Julie Wacker, Tilman Humpl, Rolf M. F. Berger, Dunbar Ivy, David Bowers, Damien Bonnet, Maurice Beghetti on behalf of the TOPP Investigators
Children’s University Hospital and University of Geneva. Hospital for Sick Children and University of Toronto. Beatrix Children’s Hospital, University Medical Center Groningen and University of Groningen. Children’s Hospital Colorado and University of Colorado School of Medicine. University of Suffolk. M3C-Necker Hospital for Sick Children, Assistance Publique des Hôpitaux de Paris and University of Paris Cité.
Switzerland, Canada, Netherlands, United States, United Kingdom and France
Frontiers in Cardiovascular Medicine
Front Cardiovasc Med 2024;
DOI: 10.3389/fcvm.2024.1344014
Abstract
Aims: A proportion of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) do not fit in the current classification. We aimed to analyse the applicability of an adapted clinical classification of PAH-CHD to pediatric patients using the TOPP-1 registry (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) and focus on atrial septal defects (ASD) and transposition of the great arteries (TGA).
Methods and results: Hemodynamic and clinical data of all patients with PAH-CHD in the TOPP cohort were reviewed. Patients were classified according to predefined ABCDE categories (A: Eisenmenger syndrome, B: left-to-right shunt, C: coincidental defects, including all ASDs, D: corrected CHD, E: TGA), or as complex CHD (group 5), by 2 independent investigators. In case of disagreement, a third reviewer could either settle a final decision, or the patient was deemed not classifiable. Survival curves were calculated for each group and compared to idiopathic PAH patients of the registry. A total of 223 out of 531 patients in the registry had PAH-CHD, and 193 were categorized to the following groups: A 39(20%), B 27(14%), C 62(32%) including 43 ASDs, D 58(30%), E 7(4%), whereas 6 patients were categorized as group 5, and 10 patients were unable to be classified. No survival difference could be demonstrated between the groups.
Conclusions: This modified classification seems to be more applicable to pediatric PAH-CHD patients than the previous classification, but some patients with PAH-CHD who never had a shunt remain unclassifiable. The role of ASD in pediatric PH should be reconsidered.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
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