Predictors of death in contemporary adult patients with Eisenmenger syndrome: a multicentre study
File(s)MUSES_paper_v_22_Nov_2015 post JW KDc.doc (2.61 MB)
Accepted version
Author(s)
Type
Journal Article
Abstract
BACKGROUND: -Eisenmenger syndrome (ES) is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of ES patients. METHODS: -We identified in a multicentre approach adults with ES under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic and laboratory parameters. RESULTS: -We studied 1098 patients (median age 34.4years, range 16.1-84.4years, 65.1% female, 31.9% with Down syndrome). The majority had a post-tricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pre-tricuspid lesion (n=, 12.7%). Over a median follow-up of 3.1years [IQR 1.4-5.9], allowing for 4361.6 patient-years observation, 278 patients died and six and six underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis only age (HR 1.41/10years, 95%CI 1.24-1.59, P<0.001), pre-tricuspid shunt (HR 1.56, 95%CI 1.02-2.39, P=0.041), oxygen saturation at rest (HR 0.53/10%, 95%CI 0.43-0.65, P<0.001), presence of sinus rhythm (HR 0.53, 95%CI 0.32-0.88, P=0.013) and presence of pericardial effusion (HR 2.41, 95%CI 1.59-3.66, P<0.001) remained significant predictors of death. CONCLUSIONS: -There is significant premature mortality amongst contemporary adults with ES. We report, herewith a multivariable mortality risk stratification model based on five simple, non-invasive predictors of death in this population.
Date Issued
2016-12-15
Date Acceptance
2016-12-06
Citation
Circulation, 2016, 135 (9), pp.1432-1440
ISSN
0009-7322
Publisher
American Heart Association
Start Page
1432
End Page
1440
Journal / Book Title
Circulation
Volume
135
Issue
9
Copyright Statement
© 2016 American Heart Association
Identifier
http://www.ncbi.nlm.nih.gov/pubmed/27979875
PII: CIRCULATIONAHA.116.023033
Subjects
Science & Technology
Life Sciences & Biomedicine
Cardiac & Cardiovascular Systems
Peripheral Vascular Disease
Cardiovascular System & Cardiology
Eisenmenger complex
mortality
pulmonary hypertension
CONGENITAL HEART-DISEASE
PULMONARY ARTERIAL-HYPERTENSION
SURVIVAL PROSPECTS
INTERNATIONAL SOCIETY
PROGNOSTIC VALUE
THERAPY
GUIDELINES
MANAGEMENT
REGISTRY
DEFECTS
Eisenmenger complex
mortality
pulmonary hypertension
Adolescent
Adult
Aged
Aged, 80 and over
Biomarkers
Echocardiography
Eisenmenger Complex
Electrocardiography
Female
Follow-Up Studies
Humans
Male
Middle Aged
Oxygen Consumption
Phenotype
Prognosis
Proportional Hazards Models
Risk Assessment
Risk Factors
Severity of Illness Index
Walk Test
Young Adult
Humans
Eisenmenger Complex
Echocardiography
Electrocardiography
Prognosis
Severity of Illness Index
Proportional Hazards Models
Risk Assessment
Risk Factors
Follow-Up Studies
Oxygen Consumption
Phenotype
Adolescent
Adult
Aged
Aged, 80 and over
Middle Aged
Female
Male
Young Adult
Biomarkers
Walk Test
Cardiovascular System & Hematology
1102 Cardiorespiratory Medicine and Haematology
1103 Clinical Sciences
1117 Public Health and Health Services
Publication Status
Published
Coverage Spatial
United States
Date Publish Online
2016-12-15