Chest pain in a woman with Marfan syndrome

CLINICAL PRESENTATION: (Dr. C. Lewis) A 32-yearold Hispanic woman with Marfan syndrome presented to the emergency room following the acute onset of left-sided chest pain radiating to her back and left shoulder. In addition, she described mild shortness of breath without cough, hemoptysis, or neurologic symptoms. Her medical history was significant for ectopia lentis as child, but she did not meet clinical criteria for Marfan syndrome until age 26 years. At that time, she developed chest pain and was found on imaging studies to have a chronic ascending aortic dissection. She underwent aortic root and aortic valve replacement using the Bentall technique. The ascending graft required revision in 1997. This was followed by the development of a Stanford type B aortic dissection, which was managed nonoperatively. She has peripheral vascular disease and hypertension, for which she takes verapamil and metoprolol. She has been on chronic anti-coagulation for her prosthetic aortic valve. Her history is also significant for a posterior hip dislocation in 1996. No known family history of Marfan syndrome exists. On initial examination, the patient was tearful and in moderate distress. Her breathing was labored, with a respiratory rate of 40 breaths/minute. Her blood pressure was 130/88, and her heart rate was regular at 129 beats/minute. On auscultation, she had diminished breath sounds at the left lung base. The right lung was clear. The radial pulse was 2 on the right and 1 on the left. Femoral pulses were 2 on the right and 1 on the left. The distal lower extremities were cool but viable, with trace pedal pulses bilaterally. Neurologic function was grossly intact. A chest radiograph was obtained that showed a widened mediastinum (12.5 cm) and a left pleural effusion (Fig. 1). An aortic dissection with a possible rupture was suspected, and she was started on esmolol and nipride drips. Laboratory studies included a hematocrit of 31.9, a platelet count of 458,000, an international normalized ratio (INR) of 2.2, and a partial thromboplastin (PTT) of 31.1. The patient was resuscitated with intravenous fluids and a transfusion of fresh frozen plasma (FFP). A chest computed tomographic (CT) scan was obtained. The CT scan identified her known type B dissection (Crawford type III) from the thoracic aorta beginning after the left subclavian artery and extending to the aortic bifurcation (Figs. 2 and 3). A large left hemothorax was present, and the right kidney was atrophic. A diagnosis of a ruptured descending aortic aneurysm was made, and the descending thoracic aorta was repaired using cardiopulmonary bypass. Intraoperatively, the patient received 16 units of packed red blood cells (PRBCs), 10 units of FFP, 20 units of cryoprecipitate, and 2 units of platelets. She was extubated and removed from intravenous antihypertensive medications by postoperative day 2. On hospital day 9, she was discharged without complications on metoprolol, fosinopril, and coumadin. At 3 weeks postoperatively, she complained of back pain. A CT scan was obtained that identified a probable pseudoaneurysm at the level of the proximal descending aorta. The aneurysmal dilation measured 5.1 cm. The patient was referred to vascular surgery. An arteriogram further defined the descending thoracic aneurysm and a pseudoaneurysm originating at the T-7 vertebral body (Figs. 4 and 5).