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Total aortic arch replacement with frozen elephant trunk in patients with Marfan syndrome
Erik Beckmann1, Andreas Martens2, Heike Krueger3
1Minneapolis Heart Institute, Minneapolis, MN, USA.
Background:
Marfan syndrome (MFS) is a connective tissue disease which can lead to aortic aneurysm and dissection. The performance outcomes of total aortic arch replacement with frozen elephant trunk (FET) are not well known in these patients. This study summarizes our experience with FET in MFS.
Methods:
Between August 2001 and December 2021, 435 patients underwent FET at Hannover Medical School. Of these, 34 patients had MFS. The mean age was 43.3±11.9 years and 27 (79%) were male. The indication for surgery was aortic aneurysm in 1 (3%), acute aortic dissection in 12 (35%), and chronic aortic dissection in 21 (62%) patients.
Results:
All patients underwent total aortic arch replacement with FET. In addition, the following procedures were performed: conventional aortic root replacement (Bentall operation, n=8), valve-sparing aortic root replacement (David procedure, n=8), coronary artery bypass grafting (n=3), mitral valve surgery (n=2), and tricuspid valve surgery (n=1). Cardiopulmonary bypass (CPB) and aortic cross clamp times were 270±87 and 139±69 minutes, respectively. Postoperatively, there were 2 (6%) disabling strokes, and 0 (0%) patients required permanent dialysis or suffered from permanent paraplegia, respectively. In-hospital mortality was 12% (n=4). The mean follow-up time was 8.4±5.9 years. The 1-, 5-, 10, and 15-year survival rates were 82%, 70%, 70% and 65, respectively. There were 18 (53%) re-interventions on the distal aorta. Mean time to re-intervention was 2.7±3.1 years. The majority of patients underwent open surgical repair (n=14, 77%), while only 4 (22%) had endovascular therapy. The freedom from distal aortic re-intervention at 1-, 5-, 10- and 15 years was 86%, 61%, 55% and 44%, respectively.
Conclusions:
The main indication for FET surgery in MFS is acute or chronic aortic dissection. Despite multiple concomitant procedures, early mortality was relatively low, suggesting that FET is feasible and effective to treat complex aortic pathology in MFS. However, our study showed a high incidence of distal aortic re-interventions, underscoring the progressive nature of the disease and the need for tailored long-term management strategies.
Insights
Frozen elephant trunk (FET) repair is feasible for Marfan syndrome (MFS) aortic dissections, showing acceptable early outcomes. However, high rates of re-intervention highlight the need for lifelong monitoring and management in MFS patients.
Area of Science:
- Cardiovascular Surgery
- Thoracic Surgery
- Genetics and Inherited Diseases
Background:
- Marfan syndrome (MFS) is a genetic connective tissue disorder predisposing to aortic aneurysm and dissection.
- The efficacy of frozen elephant trunk (FET) repair in MFS patients with complex aortic pathology remains incompletely understood.
Purpose of the Study:
- To evaluate the outcomes of total aortic arch replacement using FET in patients with Marfan syndrome.
- To assess the early and long-term results, including survival and re-intervention rates, following FET procedures in this specific patient cohort.
Main Methods:
- Retrospective analysis of 34 Marfan syndrome patients who underwent FET repair between 2001 and 2021.
- Procedures included total aortic arch replacement with FET, with various concomitant aortic root and valve surgeries.
- Data collected on perioperative outcomes, in-hospital mortality, long-term survival, and distal aortic re-interventions.
Main Results:
- The primary indications for FET were acute (35%) and chronic (62%) aortic dissection.
- In-hospital mortality was 12%, with low rates of disabling stroke (6%), permanent dialysis (0%), and paraplegia (0%).
- Long-term survival rates at 1, 5, 10, and 15 years were 82%, 70%, 70%, and 65%, respectively, with a high rate (53%) of distal aortic re-interventions.
Conclusions:
- Frozen elephant trunk repair is a feasible and effective option for managing complex aortic pathologies in Marfan syndrome patients.
- The procedure demonstrates acceptable early mortality and morbidity, supporting its use in this high-risk population.
- A high incidence of late re-interventions necessitates tailored, long-term surveillance and management strategies for Marfan syndrome patients undergoing FET repair.
