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
2011 Impact Factor: 1.035
2011 SCImago Journal Rankings: 0.092
DOIhttp://dx.doi.org/10.1016/j.avsg.2008.04.005
ISI Accession Number IDWOS:000258837700002
ReferencesReferences in Scopus
DC Field
Value
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.epage612
dc.identifier.hkuros151673
dc.identifier.isiWOS:000258837700002
dc.identifier.issn0890-5096
2011 Impact Factor: 1.035
2011 SCImago Journal Rankings: 0.092
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
Author Affiliations
  1. The University of Hong Kong