Christopher T Sower1, Jennifer C Romano2, Sunkyung Yu1, Ray Lowery1, Sara K Pasquali1, Jeffrey D Zampi3. 1. Division of Cardiology, Department of Pediatrics, University of Michigan C.S. Mott Children's Hospital, Ann Arbor, Michigan. 2. Section of Pediatric Cardiovascular Surgery, Department of Cardiac Surgery, University of Michigan C.S. Mott Children's Hospital, Ann Arbor, Michigan. 3. Division of Cardiology, Department of Pediatrics, University of Michigan C.S. Mott Children's Hospital, Ann Arbor, Michigan. Electronic address: jzampi@med.umich.edu.
Abstract
BACKGROUND: Although overall outcomes have improved for single-ventricle patients, substantial morbidity and mortality remain for certain high-risk groups. The hybrid stage I procedure is an alternative to the Norwood operation for stage I palliation, but it remains unclear whether it is associated with improved outcomes in high-risk patients. METHODS: This single-center, nested, case-control study included high-risk patients with a systemic right ventricle who underwent hybrid stage I or Norwood palliation from January 2000 to December 2016. High-risk features included prematurity < 34 weeks, birth weight < 2.5 kg, restrictive/intact atrial septum, at least moderate atrioventricular valve regurgitation or right ventricular dysfunction, genetic or extracardiac anomalies, or left ventricular sinusoids. Patients were matched by presence of genetic anomaly, restrictive/intact atrial septum, and prematurity/weight < 2 kg. Early and midterm outcomes were compared in the matched hybrid vs Norwood groups. RESULTS: The study included 96 patients (35 hybrid, 61 Norwood). Despite improved 30-day survival in hybrid patients (91% vs 66%, P < .01), 1-year survival was similar between the hybrid and Norwood groups (46% vs 48%, P = .9). No hybrid patients required dialysis or extracorporeal membrane oxygenation after stage I palliation as compared with 19% and 22% of Norwood patients, respectively (both P < .01). Hybrid patients, however, required more unplanned reinterventions (43% vs 21%, P = .02). CONCLUSIONS: There remains significant morbidity and mortality among high-risk single-ventricle infants. Despite an early survival benefit, hybrid stage I palliation has not been associated with improved midterm outcomes at our center.
BACKGROUND: Although overall outcomes have improved for single-ventricle patients, substantial morbidity and mortality remain for certain high-risk groups. The hybrid stage I procedure is an alternative to the Norwood operation for stage I palliation, but it remains unclear whether it is associated with improved outcomes in high-risk patients. METHODS: This single-center, nested, case-control study included high-risk patients with a systemic right ventricle who underwent hybrid stage I or Norwood palliation from January 2000 to December 2016. High-risk features included prematurity < 34 weeks, birth weight < 2.5 kg, restrictive/intact atrial septum, at least moderate atrioventricular valve regurgitation or right ventricular dysfunction, genetic or extracardiac anomalies, or left ventricular sinusoids. Patients were matched by presence of genetic anomaly, restrictive/intact atrial septum, and prematurity/weight < 2 kg. Early and midterm outcomes were compared in the matched hybrid vs Norwood groups. RESULTS: The study included 96 patients (35 hybrid, 61 Norwood). Despite improved 30-day survival in hybrid patients (91% vs 66%, P < .01), 1-year survival was similar between the hybrid and Norwood groups (46% vs 48%, P = .9). No hybrid patients required dialysis or extracorporeal membrane oxygenation after stage I palliation as compared with 19% and 22% of Norwood patients, respectively (both P < .01). Hybrid patients, however, required more unplanned reinterventions (43% vs 21%, P = .02). CONCLUSIONS: There remains significant morbidity and mortality among high-risk single-ventricle infants. Despite an early survival benefit, hybrid stage I palliation has not been associated with improved midterm outcomes at our center.