Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome

• DOI: 10.1161/circulationaha.116.023033
• Publication Date: 2016-12-16T03:15:43Z
• Authors: Aleksander Kempny; Cristel Sørensen ...; Hong Gu; Wei Li; Alexander R. Opot...; Michael J. Landzberg; Annette Schophuus...; Lars Søndergaard; Mette-Elise Estensen; Ulf Thilén; Werner Budts; Barbara J. Mulder; Ilja Blok; Lidia Tomkiewicz-...; Kamil Szostek; Michele D’Alto; Giancarlo Scognam...; Katja Prokšelj; Gerhard-Paul Diller; Konstantinos Dimo...; Stephen J. Wort; Michael A. Gatzoulis
• Keywords: Eisenmenger syndrome

Eisenmenger syndrome is associated with substantial morbidity and mortality. There no consensus, however, on mortality risk stratification. We aimed to investigate survival predictors of death in a large, contemporary cohort patients.In multicenter approach, we identified adults under follow-up between 2000 2015. examined its association clinical, electrocardiographic, echocardiographic, laboratory parameters.We studied 1098 patients (median age, 34.4 years; range, 16.1-84.4 65.1% female; 31.9% Down syndrome). The majority had posttricuspid defect (n=643, 58.6%), followed by complex (n=315, 28.7%) pretricuspid lesion (n=140, 12.7%). Over median 3.1 years (interquartile 1.4-5.9), allowing for 4361.6 patient-years observation, 278 died 6 underwent transplantation. Twelve parameters emerged as significant univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 95% confidence interval [CI], 1.24-1.59; P<0.001), shunt (HR, 1.56; CI, 1.02-2.39; P=0.041), oxygen saturation at rest 0.53/10%; 0.43-0.65; presence sinus rhythm 0.53; 0.32-0.88; P=0.013), pericardial effusion 2.41; 1.59-3.66; P<0.001) remained death.There premature among syndrome. report, herewith, stratification model based 5 simple, noninvasive this population.