Pulmonary vascular disease & thromboembolism
Long-term outcome of children with newly diagnosed pulmonary arterial hypertension: results from the global TOPP registry
Authors' conclusion
This comprehensive analysis of survival from time of diagnosis in a large exclusive cohort of children newly diagnosed with PAH describes current-era outcome and its predictors.
Study context
- Population
- Children aged ≥3 months and <18 years with newly diagnosed pulmonary arterial hypertension (PAH), TOPP registry, 2008–2015242 newly diagnosed PAH analysed
- Participants
- 531 children
- Outcome
- Long-term adverse outcome (death, lung transplantation, atrial septostomy, Potts shunt palliation) and its predictors
Methodological detailsglobal registry, 33 centres in 20 countries
- Global registry
- 33 centres in 20 countries
Main finding
As reported in the abstract
- Death
- 42 (17.4%); 6.2 events per 100 person-years
- Lung transplantation
- 9 (3.7%); 1.3 events per 100 person-years
- Atrial septostomy
- 3 (1.2%); 0.4 events per 100 person-years
- Potts shunt palliation
- 9 (3.7%); 1.4 events per 100 person-years
- 1-year survival free from adverse outcome
- 83.9%
- 3-year survival free from adverse outcome
- 75.2%
- 5-year survival free from adverse outcome
- 71.8%
Observational data: associations, not effects.
Further findings
Early adverse outcome (within 12 months after enrolment)
- Independent predictors: younger age, higher mean right atrial pressure, lower systemic venous oxygen saturation
Survival by cardiac shunt status
- Children with open (unrepaired or residual) cardiac shunts had the best survival rates
Original publication
Long-term outcome of children with newly diagnosed pulmonary arterial hypertension: results from the global TOPP registry. Eur Heart J Qual Care Clin Outcomes. Published 27 March 2023. doi:10.
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