Raising the bar: triple therapy for pulmonary arterial hypertension associated with congenital heart disease

Pulmonary arterial hypertension related to congenital heart disease as a paradigm of complexity Pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) really represents a paradigm of complexity. As such, some patients develop PAH-CHD during the infancy, usually as a result of a large post-tricuspid shunt, with pulmonary arterial pressures which may never drop after birth. In others, PAH may persist after late repair or develop later in life. Again, in rare cases, PAH-CHD may develop after a timely repair of the congenital defect, in the absence of any significant residual haemodynamic lesions or in the presence of a small intracardiac defect, which usually would not trigger the development of pulmonary vascular disease. The current guidelines on pulmonary hypertension1 accurately describe the different clinical scenarios of PAH-CHD, depending on the wide range of the underlying cardiac defects and the multiple and different adaptive mechanisms: Eisenmenger syndrome,...
Source: Heart - Category: Cardiology Authors: Tags: Editorials Source Type: research