Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study
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Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome : A Multicenter Study. / Kempny, Aleksander; Hjortshøj, Cristel Sørensen; Gu, Hong; Li, Wei; Opotowsky, Alexander R; Landzberg, Michael J; Jensen, Annette Schophuus; Søndergaard, Lars; Estensen, Mette-Elise; Thilén, Ulf; Budts, Werner; Mulder, Barbara J; Blok, Ilja; Tomkiewicz-Pająk, Lidia; Szostek, Kamil; D'Alto, Michele; Scognamiglio, Giancarlo; Prokšelj, Katja; Diller, Gerhard-Paul; Dimopoulos, Konstantinos; Wort, Stephen J; Gatzoulis, Michael A.
I: Circulation, Bind 135, Nr. 15, 2017, s. 1432-1440.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome
T2 - A Multicenter Study
AU - Kempny, Aleksander
AU - Hjortshøj, Cristel Sørensen
AU - Gu, Hong
AU - Li, Wei
AU - Opotowsky, Alexander R
AU - Landzberg, Michael J
AU - Jensen, Annette Schophuus
AU - Søndergaard, Lars
AU - Estensen, Mette-Elise
AU - Thilén, Ulf
AU - Budts, Werner
AU - Mulder, Barbara J
AU - Blok, Ilja
AU - Tomkiewicz-Pająk, Lidia
AU - Szostek, Kamil
AU - D'Alto, Michele
AU - Scognamiglio, Giancarlo
AU - Prokšelj, Katja
AU - Diller, Gerhard-Paul
AU - Dimopoulos, Konstantinos
AU - Wort, Stephen J
AU - Gatzoulis, Michael A
N1 - © 2016 American Heart Association, Inc.
PY - 2017
Y1 - 2017
N2 - BACKGROUND: Eisenmenger syndrome 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 Eisenmenger syndrome patients.METHODS: In a multicenter approach, we identified adults with Eisenmenger syndrome 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, 34.4 years; range, 16.1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59; P<0.001), pretricuspid 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 among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.
AB - BACKGROUND: Eisenmenger syndrome 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 Eisenmenger syndrome patients.METHODS: In a multicenter approach, we identified adults with Eisenmenger syndrome 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, 34.4 years; range, 16.1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59; P<0.001), pretricuspid 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 among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.
KW - Adolescent
KW - Adult
KW - Aged
KW - Aged, 80 and over
KW - Biomarkers
KW - Echocardiography
KW - Eisenmenger Complex/diagnosis
KW - Electrocardiography
KW - Female
KW - Follow-Up Studies
KW - Humans
KW - Male
KW - Middle Aged
KW - Oxygen Consumption
KW - Phenotype
KW - Prognosis
KW - Proportional Hazards Models
KW - Risk Assessment
KW - Risk Factors
KW - Severity of Illness Index
KW - Walk Test
KW - Young Adult
U2 - 10.1161/CIRCULATIONAHA.116.023033
DO - 10.1161/CIRCULATIONAHA.116.023033
M3 - Journal article
C2 - 27979875
VL - 135
SP - 1432
EP - 1440
JO - Circulation
JF - Circulation
SN - 0009-7322
IS - 15
ER -
ID: 196142900