Key result
A 46-year-old male with Marfan syndrome and an unusual presentation of aortic dissection successfully underwent a three-stage surgical intervention including a Bentall procedure with adequate recovery.
Why the study?
Type A aortic dissection can have fatal complications and prompt diagnosis is crucial, particularly when presenting unusually in Marfan syndrome.
Case Report (n=1)
No
Multimodality imaging is crucial for diagnosing atypical, asymptomatic, or heart failure-mimicking presentations of aortic dissection in patients with Marfan syndrome.
May inform individualized planning in complex aortic cases; leaves open need for prospective data on generalizability.
Marfan’s syndrome (MFS) is an autosomal dominant disorder characterized by aortic root dilation, mitral valve prolapse and aortic regurgitation. The typical aneurysm location involves the thoracic aorta (60%). Thoracoabdominal aortic dissections are quite infrequent (10%). A type A aortic dissection can have fatal complications, such as aortic rupture and cardiac tamponade, and a prompt diagnosis is crucial. The present case involves a 46-year-old patient with Marfan’s syndrome who was admitted to the emergency department for dyspnea. The patient presented congestive signs and manifestations suggesting acute heart failure. An initial transthoracic echocardiogram (TTE) showed ventricular hypertrophy and diastolic dysfunction. Additionally, TTE showed plurivalvular regurgitation, pulmonary hypertension (42 mmHg), aortic dilatation, and a dissection flap. Coronary computed tomography angiography (CCTA) was performed, confirming the suspicion of ascending aortic dissection. The patient underwent a three-stage intervention for the repair of all the defects, with adequate recovery. This was a rare presentation of aortic dissection in MFS, which was a diagnostic challenge for the heart team.
No takes yet. Share an insight, caveat, or question.
Espejel-Guzman et al. (2022) conducted a case report in Marfan Syndrome with Aortic Dissection (n=1). Bentall and Bono procedure, aortic and mitral valve replacement, and stenting was evaluated on Clinical recovery. A 46-year-old male with Marfan syndrome and an unusual presentation of aortic dissection successfully underwent a three-stage surgical intervention including a Bentall procedure with adequate recovery.