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.
基金:
Drs Kempny, Diller, Wort, Dimopoulos, Li, and Gatzoulis, from the Royal Brompton Hospital, have received unrestricted educational or research grants from Bayer UK, Pfizer UK, Actelion UK, Actelion Global, and GSK UK. Furthermore, they have received support from the Pulmonary Hypertension Association (UK) and the British Heart Foundation. Dr Hjortshj, from Rigshospitalet, Copenhagen, has received an educational grant from Actelion Denmark. Drs Opotowsky and Landzberg were supported by the Dunlevie Family Foundation and received grant funding from Actelion.
通讯机构:[1]Imperial Coll, Royal Brompton Hosp, Adult Congenital Heart Ctr, Natl Ctr Pulm Hypertens, London, England;[13]Royal Brompton Hosp, Sydney St, London SW3 6NP, England
推荐引用方式(GB/T 7714):
Kempny Aleksander,Hjortshoj Cristel Sorensen,Gu Hong,et al.Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study[J].CIRCULATION.2017,135(15):1432-1440.doi:10.1161/CIRCULATIONAHA.116.023033.
APA:
Kempny, Aleksander,Hjortshoj, Cristel Sorensen,Gu, Hong,Li, Wei,Opotowsky, Alexander R....&Gatzoulis, Michael A..(2017).Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study.CIRCULATION,135,(15)
MLA:
Kempny, Aleksander,et al."Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study".CIRCULATION 135..15(2017):1432-1440