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Article: Ten-Year Epidemiological Review of In-Hospital Patients with Marfan Syndrome
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TitleTen-Year Epidemiological Review of In-Hospital Patients with Marfan Syndrome
 
AuthorsChan, YC1
Ting, CW1
Ho, P1
Poon, JT1
Cheung, GC1
Cheng, SW1
 
Issue Date2008
 
PublisherElsevier Inc
 
CitationAnnals Of Vascular Surgery, 2008, v. 22 n. 5, p. 608-612 [How to Cite?]
DOI: http://dx.doi.org/10.1016/j.avsg.2008.04.005
 
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.
 
ISSN0890-5096
2013 Impact Factor: 1.029
 
DOIhttp://dx.doi.org/10.1016/j.avsg.2008.04.005
 
ISI Accession Number IDWOS:000258837700002
 
ReferencesReferences in Scopus
 
DC FieldValue
dc.contributor.authorChan, YC
 
dc.contributor.authorTing, CW
 
dc.contributor.authorHo, P
 
dc.contributor.authorPoon, JT
 
dc.contributor.authorCheung, GC
 
dc.contributor.authorCheng, SW
 
dc.date.accessioned2010-05-31T04:01:10Z
 
dc.date.available2010-05-31T04:01:10Z
 
dc.date.issued2008
 
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.
 
dc.description.natureLink_to_subscribed_fulltext
 
dc.identifier.citationAnnals Of Vascular Surgery, 2008, v. 22 n. 5, p. 608-612 [How to Cite?]
DOI: http://dx.doi.org/10.1016/j.avsg.2008.04.005
 
dc.identifier.doihttp://dx.doi.org/10.1016/j.avsg.2008.04.005
 
dc.identifier.eissn1615-5947
 
dc.identifier.epage612
 
dc.identifier.hkuros151673
 
dc.identifier.isiWOS:000258837700002
 
dc.identifier.issn0890-5096
2013 Impact Factor: 1.029
 
dc.identifier.issue5
 
dc.identifier.openurl
 
dc.identifier.pmid18562163
 
dc.identifier.scopuseid_2-s2.0-50149107560
 
dc.identifier.spage608
 
dc.identifier.urihttp://hdl.handle.net/10722/59972
 
dc.identifier.volume22
 
dc.languageeng
 
dc.publisherElsevier Inc
 
dc.publisher.placeUnited States
 
dc.relation.ispartofAnnals of Vascular Surgery
 
dc.relation.referencesReferences in Scopus
 
dc.rightsAnnals of Vascular Surgery. Copyright © Elsevier Inc.
 
dc.subject.meshAdolescent
 
dc.subject.meshAdult
 
dc.subject.meshAge Factors
 
dc.subject.meshAged
 
dc.subject.meshAorta - surgery
 
dc.subject.meshCardiac Surgical Procedures - mortality
 
dc.subject.meshCardiovascular Diseases - epidemiology - etiology - mortality - surgery
 
dc.subject.meshChild
 
dc.subject.meshChild, Preschool
 
dc.subject.meshFemale
 
dc.subject.meshHong Kong - epidemiology
 
dc.subject.meshHumans
 
dc.subject.meshInfant
 
dc.subject.meshInfant, Newborn
 
dc.subject.meshInpatients - statistics & numerical data
 
dc.subject.meshKaplan-Meier Estimate
 
dc.subject.meshLongevity
 
dc.subject.meshMale
 
dc.subject.meshMarfan Syndrome - complications - epidemiology - mortality - surgery
 
dc.subject.meshMiddle Aged
 
dc.subject.meshRetrospective Studies
 
dc.subject.meshTime Factors
 
dc.subject.meshTreatment Outcome
 
dc.subject.meshVascular Surgical Procedures - mortality
 
dc.titleTen-Year Epidemiological Review of In-Hospital Patients with Marfan Syndrome
 
dc.typeArticle
 
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Author Affiliations
  1. The University of Hong Kong