Persistent Challenges in Pediatric Pulmonary Hypertension

Abstract

Pulmonary hypertension and related pulmonary vascular diseases cause significant morbidities and high mortality and present many unique challenges toward improving outcomes in neonates, infants, and children. Differences between pediatric and adult disease are reflected in controversies regarding etiologies, classification, epidemiology, diagnostic evaluations, and therapeutic interventions. This brief review highlights several key topics reflecting recent advances in the field and identifies persistent gaps in our understanding of clinical pediatric pulmonary hypertension.

Keywords: pediatric cardiology; pediatric pulmonary; pulmonary hypertension.

Figure 1

Venn diagram illustrating the heterogeneity and multifactorial elements in pediatric pulmonary hypertensive vascular disease. Insults on the growing lung may be due to ventilator-induced lug injury. Developmental abnormalities may include conditions such as bronchopulmonary dysplasia. Examples of multifactorial conditions may include aspiration or congenital heart disease.

Figure 2

A, Postmortem lung pathology from a normal lung at 6 months of age and lung from a patient with CDH at 7 months of age. CDH lung shows alveolar simplification (top) and muscularization of the small pulmonary arteries (arrows, bottom). Images courtesy Marcus Davey, PhD. B, Muscular hypertrophy and intimal hyperplasia in pulmonary arteries from lung biopsy specimens of two pediatric patients with CDH and PH. Aw, airway; PA, pulmonary artery.

Figure 3

Adapted treatment algorithm for idiopathic or heritable pediatric pulmonary arterial hypertension. CCB = calcium channel blocker.

Figure 4

Kaplan-Meier survival curve in 77 children with PH who were treated with chronic intravenous prostacyclin analogues (including epoprostenol or treprostinil, and those who transitioned from epoprostenol to treprostinil). The shaded areas depict the 95% CI. Transplant-free 5-year survival was 70% (95% CI, 56%-80%).