Giant Pulmonary Aneurysm Associated With Pulmonary Valve Agenesia
A 54-year-old man came to our outpatient department because of ventricular extrasystole. On the basis of chest radiograph (Figure 1A), situs inversus had been diagnosed previously. This possibility was ruled out by fluoroscopy, which showed an intensely pulsating, newborn-head–sized, sharp-edged mass on the right side next to the heart, which was identified by transthoracic echocardiography as a huge, dilated (80 mm) pulmonary artery that had dislocated the heart. During transesophageal echocardiography, a secondary atrial septal defect was detected, but the pulmonary valve could not be visualized. Chest CT (Figure 2A) showed an aneurysm on the left pulmonary artery, right ventricle dilation, and normal left chambers. Because comparison of the previous chest radiographs (Figures 1A and 1B) showed progression in the size of the aneurysm, surgical treatment was chosen. Intraoperatively, a hypoplastic pulmonary valve and secondary atrial septal defect were found. A 25-mm Medtronic Hancock bioprosthesis was implanted into the pulmonary position, the atrial septal defect was closed, the pulmonary artery aneurysm was removed, and reductive angioplasty was performed. On the postoperative chest radiograph (Figure 1C), the contour of the pulmonary artery had been practically normalized. To the best of our knowledge, this is the first case in which a progressive pulmonary aneurysm associated with pulmonary valve agenesia was treated by surgical repair.