In the 1980s, paediatic pulmonary arterial hypertension (PAH) was rare but commonest in patients with late diagnosis of structural heart faults, often with associated Down syndrome, and neonates with persistent fetal circulation. Occasional children were seen with primary pulmonary hypertension/idiopathic pulmonary arterial hypertension (IPAH), sometimes in a familial context. Those who responded to calcium channel blockers (CaCBs) tended to survive and those who did not died within a few months or years of diagnosis. Patients with pulmonary hypertension associated with a structural heart fault with the potential for a large left to right shunt were likely to die at surgical repair if they had not shown evidence for significant left heart volume loading and associated heart failure.
In the 21st century, screening of Down syndrome infants for structural heart faults with earlier surgical repair and better neonatal care have almost eliminated the formerly most common causes of PAH. Their places have been taken by infants with congenital diaphragmatic hernia, occasional post repair persistent PAH in survivors and patients with a wide variety of other systemic problems including bone marrow disorders. Most diagnosed patients are treated with a variety of agents and CaCBs are uncommonly used
In this review of paediatric patients from the East of Scotland, the prevalence of different types of PAH is compared and the unexpectedly favourable outcome in some patients discussed This may have relevance to the practice of routine presciption of anti IPAH treatment for all patients.