Acute Type II Aortic Dissection with Severe Aortic Regurgitation and Chronic Descending Aortic Dissection in Pregnant Patient with Marfan Syndrome
A 37-year-old female patient with no previous medical history underwent a Cesarean section early in the morning at a local medical center, and then experienced chest pain and dyspnea. She was referred to the Yeungnam University Hospital when these symptoms worsened and when a systolic blood pressure of 60 mmHg and a heart rate of 40 beats/min were recorded. She had undergone a normal pregnancy two years prior and experienced short breath during her most recent pregnancy, but she thought nothing of it. On arrival, besides mild fever and chest discomfort, her vital signs were stable. A chest computed tomography (CT) was performed to evaluate the dyspnea and revealed sinus of Valsalva dilatation at 78 mm in diameter and descending aorta dissection from the left subclavian artery to the renal artery, but there was no dissection of the aortic arch (). On physical examination, a grade IV/VI diastolic murmur was heard, and rales in both lung fields were also heard. A transthoracic echocardiogram revealed severe aortic regurgitation (grade IV), dilated sinus of Valsalva (74 mm), and left ventricular enlargement; however, left ventricular systolic function was maintained with an ejection fraction of 56%.\nConservative treatment of the descending aorta and the Bentall procedure for the ascending aorta were planned because the descending aorta was measured at 4 cm in diameter, both the true and false lumen blood flows were stable, malperfusion symptoms were absent, and the blood pressure in both lower extremities was stable. Surgery was performed through a median sternotomy. Above the aneurysm of the ascending aorta, there was a normal aorta where aortic cross-clamping was possible; therefore, arterial cannulation was performed on the proximal aortic arch, and venous cannulation was performed on the right atrium. After aortic cross-clamping and aortotomy, the dissection flap at the aortic root was observed, and aortic dissection, which is thought to cause huge aneurysms, had occurred (). A composite graft (SJM valved graft conduit 25 mm; St. Jude Medical Inc., St. Paul, MN, USA) was used for the operation. With no total circulatory arrest, the total pump time was 208 minutes and the aortic cross clamp time was 160 minutes. After 30 hours in the intensive care unit, the patient was weaned from the ventilator and transferred to the general ward. An echocardiogram on postoperative day 20 revealed a normal graft and valve function, and the left ventricular end-diastolic volume decreased from 68 to 48 mm. The patient was followed up on postoperative day 34 with 3D CT, which revealed a well-maintained surgical region of the ascending aorta and a stable lesion on the descending aorta (). The patient has been monitored since she was discharged.