Two-Stage Total Cardioaortic Replacement for End-Stage Heart and Aortic Disease in Marfan Syndrome: Case Report and Review of the Literature

Adult Surgical Experience With Loeys-Dietz Syndrome

“…Further, case studies [6,8,9,11,12,[13][14][15][16][17] have verified many early claims regarding management of LDS patients. Tran-Fadulu and colleagues [14] described the aggressive natural history of LDS in multigenerational families with the disorder.…”

“…This patient, who was initially clinically diagnosed as having Marfan syndrome, suffered progressive deterioration of his cardiac function over the next several years due to idiopathic cardiomyopathy that ultimately necessitated orthotopic heart transplantation 4 years after index aortic operation. At the time of transplantation, his entire aorta had significantly increased in size and total arch replacement (stage I elephant trunk) was performed concomitantly with orthotopic heart transplantation [9]. After recovery from transplantation and stage I repair, he underwent successful second stage repair of the chronically dissected and aneurysmal thoracoabdominal aorta, resulting in total cardio-aortic replacement.…”

Cardiovascular Magnetic Resonance in Marfan syndrome

“…Such an approach should only be considered if the risks of open repair are prohibitive [57,78]. Aortic surgery has also been successfully combined with cardiac transplantation or left ventricular assist device implantation in patients with MFS [79,80]. …”

Type B aortic dissection triggered by heart transplantation in a patient with Marfan syndrome

“…As shown in our case, open surgery of the thoracoabdominal aorta, even in a setting after heart transplantation, is a high-risk but feasible strategy 18 20Learning points

Heart failure is an established cardiovascular feature of Marfan syndrome and may require cardiac transplantation in rare cases.

Aortic complications are frequent after heart transplantation in patients with Marfan syndrome.

Not only rapid progressive dilation of (chronic) aortic dissection but also new-onset aortic dissection may be encountered after heart transplantation in patients with Marfan syndrome, despite adequate blood-pressure control.

Open surgical repair for thoracoabdominal aortic dissection after heart transplantation is a feasible strategy.

Patient's perspective

From very early on after the heart transplant, I felt invincible.

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