An active 78-year-old woman with a lifelong asymptomatic heart murmur underwent primary care evaluation. Her physician characterized a grade 3/6, crescendo-decrescendo murmur heard best at the left sternal border, with an increased split in S2 and increased intensity with inspiration. A transthoracic echocardiogram showed elevated velocities across the pulmonic valve (PV), suggesting stenotic disease. Right-sided heart catheterization revealed a right ventricular systolic pressure of 69 mmHg, a main pulmonary artery (PA) systolic pressure of 30 mmHg, and a mean gradient across the PV of 26 mmHg.
The patient was referred to our center for cardiac magnetic resonance. A crescent-shaped orifice was consistent with a bicuspid PV (Fig. 1). The main PA was substantially dilated (maximum diameter, 5.15 cm) (Fig. 2). The right ventricle was mildly dilated without obvious free-wall hypertrophy (Fig. 3); the ejection fraction was 0.59. The left ventricular ejection fraction was 0.77. The aorta's size was normal, and the aortic valve was trileaflet. The patient had no symptoms and had a PA pressure <50 mmHg, so clinical monitoring was maintained.
Citation: Texas Heart Institute Journal 45, 3; 10.14503/THIJ-17-6347
Citation: Texas Heart Institute Journal 45, 3; 10.14503/THIJ-17-6347
Citation: Texas Heart Institute Journal 45, 3; 10.14503/THIJ-17-6347
Comment
Pulmonary artery aneurysms are rare, having been described in approximately 1 of 14,000 autopsies.1 Usually, PA aneurysms occur in clinical situations such as congenital heart disease and pulmonary hypertension.2 Bicuspid PVs have been associated with dilation of the pulmonary trunk. In patients who have a PA aneurysm and a bicuspid PV, the most likely origin is abnormal migration of the neural crest cells.3
Clinically, bicuspid PVs with PA aneurysms do not pose the same clinical risk as do their systemic counterparts, because of the lower pressure in the PAs. The greatest risk for a PA dissection from a PA aneurysm is an absolute pressure limit >50 mmHg,4 so we recommended clinical monitoring for our patient.
Cardiac magnetic resonance images. A) Steady-state free precession sequence shows the opening of the bicuspid pulmonic valve. B) Phase contrast sequence shows a crescent-shaped flow pattern across the valve.
Cardiac magnetic resonance image (steady-state free precession sequence in axial view) shows the aneurysmal, dilated main pulmonary artery.
Cardiac magnetic resonance image (steady-state free precession sequence in 4-chamber view) shows mild right ventricular enlargement without obvious free-wall hypertrophy.
Contributor Notes
Section Editor: Raymond F. Stainback, MD
From: Section of Cardiology, Department of Internal Medicine, Wake Forest University School of Medicine, Winston-Salem, North Carolina 27157
Dr. Prasada is now at the Department of Internal Medicine, University of Florida School of Medicine, Gainesville, Florida.
