Aortic aneurysm- familial thoracic 4

Synonyms

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 4 also involves another heart defect (patent ductus arteriosus) and is caused by a genetic defect on chromosome 16p13.13-p13.12.

Symptoms

• Chest pain
• Back pain
• Patent ductus arteriosus

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

Antihypertensives, negative inotropic agents, airway maintenance, opioids for pain, I.V. fluids, blood transfusions, surgery

Resources

• NIH