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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, PAH-CHD associated with prevalent systemic-to-pulmonary shunts, PAH-CHD with small/coincidental defects, PAH-CHD after defect correction.
The risk stratification models do not apply to PAH-CHD
The current scientific evidence supports a risk-based, goal-oriented treatment approach in patients with PAH,1 aimed at achieving and/or maintaining a low-risk status. However, one may wonder if the traditional risk stratification model should be used for all patients with PAH.
The risk stratification in patients with PAH-CHD is still limited, since applying the risk scores derived from idiopathic PAH to this multifaceted and varied population is questionable1 as the pathophysiology, clinical manifestations and survival rate of the latter are much …
Contributors Both authors take public responsibility for the content of the work submitted for review. The authors confirm they have both contributed to the editorial conception. Both authors reviewed and approved the final version of the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Commissioned; internally peer reviewed.