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Mid-Term Follow-Up of the One-and-a-Half Ventricle Repair for Complex Congenital Heart Defects
A. Ahmed, K. Zahka, E. Siwik, F. Erenberg, Y. Al-Khatib, A. Golden, W. Gauvin, M. Karimi, M. Uddin, J. Stork, H. Hennein. Rainbow Babies and Children's Hospital, Case Western Reserve University, Cleveland, OH,
BACKGROUND: The one-and-a-half (1½) ventricle repair consists of a bidirectional superior cavopulmonary connection along with complete separation of the pulmonary from the systemic circulation. We reviewed our mid-term results of this novel approach of incorporating a small or functionally compromised pulmonary ventricle into the pulmonary circulation.
METHODS: Over a ten-year period ending in 2006, 26 patients (mean age 5.6 ± 3.7 years) underwent a 1½ ventricle repair for a right ventricle (RV) that was either hypoplastic (n=7) or poorly functioning (n=8), or to facilitate the biventricular repair of complex cardiac anomalies (n=10). 23 patients (88%) had had at least one previous cardiac procedure. Concomitant procedures were frequent and included RV outflow tract reconstruction (n=17), tricuspid valve repair (n=11), repair of complete atrioventricular septal defect (n=2), combined Senning-Rastelli procedure (n=1), Fontan takedown (n=1), ventriculectomy (n=1), and the Damus-Kaye-Stansel procedure (n=1). The mean preoperative oxygen saturation was 72 ± 10%, tricuspid Z-score -1.4 ± 2.4, pulmonary vascular resistance 2.4 ± 0.8 wood units, and the RV was not tripartite in 14 patients (54%).
RESULTS: Three patients (11%) underwent re-operation; one at 9 months for severe tricuspid regurgitation, one at 6 years for replacement of a stenosed RV to pulmonary artery conduit, and the third at 7 years for a Konno procedure. All patients survived, and there have been no deaths at a mean follow-up of 4.2 ± 1.2 years. No patient has required the Fontan operation or cardiac transplantation, none has incapacitating cardiac failure, 19 (73%) were free of cardiac medications, and the mean late oxygen saturation was 95 ± 4.6%.
CONCLUSIONS: At mid-term follow-up, the 1½ ventricle repair appears to be a safe and effective mean of incorporating a moderately hypoplastic or dysfunctional RV into the pulmonary circulation, maintaining an adequate cardiac output, and achieving complete separation of the pulmonary and systemic circulations. While these results are encouraging, further follow-up will be necessary to assess the long-term efficacy and freedom from re-operation of this unique operative strategy.
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