Abstract
Background: Idiopathic pulmonary arterial hypertension (IPAH) in pediatric patients is a rare yet severe progressive disease characterized by complex clinical and paraclinical features that vary by age.
Objective: Our study aimed to describe the clinical and paraclinical characteristics of IPAH in pediatric patients.
Methods: A descriptive study was conducted to analyze the clinical and paraclinical characteristics of 36 IPAH children at the Vietnam National Children’s Hospital and Hanoi Medical University Hospital between June 2020 and June 2025.
Results: Among 36 children with IPAH, the mean age at diagnosis was 5.7 ± 4.1 years, with a female/male ratio of 0.8:1. Dyspnea was the most common symptom, as 47.3% of patients were classified as New York Heart Association (NYHA) functional class III–IV. Cyanosis and syncope occurred in 27.8% and 16.7% of cases, respectively. Electrocardiographic findings indicated that 35% of patients exhibited sinus tachycardia, while 91% demonstrated right-axis deviation. Chest radiographs revealed cardiomegaly in 75% of cases. The median NT-proBNP concentration was markedly elevated at 877 pmol/L [IQR:1900]. Echocardiography revealed evidence of right ventricular failure, with a mean fractional area change (FAC) of 21% and a significantly elevated pulmonary artery systolic pressure (PASP) of 90.7 ± 21.3 mmHg. Right heart catheterization confirmed pulmonary arterial hypertension, with a mean pulmonary arterial pressure of 59 mmHg and an increased pulmonary vascular resistance of 21 Wood units/m². Positive acute vasoreactivity tests were observed in 22.7% of patients.
Conclusion: IPAH in children is a rare but fatal disease; however, diagnosis is often delayed, with patients presenting severe clinical manifestations, markedly elevated pulmonary arterial pressure and resistance, and evidence of right ventricular failure on paraclinical evaluation.