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Aortic dissection in patients with Marfan syndrome based on the IRAD data

Hector W L de Beaufort1, Santi Trimarchi1, Amit Korach2

  • 1Thoracic Aortic Research Center, Policlinico San Donato IRCCS, University of Milan, San Donato Milanese, Italy.

Annals of Cardiothoracic Surgery
|December 23, 2017
PubMed

Insights

Patients with Marfan syndrome experience acute aortic dissection at a younger age. Despite this, survival rates are comparable for type A, and improved for type B dissections, emphasizing lifelong surveillance needs.

Area of Science:

  • Cardiovascular Medicine
  • Genetics
  • Thoracic Surgery

Background:

  • Marfan syndrome is a genetic disorder affecting connective tissue, predisposing individuals to aortic complications.
  • Acute aortic dissection is a life-threatening condition requiring prompt diagnosis and management.
  • Understanding Marfan syndrome's impact on aortic dissection is crucial for tailored patient care.

Purpose of the Study:

  • To compare the clinical characteristics, outcomes, and survival of patients with and without Marfan syndrome who have acute aortic dissection.
  • To investigate the specific impact of Marfan syndrome on different types of aortic dissection (Type A and Type B).
  • To evaluate the long-term prognosis and reintervention rates in Marfan syndrome patients post-aortic dissection.

Main Methods:

  • Analysis of data from the International Registry on Acute Aortic Dissections (6,424 patients, including 258 with Marfan syndrome).
  • Comparison of demographic data, comorbidities, aortic dimensions, in-hospital mortality, and long-term survival between cohorts.
  • Stratification of analysis by dissection type (Type A and Type B) and treatment modality.

Main Results:

  • Marfan syndrome patients were significantly younger at presentation (38.2 vs. 63.0 years) with fewer comorbidities but more frequent known aortic aneurysms.
  • In-hospital mortality for Type A dissection was similar between groups; however, Type B dissection mortality appeared lower in Marfan syndrome patients.
  • Marfan syndrome patients undergoing open surgery for Type B dissection had 0% mortality, and 5-year survival was 80.1% with a 55.3% reintervention rate.

Conclusions:

  • Marfan syndrome influences the presentation and potentially the outcomes of acute aortic dissection.
  • While Type A dissection outcomes are similar, Type B dissection may have better in-hospital and surgical outcomes in Marfan syndrome patients.
  • High reintervention rates necessitate rigorous long-term surveillance and management strategies for Marfan syndrome survivors of aortic dissection.
Keywords:
Aortic dissectionMarfan syndromeaortic surgeryoperative results

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