Pulmonary artery dissection is a complication associated with pulmonary arterial hypertension. This complication is described as acute in onset and is frequently fatal without intervention. We describe a patient with idiopathic pulmonary arterial hypertension and chest pain found to have an unsuspected chronic pulmonary artery dissection on postmortem examination. Chronic pulmonary artery dissection should be considered in patients with chest pain and worsening dyspnea, as the frequency this condition may be underestimated.Keywords: pulmonary arterial hypertension, pulmonary artery dissection, complication, chest pain. Pulmonary artery hypertension (PAH) is a progressive, life-threatening condition associated with symptoms that become more prevalent with disease progression. 1,2 In patients with PAH, the cause of chest pain is unclear but is commonly attributed to myocardial ischemia. [3][4][5][6] However, acute pulmonary artery dissection is also associated with new-onset chest pain, often with fatal outcomes. 7,8 We describe a patient who had progressively worsening idiopathic PAH and chest pain, which resolved after initiation of intravenous prostaglandin analog therapy, and was found to have had acute and chronic pulmonary artery dissections on postmortem examination.
CASE DESCRIPTIONA 56-year-old female with idiopathic PAH diagnosed 11 years earlier and treated with nifedipine, bosentan (Tracleer, Actelion, San Francisco), sildenafil (Revatio, Pfizer, New York), inhaled iloprost (Iloprost, Actelion, San Francisco), and warfarin presented with 2 weeks of severe, intermittent chest pain. Chest pain was sharp and nonradiating, located over the left anterior chest, lasting 20-60 minutes, and aggravated with exertion or deep inspiration. This pain was unrelieved by antacids and partially relieved by oxygen and rest. Physical examination revealed a pulse rate of 100/minute, blood pressure of 130/70 mmHg, and pulse oximetry of 96% (with oxygen administered at 2 L/min). Chest auscultation revealed clear lung fields, a 3/6 systolic murmur, and no pericardial rub. No chest wall tenderness was elicited. Ascites was absent, and lower-extremity edema was present. Initial and subsequent electrocardiographs during episodes of chest pain revealed tachycardia without ischemic changes. Concomitant serum troponin levels were normal. A single-photon emission computed tomography (CT) myocardial perfusion test with adenosine did not demonstrate perfusion defects. Echocardiography