study was conducted to analyze the mortality and morbidity of a heterogenous group of patients with a functionally univentricular heart and pulmonary artery hypertension, undergoing pulmonary artery banding followed by univentricular-type repairs. METHODS AND RESULTS: Out of 254 patients undergoing pulmonary artery banding for a functionally univentricular heart with increased pulmonary blood flow, 148 patients underwent definitive second stage surgery. Post-band hemodynamic evaluation revealed persistently high pulmonary artery pressure (> 18 mmHg), and pulmonary vascular resistance (>2.0 Woods units/m2) in 78.3% patients. Sixteen patients with moderate right ventricular hypoplasia were given a one and one-half ventricle repair (Group I), 82 patients a bidirectional Glenn connection (Group II), and 50 patients a fenestrated total cavopulmonary connection (Group III). The overall mortality following second stage surgery for the high pulmonary artery pressure group (n=116) was 30.17%, while none of the low pulmonary artery pressure group died (p=0.0009). Pulmonary hypertensive crises and/or systemic desaturation were the main causes of death at second stage repair. All mortality occurred in patients with mean pulmonary artery pressure > 18 mmHg and pulmonary vascular resistance > 3.5 Woods units/m2. Survivors from this group had persistent morbidity in the form of superior vena caval syndrome and suboptimal oxygen saturation (70-75%). CONCLUSIONS: It is advisable not to proceed with definitive second stage repair if post-pulmonary artery banding mean pulmonary artery pressure is over 25 mmHg and pulmonary vascular resistance exceeds 4.0 Woods units/m2. These patients may possibly be deemed to have undergone definitive palliation during their pulmonary artery banding.