Experience with one and a half ventricle repair.

OBJECTIVE: This article presents a 10-year experience with one and a half ventricle repair for right ventricular hypoplasia or dysfunction. METHODS: From November 1986 to December 1996, 30 patients (mean age 6.7 +/- 8.5 years, range 4 months-40 years) with functionally abnormal right ventricles underwent a bidirectional Glenn shunt as part of the repair. Diagnoses included pulmonary atresia with intact ventricular septum (n = 15), Ebstein anomaly (n = 5), levotransposition of the great arteries (n = 3), pulmonary stenosis with right ventricular hypoplasia (n = 2), tetralogy of Fallot (n = 3), dextrotransposition of the great arteries (n = l), and Uhl anomaly (n = l). Concomitantly performed cardiac procedures included atrial septal defect closure (n = 27), fenestration of the atrial septum (n = 2), right ventricular cavity augmentation (n = 8), right ventricular outflow tract enlargement (n = 6), transannular patch (n = 13), modified Blalock-Taussig shunt closure (n = 16), tricuspid replacement (n = 3), tricuspid repair (n = 2), Rastelli procedure (n = 3), tricuspid commissurotomy (n = 2), and double switch (n = l). RESULTS: There were 2 early deaths (6.6%) and 1 late death. Mean early postoperative superior vena caval pressure was 14. 12 +/- 3.55 mm Hg and mean right atrial pressure was 10.3 +/- 5.16 mm Hg. Early oxygen saturation in the operating room with an inspired oxygen fraction of 1 was 97.2 +/- 2.5; oxygen saturation was 92.3 +/- 4.8 on room air at discharge. Mean oxygen saturations were 93.6% +/- 3.6% at 1 year of follow-up (P =.10) and 93.5% +/- 4. 1% at 5 years (P =.12). Overall survival was 90% at 5 years, and 21 patients (77%) were in New York Heart Association class I, 5 (18%) were in class II, and 1 (2.7%) was in class III. CONCLUSION: This procedure provides a valid alternative for correction of right ventricle hypoplasia or dysfunction. Early and intermediate follow-up results compare favorably with those of the Fontan procedure, but long-term follow-up is needed.