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.
Coarctation of the aorta occurs in approximately 11 per 100 000 births and is more common in males.
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.
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.
The ECG is usually normal in the early years; however, later in life left ventricular hypertrophy develops.
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 .
Surgical correction is the preferred treatment unless the degree of stenosis is insignificant. Approximately 5% recur after corrective surgery.
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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