# Respiratory Syncytial Virus Infection Triggering a Pulmonary Hypertensive Crisis in a Boy With Prader–Willi Syndrome-Associated Sleep-Disordered Breathing

## TL;DR

A boy with Prader-Willi syndrome experienced a severe pulmonary crisis due to RSV infection, highlighting the need for early screening and management in similar cases.

## Contribution

The paper highlights RSV as a potential trigger for acute cardiopulmonary decompensation in children with PWS and sleep-disordered breathing.

## Key findings

- RSV infection can cause acute respiratory failure in children with PWS and severe nocturnal hypoxemia.
- Noninvasive ventilation and oxygen improved respiratory status and normalized liver enzymes.
- Polysomnography confirmed obstructive sleep apnea with significant nocturnal desaturation.

## Abstract

Prader-Willi syndrome (PWS) is a multisystem genetic disorder frequently complicated by sleep-disordered breathing, including obstructive sleep apnea. Respiratory infections may precipitate acute decompensation in vulnerable patients with PWS. A four-year-old boy with genetically confirmed PWS receiving growth hormone therapy developed respiratory distress and cyanosis. He was found to be respiratory syncytial virus-positive and presented with acute respiratory acidosis and markedly elevated aminotransferases with coagulopathy. Echocardiography showed right-sided cardiac dilation consistent with pulmonary hypertension. He was managed with noninvasive ventilation, oxygen supplementation, and diuretics, followed by step-down to nasal continuous positive airway pressure. Liver enzymes normalized as his respiratory status improved. Polysomnography after stabilization showed obstructive sleep apnea with considerable nocturnal desaturation, and home continuous positive airway pressure was initiated.

Respiratory syncytial virus (RSV) infection can trigger acute-on-chronic respiratory failure and cardiopulmonary decompensation in children with PWS and unrecognized severe nocturnal hypoxemia. Early screening and ongoing surveillance for sleep-disordered breathing, cardiopulmonary complications, and careful management during intercurrent infections are essential. RSV immunoprophylaxis, such as palivizumab, may warrant individualized consideration in high-risk young children with PWS who have impaired airway clearance or considerable respiratory comorbidity.