SEVERE AORTIC INSUFFICIENCY IN MARFAN'S SYNDROME
Authors: John F. Griffin, Gerald M. Koman
Journal: Annals of Internal Medicine (1958)

Case Reports1 January 1958SEVERE AORTIC INSUFFICIENCY IN MARFAN'S SYNDROMEJOHN F. GRIFFIN, M.D., GERALD M. KOMAN, M.D.JOHN F. GRIFFIN, M.D., GERALD M. KOMAN, M.D.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-48-1-174 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptAlthough more than 10 years have elapsed since Baer, Taussig and Oppenheimer1 first drew attention to involvement of the aortic wall as a complication of Marfan's syndrome, relatively few autopsy reports have been published with anatomic descriptions of the defect. Recent pathologic studies reported by McKusick2 and others3, 4 have stressed the variety of cardiovascular lesions that may be associated with this syndrome.Over 50 autopsied cases of Marfan's syndrome have now been reported. In 1951 Marvel reviewed the findings in the 28 autopsied cases reported up to that time.5Table 1 summarizes the significant necropsy findings in 27 autopsied...Bibliography1. BaerTaussigOppenheimer RWHBEH: Congenital aneurysmal dilatation of the aorta associated with arachnodactyly, Bull. Johns Hopkins Hosp. 74: 309-323, 1943. Google Scholar2. McKusick VA: The cardiovascular aspects of Marfan's syndrome: a heritable disorder of connective tissue, Circulation 11: 321-342, 1955. CrossrefMedlineGoogle Scholar3. AndersonPratt-Thomas MHR: Marfan's syndrome, Am. Heart J. 46: 911-917, 1953. CrossrefMedlineGoogle Scholar4. TraismanJohnson HSFR: Arachnodactyly associated with aneurysm of the aorta, Am. J. Dis. Child. 87: 156-166, 1954. Google Scholar5. MarvelGenovese RJPD: Cardiovascular disease in Marfan's syndrome, Am. Heart J. 42: 814-825, 1951. CrossrefMedlineGoogle Scholar6. Rados A: Marfan's syndrome (arachnodactyly coupled with dislocation of lens), Arch. Ophth. 27: 477-538, 1942. CrossrefGoogle Scholar7. WhitfieldArnottStafford AGWMJL: "Myocarditis" and aortic hypoplasia in arachnodactylia, Lancet 1: 1387-1391, 1951. CrossrefMedlineGoogle Scholar8. Moses MF: Aortic lesions associated with arachnodactyly, Brit. M. J. 2: 81-84, 1951. CrossrefMedlineGoogle Scholar9. ThomasBrothersAndersonCuff JGBRSJR: Marfan's syndrome; a report of three cases with aneurysm of the aorta, Am. J. Med. 12: 613-618, 1952. CrossrefMedlineGoogle Scholar10. TungLiebow HLAA: Marfan's syndrome. Observations at necropsy with special reference to medionecrosis of the great vessels, Lab. Invest 1: 382-406, 1952. MedlineGoogle Scholar11. GoyettePalmer EMPW: Cardiovascular lesions in arachnodactyly, Circulation 7: 373-379, 1953. CrossrefMedlineGoogle Scholar12. SloperStorey JCG: Aneurysms of the ascending aorta due to medial degeneration associated with arachnodactyly (Marfan's disease), J. Clin. Path. 6: 299-303, 1953. CrossrefMedlineGoogle Scholar13. Hedinger VC: Herz- und Gefabveranderungen bei Marfanschen Syndrom (Arachnodaktylie), Schweiz. Ztschr. allg. Path. 16: 977-982, 1953. MedlineGoogle Scholar14. MaierRubliSchaubHedinger NCJMFC: Cardiale Storungen bein Marfanschen Syndrom, Cardiologia 24: 106-110, 1954. CrossrefMedlineGoogle Scholar15. ReehLehman MJWL: Marfan's syndrome (arachnodactyly) with ectopia lentis, Tr. Am. Acad. Ophth. 58: 212-216, 1954. MedlineGoogle Scholar16. CoffeyBarkerFriedlander JHDEJH: Dissecting aneurysm with Marfan's syndrome, Texas State J. Med. 51: 79-81, 1955. MedlineGoogle Scholar17. BertrandWeillBurtin IJP: Histopathologie due cerveau et des globes oculaires dans un cas d'arachnodactylie du nourrisson (Maladie de Marfan), Rev. neurol. 92: 137-139, 1955. MedlineGoogle Scholar18. Pygott F: Arachnodactyly (Marfan's syndrome) with a report of two cases, Brit J. Radiol. 28: 26-29, 1955. CrossrefMedlineGoogle Scholar19. Lillian M: Multiple pulmonary artery aneurysms, Am. J. Med. 7: 280-287, 1949. CrossrefMedlineGoogle Scholar20. WhittakerSheehan SRJD: Dissecting aortic aneurysm in Marfan's syndrome, Lancet 2: 791-792, 1954. CrossrefGoogle Scholar21. Fenichel NM: Arteriosclerotic aortic insufficiency, Am. Heart J. 40: 117-124, 1950. CrossrefMedlineGoogle Scholar22. Garvin CF: Functional aortic insufficiency, Am. Heart J. 13: 1799-1803, 1940. Google Scholar23. BeanMohaupt WBFY: Rupture of an aortic valve, J. A. M. A. 150: 92-93, 1952. CrossrefMedlineGoogle Scholar24. SteinbergGeller IW: Aneurysmal dilatation of aortic sinuses in arachnodactyly: diagnosis during life in three cases, Ann. Int. Med. 43: 120-132, 1955. LinkGoogle Scholar25. SchilderHarveyHufnagel DPWPCA: Rheumatoid spondylitis and aortic insufficiency, New England J. Med. 255: 11-17 (July 5) 1956. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: *Received for publication August 6, 1956.From the Department of Medicine (Section of Cardiology), Georgetown University Medical Center, Washington, D. C.Supported in part by grants from the National Heart Institute, National Institutes of Health and the U. S. Public Health Service.†Fellow in Medicine (Cardiology), Public Health Service Trainee, National Heart Institute.‡Resident in Pathology, Georgetown University Medical Center.Requests for reprints should be addressed to John F. Griffin, M.D., 69 Chestnut Street, Springfield, Massachusetts. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byThe spectrum of cardiovascular disease in the Marfan syndrome: A clinico-morphologic study of 18 necropsy patients and comparison to 151 previously reported necropsy patientsMorphological changes of the aorta after administration of Na 2EDTA with special reference to the mucoid medial necrosisCongenital arterial diseasesANNULO-AORTIC ECTASIAAngeborene Herz- und GefäßfehlerUnusual features of Marfan's syndrome including two postmortem studiesThe Marfan SyndromeCLINICAL CONSIDERATIONS AND SURGICAL TREATMENT OF ANNULO-AORTIC ECTASIAThe electrocardiogram in Marfan's syndromeAn Electrocardiographic Pattern Associated with Mitral Valve Deformity in Marfan's SyndromeAngeborene Herz- und GefäßmißbildungenArachnodactyly HeartRupture of an aneurysm of the posterior sinus of valsalva into the right atriumMedionecrosis of the Aorta in Marfan's Syndrome.Marfan's syndrome: A case with complete dissection of the aorta 1 January 1958Volume 48, Issue 1Page: 174-187KeywordsAnatomical pathologyAortaAtrial fibrillationAuscultationAutopsyHeartLesionsMarfan syndromeMedical servicesThorax ePublished: 1 December 2008 Issue Published: 1 January 1958 PDF downloadLoading ...