PMID: 6107779Nov 29, 1980Paper

Management of cardiovascular complications in Marfan syndrome

Lancet
R M DonaldsonD N Ross

Abstract

From 1965 to 1979, 59 patients with the Marfan syndrome and cardiovascular complications underwent aortic root and aortic valve surgery. The hospital mortality was 12% (6.5% over the past 5 years). At follow-up an average of 5.1 years later, 2 patients had postoperative heart block, and late complications required reoperation in 4. There was 8 late deaths (overall mortality 25%). Survival at 6 years was 77% and improvement was noted in the functional class of 32 (74%) of the 43 survivors. 3 patients with abnormal preoperative ventricular function have continued in cardiac failure; 3 have moderate mitral regurgitation and 2 have aortic regurgitation. Aneurysms of the abdominal aorta and pulmonary artery have developed in 2 patients. Initially patients underwent surgery when their condition deteriorated despite medical treatment. Echocardiographic assessment of the aortic root has improved diagnosis and management; a large, progressively dilating aorta and evidence of ventricular dilatation were indications for surgery in 11 symptom-free patients. Although patients with Marfan syndrome are prone to other cardiovascular manifestation which affect survival, elective prophylactic surgery to the ascending aorta can be done with a low...Continue Reading

References

Sep 1, 1975·The American Journal of Cardiology·A T NasrallahR D Leachman
Jan 1, 1975·Pathologia et microbiologia·A E Becker
Jan 1, 1976·Chest·R D SpanglerJ T Okin
Mar 1, 1973·Circulation·C PhornphutkulA S Nadas
Apr 13, 1972·The New England Journal of Medicine·J L MurdochV A McKusick
May 1, 1980·The Annals of Thoracic Surgery·R Gallotti, D N Ross
Apr 1, 1962·The American Journal of Cardiology·C A WAGENVOORTJ E EDWARDS

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