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Article: Ten-Year Epidemiological Review of In-Hospital Patients with Marfan Syndrome

TitleTen-Year Epidemiological Review of In-Hospital Patients with Marfan Syndrome
Authors
Issue Date2008
PublisherElsevier Inc
Citation
Annals Of Vascular Surgery, 2008, v. 22 n. 5, p. 608-612 How to Cite?
AbstractMarfan syndrome is a connective tissue disorder with a worldwide prevalence of 1 in 5,000, without any racial predilection. Major cardiovascular manifestations of Marfan disease often require surgical intervention. The aim of this study was to examine the demographics of patients with Marfan syndrome admitted to Hong Kong hospitals over a 10-year period from 1997 to 2006. We retrospectively reviewed the prospectively collected Hong Kong Health Authority's Clinical Data Analysis and Reporting System and Clinical Medical System. Statistical analysis was performed using SPSS, version 15. A total of 525 patients with Marfan syndrome (310 male, 215 female) were included in this study. For males, mean age at first hospital admission was 19.8 years (range 0-78) and for females, 18.7 years (range 0-60). One hundred and twelve (21.3%) patients (56 male, 56 female) had documented aortoiliac aneurysms and/or dissection, with 74 (66.7%) cases involving the thoracic aorta. Forty-nine (9.3%) patients had major cardiac or aortoiliac operations, with an operative mortality of 5/49 (10.2%). Thirty-seven (7.0%) patients (23 male, 14 female) died during this period, with a mean age at death of 41.0 years (range 0-83) for males and 29.9 years (range 0-59) for females. The majority of these patients died of cardiovascular causes, with four aortic dissections, two ruptured aneurysms, seven with sudden collapse and cardiac arrest, and five with heart failure. In addition, there were other causes of mortality: five perioperative, one congenital, and four pulmonary causes, three with malignancy and one of stroke. The cause of death was unknown in five patients. Patients with aortoiliac diseases have a statistically significant higher mortality rate (p < 0.05). This population-based study shows that significant numbers of patients with Marfan syndrome are admitted to hospital per year, with a significant proportion requiring admissions at a young age. Life span in Marfan patients is markedly shortened, and aortoiliac disease is probably underdiagnosed. A standardized diagnostic and therapeutic follow-up program should be offered to these patients and their families. © 2008 Annals of Vascular Surgery Inc.
Persistent Identifierhttp://hdl.handle.net/10722/59972
ISSN
2015 Impact Factor: 1.045
2015 SCImago Journal Rankings: 0.586
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorChan, YCen_HK
dc.contributor.authorTing, CWen_HK
dc.contributor.authorHo, Pen_HK
dc.contributor.authorPoon, JTen_HK
dc.contributor.authorCheung, GCen_HK
dc.contributor.authorCheng, SWen_HK
dc.date.accessioned2010-05-31T04:01:10Z-
dc.date.available2010-05-31T04:01:10Z-
dc.date.issued2008en_HK
dc.identifier.citationAnnals Of Vascular Surgery, 2008, v. 22 n. 5, p. 608-612en_HK
dc.identifier.issn0890-5096en_HK
dc.identifier.urihttp://hdl.handle.net/10722/59972-
dc.description.abstractMarfan syndrome is a connective tissue disorder with a worldwide prevalence of 1 in 5,000, without any racial predilection. Major cardiovascular manifestations of Marfan disease often require surgical intervention. The aim of this study was to examine the demographics of patients with Marfan syndrome admitted to Hong Kong hospitals over a 10-year period from 1997 to 2006. We retrospectively reviewed the prospectively collected Hong Kong Health Authority's Clinical Data Analysis and Reporting System and Clinical Medical System. Statistical analysis was performed using SPSS, version 15. A total of 525 patients with Marfan syndrome (310 male, 215 female) were included in this study. For males, mean age at first hospital admission was 19.8 years (range 0-78) and for females, 18.7 years (range 0-60). One hundred and twelve (21.3%) patients (56 male, 56 female) had documented aortoiliac aneurysms and/or dissection, with 74 (66.7%) cases involving the thoracic aorta. Forty-nine (9.3%) patients had major cardiac or aortoiliac operations, with an operative mortality of 5/49 (10.2%). Thirty-seven (7.0%) patients (23 male, 14 female) died during this period, with a mean age at death of 41.0 years (range 0-83) for males and 29.9 years (range 0-59) for females. The majority of these patients died of cardiovascular causes, with four aortic dissections, two ruptured aneurysms, seven with sudden collapse and cardiac arrest, and five with heart failure. In addition, there were other causes of mortality: five perioperative, one congenital, and four pulmonary causes, three with malignancy and one of stroke. The cause of death was unknown in five patients. Patients with aortoiliac diseases have a statistically significant higher mortality rate (p < 0.05). This population-based study shows that significant numbers of patients with Marfan syndrome are admitted to hospital per year, with a significant proportion requiring admissions at a young age. Life span in Marfan patients is markedly shortened, and aortoiliac disease is probably underdiagnosed. A standardized diagnostic and therapeutic follow-up program should be offered to these patients and their families. © 2008 Annals of Vascular Surgery Inc.en_HK
dc.languageengen_HK
dc.publisherElsevier Incen_HK
dc.relation.ispartofAnnals of Vascular Surgeryen_HK
dc.rightsAnnals of Vascular Surgery. Copyright © Elsevier Inc.en_HK
dc.subject.meshAdolescenten_HK
dc.subject.meshAdulten_HK
dc.subject.meshAge Factorsen_HK
dc.subject.meshAgeden_HK
dc.subject.meshAorta - surgeryen_HK
dc.subject.meshCardiac Surgical Procedures - mortalityen_HK
dc.subject.meshCardiovascular Diseases - epidemiology - etiology - mortality - surgeryen_HK
dc.subject.meshChilden_HK
dc.subject.meshChild, Preschoolen_HK
dc.subject.meshFemaleen_HK
dc.subject.meshHong Kong - epidemiologyen_HK
dc.subject.meshHumansen_HK
dc.subject.meshInfanten_HK
dc.subject.meshInfant, Newbornen_HK
dc.subject.meshInpatients - statistics & numerical dataen_HK
dc.subject.meshKaplan-Meier Estimateen_HK
dc.subject.meshLongevityen_HK
dc.subject.meshMaleen_HK
dc.subject.meshMarfan Syndrome - complications - epidemiology - mortality - surgeryen_HK
dc.subject.meshMiddle Ageden_HK
dc.subject.meshRetrospective Studiesen_HK
dc.subject.meshTime Factorsen_HK
dc.subject.meshTreatment Outcomeen_HK
dc.subject.meshVascular Surgical Procedures - mortalityen_HK
dc.titleTen-Year Epidemiological Review of In-Hospital Patients with Marfan Syndromeen_HK
dc.typeArticleen_HK
dc.identifier.openurlhttp://library.hku.hk:4550/resserv?sid=HKU:IR&issn=0890-5096&volume=22&issue=5&spage=608&epage=612&date=2008&atitle=Ten-year+epidemiological+review+of+in-hospital+patients+with+marfan+syndromeen_HK
dc.identifier.emailChan, YC: ycchan88@hkucc.hku.hken_HK
dc.identifier.emailPoon, JT: tcjensen@hkucc.hku.hken_HK
dc.identifier.emailCheng, SW: wkcheng@hkucc.hku.hken_HK
dc.identifier.authorityChan, YC=rp00530en_HK
dc.identifier.authorityPoon, JT=rp01603en_HK
dc.identifier.authorityCheng, SW=rp00374en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1016/j.avsg.2008.04.005en_HK
dc.identifier.pmid18562163-
dc.identifier.scopuseid_2-s2.0-50149107560en_HK
dc.identifier.hkuros151673en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-50149107560&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume22en_HK
dc.identifier.issue5en_HK
dc.identifier.spage608en_HK
dc.identifier.epage612en_HK
dc.identifier.eissn1615-5947-
dc.identifier.isiWOS:000258837700002-
dc.publisher.placeUnited Statesen_HK
dc.identifier.scopusauthoridChan, YC=27170769400en_HK
dc.identifier.scopusauthoridTing, CW=24345325900en_HK
dc.identifier.scopusauthoridHo, P=24469553100en_HK
dc.identifier.scopusauthoridPoon, JT=7005903722en_HK
dc.identifier.scopusauthoridCheung, GC=15052803300en_HK
dc.identifier.scopusauthoridCheng, SW=7404684779en_HK

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