Sinus of Valsalva Aneurysm

KEY FEATURES

ESSENTIALS OF DIAGNOSIS

• Diverticulum of a coronary sinus of the aortic valve

GENERAL CONSIDERATIONS

• Rare congenital cardiac defect due to a defect in the aortic media that leads to aortic root dilation and usually including the right aortic sinus

• Acquired sinus of Valsalva aneurysm may be due to Marfan’s syndrome or syphilitic aortitis or is a function of aging

• Other generalized disorders such as Ehlers-Danlos, Turner’s, and Williams’ syndromes, osteogenesis imperfecta, and traumatic injury may be associated with aortic root dilation and/or distortion

• Associated with a ventricular septal defect (usually supracristal) in 40% of patients

• Often associated with aortic insufficiency

• Leads to progressive dilation of the aorta usually over many years

• Rupture into any chamber (usually the right ventricle followed by the right atrium) possible

• Compression of the coronary arteries and/or the conduction system may lead to ischemia and/or heart block, respectively

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

• Chest and/or epigastric pain similar to that in acute myocardial infarction, which occurs suddenly after strenuous exertion due to sudden rupture of the aneurysm

• Progressive exertional dyspnea and/or chest pain in patients with advancing heart failure related to smaller insidious ruptures

• Patients with unruptured aneurysms may be asymptomatic or have angina from coronary artery compression or symptoms of heart block related to compression of the conduction system

PHYSICAL EXAM FINDINGS

• Patients without aneurysm rupture often have normal exam findings

• An ejection murmur may be present at the left base radiating into the back with an unruptured aneurysm partially obstructing the right ventricular outflow tract

• Bounding pulses, aortic regurgitation in patients with rupture into the left ventricle

• Loud continuous murmur with the diastolic component best heard along the sternal border in patients with rupture into the right cardiac chambers

• Pulmonary rales may be present

DIFFERENTIAL DIAGNOSIS

• Aortic dissection

• Aortic regurgitation with aortic root dilatation

• Aorta to pulmonary artery fistula (anterior-posterior window)

• Ventricular septal defect

• Coronary artery aneurysm with fistula to right heart

• Rupture may simulate other causes of acute chest pain or heart failure

DIAGNOSTIC EVALUATION

LABORATORY TESTS

• No specific serologic or genetic markers have been identified

ELECTROCARDIOGRAPHY

• Biventricular hypertrophy by voltage in patients with ruptured aneurysm

• ST-T depression caused by myocardial ischemia

• Second- or third-degree heart block in patients with conduction system involvement

IMAGING STUDIES

• Echocardiography:

  • – Provides an accurate description of the aortic root, proximal aorta, aortic valve, and surrounding structures

  • – Doppler findings can provide an accurate indication of the shunt location and magnitude

• Chest x-ray:

  • – Cardiomegaly—right heart enlargement in the setting of rupture into the right ventricle

  • – ...