Echocardiographic Predictors of Outcome in Eisenmenger Syndrome

Study Questions:

What is the relationship between the echocardiographic characteristics of patients with Eisenmenger syndrome and mortality?

Methods:

A longitudinal study was performed at a single tertiary center. Clinical and echocardiographic variables were assessed in 181 consecutive patients with Eisenmenger syndrome. Patients with complex disease, such as those with single-ventricle physiology and systemic right ventricles (RVs), were excluded from the study. The primary outcome measure was death.

Results:

The mean age of participants was 39.1 ± 12.8 years. A majority of patients (67.4%) were in functional class III or higher, mean oxygen saturation was 85.1 ± 7.8%, 40.9% of patients were on advanced medical therapies, and median B-type natriuretic peptide (BNP) was 55.4 ng/L. Over a median follow-up period of 16.4 months, 19 patients died. The strongest predictors of mortality were tricuspid annular plane systolic excursion (TAPSE), myocardial performance (expressed as total isovolumic time and systolic to diastolic duration ratio) and elevated central venous pressure (expressed as right atrial [RA] area, RA pressure, and RA to left atrial area ratio). A composite score including TAPSE <15 mm, RV effective systolic to diastolic duration ratio ≥1.5, and RA area ≥was predictive of clinical outcome. Addition of BNP and resting saturations to the model did not improve the score’s predictive value.

Conclusions:

The authors concluded that echocardiographic parameters of RV function and RA area predict mortality in Eisenmenger patients.

Perspective:

As patients with Eisenmenger physiology may remain stable for years with a disease course with a different natural history than other types of pulmonary hypertension, prognostication can be difficult. Recent studies have shown the prognostic valve of serum BNP as well as the echocardiographic parameter TAPSE. This study reinforces the role for TAPSE and includes the additional echocardiographic parameters of myocardial performance and estimated central venous pressure. As it can be difficult to determine the optimal timing for initiation of pulmonary vasodilators, it is hoped that a better understanding of prognosis can help to determine timing and optimization of medical therapy in this complex patient population.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Pulmonary Hypertension and Venous Thromboembolism, Congenital Heart Disease, CHD & Pediatrics and Prevention, CHD & Pediatrics and Quality Improvement, Heart Failure and Cardiac Biomarkers, Pulmonary Hypertension

Keywords: Outcome Assessment (Health Care), Tricuspid Valve, Ventricular Function, Right, Eisenmenger Complex, Heart Defects, Congenital, Cardiac Catheterization, Hypertension, Pulmonary, Central Venous Pressure, Heart Ventricles, Vasodilator Agents, Natriuretic Peptide, Brain


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