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 2). Cardiac magnetic resonance images showed substantial aneurysmal dilation of the main PA (maximal diameter, 7.5 × 7.4 cm) (Figs. 3 and 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.
AAo = ascending aorta; LV = left ventricle;
RV = right ventricle Supplemental motion image is available for Figure 3 (1.3MB, mp4) .
Fig. 4.
Cardiovascular magnetic resonance image (sagittal view) shows a severely dilated main pulmonary artery (MPA) with a giant aneurysm. Pulmonary regurgitation was severe.
RV = right ventricle
Supplemental motion image is available for Figure 4 (1.3MB, mp4) .
The patient underwent replacement of the pulmonary valve and the aneurysmal PA with a 30-mm pulmonary homograft (Figs. 5 and 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.
RVOT = right ventricular outflow tract
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.
Comment
Pulmonary artery aneurysm is on rare occasion a congenital anomaly (found in 8 of 109,571 autopsy cases).1 It can develop consequent to structural, infectious, and traumatic causes, but idiopathic congenital presentation, as in our patient, has been reported.2 The aneurysm can compress the left main coronary artery, so coronary imaging should be considered during evaluation. Pulmonary artery aneurysm is frequently associated with untreated PDA, perhaps because of higher PA pressure and volume overload. Even though our patient's PDA was ligated early enough in life to avert aneurysm formation, a giant PA aneurysm developed.
Supplementary Material
Footnotes
Section Editor: Raymond F. Stainback, MD, Department of Adult Cardiology, Texas Heart Institute, 6624 Fannin St., Suite 2480, Houston, TX 77030
From: Departments of Cardiovascular Medicine (Drs. Brody, Janardhanan, and Kannan), Sarver Heart Center; and Cardiothoracic Surgery (Drs. Lick and Teodori); University of Arizona, Tucson, Arizona 85724
References
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