Clinical History
A 24-year-old man who known as a marfan syndrome, complained of severe chest pain for 2 days. For suspicion of aortic dissection, first TEE, then CE-MRA examinations were performed. Coarctation and the dissection at the proximal portion of descending thoracic aorta were diagnosed by TEE. CE-MRA showed the dissection which was beginning at the proximal portion of the left subclavian artery and ending at the coarctation and also aortoannular ectasia at the ascending thoracic aorta.
Imaging Findings
A 24-year-old man who known as a marfan syndrome or related connective tissue disorder, complained of severe chest pain for 2 days. Although any differences in blood pressure and pulses between both arms couldn’t be detected, a clinical diagnosis of acute aortic dissection was proposed and transthoracic and transesophageal echocardiographic (TEE) examinations were performed. TEE examination showed aortic coarctation and dissecan aneurysm at the proximal portion of descending thoracic aorta. Any aortic regurtition wasn’t seen during transthoracic echocardiography. Contrast-enhanced magnetic resonance angiography (CE-MRA) showed the dissection which was beginning at the proximal portion of the left subclavian artery and ending at the coarctation (Fig 1). Dissection couldn’t continue to the distal segment of the descenden thoracic aorta due to coarctation (Fig 2). The diameter of the ascending thoracic aorta was measured 5.4 cm in diameter and aortoannular ectasia was also diagnosed on MRA. Elective surgery was considered because patient’s clinical and hemodynamic conditions were stable.
Discussion
It is difficult to diagnose the marfan syndrome because there is no specific laboratory test for the syndrome. Clinic and radiographic findings vary greatly among the affected individuals and most people affected by the marfan syndrome do not have all the possible signs and complications of the syndrome. Dilatation of the ascending aorta and dissection are two primary manifestations and presented case has these two. Symptoms of the dissections depend on the affected vessels. If the dissection progresses to the abdominal aorta, it can cause the ischemic events to the viscera. In this case, aortic coarctation prevented the progress of the dissection to the distal segments of the thoracic and abdominal aorta. Acute severe chest pain should proposed to the acute aortic dissection especially in a young patient. Once the possibility of the a dissection has been raised, an imaging study is needed and TEE should be the first imaging modality. Although contrast enhanced MRA can give more information about the progress of the dissection, MRA should be chosen as a complementary imaging modality in cooperative and hemodinamically stable patients.
Differential Diagnosis List
Marfan syndrome who has type B acute dissecan aneurysm ending at the aortic coarctation and aortoannular ectasia.
Final Diagnosis
Marfan syndrome who has type B acute dissecan aneurysm ending at the aortic coarctation and aortoannular ectasia.
