Paediatric pulmonary arterial hypertension following congenital heart defect repair: enhanced risk stratification and outcomes in a national cohort
Authors' conclusion
In this national cohort of children with repaired PAH-CHD, mortality is significant. This novel, simple risk score has been developed and validated specifically for children with repaired PAH-CHD, useful at the time of post-operative assessment to predict outcome and direct management.
Study context
- Population
- Children with pulmonary arterial hypertension following congenital heart defect repair (repaired PAH-CHD), UK National Paediatric PH Servicemedian age 3.2 years, 58.4% female, 73.0% referred post-CHD repair, median six-year follow-up of 156 patients
- Participants
- 178 children
- Outcome
- Characteristics
Methodological detailsnational, externally validated using the national Spanish PH (REHIPED) registry
- National
- Externally validated using the national Spanish PH (REHIPED) registry
Main finding
As reported in the abstract
Complex CHD
61.2%
Both pre- and post-tricuspid shunts
48.9%
Down syndrome
33.1%
Observational data: associations, not effects.
Further findings
Findings at initial post-operative assessment
- Symptoms like breathlessness
- 53.1%
- Moderate-severe right ventricular dilatation
- 30.9%
- Right ventricular systolic impairment
- 23.7%
Death, lung transplantation and survival · median six-year follow-up
- Died
- 19.2%
- Required lung transplantation
- 3.2%
- Survival at one year
- 94.7%
- Survival at five years
- 85.9%
- Survival at ten years
- 80.1%
- 156 patients followed
Risk score performance (absence of pre-operative PH, breathlessness, right ventricular dysfunction, pulmonary vascular resistance index)
- Good performance and calibration in predicting outcomes
Original publication
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