Key result
Advanced pharmacological therapy with bosentan, with or without sildenafil, improved functional capacity and hemodynamics in three adult patients with congenital heart disease-associated pulmonary arterial hypertension.
Case Report (n=3)
Advanced pharmacological therapy should be considered for patients with pulmonary arterial hypertension due to congenital heart disease.
May support bosentan-based therapy in adult CHD-PAH; hypothesis-generating from three cases and requires prospective trials.
Pulmonary arterial hypertension (PAH) is a progressive disease with poor survival. PAH is classified by the 2009 updated clinical classification of pulmonary hypertension and a major subgroup is PAH due to congenital heart disease (CHD) with systemic-to-pulmonary shunt [ 1 ]. This shunting may lead to extensive histological changes in the distal pulmonary arteries resulting in an irreversible increase in pulmonary vascular resistance (PVR). The classification of CHD-PAH includes four categories: (1) Eisenmenger syndrome, (2) PAH associated with systemic-to-pulmonary shunts, (3) PAH with small septal defects, and (4) PAH after corrective cardiac surgery [ 2 ]. In these patients advanced pharmacological therapy should be considered.
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Schuuring et al. (2011) conducted a case report in Pulmonary arterial hypertension due to congenital heart disease (n=3). Advanced pharmacological therapy (bosentan, sildenafil) was evaluated on Clinical and functional improvement (6-minute walking distance, NYHA class, hemodynamics). Advanced pharmacological therapy with bosentan, with or without sildenafil, improved functional capacity and hemodynamics in three adult patients with congenital heart disease-associated pulmonary arterial hypertension.
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