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Published on: May 11, 2015
Pediatric pulmonary hypertension registry: Single-center experience from India
Wadile Santosh1, Mishra Arpita1, Banpurkar Ashishkumar Moreshwar1
1Department of Pediatric Cardiology, Sri Sathya Sai Sanjeevani Centre for Child Heart Care and Training in Pediatric Cardiac Skills, Navi Mumbai, Maharashtra, India.
Insights
This study highlights that pediatric pulmonary hypertension (PH) in India often stems from congenital heart defects (CHD-PAH), with idiopathic pulmonary artery hypertension (IPH) showing higher mortality. Registries are crucial for understanding this rare disease in developing nations.
Area of Science:
- Pediatric Cardiology
- Pulmonary Hypertension Research
- Rare Disease Registries
Background:
- Pulmonary hypertension (PH) is a severe, irreversible condition leading to right heart failure (RHF).
- Existing PH registries primarily focus on adults, with a scarcity of pediatric data, especially from developing countries.
- This study addresses the need for pediatric PH data in the Indian context.
Purpose of the Study:
- To establish a pediatric PH registry in India.
- To characterize the etiology, clinical presentation, and outcomes of pediatric PH in the Indian population.
- To compare findings with Western data and identify unique challenges in developing countries.
Main Methods:
- Enrolled 100 children (3 months-18 years) diagnosed with PH.
- Defined PH using standard echocardiographic and cardiac catheterization criteria.
- Excluded transient or reversible PH, including hyperkinetic PH associated with congenital heart defects (CHDs).
Main Results:
- The registry included 100 children (50% male, 50% female) from June 2019 to January 2024.
- Congenital heart defect-associated pulmonary artery hypertension (CHD-PAH) constituted the majority (81%), with idiopathic pulmonary artery hypertension (IPH) at 15%.
- Mortality was 5% overall, with significantly higher mortality in the IPH group (20%) compared to the CHD-PAH group (0%).
Conclusions:
- Pediatric PH in India differs from Western populations, with a higher prevalence of CHD-PAH potentially due to late referrals and resource limitations.
- Idiopathic pulmonary artery hypertension (IPH) in children carries a poor prognosis and high mortality risk.
- Multicenter registries are essential for gathering accurate statistics and advancing the understanding of pediatric PH in diverse settings.
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.
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