A Rare Case of Continuous Murmur

JOURNAL OF CARDIAC FAILURE(2016)

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Introduction: The patient is a 59 YO male who presented with worsening SOB. He was recently evaluated for his SOB 1 week prior at an outside hospital, where he underwent right and left heart catheterization showing Qp/Qs of 1.6 with normal coronary angiography, TEE, and TTE and was diagnosed as having a perimebrenous VSD and was treated for heart failure. At time of presentation his heart rate was 125 BPM, blood pressure 84/46 mm Hg, respiratory rate of 30, oxygen saturation of 96% on 2 liters. His cardiac exam revived a continuous murmur at the left sternal boarder 3rd intercostal space. EKG showed a sinus tachycardia with RBBB with left anterior fascicular block. Troponin and BNP were elevated. Emergent Color Doppler on TTE showed a continuous flow into right atrium. Rupture of ruptured non-coronary sinus of Valsalva aneurysm with a fistula into the right atrium was the diagnosis. Surgeon was consulted and patient was taking to OR. Sinus of Valsalva of aneurysm (SVA) is a rare congenital heart disease, constituting approximately 3.5 % of all congenital heart diseases. SVA usually results from a separation between the aortic media and annulus fibrosis. It arises from the right coronary (77%), non- coronary (23%) and left coronary sinus of Valsalva in less than 5%. Unruptured SVA is usually asymptomatic, with most of the patients becoming symptomatic at age 30–45 years, however presentation from infancy to 70 years has been documented. Symptoms are likely to show once enlargement causes RVOT, coronary artery compression or conduction abnormalities. Chest pain, SOB and feeling fatigue are symptoms associated with rupture of SVA. It may be spontaneous, triggered by chest trauma or exertion, or iatrogenic like cardiac catheterization. TTE has successfully identified SVA in most cases, however TEE may be needed in 25% of cases to delineate the anatomy. CMR may be used in diagnosing the coexistence cardiac lesion, while cardiac angiography is also performed to evaluate coronary perfusion prior to surgery. Intact non-symptomatic aneurysm is usually managed conservatively with close follow-up in the absence of coexistence cardiac disease, while the optimal care for a ruptured SVAs is surgical repair. The diagnose of a rupture of Sinus of Valve aneurysm can be challenging. The physical exam finding of a new continuous murmur can be helpful in many cases but can be difficult to interpret depending on the amount of flow, the degree of tachycardia and the patients body habitus. If the physical exam is not conclusive one should always analyze the objective data such as TTE or TEE carefully in relation of physical exam findings. Any continuous flow into the left atrium or ventricle seen by Doppler imaging on echocardiography should be further investigated if the diagnosis still remains elusive. The importance of a detailed physical exam which is not consistent the current working diagnosis should be further investigated. If the diagnosis is not made in a timely manner this could result in a fatal outcome which can been prevented by a through physical exam and a detailed evaluation by echocardiography. Introduction: The patient is a 59 YO male who presented with worsening SOB. He was recently evaluated for his SOB 1 week prior at an outside hospital, where he underwent right and left heart catheterization showing Qp/Qs of 1.6 with normal coronary angiography, TEE, and TTE and was diagnosed as having a perimebrenous VSD and was treated for heart failure. At time of presentation his heart rate was 125 BPM, blood pressure 84/46 mm Hg, respiratory rate of 30, oxygen saturation of 96% on 2 liters. His cardiac exam revived a continuous murmur at the left sternal boarder 3rd intercostal space. EKG showed a sinus tachycardia with RBBB with left anterior fascicular block. Troponin and BNP were elevated. Emergent Color Doppler on TTE showed a continuous flow into right atrium. Rupture of ruptured non-coronary sinus of Valsalva aneurysm with a fistula into the right atrium was the diagnosis. Surgeon was consulted and patient was taking to OR. Sinus of Valsalva of aneurysm (SVA) is a rare congenital heart disease, constituting approximately 3.5 % of all congenital heart diseases. SVA usually results from a separation between the aortic media and annulus fibrosis. It arises from the right coronary (77%), non- coronary (23%) and left coronary sinus of Valsalva in less than 5%. Unruptured SVA is usually asymptomatic, with most of the patients becoming symptomatic at age 30–45 years, however presentation from infancy to 70 years has been documented. Symptoms are likely to show once enlargement causes RVOT, coronary artery compression or conduction abnormalities. Chest pain, SOB and feeling fatigue are symptoms associated with rupture of SVA. It may be spontaneous, triggered by chest trauma or exertion, or iatrogenic like cardiac catheterization. TTE has successfully identified SVA in most cases, however TEE may be needed in 25% of cases to delineate the anatomy. CMR may be used in diagnosing the coexistence cardiac lesion, while cardiac angiography is also performed to evaluate coronary perfusion prior to surgery. Intact non-symptomatic aneurysm is usually managed conservatively with close follow-up in the absence of coexistence cardiac disease, while the optimal care for a ruptured SVAs is surgical repair. The diagnose of a rupture of Sinus of Valve aneurysm can be challenging. The physical exam finding of a new continuous murmur can be helpful in many cases but can be difficult to interpret depending on the amount of flow, the degree of tachycardia and the patients body habitus. If the physical exam is not conclusive one should always analyze the objective data such as TTE or TEE carefully in relation of physical exam findings. Any continuous flow into the left atrium or ventricle seen by Doppler imaging on echocardiography should be further investigated if the diagnosis still remains elusive. The importance of a detailed physical exam which is not consistent the current working diagnosis should be further investigated. If the diagnosis is not made in a timely manner this could result in a fatal outcome which can been prevented by a through physical exam and a detailed evaluation by echocardiography.
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