Group D of pulmonary arterial hypertension and its relationship to congenital heart disease: Is there a non-invasive way to predict the unpredictable?

Antoine Fakhry AbdelMassih, Alyaa AlAli, Emad Nasr, Eman Hanafy, Musaab Ramsi
Cairo University. heikh Khalifa Medical City and Pure Health Group. Aswan Heart Centre. Helwan University.
Egypt and United Arab Emirates

Global Cardiology Science and Practice
Glob Cardiol Sci Pract 2025;
DOI: 10.21542/gcsp.2025.5

Abstract
Pulmonary arterial hypertension related to congenital heart disease (PAH-CHD), can pose a few challenging therapeutic challenges. PAH related to CHD can be classified into 4 clinical groups: Group A, which includes patients with Eisenmenger syndrome; Group B, which includes patients with severe PAH due to significant shunt lesions with no reversal of the shunt and no cyanosis; Group C, which includes patients with PAH due to small defects whose clinical picture is comparable to that of IPAH (idiopathic PAH) patients; and Group D, which includes patients with persistent PAH following CHD repair. This review aims to shed light on the possible laboratory markers that can predict whether pulmonary arterial hypertension secondary to a congenital heart defect will improve after repair of the defect or will continue to progress because the patient’s PAH is mediated by idiopathic changes and not the shunt lesion itself. This differentiation is crucial for predicting PH prognosis after cardiac repair.

Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Potential Biomarkers Associated with Pulmonary Vascular Disease
Review Articles Concerning Pulmonary Vascular Disease

Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease

Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication

Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes

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