Sinus Of Valsalva Aneurysm


Article Author:
David Bass


Article Editor:
Vijai Tivakaran


Editors In Chief:
Yvonne Carter
Jason Wallen


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Orawan Chaigasame
Carrie Smith
Abdul Waheed
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Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Hajira Basit
Phillip Hynes
Kavin Sugumar


Updated:
6/4/2019 5:45:35 PM

Introduction

Sinus of Valsalva aneurysm (SOVA) is an abnormal dilatation of the aortic root located between the aortic valve annulus and the sinotubular junction. This occurs as a consequence of the weakness of the elastic lamina at the junction of the aortic media and the annulus fibrosis. The function of the normal sinuses is to prevent occlusion of the coronary artery ostia during systole when the aortic valve opens. The normal sinus diameter is less than 4.0 cm for men and 3.6 cm for women. Sinus of Valsalva aneurysm can be either congenital or acquired. They are usually isolated, rare case reports describe aneurysm of two to three sinuses. Sinus of Valsalva aneurysm rupture is a potentially fatal complication of sinus of Valsalva aneurysm and should prompt urgent referral to a cardiothoracic surgeon for consideration of repair.[1][2][3][4]

Etiology

Sinus of Valsalva aneurysm can be either congenital or acquired. Embryologically, sinus of Valsalva aneurysm forms first as a blind diverticulum secondary to pressure forces on the aortic root. Congenital sinus of Valsalva aneurysm has been linked to connective tissue diseases, such as Marfan's syndrome and Ehlers-Danlos syndrome. Congenital Sinus of Valsalva aneurysm may be associated with bicuspid aortic valves.[5][6][7]

Acquired forms of sinus of Valsalva aneurysm may also be seen with connective tissue disease. Infectious etiologies that weaken elastic tissue and are associated with acquired Sinus of Valsalva aneurysm include syphilis, bacterial endocarditis, and tuberculosis. Sinus of Valsalva aneurysm may also occur secondary to chronic changes of atherosclerosis and medial cystic necrosis. Chest trauma and iatrogenic injury during aortic valve surgery have been reported as causes of acquired Sinus of Valsalva aneurysm. Vasculitic diseases, such as Takayasu's Arteritis, may lead to a formation of the sinus of Valsalva aneurysm. 

Epidemiology

The estimated rate of Sinus of Valsalva aneurysm is approximately 0.09% of the general population, based on large autopsy series. SOVAs comprise up to 3.5% of all congenital heart defects. Sinus of Valsalva aneurysms usually affect the right coronary sinus, followed by the noncoronary sinus, and finally the left coronary sinus. Males are four times more likely to be affected than females. There is a higher reported incidence in Asian groups.[8][9]

Pathophysiology

Non-ruptured sinus of Valsalva aneurysm is usually asymptomatic. However, non-ruptured sinus of Valsalva aneurysm can lead to cardiac arrhythmias; atrial fibrillation and complete heart block have both been described. Non-ruptured Sinus of Valsalva aneurysm thrombosis can lead to coronary ostia occlusion. These patients frequently present with acute coronary syndrome. Non-ruptured sinus of Valsalva aneurysm is associated with significant aortic valve regurgitation in 30% to 50% of cases.

Ruptured sinus of Valsalva aneurysm is a feared complication of sinus of Valsalva aneurysm. Consequences of rupture typically depend on the anatomical location of the aneurysm. Rupture of the right and noncoronary sinuses typically results in communication between the aorta and either the right atrium or the right ventricular outflow tract, thus creating a left to right shunt, which can lead to right ventricular overload and right-sided heart failure. Left sinus of Valsalva aneurysm rupture is clinically less significant, causing communication to the left atrium or left ventricular outflow tract.[10][11][12]

Histopathology

Sinus of Valsalva aneurysm occurs due to a weakening of elastic lamina between the aortic media and the annulus fibrosis.

History and Physical

Patients that have sinus of Valsalva aneurysm may be completely asymptomatic or may present with non-specific complaints, such as dyspnea, chest pain, palpitations, or loss of consciousness. Physical exam findings are usually not present unless an aneurysm is large or has ruptured. The classic finding on auscultation is a continuous sawing-like murmur that occurs over both heart sounds. There may be a diastolic descrescendo murmur suggestive of aortic regurgitation. Patients with ruptured sinus of Valsalva aneurysm will frequently present with dyspnea and decreased exercise tolerance. In advanced stages, congestive heart failure may occur.

Evaluation

Cardiac computed tomography is the test of choice for quantifying size and morphology of sinus of Valsalva aneurysm. Echocardiography, usually transesophageal, will demonstrate flow and hemodynamic significance if rupture is suspected. Cardiac MRI will demonstrate anatomy as well as hemodynamic significance as well. Cine Cardiac MRI is considered the gold standard for diagnosis but is not required if other imaging modalities sufficiently give the diagnosis as well as pertinent anatomic and physiologic details. Patients will undergo coronary angiography before cardiac surgery to assess coronary anatomy. Patients with low risk of coronary disease may have an evaluation of coronary anatomy using cardiac computed tomography, and may not need to undergo cardiac catheterization. Patients with intermediate or high risk for coronary artery disease will usually undergo cardiac catheterization for assessment of possible bypass grafting at the time of cardiac surgery.

Treatment / Management

Ruptured sinus of Valsalva aneurysm traditionally requires surgical management, although endovascular closure devices have been used with good outcomes. Surgical management of sinus of Valsalva aneurysm is preferred when there is significant aortic regurgitation or a ventricular septal defect. Surgical management remains the preferred method of treatment of ruptured sinus of Valsalva aneurysm. Ruptured sinus of Valsalva aneurysm warrants urgent cardiothoracic surgical evaluation, as patients may quickly deteriorate.[13][14]

Non-ruptured sinus of Valsalva aneurysm should be surgically repaired if there is associated with significant symptoms or are rapidly enlarging. The 2010 American Guidelines for Thoracic Aortic Disease recommend surgical repair to be considered in those with aneurysms greater than 5.5 cm, greater than 5 cm in those with bicuspid valves, and greater than 4.5 cm in the setting of connective tissue disease. Sinus of Valsalva aneurysm repair should be considered when there is a growth rate of more than 0.5 cm/year. Surgical repair entails the use of cardiopulmonary bypass, cardioplegia, and either primary closure or patch closure. Surgical mortality ranges from 1.9% to 3.6%. Survival rates are close to 90% after 15 years.

Medical management of Sinus of Valsalva aneurysm rupture is insufficient for definitive treatment. Medical management should serve as a bridge to help temporarily stabilize patients until surgical or transcatheter definitive therapy is available. Medical management involves treating arrhythmias as needed, treating endocarditis if present, and treating heart failure if present. Patients with ruptured Sinus of Valsalva aneurysm typically succumb to their disease state within one year of diagnosis due to congestive heart failure in the setting of left to right shunting. Left to right shunting is the usual underlying etiology of decompensation. 

Pearls and Other Issues

Although rare, sinus of Valsalva aneurysm can be a significant cause of morbidity. Diagnosis requires a low index of suspicion and proper use of cardiac imaging. Ruptured sinus of Valsalva aneurysm is a surgical urgency with a high rate of mortality if left untreated. Surgical repair remains the preferred method of treatment, although transcatheter closure devices are an acceptable treatment for patients with suitable anatomy and high risk for cardiac surgery.

Enhancing Healthcare Team Outcomes

Sinus of valsalva aneurysms are best managed by a multidisciplinary team including cardiac nurses. Medical management is only done to stabilize the patient. These patients need surgery as it can be life saving. Once a sinus of valsalva aneurysm ruptures, the prognosis is very poor.


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Sinus Of Valsalva Aneurysm - Questions

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Transesophageal echocardiography reveals an unruptured sinus of valsalva aneurysm in a patient evaluated for widened mediastinum on chest x-ray. What is the next best step in management?



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A 25-year-old female with Marfan syndrome presents to your office for routine follow up. She is in her usual state of health and denies medical complaints. A focused physical exam reveals regular heart sounds without murmur or gallop and no evidence of volume overload. She has a known right sinus of Valsalva aneurysm that was 4.2 cm in diameter last year and demonstrated no evidence of rupture on transesophageal echocardiogram. Repeat cardiac computed tomography one year after the last imaging study shows that the right sinus of Valsalva is now 4.6 cm in diameter, indicating a 0.3 cm increase in one year. What is the next step in management?



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A 45-year-old male with a history of bacterial endocarditis presents for routine follow up. He has no symptoms. A murmur is auscultated on exam which prompts a referral for an echocardiogram. Which of the following fistulous formations would be most likely to occur?



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Which of the following is not known to cause sinus of Valsalva aneurysms?



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A 45-year-old female with hypertension presented to an emergency department with chest pain and undergoes cardiac computed tomography to assess for obstructive coronary artery disease. Her coronary arteries are widely patent, but she is found to have a 4.7 cm sinus of Valsalva aneurysm on the right coronary sinus. Which of the following are not an indication for repair of the sinus of Valsalva aneurysm?



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Sinus Of Valsalva Aneurysm - References

References

Serban AM,Bătrâna N,Cocoi M,Ianoș R,Moț Ș,Kovacs E,Axente DD,Man C,Molnar A, The role of echocardiography in the diagnosis and management of a giant unruptured sinus of Valsalva aneurysm. Medical ultrasonography. 2019 May 2;     [PubMed]
Galeczka M,Glowacki J,Yashchuk N,Ditkivskyy I,Rojczyk D,Knop M,Smerdzinski S,Cherpak B,Szkutnik M,Bialkowski J,Fiszer R,Lazoryshynets V, Medium- and long-term follow-up of transcatheter closure of ruptured sinus of Valsalva aneurysm in Central Europe population. Journal of cardiology. 2019 Apr 22;     [PubMed]
Wierda E,Koolbergen DR,de Mol BAJM,Bouma BJ, Rupture of a giant aneurysm of the sinus of Valsalva leading to acute heart failure: a case report demonstrating the excellence of echocardiography. European heart journal. Case reports. 2018 Sep;     [PubMed]
Hajizeinali A,Hosseinsabet A, Percutaneous device closure of a ruptured aortic sinus of Valsalva aneurysm in a patient with a mechanical bileaflet aortic valve. Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir. 2019 Apr;     [PubMed]
Wingo M,de Angelis P,Worku BM,Leonard JR,Khan FM,Hameed I,Lau C,Gaudino M,Girardi LN, Sinus of Valsalva aneurysm repairs: Operative technique and lessons learned. Journal of cardiac surgery. 2019 Apr 6;     [PubMed]
Ohno N,Watanabe K,Maeda T,Kato O,Ueno G,Yoshizawa K,Fujiwara K, A rare case of unruptured extracardiac multiple sinus of Valsalva aneurysms originating from the orifices with partial aortic wall defects. Surgical case reports. 2019 Mar 27;     [PubMed]
Urbanski PP,Hirao S,Irimie V, Root repair in patient with huge sinus Valsalva aneurysm and severe aortic regurgitation. General thoracic and cardiovascular surgery. 2019 Mar 13;     [PubMed]
Seo KW,Park JS, Sinus of Valsalva Aneurysm and Multiple Aortic Aneurysms Provoked by Viral Myocarditis. Korean circulation journal. 2019 Feb;     [PubMed]
Stöger G,Borger MA,Misfeld M, A giant aneurysm of the left sinus of Valsalva. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery. 2019 Jan 25;     [PubMed]
Diwakar A,Patnaik SS,Hiremath CS,Chalam KS,Dash P, Rupture of sinus of valsalva - A 15 years single institutional retrospective review: Preoperative heart failure has an impact on post operative outcome? Annals of cardiac anaesthesia. 2019 Jan-Mar;     [PubMed]
Chen J,Liang HN,Wu L,Dong SH,Li JH, Right sinus of Valsalva aneurysm spontaneously dissecting into the interventricular septum in a rare case of Behcet's disease. European heart journal cardiovascular Imaging. 2019 Jan 9;     [PubMed]
Stróżyk A,Kołaczkowska M,Fijałkowska J,Siondalski P,Fijałkowski M, Sinus of Valsalva rupture in a patient with a mechanical aortic prosthesis: aneurysm dissecting into the interventricular septum. Kardiologia polska. 2018;     [PubMed]
Sarkar M,Wehman B,Mukherjee R,Taylor BS, Left sinus of Valsalva aneurysm presenting as myocardial ischemia. The Journal of thoracic and cardiovascular surgery. 2018 Oct 10;     [PubMed]
Chan N,Charalambous M,Fuschetto DP,Fuschetto O,Makaryus JN, Severe compression of the left circumflex coronary artery by a large sinus of Valsalva aneurysm. Journal of cardiovascular computed tomography. 2018 Nov 3;     [PubMed]

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