Usefulness of cardiac transplantation in children with visceral heterotaxy (asplenic and polysplenic syndromes and single right-sided spleen with levocardia) and comparison of results with cardiac transplantation in children with dilated cardiomyopathy

• Published in: The American journal of cardiology Vol. 89; no. 11; pp. 1275 - 1279
• Main Authors: Larsen, Ranae L., Eguchi, Jim H., Mulla, Neda F., Johnston, Joyce K., Fitts, James, Kuhn, Micheal A., Razzouk, Anees J., Chinnock, Richard E., Bailey, Leonard L.
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01-06-2002; Elsevier
• Subjects: Abnormalities, Multiple - surgery; Adolescent; Biological and medical sciences; Cardiomyopathy, Dilated - surgery; Child; Child, Preschool; Coronary Disease; Female; Graft Survival; Heart Defects, Congenital - surgery; Heart Transplantation; Humans; Infant; Levocardia - surgery; Male; Medical sciences; Postoperative Complications; Retrospective Studies; Spleen - abnormalities; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of the heart; Survival Analysis; Syndrome; Treatment Outcome; Viscera - abnormalities; Human; Heart; Prognosis; Congenital; Treatment efficiency; Splenic disease; Clinical form; Situs inversus; Cardiovascular disease; Transplantation; Homotransplantation; Congenital disease; Concomitant disease; Treatment; Malformation; Abdominal disease; Surgery; Heart disease; Child; Hétérotaxy
• ISSN: 0002-9149; 1879-1913
• DOI: 10.1016/S0002-9149(02)02325-1

Surgical mortality is high in children with visceral heterotaxy (VH), particularly if atrioventricular valve insufficiency, ventricular dysfunction, or aortic atresia is present. This study reviews the outcome of cardiac transplantation (CT) in infants and children with VH and congenital heart disease who are at high risk for standard palliative or corrective surgery. We reviewed CT outcomes in 29 children with VH, congenital heart disease, atrioventricular valve insufficiency, ventricular dysfunction, and/or aortic atresia. Median age at CT was 3.1 years. Cardiac surgery had been performed in 20 patients (69%) before CT. Follow-up since CT has been 8.5 ± 2.2 years. Outcomes were compared with 45 children who underwent transplantation for dilated cardiomyopathy. Actuarial graft survival in the VH group at 30 days and 1, 5, and 10 years was 100%, 86%, 68%, and 50%, respectively, compared with 100%, 96%, 83%, and 68% in children who underwent transplantation for dilated cardiomyopathy (p = 0.12). Splenic status, cardiac position, age at CT, number of prior cardiac surgeries, or systemic venous anomalies were not predictors of mortality after CT. Cardiopulmonary bypass and graft ischemic times were longer in the VH group; time on the ventilator after CT, length of hospitalization, and rejection, infection, post-transplant lymphoproliferative disease, and transplant coronary artery disease rates were equal. Thus, CT is a viable alternative therapy for high-risk patients with VH, possibly offering improved survival over standard surgical management.