{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T02:04:49Z","timestamp":1789524289744,"version":"build-2803163510"},"reference-count":52,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,1]]},"abstract":"<jats:sec>\n                    <jats:title\/>\n                    <jats:p>Marfan syndrome (MFS), a multisystemic connective disorder, caused by fibrillin 1 gene mutations with autosomal dominant inheritance. The disease spectrum is wide and the major causes of death are related to aortic root aneurysm or dissection. The purposes of medical treatment are to reduce structural changes in the aortic wall and slow aortic root dilatation. Advance in medical researches have provided new insights into the pathogenesis of disease and opened up new horizons for treatments. Several medications such as angiotensin II type I receptor blockers, \u03b2-blockers, angiotensin-converting enzyme inhibitors, calcium channel blockers, tetracyclines, and statins have been studied for the purpose. Currently, the life expectancy of Marfan patients improves significantly and is closes to the general population with proper treatment. In this article, we review and update the medical treatments for patients with MFS.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/tcmj.tcmj_95_20","type":"journal-article","created":{"date-parts":[[2021,9,9]],"date-time":"2021-09-09T08:30:47Z","timestamp":1631176247000},"page":"44-48","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":9,"title":["An update of medical care in Marfan syndrome"],"prefix":"10.4103","volume":"34","author":[{"given":"Hsin-Hui","family":"Chiu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-7-20240828","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1038\/352337a0","article-title":"Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene","volume":"352","author":"Dietz","year":"1991","journal-title":"Nature"},{"key":"R2-7-20240828","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1111\/j.1399-0004.2007.00845.x","article-title":"Utility of molecular analyses in the exploration of extreme intrafamilial variability in the Marfan syndrome","volume":"72","author":"De Backer","year":"2007","journal-title":"Clin Genet"},{"key":"R3-7-20240828","doi-asserted-by":"crossref","first-page":"1965","DOI":"10.1016\/S0140-6736(05)67789-6","article-title":"Marfan's syndrome","volume":"366","author":"Judge","year":"2005","journal-title":"Lancet"},{"key":"R4-7-20240828","doi-asserted-by":"crossref","first-page":"1978","DOI":"10.1016\/S0140-6736(05)66995-4","article-title":"Marfan's syndrome","volume":"366","author":"Ho","year":"2005","journal-title":"Lancet"},{"key":"R5-7-20240828","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1161\/CIRCULATIONAHA.108.783753","article-title":"Report of the National Heart, Lung, and Blood Institute and National Marfan Foundation Working Group on research in Marfan syndrome and related disorders","volume":"118","author":"Pearson","year":"2008","journal-title":"Circulation"},{"key":"R6-7-20240828","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1136\/hrt.2008.160515","article-title":"Marfan syndrome: 30 years of research equals 30 years of additional life expectancy","volume":"95","author":"Pyeritz","year":"2009","journal-title":"Heart"},{"key":"R7-7-20240828","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/S0002-9149(00)80066-1","article-title":"Life expectancy in the Marfan syndrome","volume":"75","author":"Silverman","year":"1995","journal-title":"Am J Cardiol"},{"key":"R8-7-20240828","doi-asserted-by":"crossref","first-page":"804","DOI":"10.1056\/NEJM197204132861502","article-title":"Life expectancy and causes of death in the Marfan syndrome","volume":"286","author":"Murdoch","year":"1972","journal-title":"N Engl J Med"},{"key":"R9-7-20240828","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1038\/nrm2262","article-title":"Extracellular control of TGFbeta signalling in vascular development and disease","volume":"8","author":"Ten Dijke","year":"2007","journal-title":"Nat Rev Mol Cell Biol"},{"key":"R10-7-20240828","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1083\/jcb.200608167","article-title":"Fibrillin-1 regulates the bioavailability of TGFbeta1","volume":"176","author":"Chaudhry","year":"2007","journal-title":"J Cell Biol"},{"key":"R11-7-20240828","doi-asserted-by":"crossref","first-page":"I365","DOI":"10.1161\/CIRCULATIONAHA.105.000810","article-title":"Expression of matrix metalloproteinases and endogenous inhibitors within ascending aortic aneurysms of patients with Marfan syndrome","volume":"114","author":"Ikonomidis","year":"2006","journal-title":"Circulation"},{"key":"R12-7-20240828","doi-asserted-by":"crossref","first-page":"I371","DOI":"10.1161\/CIRCULATIONAHA.105.000927","article-title":"Overexpression of transforming growth factor-beta is associated with increased hyaluronan content and impairment of repair in Marfan syndrome aortic aneurysm","volume":"114","author":"Nataatmadja","year":"2006","journal-title":"Circulation"},{"key":"R13-7-20240828","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1136\/jmg.2009.072785","article-title":"The revised Ghent nosology for the Marfan syndrome","volume":"47","author":"Loeys","year":"2010","journal-title":"J Med Genet"},{"key":"R14-7-20240828","doi-asserted-by":"crossref","first-page":"524","DOI":"10.1002\/humu.22977","article-title":"Marfan syndrome and related disorders: 25 years of gene discovery","volume":"37","author":"Verstraeten","year":"2016","journal-title":"Hum Mutat"},{"key":"R15-7-20240828","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1136\/hrt.2006.098798","article-title":"Marfan syndrome: An update of genetics, medical and surgical management","volume":"93","author":"von Kodolitsch","year":"2007","journal-title":"Heart"},{"key":"R16-7-20240828","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.1056\/NEJM199904293401702","article-title":"Replacement of the aortic root in patients with Marfan's syndrome","volume":"340","author":"Gott","year":"1999","journal-title":"N Engl J Med"},{"key":"R17-7-20240828","doi-asserted-by":"crossref","first-page":"1335","DOI":"10.1056\/NEJM199405123301902","article-title":"Progression of aortic dilatation and the benefit of long-term beta-adrenergic blockade in Marfan's syndrome","volume":"330","author":"Shores","year":"1994","journal-title":"N Engl J Med"},{"key":"R18-7-20240828","doi-asserted-by":"crossref","first-page":"1364","DOI":"10.1016\/S0002-9149(99)00101-0","article-title":"Phenotypic features and impact of beta blocker or calcium antagonist therapy on aortic lumen size in the Marfan syndrome","volume":"83","author":"Rossi-Foulkes","year":"1999","journal-title":"Am J Cardiol"},{"key":"R19-7-20240828","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1016\/0002-9149(94)90762-5","article-title":"Effect of beta-adrenergic blockade on aortic root rate of dilation in the Marfan syndrome","volume":"74","author":"Salim","year":"1994","journal-title":"Am J Cardiol"},{"key":"R20-7-20240828","first-page":"123","article-title":"A prospectus on the prevention of aortic rupture in the Marfan syndrome with data on survivorship without treatment","volume":"129","author":"Halpern","year":"1971","journal-title":"Johns Hopkins Med J"},{"key":"R21-7-20240828","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1016\/S0002-8703(97)70248-5","article-title":"Heterogeneous aortic response to acute beta-adrenergic blockade in Marfan syndrome","volume":"133","author":"Haouzi","year":"1997","journal-title":"Am Heart J"},{"key":"R22-7-20240828","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/j.jpeds.2006.09.003","article-title":"Beta-Blocker therapy does not alter the rate of aortic root dilation in pediatric patients with Marfan syndrome","volume":"150","author":"Selamet Tierney","year":"2007","journal-title":"J Pediatr"},{"key":"R23-7-20240828","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1016\/j.ijcard.2005.11.116","article-title":"The effect of b-blocker therapy on clinical outcome in patients with Marfan's syndrome: A meta-analysis","volume":"114","author":"Gersony","year":"2007","journal-title":"Int J Cardiol"},{"key":"R24-7-20240828","doi-asserted-by":"crossref","first-page":"266","DOI":"10.1038\/nrcardio.2010.31","article-title":"Marfan syndrome. Part 2: Treatment and management of patients","volume":"7","author":"Ca\u00f1adas","year":"2010","journal-title":"Nat Rev Cardiol"},{"key":"R25-7-20240828","doi-asserted-by":"crossref","first-page":"2802","DOI":"10.1161\/CIRCULATIONAHA.107.693523","article-title":"Medical management of Marfan syndrome","volume":"117","author":"Keane","year":"2008","journal-title":"Circulation"},{"key":"R26-7-20240828","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1038\/ng1116","article-title":"Dysregulation of TGF-beta activation contributes to pathogenesis in Marfan syndrome","volume":"33","author":"Neptune","year":"2003","journal-title":"Nat Genet"},{"key":"R27-7-20240828","doi-asserted-by":"crossref","first-page":"2750","DOI":"10.1074\/jbc.M209256200","article-title":"Latent transforming growth factor b-binding protein 1 interacts with fibrillin and is a microfibril-associated protein","volume":"278","author":"Isogai","year":"2003","journal-title":"J Biol Chem"},{"key":"R28-7-20240828","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1126\/science.1124287","article-title":"Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome","volume":"312","author":"Habashi","year":"2006","journal-title":"Science"},{"key":"R29-7-20240828","doi-asserted-by":"crossref","first-page":"2994","DOI":"10.1007\/s10439-016-1616-4","article-title":"Losartan attenuates degradation of aorta and lung tissue micromechanics in a mouse model of severe Marfan syndrome","volume":"44","author":"Lee","year":"2016","journal-title":"Ann Biomed Eng"},{"key":"R30-7-20240828","doi-asserted-by":"crossref","first-page":"2787","DOI":"10.1056\/NEJMoa0706585","article-title":"Angiotensin II blockade and aortic-root dilation in Marfan's syndrome","volume":"358","author":"Brooke","year":"2008","journal-title":"N Engl J Med"},{"key":"R31-7-20240828","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1016\/j.mayocp.2012.11.005","article-title":"Losartan added to \u03b2\u2013blockade therapy for aortic root dilation in Marfan syndrome: A randomized, open-label pilot study","volume":"88","author":"Chiu","year":"2013","journal-title":"Mayo Clin Proc"},{"key":"R32-7-20240828","doi-asserted-by":"crossref","first-page":"3491","DOI":"10.1093\/eurheartj\/eht334","article-title":"Losartan reduces aortic dilatation rate in adults with Marfan syndrome: A randomized controlled trial","volume":"34","author":"Groenink","year":"2013","journal-title":"Eur Heart J"},{"key":"R33-7-20240828","doi-asserted-by":"crossref","first-page":"1477","DOI":"10.1016\/j.amjcard.2013.06.019","article-title":"Usefulness of losartan on the size of the ascending aorta in an unselected cohort of children, adolescents, and young adults with Marfan syndrome","volume":"112","author":"Pees","year":"2013","journal-title":"Am J Cardiol"},{"key":"R34-7-20240828","doi-asserted-by":"crossref","first-page":"2061","DOI":"10.1056\/NEJMoa1404731","article-title":"Pediatric Heart Network Investigators. Atenolol versus losartan in children and young adults with Marfan's syndrome","volume":"371","author":"Lacro","year":"2014","journal-title":"N Engl J Med"},{"key":"R35-7-20240828","doi-asserted-by":"crossref","first-page":"616","DOI":"10.1080\/00015385.2017.1314134","article-title":"Efficacy of losartan as add-on therapy to prevent aortic growth and ventricular dysfunction in patients with Marfan syndrome: A randomized, double-blind clinical trial","volume":"72","author":"Mui\u00f1o-Mosquera","year":"2017","journal-title":"Acta Cardiol"},{"key":"R36-7-20240828","doi-asserted-by":"crossref","first-page":"1613","DOI":"10.1016\/j.jacc.2018.07.052","article-title":"Losartan versus atenolol for prevention of aortic dilation in patients with Marfan syndrome","volume":"72","author":"Teixido-Tura","year":"2018","journal-title":"J Am Coll Cardiol"},{"key":"R37-7-20240828","doi-asserted-by":"crossref","first-page":"2263","DOI":"10.1016\/S0140-6736(19)32518-8","article-title":"Irbesartan in Marfan syndrome (AIMS): A double-blind, placebo-controlled randomised trial","volume":"394","author":"Mullen","year":"2019","journal-title":"Lancet"},{"key":"R38-7-20240828","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1016\/j.jfma.2019.03.018","article-title":"The effects of losartan versus beta-blockers on cardiovascular protection in Marfan syndrome: A systemic review and meta-analysis","volume":"119","author":"Kang","year":"2020","journal-title":"J Formos Med Assoc"},{"key":"R39-7-20240828","doi-asserted-by":"crossref","first-page":"879","DOI":"10.1161\/01.RES.82.8.879","article-title":"Protection of the arterial internal elastic lamina by inhibition of the renin-angiotensin system in the rat","volume":"82","author":"Huang","year":"1998","journal-title":"Circulation Res"},{"key":"R40-7-20240828","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1016\/S0140-6736(06)69250-7","article-title":"Angiotensin-converting enzyme inhibitors and aortic rupture: A population-based case-control study","volume":"368","author":"Hackam","year":"2006","journal-title":"Lancet"},{"key":"R41-7-20240828","doi-asserted-by":"crossref","first-page":"1125","DOI":"10.1016\/j.amjcard.2005.01.032","article-title":"Usefulness of enalapril versus propranolol or atenolol for prevention of aortic dilation in patients with the Marfan syndrome","volume":"95","author":"Yetman","year":"2005","journal-title":"Am J Cardiol"},{"key":"R42-7-20240828","doi-asserted-by":"crossref","first-page":"1539","DOI":"10.1001\/jama.298.13.1539","article-title":"Effect of perindopril on large artery stiffness and aortic root diameter in patients with Marfan syndrome: A randomized controlled trial","volume":"298","author":"Ahimastos","year":"2007","journal-title":"JAMA"},{"key":"R43-7-20240828","doi-asserted-by":"crossref","first-page":"951","DOI":"10.1016\/j.jpeds.2014.07.008","article-title":"\u03b2-Blockers and angiotensin converting enzyme inhibitors: Comparison of effects on aortic growth in pediatric patients with Marfan syndrome","volume":"165","author":"Phomakay","year":"2014","journal-title":"J Pediatr"},{"key":"R44-7-20240828","doi-asserted-by":"crossref","first-page":"891","DOI":"10.1111\/j.1365-2362.2012.02668.x","article-title":"Effects of atenolol, perindopril and verapamil on haemodynamic and vascular function in Marfan syndrome \u2013 A randomised, double-blind, crossover trial","volume":"42","author":"Williams","year":"2012","journal-title":"Eur J Clin Invest"},{"key":"R45-7-20240828","doi-asserted-by":"crossref","first-page":"e08648","DOI":"10.7554\/eLife.08648","article-title":"A deleterious gene-by-environment interaction imposed by calcium channel blockers in Marfan syndrome","volume":"4","author":"Doyle","year":"2015","journal-title":"Elife"},{"key":"R46-7-20240828","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1067\/mva.2002.123680","article-title":"A 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitor, cerivastatin, suppresses production of matrix metalloproteinase-9 in human abdominal aortic aneurysm wall","volume":"36","author":"Nagashima","year":"2002","journal-title":"J Vasc Surg"},{"key":"R47-7-20240828","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1016\/j.ejvs.2005.12.024","article-title":"Statins are associated with a reduced infrarenal abdominal aortic aneurysm growth","volume":"32","author":"Schouten","year":"2006","journal-title":"Eur J Vasc Endovasc Surg"},{"key":"R48-7-20240828","doi-asserted-by":"crossref","first-page":"S168","DOI":"10.1161\/CIRCULATIONAHA.110.012187","article-title":"Pravastatin reduces Marfan aortic dilation","volume":"124","author":"McLoughlin","year":"2011","journal-title":"Circulation"},{"key":"R49-7-20240828","doi-asserted-by":"crossref","first-page":"e008543","DOI":"10.1161\/JAHA.118.008543","article-title":"Statins reduce thoracic aortic aneurysm growth in Marfan syndrome mice via inhibition of the ras-induced ERK (Extracellular Signal-Regulated Kinase) signaling pathway","volume":"7","author":"Sato","year":"2018","journal-title":"J Am Heart Assoc"},{"key":"R50-7-20240828","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1016\/j.jvs.2007.09.016","article-title":"Doxycycline delays aneurysm rupture in a mouse model of Marfan syndrome","volume":"47","author":"Xiong","year":"2008","journal-title":"J Vasc Surg"},{"key":"R51-7-20240828","doi-asserted-by":"crossref","first-page":"e73","DOI":"10.1161\/CIRCRESAHA.108.174367","article-title":"Long-term doxycycline is more effective than atenolol to prevent thoracic aortic aneurysm in marfan syndrome through the inhibition of matrix metalloproteinase-2 and -9","volume":"102","author":"Chung","year":"2008","journal-title":"Circ Res"},{"key":"R52-7-20240828","doi-asserted-by":"crossref","first-page":"606","DOI":"10.1067\/mva.2001.117891","article-title":"Use of doxycycline to decrease the growth rate of abdominal aortic aneurysms: A randomized, double-blind, placebo-controlled pilot study","volume":"34","author":"Mosorin","year":"2001","journal-title":"J Vasc Surg"}],"container-title":["Tzu Chi Medical Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/tcmj.tcmj_95_20","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,14]],"date-time":"2026-08-14T19:34:24Z","timestamp":1786736064000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/tcmj.tcmj_95_20"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,1]]},"references-count":52,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2022]]}},"URL":"https:\/\/doi.org\/10.4103\/tcmj.tcmj_95_20","relation":{},"ISSN":["1016-3190"],"issn-type":[{"value":"1016-3190","type":"print"}],"subject":[],"published":{"date-parts":[[2022,1]]}}}