Hybrid Repair of Type B Aortic Dissection With Thoracoabdominal Aortic Aneurysmal Degeneration in the Setting of Marfan Syndrome

Aru, Roberto G.; Richie, Cheryl D.; Badia, D.; Romesberg, Amanda M; Sheppard, Mary B.; Minion, David J.; Tyagi, Suresh C.

doi:10.1177/1538574420988279

Cited by 4 publications

(4 citation statements)

References 12 publications

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“…Open surgical approaches might be preferred to patients with MFS mainly because of the complex and extensive involvement of the aorta (with the chance of also requiring the intervention of the aortic valve), the increased risk for endoleaks and endoprosthesis migration, and the young age of the patients that may face a lifetime risk of aortic complications [15]. However, endovascular and hybrid approaches have been reported in the literature [13,14,[20][21][22]. In our case series, 88% of the procedures were performed with open approaches.…”

Section: Discussionmentioning

confidence: 99%

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Challenges in prompt identification and surgical correction of Marfan Syndrome aortic disease in a middle-income country: a case series study

Velandia-Sánchez,

Polanía-Sandoval,

Senosiain-González

et al. 2024

J Cardiothorac Surg

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Background Marfan Syndrome is an autosomal dominant disease caused by pathogenetic variants in the FBN1 gene. The progressive dilatation of the aorta and the potential risk of acute aortic syndromes influence the prognosis of these patients. We aim to describe population characteristics, long-term survival, and re-intervention patterns in patients who underwent aortic surgery with a previously confirmed clinical diagnosis of Marfan Syndrome in a middle-income country. Methods A retrospective single-center case series study was conducted. All Marfan Syndrome patients who underwent aortic procedures from 2004 until 2021 were included. Qualitative variables were frequency-presented, while quantitative ones adopted mean ± standard deviation. A subgroup analysis between elective and emergent procedures was conducted. Kaplan-Meier plots depicted cumulative survival and re-intervention-free. Control appointments and government data tracked out-of-hospital mortality. Results Fifty patients were identified. The mean age was 38.79 ± 14.41 years, with a male-to-female ratio of 2:1. Common comorbidities included aortic valve regurgitation (66%) and hypertension (50%). Aortic aneurysms were observed in 64% without dissection and 36% with dissection. Surgical procedures comprised elective (52%) and emergent cases (48%). The most common surgery performed was the David procedure (64%), and the Bentall procedure (14%). The in-hospital mortality rate was 4%. Complications included stroke (10%), and acute kidney injury (6%). The average follow-up was 8.88 ± 5.78 years. Survival rates at 5, 10, and 15 years were 89%, 73%, and 68%, respectively. Reintervention rates at 1, 2.5, and 5 years were 10%, 14%, and 17%, respectively. The emergent subgroup was younger (37.58 ± 14.49 years), had the largest number of Stanford A aortic dissections, presented hemodynamic instability (41.67%), and had a higher requirement of reinterventions in the first 5 years of follow-up (p = 0.030). Conclusion In our study, surveillance programs played a pivotal role in sustaining high survival rates and identifying re-intervention requirements. However, challenges persist, as 48% of the patients required emergent surgery. Despite not affecting survival rates, a greater requirement for reinterventions was observed, emphasizing the necessity of timely diagnosis. Enhanced educational initiatives for healthcare providers and increased patient involvement in follow-up programs are imperative to address these concerns.

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Section: Discussionmentioning

confidence: 99%

“…Current interventional treatment options include different techniques, including composite valve graft replacement of the aortic valve and ascending aorta (Bentall procedure), VSRR with reimplantation of the coronary arteries (David procedure), and less often, endovascular or hybrid interventions [9,13,14].…”

Section: Introductionmentioning

confidence: 99%

Challenges in prompt identification and surgical correction of Marfan Syndrome aortic disease in a middle-income country: a case series study

Velandia-Sánchez,

Polanía-Sandoval,

Senosiain-González

et al. 2024

J Cardiothorac Surg

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show abstract

“…Open surgical approaches might be preferred to patients with MFS mainly because of the complex and extensive involvement of the aorta (with the chance of also requiring intervention of the aortic valve), the increased risk for endoleaks and endoprosthesis migration, and the young age of the patients that may face a lifetime risk of aortic complications (15). However, endovascular and hybrid approaches have been reported in the literature (13,14,(20)(21)(22). In our case series, 88% of the procedures were performed with open approaches.…”

Section: Discussionmentioning

confidence: 99%

“…Current interventional treatment options include valve-sparing root replacement (VSRR). Which have different techniques, including composite valve graft replacement of the aortic valve and ascending aorta (Bentall procedure), VSRR with reimplantation of the coronary arteries (David procedure), and less often, endovascular or hybrid interventions (9,13,14).…”

Section: Introductionmentioning

confidence: 99%

Challenges in Prompt Identification and Surgical Correction of Marfan Syndrome Aortic Disease in a middle-income Country: A Case Series Study.

Velandia-Sánchez,

Polanía-Sandoval,

Senosiain-González

et al. 2023

Preprint

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Background: Marfan Syndrome is an autosomal dominant disease caused by pathogenetic variants in the FBN1 gene. The progressive dilatation of the aorta and the potential risk of acute aortic syndromes influence the prognosis of these patients. We aim to describe population characteristics, long-term survival, and re-intervention patterns in patients who underwent aortic surgery with a previously confirmed clinical diagnosis of Marfan Syndrome in a middle-income country. Methods: A retrospective single-center case series study was conducted. All Marfan Syndrome patients who underwent aortic procedures from 2004 until 2021 were included. Qualitative variables were frequency-presented, while quantitative ones adopted mean ± standard deviation. A subgroup analysis between elective and emergent procedures was conducted. Kaplan-Meier plots depicted cumulative survival and re-intervention-free. Control appointments and government data tracked out-of-hospital mortality. Results: 50 patients were identified. Mean age was 38.79 ± 14.41 years, with a male-to-female ratio of 2:1. Common comorbidities included aortic valve regurgitation (66%) and hypertension (50%). Aortic aneurysms were observed in 64% without dissection and 36% with dissection. Surgical procedures comprised elective (52%) and emergent cases (48%). The most common surgery performed was valve-sparing root replacement, specifically the David procedure (64%), and the Bentall procedure (14%). The in-hospital mortality rate was 4%. Complications included stroke (10%), and acute kidney injury (6%). The average follow-up was 8.88 ± 5.78 years. Survival rates at 5, 10, and 15 years were 89%, 73%, and 68%, respectively. Reintervention rates at 1, 2.5, and 5 years were 10%, 14%, and 17%, respectively. The emergent subgroup was younger (37.58 ± 14.49 years), had the largest number of Stanford A aortic dissections, presented hemodynamic instability (41.67%), and had a higher requirement of reinterventions in the first 5 years of follow-up (p=0.030). Conclusion: In our study, surveillance programs played a pivotal role in sustaining high survival rates and identifying re-intervention requirements. However, challenges persist, as 48% of the patients required emergent surgery. Despite not affecting survival rates, a greater requirement for reinterventions was observed, emphasizing the necessity of timely diagnosis. Enhanced educational initiatives for healthcare providers and increased patient involvement in follow-up programs are imperative to address these concerns.

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Thoracoabdominal aortic aneurysm in connective tissue disorder patients

Loschi

Rinaldi

Melissano

2022

Indian J Thorac Cardiovasc Surg

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Hybrid Repair of Type B Aortic Dissection With Thoracoabdominal Aortic Aneurysmal Degeneration in the Setting of Marfan Syndrome

Cited by 4 publications

References 12 publications

Challenges in prompt identification and surgical correction of Marfan Syndrome aortic disease in a middle-income country: a case series study

Challenges in prompt identification and surgical correction of Marfan Syndrome aortic disease in a middle-income country: a case series study

Challenges in Prompt Identification and Surgical Correction of Marfan Syndrome Aortic Disease in a middle-income Country: A Case Series Study.

Thoracoabdominal aortic aneurysm in connective tissue disorder patients

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