Long-term outcome for right heart function after biventricular repair of pulmonary atresia and intact ventricular septum.

OBJECTIVES: The right heart function of the patients with pulmonary atresia and intact ventricular septum was assessed periodically during the process of staged biventricular repair, and the implications for its long-term outcome were analyzed. SUBJECTS AND METHODS: During the period from 1971 to 1990, 21 neonates or infants with pulmonary atresia and intact ventricular septum had undergone initial palliative surgery. There were seven early postoperative deaths and one late death. Of the 13 survivors, 10 patients underwent subsequent biventricular repair and form the basis of this study. Their clinical records of roentgenography, electrocardiography, and catheterization studies at each staged period were reviewed retrospectively.
RESULTS: Arrhythmia occurred late in 2 patients, one of whom died by arrhythmia at 11 years after definitive surgical repair. Therefore the actuarial survival rate was 85.7% at 14 years. The catheterization study after the definitive biventricular repair revealed a significant fall in the right heart pressure (p = 0.0005) and significant improvement in the right ventricular ejection fraction (p = 0.0004). In angiocardiography, dilatation of the right atrium was noted in all patients and was more marked in those who developed arrhythmia in conjunction with rapid growth of the right ventricle. Moreover, the serial repeated electrocardiography disclosed progressive and significant prolongation of both PQ interval (p = 0.003) and QRS duration (p = 0.021).
CONCLUSIONS: Although biventricular repair for pulmonary atresia and intact ventricular septum proved to attain a satisfactory long-term result, it failed to resolve right heart dysfunction. Postoperative arrhythmia was prone to precipitate progressive dilatation of the right atrium.