BACKGROUND: Optimal management, including timing of surgery, remains debated in Marfan syndrome because of a lack of data on aortic risk associated with this disease. METHODS AND RESULTS: We used our database to evaluate aortic risk associated with standardized care. Patients who fulfilled the international criteria, had not had previous aortic surgery or dissection, and came to our center at least twice were included. Aortic measurements were made with echocardiography (every 2 years); patients were given systematic β-blockade and advice about sports activities. Prophylactic aortic surgery was proposed when the maximal aortic diameter reached 50 mm. Seven hundred thirty-two patients with Marfan syndrome were followed up for a mean of 6.6 years. Five deaths and 2 dissections of the ascending aorta occurred during follow-up. Event rate (death/aortic dissection) was 0.17%/y. Risk rose with increasing aortic diameter measured within 2 years of the event: from 0.09%/y per year (95% confidence interval, 0.00-0.20) when the aortic diameter was <40 mm to 0.3% (95% confidence interval, 0.00-0.71) with diameters of 45 to 49 mm and 1.33% (95% confidence interval, 0.00-3.93) with diameters of 50 to 54 mm. The risk increased 4 times at diameters ≥50 mm. The annual risk dropped below 0.05% when the aortic diameter was <50 mm after exclusion of a neonatal patient, a woman who became pregnant against our recommendation, and a 72-year-old woman with previous myocardial infarction. CONCLUSIONS: Risk of sudden death or aortic dissection remains low in patients with Marfan syndrome and aortic diameter between 45 and 49 mm. Aortic diameter of 50 mm appears to be a reasonable threshold for prophylactic surgery.
BACKGROUND: Optimal management, including timing of surgery, remains debated in Marfan syndrome because of a lack of data on aortic risk associated with this disease. METHODS AND RESULTS: We used our database to evaluate aortic risk associated with standardized care. Patients who fulfilled the international criteria, had not had previous aortic surgery or dissection, and came to our center at least twice were included. Aortic measurements were made with echocardiography (every 2 years); patients were given systematic β-blockade and advice about sports activities. Prophylactic aortic surgery was proposed when the maximal aortic diameter reached 50 mm. Seven hundred thirty-two patients with Marfan syndrome were followed up for a mean of 6.6 years. Five deaths and 2 dissections of the ascending aorta occurred during follow-up. Event rate (death/aortic dissection) was 0.17%/y. Risk rose with increasing aortic diameter measured within 2 years of the event: from 0.09%/y per year (95% confidence interval, 0.00-0.20) when the aortic diameter was <40 mm to 0.3% (95% confidence interval, 0.00-0.71) with diameters of 45 to 49 mm and 1.33% (95% confidence interval, 0.00-3.93) with diameters of 50 to 54 mm. The risk increased 4 times at diameters ≥50 mm. The annual risk dropped below 0.05% when the aortic diameter was <50 mm after exclusion of a neonatal patient, a woman who became pregnant against our recommendation, and a 72-year-old woman with previous myocardial infarction. CONCLUSIONS: Risk of sudden death or aortic dissection remains low in patients with Marfan syndrome and aortic diameter between 45 and 49 mm. Aortic diameter of 50 mm appears to be a reasonable threshold for prophylactic surgery.
Authors: Ellen S Regalado; Dong-chuan Guo; Siddharth Prakash; Tracy A Bensend; Kelly Flynn; Anthony Estrera; Hazim Safi; David Liang; James Hyland; Anne Child; Gavin Arno; Catherine Boileau; Guillaume Jondeau; Alan Braverman; Rocio Moran; Takayuki Morisaki; Hiroko Morisaki; Reed Pyeritz; Joseph Coselli; Scott LeMaire; Dianna M Milewicz Journal: Circ Cardiovasc Genet Date: 2015-03-10
Authors: Federico M Asch; Eugene Yuriditsky; Siddharth K Prakash; Mary J Roman; Jonathan W Weinsaft; Gaby Weissman; Wm Guy Weigold; Shaine A Morris; William J Ravekes; Kathryn W Holmes; Michael Silberbach; Rita K Milewski; Barbara L Kroner; Ryan Whitworth; Kim A Eagle; Richard B Devereux; Neil J Weissman Journal: JACC Cardiovasc Imaging Date: 2016-02-17
Authors: Kristian A Groth; Kirstine Stochholm; Hanne Hove; Kasper Kyhl; Pernille A Gregersen; Niels Vejlstrup; John R Østergaard; Claus H Gravholt; Niels H Andersen Journal: Clin Res Cardiol Date: 2016-08-22 Impact factor: 5.460
Authors: Parmanand Singh; Zaid Almarzooq; Noel C F Codell; Yi Wang; Mary J Roman; Richard B Devereux; Jonathan W Weinsaft Journal: J Thorac Dis Date: 2017-04 Impact factor: 2.895