Pediatric pulmonary hypertension registry: Single-center experience from India.

Background: Pulmonary hypertension (PH) is a rare and usually irreversible disease, eventually leading to right heart failure (RHF). There are multiple PH registries in adults with PH, but very few pediatric PH registries, especially from developing countries. Methods: All children (aged 3 months-18 years) diagnosed with PH were enrolled in the study. PH was defined by standard echocardiographic and cardiac catheterization criteria. We excluded patients with transient or reversible PH, including hyperkinetic PH associated with congenital heart defects (CHDs). Results: Our registry included 100 children from June 2019 to January 2024, with an equal gender ratio (male: female = 1:1). Easy fatigability, syncope, and RHF were the most common presenting features. Only 15% of children were classified as idiopathic pulmonary artery hypertension (IPH). The majority (81%) of children belonged to the CHD-pulmonary artery hypertension (CHD-PAH) group (49% Eisenmenger syndrome, 26% CHD with systemic to pulmonary shunt and severe PAH falling in the inoperable category, and 6% had post-operative residual PH). All patients in the IPH group and 42 patients (48.2%) in other PH were on pulmonary vasodilators. The median follow-up duration was 13 months (range: 4-56 months). Overall, there were 5 deaths (5% mortality), with 3 deaths in the IPH (20%) group. Children with CHD-PAH had better functional class, and no patient in this group died. Conclusions: The underlying etiology and prognosis in children with PH may differ in the Indian context compared to the Western world. Different centers across India may still face a higher proportion of unoperated CHD due to late referral, a limited number of specialized centers, and financial constraints. Children with IPH have poor long-term outcomes and a high risk of mortality. Multicenter PH registries may provide actual statistics and further insights into the disease.