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The presence of systemic and pulmonary venous anomalies in late presenting transposition of great arteries (TGA) may make standard atrial switch procedures difficult, necessitating modification in the surgical procedure. We present a case report of a 13-year-old boy with a partial anomalous connection of right superior pulmonary vein with TGA and regressed left ventricle, where a combination of Warden procedure and Mustard procedure was used.
RESUMO
Double-outlet left ventricle (DOLV) is a rare congenital cardiac anomaly in which both the aorta and pulmonary artery arise completely or predominantly from the left ventricle. DOLV is a spectrum and can be classified depending on the position of the ventricular septal defect (VSD) relative to the great vessels, the relationship of the great vessels, and the presence or absence of pulmonary or aortic outflow obstruction. In the absence of tricuspid atresia or hypoplastic right ventricle, two ventricle repair is the preferred surgical treatment. We report a 31-day-old, 2.1 kg neonate with DOLV, subaortic VSD who underwent a successful arterial switch with VSD closure.
RESUMO
AIMS AND OBJECTIVES: There is a paucity of data regarding the outcomes of Heart transplantation in children from the Indian subcontinent. The data of patients under the age of 18 undergoing an isolated heart transplantation was analyzed for patient clinical profiles and risk factors for early and medium-term mortality. Hospital mortality was defined as death within 90 days of transplantation and medium-term survival as follow up of up to 6 years. MATERIALS AND METHODS: A total of 97 patients operated between March 2014 and October 2019 were included in this study. Data was collected about their INTERMACS status, pulmonary vascular resistance, donor heart ischemic times, donor age, donor to recipient weight ratio and creatinine levels. RESULTS: The age range was from 1 to 18 with a mean of 10.6 ± 4.6 years. 67 % patients were in INTERMACS category 3 or less.12 children were on mechanical circulatory support at the time of transplant. The 90 day survival was 89 %. The risk factors for hospital mortality was lower INTERMACS category (odd's ratio 0.2143, P = 0.026), elevated creatinine (odd's ratio 5.42, P = 0.076) and elevated right atrial pressure (odd's ratio 1.19, P = 0.015). Ischemic time, pulmonary vascular resistance (PVR) and PVR index (PVRI) had no effect on 90 day survival. Kaplan Meier estimates for 5 year survival was 73 %. The medium term survival was affected by INTERMACS category (Hazard ratio 0.7, P = .078), donor age > 25 (Hazard ratio 1.6, P = 0.26) and raised serum creatinine values.(Hazard ratio 2.7, P = 0.012). All the survivors are in good functional class. CONCLUSIONS: Excellent outcomes are possible after heart transplantation in a pediatric population even in a resource constrained environment of a developing economy. More efforts are needed to promote pediatric organ donation and patients need to be referred in better INTERMACS category for optimal outcomes.
