Aneurysm An aneurysm is an outward bulging, likened to a bubble or balloon, caused by a localized, abnormal, weak spot on a blood vessel wall. Aneurysms may be a result of a hereditary condition or an acquired disease. Aneurysms can also be a nidus ( ...

of the aortic sinus, also known as the sinus of Valsalva, is a rare abnormality of the

aorta The aorta ( ) is the main and largest artery in the human body, originating from the left ventricle of the heart and extending down to the abdomen, where it splits into two smaller arteries (the common iliac arteries). The aorta distributes o ...

, the largest artery in the body. The aorta normally has three small pouches that sit directly above the

aortic valve The aortic valve is a valve in the heart of humans and most other animals, located between the left ventricle and the aorta. It is one of the four valves of the heart and one of the two semilunar valves, the other being the pulmonary valve. Th ...

(the sinuses of Valsalva), and an aneurysm of one of these sinuses is a thin-walled swelling. Aneurysms may affect the right (65–85%), non-coronary (10–30%), or rarely the left (< 5%) coronary sinus. These aneurysms may not cause any symptoms but if large can cause

shortness of breath Shortness of breath (SOB), also medically known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing di ...

palpitations Palpitations are perceived abnormalities of the heartbeat characterized by awareness of cardiac muscle contractions in the chest, which is further characterized by the hard, fast and/or irregular beatings of the heart. Symptoms include a rap ...

or blackouts. Aortic sinus aneurysms can burst or rupture into adjacent cardiac chambers, which can lead to

heart failure Heart failure (HF), also known as congestive heart failure (CHF), is a syndrome, a group of signs and symptoms caused by an impairment of the heart's blood pumping function. Symptoms typically include shortness of breath, excessive fatigue, ...

if untreated. Aortic sinus aneurysms may occur in isolation, or may been seen in association with other diseases of the aorta including

Marfan syndrome Marfan syndrome (MFS) is a multi-systemic genetic disorder that affects the connective tissue. Those with the condition tend to be tall and thin, with long arms, legs, fingers, and toes. They also typically have exceptionally flexible joints a ...

, Loeys-Dietz syndrome, and

bicuspid aortic valve Bicuspid aortic valve (aka BAV) is a form of heart disease in which two of the leaflets of the aortic valve fuse during development in the womb resulting in a two-leaflet (bicuspid) valve instead of the normal three-leaflet (tricuspid) valve. BA ...

. They can be diagnosed using an

echocardiogram An echocardiography, echocardiogram, cardiac echo or simply an echo, is an ultrasound of the heart. It is a type of medical imaging of the heart, using standard ultrasound or Doppler ultrasound. Echocardiography has become routinely used in th ...

or cardiac

magnetic resonance imaging Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio wave ...

(MRI) scan. Treatment includes blood pressure control but surgical repair may be needed, especially if the aneurysm ruptures.

Symptoms and signs

If unruptured, sinus of Valsalva aneurysm (commonly abbreviated SVA or SOVA) is usually asymptomatic and typically goes undetected until symptoms appear or

medical imaging Medical imaging is the technique and process of imaging the interior of a body for clinical analysis and medical intervention, as well as visual representation of the function of some organs or tissues (physiology). Medical imaging seeks to re ...

is performed for other reasons. If symptoms do occur, the most common are

myocardial ischemia Coronary artery disease (CAD), also called coronary heart disease (CHD), ischemic heart disease (IHD), myocardial ischemia, or simply heart disease, involves the reduction of blood flow to the heart muscle due to build-up of atherosclerotic pla ...

, and syncope. Even less common, but more serious, presentations are

embolic stroke A stroke is a medical condition in which poor blood flow to the brain causes cell death. There are two main types of stroke: ischemic, due to lack of blood flow, and hemorrhagic, due to bleeding. Both cause parts of the brain to stop functioni ...

and

myocardial infarction A myocardial infarction (MI), commonly known as a heart attack, occurs when blood flow decreases or stops to the coronary artery of the heart, causing damage to the heart muscle. The most common symptom is chest pain or discomfort which ma ...

due to blockage of a

coronary artery The coronary arteries are the arterial blood vessels of coronary circulation, which transport oxygenated blood to the heart muscle. The heart requires a continuous supply of oxygen to function and survive, much like any other tissue or organ o ...

by the aneurysm. A ruptured aneurysm typically leads to an aortocardiac shunt and progressively worsening heart failure. An aneurysm of the aortic sinus may rupture due to

infective endocarditis Infective endocarditis is an infection of the inner surface of the heart, usually the valves. Signs and symptoms may include fever, small areas of bleeding into the skin, heart murmur, feeling tired, and low red blood cell count. Complicatio ...

involving the aortic wall and tertiary-stage

syphilis Syphilis () is a sexually transmitted infection caused by the bacterium '' Treponema pallidum'' subspecies ''pallidum''. The signs and symptoms of syphilis vary depending in which of the four stages it presents (primary, secondary, latent, a ...

. The manifestations appear depending on the site where the sinus has ruptured. For example, if the sinus ruptures in a low pressure area like the right atrium or right ventricle then a continuous type of murmur is heard. The murmur is located in the left parasternal region mainly confined to the lower sternum. It is also accompanied by a superficial thrill. A ruptured Sinus of Valsalva abscess represents a surgical emergency.

Causes

This type of aneurysm is typically

congenital A birth defect, also known as a congenital disorder, is an abnormal condition that is present at birth regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can ...

and may be associated with

heart defects A congenital heart defect (CHD), also known as a congenital heart anomaly and congenital heart disease, is a defect in the structure of the heart or great vessels that is present at birth. A congenital heart defect is classed as a cardiovascular ...

. It is sometimes associated with

or Loeys–Dietz syndrome, but may also result from Ehlers–Danlos syndrome,

atherosclerosis Atherosclerosis is a pattern of the disease arteriosclerosis in which the wall of the artery develops abnormalities, called lesions. These lesions may lead to narrowing due to the buildup of atheromatous plaque. At onset there are usually no s ...

hypoplastic left heart syndrome Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely underdeveloped and incapable of supporting the systemic circulation. It is estimated to account for 2-3% of all congenital he ...

cystic medial necrosis Familial thoracic aortic aneurysm is an autosomal dominant disorder of large arteries. There is an association between familial thoracic aortic aneurysm and Marfan syndrome as well as other hereditary connective tissue disorders. Signs and sympt ...

, chest injury, or infective

endocarditis Endocarditis is an inflammation of the inner layer of the heart, the endocardium. It usually involves the heart valves. Other structures that may be involved include the interventricular septum, the chordae tendineae, the mural endocardium, or the ...

Diagnosis

The first step in diagnosis is typically

transthoracic echocardiography An echocardiography, echocardiogram, cardiac echo or simply an echo, is an ultrasound of the heart. It is a type of medical imaging of the heart, using standard ultrasound or Doppler ultrasound. Echocardiography has become routinely used in ...

. However, if surgery is planned or if the standard echocardiogram lacks sufficient detail, then one or more additional studies are recommended. These studies include transesophageal echocardiography, 3D echocardiography,

CT Angiography Computed tomography angiography (also called CT angiography or CTA) is a computed tomography technique used for angiography—the visualization of arteries and veins—throughout the human body. Using contrast injected into the blood vessels, im ...

and aortic angiography.

Cardiac MRI Cardiac magnetic resonance imaging (cardiac MRI), also known as cardiovascular MRI, is a magnetic resonance imaging (MRI) technology used for non-invasive assessment of the function and structure of the cardiovascular system. Conditions in which i ...

may be another option.

Treatment

Medical therapy of

aneurysm An aneurysm is an outward bulging, likened to a bubble or balloon, caused by a localized, abnormal, weak spot on a blood vessel wall. Aneurysms may be a result of a hereditary condition or an acquired disease. Aneurysms can also be a nidus ( ...

of the aortic sinus includes

blood pressure Blood pressure (BP) is the pressure of circulating blood against the walls of blood vessels. Most of this pressure results from the heart pumping blood through the circulatory system. When used without qualification, the term "blood pressure ...

control through the use of drugs, such as

beta blockers Beta blockers, also spelled β-blockers, are a class of medications that are predominantly used to manage abnormal heart rhythms, and to protect the heart from a second heart attack after a first heart attack ( secondary prevention). They are ...

. Another approach is surgical repair. The determination to perform surgery is usually based upon the diameter of the aortic root (with 5 centimeters being a rule of thumb - a normal size is 2-3 centimeters) and the rate of increase in its size (as determined through repeated

echocardiography An echocardiography, echocardiogram, cardiac echo or simply an echo, is an ultrasound of the heart. It is a type of medical imaging of the heart, using standard ultrasound or Doppler ultrasound. Echocardiography has become routinely used in ...

). An alternative to surgical repair or a ruptured aneurysm is percutaneous closure. In this technique, a wire is introduced via a small incision in the groin and advanced through the vascular system to the aneurysm. A closure device is advanced along the wire before being expanded to straddle the site of rupture.

Prognosis

If a sinus of Valsalva aneurysm ruptures, the life expectancy without treatment is approximately four years. Surgery carries a 1% risk of intra-operative death with higher risks associated with infected aneurysms, and 5- to 10-year survival rates following surgery range from 82-97%.

Epidemiology

Aneurysms of the sinuses of Valsalva are estimated to affect 0.09% of the population. Rupture of a sinus of Valsalva can occur at any age. Rupture is five times more likely to occur in those of far eastern than western ethnic backgrounds, and is twice as likely to occur in males than females.

Symptoms and signs

Causes

Diagnosis

Treatment

Prognosis

Epidemiology

History

See also

References

External links