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Exp Clin Cardiol. Sep 2012; 17(3): 148–149.
PMCID: PMC3628432
Clinical Cardiology: Case Report
An uncommon cause of a continuous murmur
Bernard Topi, MD,1 Jinu John, MD,1 Amit Agarwal, MD,1 Nishant Nerella, MD,2 Vijay Shetty, MD,2 Adnan Sadiq, MD FACC FASE,2 and Jacob Shani, MD FACP FACC FSCAI2
1Department of Medicine;
2Division of Cardiology, Maimonides Medical Center, Brooklyn, New York, USA
Correspondence: Dr Jinu John, Department of Internal Medicine, Maimonides Medical Center, 4802 10th Avenue, Brooklyn, New York 11219, USA. Telephone 718-283-6000, fax 718-283-8498, e-mail jjohn3/at/maimonidesmed.org, doctorjacob/at/gmail.com
Abstract
Aneurysms of the sinus of Valsalva are rare congenital lesions. Less often, they are encountered secondary to trauma, infective endocarditis or syphilis. The majority of these aneurysms arise from the right coronary sinus. The present report describes a rare case of an aneurysm arising from the noncoronary sinus of Valsalva and rupturing into the right atrium. Patients with unruptured aneurysms often remain asymptomatic. Rupture of the aneurysm usually causes the appearance of a continuous murmur in the left sternal border. Common sites of rupture include the right ventricle, right atrium or left atrium. Surgical repair is usually associated with a favourable outcome.
Keywords: Congenital heart disease, Continuous murmur, Hypertension, Sinus of Valsalva aneurysm
A 30-year-old construction worker with a strong family history of hypertension presented to the emergency department with worsening exertional dyspnea of one months’ duration. Symptoms began four weeks previously when he developed central chest tightness and shortness of breath while pushing a heavy box. At the same time, he also felt weak and diaphoretic. These symptoms lasted for 10 min and subsided on taking rest. There was no associated nausea or radiation of chest symptoms. The next day, he visited his primary doctor who recorded a blood pressure of 190/110 mmHg and began treatment with hydrochlorothiazide 25 mg daily and metoprolol 25 mg twice daily. As his symptoms continued to worsen, he was referred to the Maimonides Medical Center, Brooklyn, New York, USA. He denied any fever, paroxysmal nocturnal dyspnea or recent chest trauma.
On physical examination, the patient was in mild respiratory distress, and had a regular pulse of 90 beats/min and a blood pressure of 140/90 mmHg. He had an elevated jugular venous pulse rate and pitting ankle edema. His lungs were clear on auscultation. Examination of the precordium revealed a grade 3/6 continuous murmur, which was best heard in the left lower sternal border. The electrocardiogram was normal except for T wave inversions in the inferior leads. A chest x-ray revealed cardiomegaly and pulmonary venous congestion. Cardiac enzyme levels were within normal limits.
A transesophageal echocardiogram was performed, which revealed a ruptured sinus of Valsalva aneurysm (Figures 1 and and2;2; videos 1 and 2) arising from the noncoronary sinus and communicating with the right atrium. Colour Doppler confirmed the presence of an aorto-right atrial fistula (Figures 3 and and4;4; videos 3 and 4). A patent foramen ovale with a right to left shunt was also detected.
Figure 1)
Figure 1)
Transesophageal echocardiogram. Midesophageal, two-dimensional short-axis view showing a sinus of Valsalva aneurysm (arrows) arising from the noncoronary sinus (NCS) and bulging into right atrium (RA). LA Left atrium
Figure 2)
Figure 2)
Transesophageal echocardiogram. Transgastric long-axis view showing a sinus of Valsalva aneurysm (arrows) with a characteristic ‘wind-sock’ appearance arising from the aortic root. RA Right atrium; RV Right ventricle
Figure 3)
Figure 3)
Transesophageal echocardiogram. Midesophageal, short-axis view with colour Doppler showing fistulous flow (arrows) from the aorta into the right atrium (RA) through the ruptured sinus of Valsalva aneurysm. LA Left atrium; RV Right ventricle
Figure 4)
Figure 4)
Transesophageal echocardiogram. Transgastric long-axis view with colour Doppler showing aorto-right atrial fistula (arrow) due to rupture of a sinus of Valsalva aneurysm. RA Right atrium; RV Right ventricle
The patient was admitted to the cardiothoracic intensive care unit and was medically optimized. The next day, he underwent a successful Dacron patch closure of the aorto-atrial fistula along with a closure of the patent foramen ovale. The postoperative period was uneventful and the patient was discharged on postoperative day 7. He remained asymptomatic on the first follow-up after discharge.
Sinus of Valsalva aneurysms are rare congenital lesions that can arise from the right coronary (77%), noncoronary (23%) and, sometimes, the left coronary sinus of Valsalva (1). Other less common etiologies include infective endocarditis and syphilis. More frequently encountered in men (2), these aneurysms are usually detected in the third or fourth decade of life.
Often, unruptured sinus of Valsalva aneurysms are asymptomatic. These can become symptomatic once enlargement causes right ventricular outflow tract obstruction, coronary artery compression or conduction abnormalities (3).
Rupture of sinus of Valsalva aneurysms may be spontaneous, triggered by chest trauma or exertion, or iatrogenic (cardiac catheterization). Rupture can occur into the right ventricle, right atrium or left atrium in decreasing order of frequency. Although uncommon, other sites into which rupture can occur are the left ventricle, interventricular septum, pericardium, pulmonary artery and superior vena cava. A transthoracic echocardiogram is diagnostic in the majority of cases. However, in one-quarter of patients, a transesophageal echocardiogram is required to delineate the anatomy.
Once these aneurysms rupture, the median survival is reduced to one to two years (if left untreated) and patients usually succumb to cardiac failure or infective endocarditis (4). Early correction of the defect, either by direct closure or patch repair, is generally associated with a good outcome. Successful percutaneous closure of suitable lesions has also been reported.
In the present case, the patient probably had a congenital sinus of Valsalva aneurysm that enlarged gradually over time and ruptured into the right atrium causing right-sided heart failure. His uncontrolled hypertension and strenuous physical activity at work may have further promoted the rupture of the aneurysm.
Footnotes
DISCLOSURES: The authors have no financial disclosures or conflicts of interest to declare.
REFERENCES
1. Meier JH, Seward JB, Miller FA, Jr, Oh JK, Enriquez-Sarano M. Aneurysms of the left ventricular outflow tract: Clinical presentation, causes, and echocardiographic features. J Am Soc Echocardiogr. 1998;11:729–45. [PubMed]
2. Takach TJ, Reul GJ, Duncan JM, et al. Sinus of Valsalva aneurysm or fistula: Management and outcome. Ann Thorac Surg. 1999;68:1573–7. [PubMed]
3. Bulkley BH, Hutchins GM, Ross RS. Aortic sinus of Valsalva aneurysms simulating primary right-sided valvular heart disease. Circulation. 1975;52:696–9. [PubMed]
4. Moustafa S, Mookadam F, Cooper L, et al. Sinus of Valsalva aneurysms – 47 years of a single center experience and systematic overview of published reports. Am J Cardiol. 2007;99:1159–64. [PubMed]
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