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Pulmonary arterial hypertension (PAH) is relatively uncommon yet life-threatening morbidity in neonates and infants. South Asian countries including India may record a relatively higher incidence of pediatric PAH. The etiology of pediatric PAH is heterogeneous, and little evidence is available on the clinical spectrum of pediatric PAH from low- and middle-income countries. This descriptive study aims to describe the incidence and underlying causes of pediatric PAH at a tertiary care referral center in coastal Karnataka, India.

This is a retrospective study which involved complete enumeration of pediatric PAH cases admitted at a tertiary care referral center in coastal Karnataka between January 2018 and December 2021 after prior approval from the Institutional Ethics Committee. Clinical data were retrieved from the electronic medical records.

The center documented a total of 165 cases of pediatric PAH, with an incidence of 1.19% among the total neonatal intensive care unit admissions within the study period. Persistent fetal circulation was the most common sub-classification (77.44%), with underlying inherited metabolic disorders (30%), infectious pathologies, and malnutrition being the final diagnosis in this group. Neonates subclassified under neonatal cardiac failure majorly showed underlying cardiac anomalies (80%), while 4% of the cases were attributed to other causes including hyperbilirubinemia-induced encephalopathy and COVID-induced multisystem inflammatory syndrome, viral endocarditis, etc.

Inherited metabolic and cardiac anomalies, antenatal and postnatal maternal morbidities, nutritional deficiencies, and secondary infections are the major causes of neonatal and infantile PAH. Multilevel screening could substantially reduce the incidence and the morbidities associated with infantile PAH.