A Silent and Catastrophic Cardiac Complication in a Marfan Syndrome Patient With Blurred Vision: A Case Report

被引:0
作者
Fesharaki, Mehrdad Jafari [1 ]
Anvari, Shideh [1 ]
Nazhand, Hadi Allahverdi [2 ]
机构
[1] Shahid Beheshti Univ Med Sci, Dept Cardiol, Sch Med, Tehran, Iran
[2] Shahid Beheshti Univ Med Sci, Sch Med, Tehran, Iran
来源
IRANIAN HEART JOURNAL | 2024年 / 25卷 / 01期
关键词
Marfan syndrome; Aortic dissection; Aortic aneurysm; Aortic valve regurgitation; THORACIC AORTIC-ANEURYSM; RETINAL-DETACHMENT; GHENT NOSOLOGY; MANIFESTATIONS; ROOT; DILATATION; DISSECTION; DILATION; SURGERY; DISEASE;
D O I
暂无
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Marfan syndrome is an autosomal dominant genetic disorder owing to insufficient fibrillin-1 that involves connective tissue, with an incidence rate of approximately 2-3/10000 in most populations. Almost 25% of cases are caused by new mutations and are sporadic. Marfan syndrome leads to not only skeletal and ocular complications (eg, ectopia lentis and retinal detachment) but also cardiac complications, which are the most significant. Critical cardiovascular complications that can occur include mitral valve prolapse, mitral regurgitation, aortic regurgitation, aortic aneurysms, dilation of the sinus of Valsalva, aortic dissection, and rupture. The diagnosis of Marfan syndrome is based on the Ghent nosology. Since cardiac manifestations are life-threatening, they need to be diagnosed and treated promptly. The treatment of cardiac complications comprises surgical and pharmacological therapy. Here, we introduce an asymptomatic case of Marfan syndrome with blurred vision and severe cardiac manifestations discovered during cardiac assessments before eye surgery.
引用
收藏
页码:112 / 117
页数:6
相关论文
共 32 条
  • [1] Abboud EB, 1998, RETINA-J RET VIT DIS, V18, P405
  • [2] Baumgartner FJ, 1997, ANN THORAC SURG, V64, P1871
  • [3] AORTIC ROOT DILATATION AND MITRAL-VALVE PROLAPSE IN MARFANS-SYNDROME - ECHOCARDIOGRAPHIC STUDY
    BROWN, OR
    DEMOTS, H
    KLOSTER, FE
    ROBERTS, A
    MENASHE, VD
    BEALS, RK
    [J]. CIRCULATION, 1975, 52 (04) : 651 - 657
  • [4] Child A.H., 2016, Diagnosis and Management of Marfan Syndrome
  • [5] Non-cardiac manifestations of Marfan syndrome
    Child, Anne H.
    [J]. ANNALS OF CARDIOTHORACIC SURGERY, 2017, 6 (06) : 599 - 609
  • [6] Mechanical and pharmacological approaches to investigate the pathogenesis of Marfan syndrome in the abdominal aorta
    Chung, Ada W. Y.
    Yang, H. H. Clarice
    Yeung, Karen Au
    van Breemen, Cornelis
    [J]. JOURNAL OF VASCULAR RESEARCH, 2008, 45 (04) : 314 - 322
  • [7] Yearly rupture or dissection rates for thoracic aortic aneurysms: Simple prediction based on size
    Davies, RR
    Goldstein, LJ
    Coady, MA
    Tittle, SL
    Rizzo, JA
    Kopf, GS
    Elefteriades, JA
    [J]. ANNALS OF THORACIC SURGERY, 2002, 73 (01) : 17 - 27
  • [8] Primary impairment of left ventricular function in Marfan syndrome
    De Backer, Julie F.
    Devos, Daniel
    Segers, Patrick
    Matthys, Dirk
    Francois, Katrien
    Gillebert, Thierry C.
    De Paepe, Anne M.
    De Sutter, Johan
    [J]. INTERNATIONAL JOURNAL OF CARDIOLOGY, 2006, 112 (03) : 353 - 358
  • [9] Cardiovascular manifestations in men and women carrying a FBN1 mutation
    Detaint, Delphine
    Faivre, Laurence
    Collod-Beroud, Gwenaelle
    Child, Anne H.
    Loeys, Bart L.
    Binquet, Christine
    Gautier, Elodie
    Arbustini, Eloisa
    Mayer, Karin
    Arslan-Kirchner, Mine
    Stheneur, Chantal
    Halliday, Dorothy
    Beroud, Christophe
    Bonithon-Kopp, Claire
    Claustres, Mireille
    Plauchu, Henri
    Robinson, Peter N.
    Kiotsekoglou, Anatoli
    De Backer, Julie
    Ades, Lesley
    Francke, Uta
    De Paepe, Anne
    Boileau, Catherine
    Jondeau, Guillaume
    [J]. EUROPEAN HEART JOURNAL, 2010, 31 (18) : 2223 - 2229
  • [10] Thoracic aortic aneurysm: Reading the enemy's playbook
    Elefteriades, John A.
    [J]. CURRENT PROBLEMS IN CARDIOLOGY, 2008, 33 (05) : 203 - 277