Long-term results of one-and-a-half ventricle repair in complex cardiac anomalies

Objective: One-and-a-half ventricle repair is a surgical option for complex cardiac anomalies characterized by right-ventricle hypoplasia or dysfunction. The long-term result analyses or large clinical reviews are rare. The aim of this study is to evaluate the long-term functional results of this surgical procedure. Methods: The 29 patients, who underwent one-and-a-half ventricle repair from June 1993 to June 2007, at our Institution, were included. The median age was 26 months (range 6 months to 26 years). One-and-a-half-ventricle repair was performed for volume unloading the small right ventricle (group A, n = 18), for work unloading in patients with chronic right-ventricle dysfunction (group B, n = 9), and with the acute postoperative right-ventricular dysfunction (group C, n = 2). The mean Z value of the tricuspid valve in group A was −3.6 ± 0.7 (range −2.6 to −4.8). The median follow-up duration of hospital survivors was 82 months (range 3 months to 16 years). Results: There were four early deaths (two in group A and C, respectively) and no late cardiac death. During follow-up, no patient had superior vena cava (SVC) hypertension or chronic atrial arrhythmia. There was one patient with protein-losing enteropathy. Functional status was New York Heart Association Functional Class I in 21 patients and class II in three patients. Arterial oxygen saturation increased significantly after operation, compared with the preoperative saturation (86.6 ± 9.7-96.8 ± 4.0%, p ≪ 0.01). Two patients in group B needed medications related to the cardiac function. Four patients underwent reoperation. The 10-year freedoms from late reoperation were 80.0 ± 12.6% in group A and 51.4 ± 20.4% in group B. Conclusions: The patients with one-and-a-half ventricle repair resulted in favorable late survival in this series. During the follow-up period, most surviving patients showed good functional status without common late complications of the Fontan procedure such as, recurrent cyanosis, pulmonary arteriovenous fistulas, chronic arrhythmias, and SVC syndrome. This procedure appears to be a valid alternative to Fontan and biventricular repairs in patients with right-ventricular dysfunction or hypoplasia.