Pulmonary artery dissection in long standing idiopathic pulmonary arterial hypertension: A case report.

Pulmonary arterial dissection (PAD) is a rare and often lethal complication of chronic pulmonary arterial hypertension (PAH), which may occurs in patients with idiopathic pulmonary arterial hypertension (IPAH) and potentially in those with connective tissue disorders. While rare, sudden death often occurs secondary to acute cardiac tamponade, as the pulmonary artery dissects into the pericardium; this diagnosis is often made postmortem. Nevertheless, with the proliferation of multidetector computed tomography (MDCT) as a diagnostic test, patients may be identified very early after symptom onset, prompting rapid intervention with decreased morbidity and mortality. We report a case of IPAH complicated by pulmonary artery aneurysm (PAA) and PAD, diagnosed by CT pulmonary angiogram (CTPA), and treated with bilateral lung transplantation, pulmonic valve replacement, and re-anastomosis of the donor main PA to a pulmonary valve conduit.