Extract

A 29-year-old man with pulmonary hypertension who was undergoing treatment with bosentan and sildenafil was admitted with sudden-onset dyspnoea and anterior chest pain. He had a patent ductus arteriosus that had been closed percutaneously using coils at the age of 24 years. Chest radiography revealed enlargement of the cardiac silhouette, a marked upper left cardiac border, and a prominent right pulmonary artery (PA). Transthoracic echocardiography revealed a markedly dilated main PA and a dissection with a flap extending from the main PA to the left PA. Meticulous Doppler echocardiography also revealed an intimal flap and entry site in the PA. Rotation of the probe enabled direct visualization of a laceration of the wall of the main PA, which was considered the entry site of the PA dissection. Contrast-enhanced computed tomography also confirmed the enlarged PA (maximum diameter, 105 mm), dissection flap, and entry site in the main PA. After prompt diagnosis, emergent right ventricular outflow tract repair (using a 22-mm Yamagishi conduit) and bilateral PA plication were successfully performed. Intraoperative examination revealed an enlarged PA and a dissection flap in the pulmonary trunk; these were exactly the same morphological abnormalities shown on preoperative transthoracic echocardiography. Pulmonary artery dissection is an extremely rare and lethal complication of chronic pulmonary hypertension. We directly visualized the laceration of the PA wall by two-dimensional echocardiography.

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