acyanotic heart disease- coarctation of aorta

Coarctation of the aorta

Coarctation of the aorta is narrowing of the aortic lumen-usually a discrete area of stenosis distal to the lumen of the left subclavian artery.

Epidemiology

Coarctation of the aorta occurs in approximately 11 per 100 000 births and is more common in males.

Pathology

Although the most common site is just distal to the left subclavian artery, a spectrum of locations and lengths of stenosis may occur. The effects of the coarctation depend on the severity of the stenosis. As the heart pumps more forcefully to overcome the resistance from the narrowing, hypertension develops in the head and upper limb vessels. Collateral blood supply to the body and lower limbs includes the intercostal arteries that may dilate sufficiently to produce rib notching. Complications of coarctation include systemic hypertension, cardiac failure and predisposition to aortic dissection and endocarditis.

Clinical features

Most patients are asymptomatic and the coarctation is detected on routine clinical examination. Differential hypertension is confined to the upper limbs, with normal blood pressure in the lower limbs accompanied by radio-femoral delay. A systolic murmur is usually audible in the aortic area and is loudest posteriorly in the left 4th intercostal space. In addition, bruits may be audible over the intercostal arteries.

Investigations

Electrocardiogram

The ECG is usually normal in the early years; however, later in life left ventricular hypertrophy develops.

Chest X-ray

The chest X-ray may reveal a prominent aortic knuckle and cardiomegaly from ventricular hypertrophy. Rib notching may be visible.

Computed tomography (CT)/magnetic resonance imaging (MRI)

The site of the coarctation may be visualized on echocardiography, but better anatomical definition is obtained from CT or MRI with the possibility of three-dimensional reconstruction .

Management

Surgical correction is the preferred treatment unless the degree of stenosis is insignificant. Approximately 5% recur after corrective surgery.

Prognosis

The prognosis of patients with coarctation depends on the severity of the luminal stenosis. In the majority, hypertension develops by the age of 30 years.


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