Aortic aneurysm- familial thoracic 3

Overview

A rare familial disorder where the aorta has a weak, bulging portion. The condition is asymptomatic but can result in death if it bursts. Type 3 is caused by a genetic defect on chromosome 3p22

Symptoms

• Chest pain
• Back pain

Causes

Commonly, a thoracic aortic aneurysm results from atherosclerosis, which weakens the aortic wall and gradually distends the lumen. An intimal tear in the ascending aorta initiates a dissecting aneurysm in about 60% of patients. Aneurysm of the ascending part of the aorta is usually seen in hypertensive men who are younger than age 60. Aneurysm of the descending part of the aorta, usually found just below the origin of the subclavian artery, is most common in elderly hypertensive men. It’s also seen in younger patients with a history of traumatic chest injury, although less often in those with infection. Aneurysm of the transverse part of the aorta is the least common.

Treatment

A dissecting aneurysm is an emergency that requires prompt surgery and stabilizing measures: an antihypertensive such as nitroprusside; a negative inotropic agent that decreases contractility force, such as propranolol; oxygen for respiratory distress; a narcotic for pain; I.V. fluids; and, possibly, whole blood transfusions. Surgery consists of resecting the aneurysm, restoring normal blood flow through a Dacron or Teflon graft replacement and, with aortic valve insufficiency, replacing the aortic valve. Postoperative measures include careful monitoring and continuous assessment in the intensive care unit, an antibiotic, endotracheal (ET) intubation, chest tube insertion, ECG monitoring, and pulmonary artery (PA) catheterization.