CHIẾN LƯỢC HYBRID NHIỀU GIAI ĐOẠN CHO PHÌNH ĐỘNG MẠCH CHỦ NGỰC-BỤNG DỌA VỠ KÈM HỞ VAN HAI LÁ NẶNG VÀ BẤT THƯỜNG XUẤT PHÁT ĐỘNG MẠCH DƯỚI ĐÒN PHẢI Ở BỆNH NHÂN MARFAN CÓ LÓC ĐỘNG MẠCH CHỦ NON-A NON-B: BÁO CÁO CA BỆNH

Abstract

Managing complex aortic disease in patients with Marfan syndrome is especially challenging when accompanied by valvular heart disease and anatomical abnormalities of the aortic arch branches. We present the case of a 28-year-old woman with Marfan syndrome who had severe mitral regurgitation, a non-A non-B aortic dissection extending from the aortic arch to the aortic bifurcation, a large aneurysm of the aortic arch and descending thoracic aorta measuring 78 mm in diameter with an impending risk of rupture, and an anomalous origin of the right subclavian artery. A comprehensive, multistage hybrid strategy was implemented to address all lesions. In the first stage, the patient underwent mitral valve repair with an annuloplasty ring and a modified frozen elephant trunk procedure. The following day, the infrarenal abdominal aorta and both common iliac arteries were replaced with a bifurcated Y-shaped vascular graft, and total debranching of the visceral arteries was performed, including the coeliac artery, superior mesenteric artery, and both renal arteries. Finally, the entire diseased thoracoabdominal aortic segment was excluded using a series of stent grafts. Although the patient underwent multiple major surgeries, postoperative recovery was uneven, and discharge occurred after 37 days. Pre-discharge echocardiography demonstrated no residual mitral regurgitation and preserved left ventricular ejection fraction. Follow-up computed tomography confirmed complete replacement of the aorta with vascular and stent grafts without endoleak. The supra-arch and visceral arteries were well debranched and contracted. This case illustrates that a multistage hybrid strategy planned for the entire aorta may be a feasible treatment option for selected patients with Marfan syndrome and complex concomitant cardiac and aortic disease.