Assessment of the Reversibility of Pulmonary Hypertension in Adult Congenital Heart Defects: When, how and why?

Abstract

Pulmonary arterial hypertension (PAH) in the context of adult congenital heart disease (ACHD) can be reversed by early closure of the communication or pre-existing shunt. This "window of opportunity" is lost beyond a certain point of no return. Therefore, it is crucial to accurately assess the reversibility of this progressive vascular disease, which usually begins in early stages. The reversibility assessment is currently based on a combination of clinical symptoms, hemodynamic changes and fundamental variables such as pulmonary vascular resistance. However, its measurement has limited predictive value and leaves many patients in a “gray area” regarding decision-making. This review provides a concise overview of the mechanisms involved in the flow-dependent progression of PAH in CHD and assesses existing and future alternatives for a thorough assessment of existing pulmonary artery disease. The structural quantification of the pulmonary arterial tree using fractal branching algorithms, functional images with intravascular ultrasound, nuclear imaging, new serum biomarkers, genetic tests and the potential for transcriptomic analysis of circulating endothelial cells and platelets are being incorporated into the evaluation of this type of patients. Keywords: Pulmonary Hypertension-Congenital Heart Diseases-Pulmonary Vasculature-Heart Failure. Read more →