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Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome

2016; Lippincott Williams & Wilkins; Volume: 135; Issue: 15 Linguagem: Inglês

10.1161/circulationaha.116.023033

1524-4539

Aleksander Kempny, Cristel Sørensen Hjortshøj, Hong Gu, Wei Li, Alexander R. Opotowsky, Michael J. Landzberg, Annette Schophuus Jensen, Lars Søndergaard, Mette‐Elise Estensen, Ulf Thilén, Werner Budts, Barbara J.M. Mulder, Ilja M. Blok, Lidia Tomkiewicz‐Pająk, Kamil Szostek, Michele D’Alto, Giancarlo Scognamiglio, Katja Prokšelj, Gerhard‐Paul Diller, Konstantinos Dimopoulos, Stephen J. Wort, Michael Α. Gatzoulis,

Congenital Heart Disease Studies

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.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.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.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.