Abstract

A 40-year-old woman presented with recent-onset left-sided chest pain and orthopnea. She had undergone patent ductus arteriosus (PDA) ligation at the age of 2 years. A 2-dimensional transthoracic echocardiogram during the current admission revealed a dilated main pulmonary artery (PA) with significant pulmonary regurgitation (Figs. 1 and ​and2).2). Cardiac magnetic resonance images showed substantial aneurysmal dilation of the main PA (maximal diameter, 7.5 × 7.4 cm) (Figs. 3 and ​and4).4). The pulmonary valve was dysplastic with significant pulmonary regurgitation. Right-sided heart catheterization revealed PA pressures of 55/22 mmHg and a PA saturation of 65%. The coronary arteries were normal. Fig. 1 Transthoracic echocardiogram (basal short-axis view) shows a dilated main pulmonary artery (MPA) and right ventricular outflow tract (RVOT). Fig. 2 Transthoracic echocardiogram (basal short-axis view) in color-flow Doppler mode shows significant pulmonary regurgitation (arrow) across the pulmonary valve. Fig. 3 Cardiovascular magnetic resonance image (coronal view) shows a severely dilated main pulmonary artery (MPA) with a giant aneurysm. Fig. 4 Cardiovascular magnetic resonance image (sagittal view) shows a severely dilated main pulmonary artery (MPA) with a giant aneurysm. Pulmonary regurgitation was severe. The patient underwent replacement of the pulmonary valve and the aneurysmal PA with a 30-mm pulmonary homograft (Figs. 5 and ​and6).6). Her postoperative course was uneventful. Pathologic analysis yielded mild cystic medial degeneration without dissection. Fig. 5 Intraoperative photograph shows the dysplastic native pulmonary valve (PV) and giant pulmonary artery (PA) aneurysm before surgery. Fig. 6 Intraoperative photograph shows the posterior half of the homograft (arrow) sewn to the pulmonary valve annulus (arrowhead) after excision of the native valve.

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