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Marfan syndrome. Part 2: treatment and management of patients

Nature Reviews Cardiology volume 7, pages 266–276 (2010)Cite this article

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Abstract

Aortic disease is the main cause of death among patients with Marfan syndrome. Before the development of open surgery, most patients died in the fourth decade of life. Improvements in surgical techniques have facilitated prophylactic surgery and have dramatically changed the life expectancy of patients with Marfan syndrome. Valve-sparing techniques are becoming the standard surgical treatment for these patients, since the operative and long-term results are comparable with those obtained with the Bentall and De Bono procedure and their theoretical advantages over the Bentall and De Bono procedure are attractive for young patients. Distal aortic complications still cause substantial morbidity in patients who have undergone surgery. On the other hand, several medical approaches have appeared as alternatives or adjuncts to the standard treatment with β-blockers. Mouse models of the disease have shown that the angiotensin II receptor blocker losartan can rescue the phenotype. Among female patients, pregnancy deserves special consideration. Aortic dissection occurs mainly in the third trimester of gestation and in patients with dilated aortas. As aortic dissection carries a high risk of maternal mortality and fetal demise, prophylactic aortic surgery is recommended before attempting pregnancy for those women with an aortic diameter exceeding 40 mm.

Key Points

  • Prognosis in Marfan syndrome is mainly determined by aortic complications

  • Prophylactic aortic surgery has dramatically changed the prognosis of Marfan syndrome in the past few decades

  • Aortic root replacement is indicated if aortic diameter exceeds 50 mm; however, a lower threshold is often considered

  • Despite the lack of solid evidence, β-blockers are the standard medical treatment for patients with Marfan syndrome

  • Angiotensin II receptor blockers have emerged as a potentially better alternative to β-blockers for patients with Marfan syndrome

  • Pregnancy represents a challenge for female patients with Marfan syndrome, with the most feared complication being the risk of aortic dissection

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Figure 1: Possible pathogenic targets for medical treatment of patients with Marfan syndrome.
Figure 2: Prophylatic surgical techniques used in patients with Marfan syndrome.

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Authors and Affiliations

  1. Instituto Cardiovascular, Hospital Clínico San Carlos, C/Profesor Martín Lagos, sn, 28024, Madrid, Spain

    Victoria Cañadas & Isidre Vilacosta

  2. Servicio de Ginecología y Obstetricia, Hospital Universitario Madrid-Montepríncipe, Avenida de Montepríncipe, 25, Boadilla del Monte, 28660, Madrid, Spain

    Isidoro Bruna

  3. The Zena and Michael A. Wiener Cardiovascular Institute, Mount Sinai Hospital, 1190 Fifth Avenue, New York, 10029, NY, USA

    Valentin Fuster

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Correspondence to Victoria Cañadas.

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Cañadas, V., Vilacosta, I., Bruna, I. et al. Marfan syndrome. Part 2: treatment and management of patients. Nat Rev Cardiol 7, 266–276 (2010). https://doi.org/10.1038/nrcardio.2010.31

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