1. Prognostic Value of Early Risk Stratification in Pediatric Pulmonary Arterial Hypertension
- Author
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György Reusz, Orsolya Somoskövi, Tamás Ferenci, László Ablonczy, Réka Osváth, and Eva Kis
- Subjects
Heart Defects, Congenital ,Male ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,Disease ,World health ,Risk Factors ,Internal medicine ,medicine ,Humans ,Familial Primary Pulmonary Hypertension ,Child ,Survival analysis ,Retrospective Studies ,Cardiac catheterization ,Pulmonary Arterial Hypertension ,Transplantation ,business.industry ,Hemodynamics ,Infant, Newborn ,Infant ,Wood units ,Prognosis ,Survival Analysis ,Echocardiography ,Sample size determination ,Child, Preschool ,Risk stratification ,Etiology ,Cardiology ,Female ,Surgery ,business - Abstract
Background Pulmonary arterial hypertension (PAH) is a life-threatening disease with risk stratification-based treatment strategy in adults. Although the risk factors have been studied individually in children, effective risk stratification is still lacking. We have tested the prognostic accuracy of pediatric PAH risk factors in our patient group. Patients and methods Records of 58 PAH patients treated between 1995 and 2019 were reviewed retrospectively. Median age at diagnosis was 4.2 years (range, 0.1-16.1 years), and follow-up was 5.4 years (range, 0.01-24.1 years). Data collected at diagnosis were demographics, World Health Organization functional class, evidence of right ventricular failure, and parameters of echocardiography and cardiac catheterization. Results Mortality was 29% and 33% reached the composite endpoint. Patients with idiopathic PAH (n = 12) had increased risk of mortality compared with the congenital heart disease-associated PAH group (n = 32) (P = .0024). Neither the initial World Health Organization functional class staging nor the echocardiographic parameters significantly predicted the prognosis. The number of risk factors had no significant prognostic value either. In contrast, patients with higher pulmonary vascular resistance index (PVRI) had significantly increased risk (each 10 Wood units ⋅ m2 increase in PVRI being associated with 49.1% higher hazard, P = .0048). Conclusions Survival analysis showed that PAH etiology might be an important determinant in pediatric PAH risk stratification. We confirmed that PVRI has predictive value in prognostic assessment. We could not establish the prognostic value of nonweighted single risk factors or their combination to predict pediatric PAH outcome due to the low sample size, but these results indicate that such studies are warranted.
- Published
- 2021
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