Abstract
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A 34-year-old woman presented with cyanosis. Her exercise tolerance (poor for most of her life) had worsened recently. She had been diagnosed with Swyer-James syndrome, a manifestation of postinfectious obliterative bronchiolitis wherein the involved lung does not grow normally and is slightly smaller than the other lung.1 On examination, she was cyanotic and in mild respiratory distress, with an oxygen saturation of 82% on 6 L/min of oxygen through a nasal cannula. Auscultation revealed diminished breath sounds in the left upper lung field. There were no murmurs or extra heart sounds, and no persistent splitting of the S2 was noted. The patient's hematocrit level was 61.3%, and arterial blood gas analysis revealed a PO2 of 33 mmHg.
Transesophageal echocardiography showed an atrial septal defect (ASD) and a large aneurysm of the noncoronary sinus of Valsalva. The aneurysm protruded into the right atrium (Fig. 1). Right-heart angiography showed a large filling defect in the right atrium with nearly total obstruction of right ventricular (RV) inflow, resulting in right-to-left shunting across a secundum ASD rather than across the tricuspid valve. Oxygen saturations of 55% to 65% in the right heart, 82.5% in the aorta, and 99% in the left pulmonary vein confirmed right-to-left intracardiac shunting. Aortography showed the aneurysm in the right atrium (Fig. 2). We resected the aneurysm, closed the ASD, and inserted a prosthetic aortic valve. The patient's postoperative oxygen saturation was 95% by pulse oximetry on room air.
Fig. 1 Transesophageal echocardiogram. A large, balloon-like aneurysm of the noncoronary sinus of Valsalva (SVA) occupies almost the entire right atrium (RA) and obstructs the tricuspid valve, resulting in preferential flow from the RA to the left atrium (LA) and causing cyanosis.
LV = left ventricle; RV = right ventricle
Real-time motion image is available at www.texasheart.org/journal.
Fig. 2 Angiogram of the ascending aorta (Ao) shows the large aneurysm of the noncoronary sinus of Valsalva (SVA) in the right atrium.
Real-time motion image is available at www.texasheart.org/journal.
Comment
Aneurysms of the sinus of Valsalva arise from congenital or acquired weakening of the aortic medium at the junction with the aortic annulus. The weakening can occur secondary to infection, atherosclerosis, or cystic medial necrosis, as in Marfan syndrome. The aneurysms are typically detected only after rupture that results in a left-to-right shunt and a loud continuous murmur. They also may occur in the presence of ASD, ventricular septal defect, bicuspid aortic valve, aortic stenosis, or aortic insufficiency. Known complications of unruptured aneurysms of the sinus of Valsalva include heart failure, myocardial ischemia,2 arrhythmias,3 syncope,4 and RV outflow obstruction.5
This patient's cyanosis was due to a right-to-left shunt across a secundum ASD associated with a large, unruptured aneurysm of the noncoronary sinus of Valsalva. The intact aneurysm obstructed RV inflow and caused right-to-left shunting at the atrial level. This pathophysiology is an unusual example of reversible cyanosis associated with congenital heart disease.
Supplementary Material
Footnotes
Address for reprints: Andreas P. Kalogeropoulos, MD, Emory Clinical Cardiovascular Research Institute, Suite 535B, 1462 Clifton Rd. NE, Atlanta, GA 30322
E-mail: akaloge@emory.edu
References
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