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A continuous cardiac murmur

Cleveland Clinic Journal of Medicine. 2015 July;82(7):414-418 | 10.3949/ccjm.82a.14068
July 1, 2015|Cleveland Clinic Journal of Medicine
Rebecca Napier, MD;Jonathan D. Gardner, MD;Susan Noe, MD, FACC, FACP;Joe B. Calkins, Jr., MD, FACC, FACP, FASE
Author and Disclosure Information

Rebecca Napier, MD
Georgia Regents University, Medical College of Georgia, Augusta

Jonathan D. Gardner, MD
Georgia Regents University, Medical College of Georgia, Augusta

Susan Noe, MD, FACC, FACP
Charlie Norwood VA Medical Center and Georgia Regents University, Medical College of Georgia, Augusta

Joe B. Calkins, Jr., MD, FACC, FACP, FASE
Charlie Norwood VA Medical Center and Georgia Regents University, Medical College of Georgia, Augusta

Address: Rebecca Napier, MD, Georgia Regents University, 1120 15th Street, BBR 6518, Augusta, GA 30912; e-mail: rnapier@gru.edu

SINUS OF VALSALVA ANEURYSM

Sinus of Valsalva aneurysm is rare, with an incidence of 0.09% to 0.15%. From 65% to 85% are in the right coronary cusp, 10% to 30% are in the noncoronary cusp, and fewer than 5% are in the left coronary cusp.5

This condition is most often congenital, accounting for up to 3.5% of congenital cardiac anomalies, though it can be acquired. Formation of the aneurysm is generally related to weakening of elastic fibers and muscular tissues that progresses over time.

Many cases of sinus of Valsalva aneurysm are associated with additional cardiac defects.1 Ventricular septal defect is the most common coexisting congenital anomaly, occurring in up to 53% of patients and frequently associated with aneurysms involving the right coronary cusp and with sinus of Valsalva aneurysm.6 Other congenital anomalies often accompanying sinus of Valsalva aneurysm include pulmonary stenosis, atrial septal defect, bicuspid aortic valve, tetralogy of Fallot, patent ductus arteriosus, coarctation of the aorta, and subaortic stenosis. Another associated condition is aortic regurgitation, for which more than half of affected patients eventually require aortic valve replacement.2

Acquired sinus of Valsalva aneurysm can be the result of endocarditis, trauma, surgery, cardiac catheterization, or inflammatory or degenerative processes including, rarely, tertiary syphilis.3

Sinus of Valsalva aneurysm often remains asymptomatic, but symptoms may arise if the aneurysm ruptures, resulting in intracardiac shunting or aneurysm-associated compression of adjacent cardiac structures such as coronary arteries. Rupture may be spontaneous, secondary to chest trauma or excess exertion, or iatrogenic.

Imaging studies such as echocardiography, cardiac computed tomography, and cardiac magnetic resonance imaging are essential in diagnosing and managing sinus of Valsalva aneurysm and identifying coexisting cardiac anomalies.

Rupture occurs most commonly into the right ventricle, followed in frequency by the right atrium or left atrium. Once rupture occurs, median survival is 1 to 2 years if left untreated, with death often secondary to congestive heart failure or infective endocarditis.7

Surgery remains the preferred approach to the treatment of ruptured sinus of Valsalva aneurysm. Operative risk is reasonably low and long-term outcomes are good. The appropriate therapy for unruptured and asymptomatic sinus of Valsalva aneurysm remains less clear.

Successful transcatheter closure of ruptured sinus of Valsalva aneurysm has been described using Amplatzer devices, a procedure that avoids sternotomy and cardiopulmonary bypass. Despite advances in percutaneous techniques, open surgery with or without aortic valve replacement remains the current standard of care.8

BACK TO OUR PATIENT

In the case described above, the initial diagnostic study done to evaluate the patient’s dyspnea and murmur was transthoracic echocardiography, which demonstrated a relatively preserved ejection fraction with mild aortic regurgitation and an aneurysmal structure extending from the aortic root toward the right atrium.

Transesophageal echocardiography confirmed this finding (Figure 1). Cross-sectional imaging of the aortic valve (Figure 2) showed the aneurysm arising from the noncoronary cusp and communicating with the right atrium. Color flow Doppler (Figure 3) confirmed continuous flow between the aneurysmal sinus and right atrium throughout the cardiac cycle, consistent with the continuous murmur noted on physical examination.

Figure 1. Transesophageal echocardiography shows a sinus of Valsalva aneurysm (large arrow) extending from the aortic root into the right atrium. The small arrow points to the tricuspid valve. (LA, left atrium; RA, right atrium; RV, right ventricle.)
Figure 2. Transesophageal echocardiography of the aortic valve shows communication of the aneurysm (arrow) with the noncoronary cusp and extension into the right atrium. (LA, left atrium; RA, right atrium; NC, noncoronary cusp of the aortic valve; RC, right coronary cusp; LC, left coronary cusp.)
Figure 3. Color flow Doppler imaging confirmed that blood was flow-ing from the aneurysmal sinus (small arrow) into the right atrium (large arrow). This flow was present throughout the cardiac cycle because a constant pressure gradient exists between the aorta and the right atrium, resulting in a continuous murmur. A small jet of centrally located aortic regurgitation is also seen.

The aneurysm was also noted on aortography (Figure 4) obtained before the patient underwent surgery to correct it. The surgery was successful, no complications occurred, and the murmur and associated dyspnea had completely resolved at subsequent follow-up.

Figure 4. Aortography also showed the aneurysm (arrow).

This case highlights the importance of imaging studies such as echocardiography in diagnosing and managing sinus of Valsalva aneurysm, and also the importance of physical examination in guiding the diagnostic evaluation and differentiating this condition from other cardiac disorders.

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