Clinical analysis of 38 infants with hypoplastic left heart syndrome

Abstract

Objective To discuss the clinical features and treatment of hypoplastic left heart syndrome(HLHS). Methods A single-center retrospective study of infants with HLHS who were admitted to the Department of Pediatric Cardiology of Guangdong General Hospital from January 2004 to December 2014 was performed, and the infants were followed-up. Results There were 38 cases of HLHS, including 23 boys and 15 girls, accounting for 0.15% of the 25 698 in-patients with congenital heart disease during the corresponding period.The ages of onset ranged from 1 hour to 5 months, while the ages at admission ranged from 1 day to 11 months.Weight at admission ranged from 1.55 to 14.00 kg (the mean weight was 3.9 kg), and hospital day ranged from 1 to 88 days (the mean hospital day was 46 days). Twenty-four of the patients had comorbidities at admission, including 20 patients with pneumonia, 2 with septicemia, 3 developed multi-organ dysfumction, 1 with cytomegalovirus infection, 4 with brain injury caused by hypoxia, 1 with congenital airway constriction, and 1 with renal failure.Eighteen patients received operation with 8 survi-vals, 16 patients gave up and were discharged, 3 died before operation because of give-up, 10 patients died during or after operation.One of the children who did not receive the operation lived until 4 years old.Patients underwent operation at the ages between 2 days and 4 months, and 10 of them needed ventilation before operation, 8 needed emergency operation.Modified Blalock-Taussig shunt(MBTS) was applied in 10 patients while sano-shunt in 8 patients.In the 8 survivals, MBTS and sano-shunt was performed equally in 4 patients in Norwood stage Ⅰ, while 5 received MBTS and 3 sano-shunt who died after stage Ⅰ.After the operation, 7 patients developed severe low cardiac output, and 1 had pericardial tamponade, 3 suffered from pulmonary infection, 1 had to use extracorporeal membrane oxygenation, and 1 underwent seizure. Conclusions HLHS is a rare malformation of heart.Norwood Procedure Staged palliative surgery is the predominant management option for HLHS. Key words: Infant; Hypoplastic left heart syndrome; Treatment; Prognosis