Prenatal diagnosis and early neonatal management of patients with hypoplastic left heart syndrome: experience of a perinatal center

Abstract

Hypoplastic Left Heart Syndrome (HLHS) is the most common form of functional single-ventricle heart disease. Despite improvements in medical and surgical care, morbidity and mortality remain high, especially around the first surgical stage.

Objective: To describe the clinical characteristics of newborns with HLHS, seen at a perinatal referral center.

Patients and Method: Retrospective descriptive study of all newborns with pre and/or postnatal diagnosis of HLHS between 2014 and 2019 in the Complejo Asistencial Dr. Sótero del Rio. Medical records were reviewed collecting demographic and perinatal variables, clinical evolution, mortality associated with the first surgery, and survival at one year.

Results: Nineteen patients were included. Most were term newborns and 13/19 were female. All had a prenatal diagnosis. Five patients had associated extracardiac anomalies. 16/19 required vasoactive drugs and 17/19 used invasive mechanical ventilation. Seven patients received enteral feeding, without episodes of enterocolitis. The median age at transfer for surgery was 8 days. Thirteen patients underwent the Norwood procedure, with 31% of mortality, and 47% of survival at one year.

Conclusions: Neonatal management of patients with HLHS is complex and the multidisciplinary approach is fundamental. Mortality around the first surgical stage of our cohort is greater than the international reports which could be influenced by the absence of fetal therapy and the higher incidence of extracardiac malformations.