Outcome in Adult Eisenmenger Syndrome Patients

Study Questions:

What are predictors for death in adults with Eisenmenger syndrome (ES)?


A retrospective review of patients >16 years old followed at 11 international adult congenital heart disease (ACHD) clinics was performed. Patients were categorized as having pretricuspid lesions, post-tricuspid lesions, and mixed lesions (combined pre- and post-tricuspid lesions). Data examined included clinical status, oxygen saturation, electrocardiography, transthoracic echocardiography (including tricuspid annular plane systolic excursion [TAPSE]) and presence or absence of pericardial effusion, 6-minute walk, albumin, sodium, B-type natriuretic peptide (BNP), and C-reactive protein (CRP). Survival was calculated using the Kaplan-Meier method, and both univariable and multivariable Cox regression analyses were performed.


A total of 1,098 patients were studied. The median age was 34.4 years (range 16.1-84.4 years), 65.1% of patients were female, and 31.9% had Down syndrome. The majority of patients had post-tricuspid lesions (n = 643, 58.6%), followed by those with complex lesions (n = 315, 28.7%) and pretricuspid lesions (n = 140, 12.8%). Over a median follow-up of 3.1 years [interquartile range, 1.4-5.9], 278 patients died and six underwent transplantation. On univariable analysis, 12 parameters were significant predictors of death, although only five remained significant on multivariable analysis. Predictors of death included age (hazard ratio [HR], 1.41/10 years), pretricuspid shunt (HR, 1.56), oxygen saturation at rest (HR, 0.53/10%), presence of sinus rhythm (HR, 0.53), and presence of pericardial effusion (HR, 2.41).


The authors concluded that there is significant premature mortality in adults with ES, and describe a multivariable mortality risk stratification model based on five noninvasive predictors.


Despite advances in congenital heart surgery resulting in early repair of left-to-right shunt lesions, patients with Eisenmenger syndrome continue to be seen regularly in ACHD clinics, and face significant morbidity and mortality. The patient population in this study appears representative of patients seen in tertiary ACHD centers, with a majority of patients with pretricuspid and complex lesions, and a significant proportion (31.9%) with Down syndrome. Some factors felt to be important on previous studies (including BNP and use of advanced therapies for pulmonary arterial hypertension) were not found to be significant on multivariable analysis. A relatively low use of advanced therapies at the beginning of the study period (36.9%) as compared with the end of the study (62.8%) may have been one of the factors contributing to this finding. The etiology of pericardial effusion and impact on mortality in this patient group remains unclear. Patients with pericardial effusion appeared to have higher CRP levels, suggesting an inflammatory component and need for further study.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Pericardial Disease, Pulmonary Hypertension and Venous Thromboembolism, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, CHD and Pediatrics and Prevention, Heart Failure and Cardiac Biomarkers, Pulmonary Hypertension, Interventions and Imaging, Interventions and Structural Heart Disease, Echocardiography/Ultrasound

Keywords: Cardiac Surgical Procedures, C-Reactive Protein, Down Syndrome, Echocardiography, Eisenmenger Complex, Electrocardiography, Heart Defects, Congenital, Hypertension, Pulmonary, Mortality, Natriuretic Peptide, Brain, Pericardial Effusion, Systole, Tricuspid Valve

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