Transient isolated right ventricular hypertrophy: A benign phenotype in neonates: Case series and review of the literature

Abstract

Background

Transient isolated right ventricular hypertrophy (RVH) is an infrequently recognized cause of early neonatal cyanosis and prolonged oxygen requirement, closely mimicking cyanotic congenital heart disease or persistent pulmonary hypertension of the newborn.

Methods

We present a narrative review of published neonatal cases of transient isolated RVH and describe illustrative observations from four term neonates managed at a tertiary neonatal unit over a 3-year period. Clinical features, antenatal exposures, echocardiographic findings, management, and outcomes were reviewed. A focused literature search identified previously reported neonatal cases.

Results

Including the present cohort, 18 neonatal cases have been described to date. Most neonates presented within the first day of life with cyanosis. Echocardiography demonstrated isolated RVH with preserved biventricular function and absence of structural heart disease; premature ductal closure was documented in several cases. Identified risk factors included fetal or perinatal distress, maternal exposure to nonsteroidal anti-inflammatory drugs or corticosteroids, maternal diabetes, and polyphenol-rich substances. Clinical improvement occurred with supportive care, and follow-up echocardiography showed regression of RVH within 4–12 weeks.

Conclusions

Transient isolated RVH represents a benign, self-limiting cause of early neonatal cyanosis. Awareness of this phenotype and its natural history may help avoid unnecessary investigations/interventions.