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Published on: May 11, 2015
Clinical differences between children and adults with idiopathic and heritable pulmonary arterial hypertension
Shinichi Takatsuki1, Hiroto Shimokawahara2, Yurika Shimizu1
1Department of Pediatrics, Toho University Omori Medical Center, Tokyo, Japan.
Insights
Pediatric pulmonary arterial hypertension (PAH) patients present with worse hemodynamics but achieve similar survival rates as adults with PAH. Effective therapeutic strategies ensure comparable long-term outcomes for children and adults with PAH.
Area of Science:
- Cardiology
- Pulmonology
- Pediatrics
Background:
- Pediatric pulmonary arterial hypertension (PAH) differs from adult PAH, yet direct comparative studies are limited.
- Understanding these distinctions is crucial for effective management and treatment strategies.
Purpose of the Study:
- To conduct a head-to-head comparison of gender, hemodynamic parameters, and prognosis between pediatric and adult PAH patients.
- To identify key differences and similarities in disease presentation and outcomes.
Main Methods:
- Retrospective analysis of 40 childhood-onset (<20 years) and 40 adult-onset idiopathic/heritable PAH patients.
- Comparison of demographic data, hemodynamic parameters (mean pulmonary artery pressure), functional class (NYHA IV), and event-free survival.
Main Results:
- No female predominance in pediatric PAH (42.5% female) versus adult PAH (80% female).
- Pediatric PAH patients exhibited worse baseline hemodynamics (mean pulmonary artery pressure: 65 mmHg vs. 49 mmHg).
- Similar event-free survival rates (95% vs. 85%) despite initial hemodynamic differences.
Conclusions:
- Pediatric PAH patients face more severe hemodynamic challenges at diagnosis compared to adults.
- Appropriate therapeutic strategies enable pediatric PAH patients to achieve survival rates comparable to adults.
Background:
Although previous studies have demonstrated that paediatric pulmonary arterial hypertension remains distinct from that in adults, there are limited studies evaluating a direct comparison between children and adults. The aim of this head-to-head comparison study was to compare the gender, haemodynamic parameters, and prognosis between paediatric and adult pulmonary arterial hypertension.
Methods And Results:
We retrospectively assessed the clinical differences in 40 childhood-onset (under 20 years old) patients and 40 adult-onset patients with idiopathic and heritable pulmonary arterial hypertension who were followed up at two centres. There was no female predominance among patients with childhood-onset pulmonary arterial hypertension (child female: 42.5%, adult female: 80%). The percent of New York Heart Association functional class IV in adult-onset pulmonary arterial hypertension tended to be higher than those in childhood-onset pulmonary arterial hypertension (22.5 and 10%, respectively), although children had worse haemodynamic parameters at diagnosis (mean pulmonary artery pressure (children versus adults); median 65 mmHg versus 49 mmHg, p < 0.001). There was no significant difference in the event-free survival rate between the two groups (95% vs. 85%) during the follow-up period (median, 96 months; range, 1-120 months).
Conclusions:
Although paediatric pulmonary arterial hypertension patients had worse haemodynamic parameters at diagnosis than adults, children survived as long as adults with appropriate therapeutic strategies.
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