Recurrent chest pain and dyspnoea in a patient with pulmonary arterial hypertension.

: Pulmonary arterial hypertension (PAH) is a devastating, life-threatening disease with poor prognosis when left untreated. The long-term prognosis is definitely influenced by the natural progression of PAH but late disease-specific complications may also contribute. : We present a patient with a long-standing idiopathic PAH in whom progressive dilatation of pulmonary trunk and pulmonary arteries leads to compression of the left main coronary artery and the left atrium with hemodynamic compromise. : With the current treatment options, survival in PAH has improved. Guidelines focus on more aggressive treatment with initial combination therapy and earlier referral for transplantation.