Portopulmonary hypertension in children: a rare but potentially lethal and under-recognized disease

Area of Science:

• Pediatric Cardiology
• Hepatology
• Pulmonology

Background:

• Portopulmonary hypertension (PoPH) involves co-occurring portal hypertension and precapillary pulmonary arterial hypertension (PAH).
• Limited data exists on pediatric PoPH, with a generally poor prognosis reported.

Purpose of the Study:

• To review institutional experience with pediatric PoPH.
• To describe clinical characteristics, treatment, and outcomes in children diagnosed with PoPH.

Main Methods:

• Retrospective review of five pediatric patients with PoPH.
• Diagnosis confirmed via echocardiogram and cardiac catheterization in most cases.
• Assessment of pulmonary artery pressures and pulmonary vascular resistance index.

Main Results:

• Median age at PoPH diagnosis was 6 years; PAH diagnosis at 14 years.
• Median mean pulmonary artery pressure (mPAP) was 48.5 mmHg and PVRi was 9 WU*M^2.
• All patients were non-responsive to pulmonary vasodilator testing; 60% (3/5) died despite evidence-based care.

Conclusions:

• Pediatric PoPH is associated with significant childhood mortality and treatment challenges.
• Current evidence-based approaches show limited efficacy in improving survival.
• Future research should focus on early screening and novel treatment strategies to improve outcomes.