Nitric oxide-sensitive pulmonary hypertension in congenital rubella syndrome.

Francesco Raimondi,Elisa Di Pietro,Fiorella Migliaro,Letizia Capasso,Antonio Rapacciuolo,Francesco Borgia

doi:10.1155/2015/198570

Francesco Raimondi, Elisa Di Pietro + Show 4 more

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https://doi.org/10.1155/2015/198570

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Abstract

Persistent pulmonary hypertension is a very rare presentation of congenital virus infection. We discuss the case of complete congenital rubella syndrome presenting at echocardiography with pulmonary hypertension that worsened after ductus ligation. Cardiac catheterization showed a normal pulmonary valve and vascular tree but a PAP = 40 mmHg. The infant promptly responded to inhaled nitric oxide while on mechanical ventilation and was later shifted to oral sildenafil. It is not clear whether our observation may be due to direct viral damage to the endothelium or to the rubella virus increasing the vascular tone via a metabolic derangement.

Highlights

Congenital rubella syndrome (CRS) results from an invalidating vertical infection originally described by Gregg as the association of cataracts, deafness, and cardiac defects
WalterNicolet et al recently reported two CMV neonates presenting with progressive pulmonary hypertension of the newborn (PPHN) where inhaled nitric oxide (iNO) was not contributory to the outcome [2]
Toizumi et al published the largest series of CRS affected infants with echocardiographic evidence of PPHN [1]

Summary

Introduction

Congenital rubella syndrome (CRS) results from an invalidating vertical infection originally described by Gregg as the association of cataracts, deafness, and cardiac defects. An interesting Vietnamese series of infants affected from congenital rubella was published, some of whom had developed PPHN [1]. We discuss the case of an infant affected from full CRS presenting at birth with PPHN showing a progressive course and requiring emergency inhaled nitric oxide (iNO) and later chronic sildenafil treatment.

Case Report

Discussion