B-Type Natriuretic Peptide Concentrations in Contemporary Eisenmenger Syndrome Patients: Predictive Value and Response to Disease Targeting Therapy

Study Questions:

What is the relationship between elevated levels of B-type natriuretic peptide (BNP) and outcome in adults with Eisenmenger syndrome?


A retrospective review was performed at a single center. Patients enrolled in the study had undergone measurement of BNP concentrations as part of routine clinical care. Correlation between patient demographics, clinical data, exercise measures, and BNP were studied.


A total of 181 patients (mean age 36.9 ± 12.1 years) were studied, of which 31% had Down syndrome. During the median follow-up period of 3.3 years, 20 (11%) patients died. On univariate analysis, higher BNP concentrations were predictive of all-cause mortality. BNP levels >100 pg/ml were associated with a 4.6x increase in mortality. Increasing BNP concentration with time was also predictive of mortality. Treatment with pulmonary vasodilators was associated with significant reduction in BNP concentrations.


The authors concluded that BNP concentrations predict outcome in patients with Eisenmenger syndrome. Disease-targeting therapies may help to reduce BNP concentrations.


Patients with Eisenmenger physiology are a complex and high-risk patient population. This retrospective study assessed the role of BNP as a predictor of risk in patients with Eisenmenger syndrome. This study suggested that elevated BNP is associated with poor outcomes, as well as a decrease in BNP with pulmonary vasodilators. Early studies of pulmonary vasodilator therapy demonstrated an improvement in exercise capacity. Subsequent retrospective studies have suggested a survival benefit as well. This study showed improvement in BNP concentrations for patients started on pulmonary vasodilators (approximately one-half patients on sildenafil and one-half on bosentan or similar agent) during the study period. Although this study was not designed to document a causal relationship between pulmonary vasodilator use and outcomes of decreased BNP or decreased mortality, this study provides additional support for the use of pulmonary vasodilators in adults with Eisenmenger physiology.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Diabetes and Cardiometabolic Disease, Heart Failure and Cardiomyopathies, Prevention, Congenital Heart Disease, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Acute Heart Failure, Heart Failure and Cardiac Biomarkers, Exercise

Keywords: Heart Diseases, Natriuretic Peptides, Down Syndrome, Follow-Up Studies, Eisenmenger Complex, Biological Markers, Demography, Exercise, Heart Failure, Vasodilator Agents

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