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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

    • – Pulmonary congestion may be present

  • MRI: identifies ruptured and unruptured sinus ...

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