Objective: To explore the clinical experiences of combined palliative procedures for cyanotic congenital heart defects with intractable hypoplasia of pulmonary arteries.
Methods: From August 2001 to September 2009, 31 patients with cyanotic congenital heart defects and intractable hypoplasia of pulmonary arteries underwent surgical procedures. Among them, 26 patients were pulmonary atresia with ventricular septal defect and the other 5 cases complicated congenital heart defects with pulmonary stenosis. Different kinds of palliative procedures were performed according to the morphology of right and left pulmonary arteries in every patient. If the pulmonary artery was well developed, Glenn procedure was performed. Modified Blalock-Taussig or Waterston shunt was performed if pulmonary arteries had hypoplasia. If the pulmonary arteries were of severe hypoplasia, Melbourne shunt was performed. Systemic-pulmonary artery shunts were performed bilaterally in 23 cases. Systemic-pulmonary shunt was performed in one side and Glenn procedure contralaterally in 8 cases.
Results: There was one early death because of cardiac arrest. The number of patients suffered from low cardiac output syndrome, perfusion lung and pulmonary infection postoperatively was 5, 3 and 2, respectively. Systemic-pulmonary shunts were reperformed after the original operation in 3 cases because of occlusion of conduits. The mean follow-up time was 25 ± 16 months (6 - 72 months). Left pulmonary index (8.1 ± 3.7 vs 14.9 ± 6.2), right pulmonary index (12.7 ± 8.1 vs 17.7 ± 7.8) and pulmonary index (20.9 ± 9.4 vs 32.6 ± 11.7) increased significantly (all P < 0.001). The pulmonary diameter increased significantly after modified Blalock-Taussig shunt (5.5 ± 1.0 vs 7.0 ± 2.0), modified Waterston shunt (5.7 ± 4.0 vs 9.2 ± 3.6) and melbourne shunt (2.2 ± 0.4 vs 7.4 ± 2.5) (all P < 0.05). Bilateral pulmonary arteries developed well compared with that of preoperative condition. Hemoglobin decreased from (194 ± 27) g/L to (174 ± 24) g/L (P < 0.05) and peripheral oxygen saturation increased from (65 ± 11)% to (84 ± 6)% (P < 0.001). During the follow-up ultimate complete repair were performed in 3 cases and one patients underwent Glenn procedure.
Conclusions: The procedures should be considered for hypoplasia of pulmonary arteries in cyanotic congenital heart defects. Combined palliative operation is an adequate therapy.