title: Aortic event rate in the Marfan population: a cohort study.
abstract: 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.
  
  
  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: Centre de Référence pour le Syndrome de Marfan et Apparentés, Hôpital Bichat, 75018 Paris, France. guillaume.jondeau@bch.aphp.fr
authors: Guillaume
authors: G
authors: Jondeau
authors: Delphine
authors: D
authors: Detaint
authors: Florence
authors: F
authors: Tubach
authors: Florence
authors: F
authors: Arnoult
authors: Olivier
authors: O
authors: Milleron
authors: Francois
authors: F
authors: Raoux
authors: Gabriel
authors: G
authors: Delorme
authors: Lea
authors: L
authors: Mimoun
authors: Laura
authors: L
authors: Krapf
authors: Dalil
authors: D
authors: Hamroun
authors: Christophe
authors: C
authors: Beroud
authors: Carine
authors: C
authors: Roy
authors: Alec
authors: A
authors: Vahanian
authors: Catherine
authors: C
authors: Boileau
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000784:Aortic Dissection; D001011:Aorta / Q000473:pathology; D001014:Aortic Aneurysm; D001018:Aortic Diseases / Q000453:epidemiology* / Q000209:etiology / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008382:Marfan Syndrome / Q000453:epidemiology* / Q000401:mortality / Q000601:surgery; D008875:Middle Aged; D055815:Young Adult