Application of a modified clinical classification for pulmonary arterial hypertension associated with congenital heart disease in children: emphasis on atrial septal defects and transposition of the great arteries. An analysis from the TOPP registry

Julie Wacker; Tilman Humpl; Rolf M F Berger; Dunbar Ivy; David Bowers; Damien Bonnet; Maurice Beghetti

doi:10.3389/fcvm.2024.1344014

Application of a modified clinical classification for pulmonary arterial hypertension associated with congenital heart disease in children: emphasis on atrial septal defects and transposition of the great arteries. An analysis from the TOPP registry

Front Cardiovasc Med. 2024 Feb 2:11:1344014. doi: 10.3389/fcvm.2024.1344014. eCollection 2024.

Authors

Julie Wacker¹, Tilman Humpl², Rolf M F Berger³, Dunbar Ivy⁴, David Bowers⁵, Damien Bonnet⁶, Maurice Beghetti¹

Affiliations

¹ Pediatric Cardiology Unit, Department of Women, Child and Adolescent, Children's University Hospital, and University of Geneva, Geneva, Switzerland.
² Pediatric Cardiology and Critical Care Medicine, The Hospital for Sick Children University of Toronto, Toronto, ON, Canada.
³ Centre for Congenital Heart Diseases, Pediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, Netherlands.
⁴ Pediatric Cardiology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, United States.
⁵ School of Allied Health Sciences, University of Suffolk, Ipswich, United Kingdom.
⁶ Centre de Référence Malformations Cardiaques Congénitales Complexes, M3C-Necker Hospital for Sick Children, Assistance Publique des Hôpitaux de Paris, University of Paris Cité, Paris, France.

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.

Keywords: Eisenmenger syndrome; atrial septal defect; classification; congenital heart disease; pediatrics; pulmonary arterial hypertension; pulmonary hypertension; transposition of the great arteries.

Grants and funding

The author(s) declare that financial support was received for the research, authorship, and/or publication of this article.