Predictors of Death in Contemporary Adult Patients with Eisenmenger Syndrome : a Multicentre Study
| Autor: | A. S. Jensen, Lars Søndergaard, Wei Li, Gerhard-Paul Diller, Cristel S. Hjortshøj, Michael J. Landzberg, Alexander R. Opotowsky, Ilja M. Blok, Hong Gu, Stephen J. Wort, Katja Prokšelj, Michael A. Gatzoulis, Ulf Thilén, Konstantinos Dimopoulos, Kamil Szostek, Michele D'Alto, Giancarlo Scognamiglio, Werner Budts, Aleksander Kempny, Mette-Elise Estensen, Lidia Tomkiewicz-Pająk, Barbara J. Mulder |
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| Přispěvatelé: | Cardiology, APH - Personalized Medicine, APH - Aging & Later Life, ACS - Heart failure & arrhythmias |
| Jazyk: | angličtina |
| Rok vydání: | 2017 |
| Předmět: |
Male
Pediatrics Cardiac & Cardiovascular Systems Heart malformation 030204 cardiovascular system & hematology GUIDELINES THERAPY Severity of Illness Index Electrocardiography 0302 clinical medicine Risk Factors pulmonary hypertension Medicine 030212 general & internal medicine Young adult 1102 Cardiorespiratory Medicine and Haematology Aged 80 and over SURVIVAL PROSPECTS DEFECTS Middle Aged Prognosis CONGENITAL HEART-DISEASE PROGNOSTIC VALUE Phenotype Echocardiography Female Cardiology and Cardiovascular Medicine Risk assessment Life Sciences & Biomedicine Adult PULMONARY ARTERIAL-HYPERTENSION medicine.medical_specialty Adolescent INTERNATIONAL SOCIETY Walk Test Risk Assessment 1117 Public Health and Health Services 03 medical and health sciences Young Adult Oxygen Consumption Physiology (medical) Severity of illness MANAGEMENT Humans Intensive care medicine Aged Proportional Hazards Models Science & Technology business.industry Proportional hazards model 1103 Clinical Sciences Eisenmenger Complex medicine.disease mortality Pulmonary hypertension Peripheral Vascular Disease Cardiovascular System & Hematology REGISTRY Eisenmenger syndrome Cardiovascular System & Cardiology business Biomarkers Follow-Up Studies |
| Zdroj: | Circulation, 135(15), 1432-1440. Lippincott Williams and Wilkins |
| ISSN: | 0009-7322 |
| Popis: | 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 P =0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43–0.65; P P =0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59–3.66; P 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. |
| Databáze: | OpenAIRE |
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