Right Ventricular Function in Patients With Eisenmenger Syndrome

Study Questions:

Is right ventricular (RV) dysfunction, as measured by tricuspid annular plane systolic excursion (TAPSE), associated with worse outcomes in patients with Eisenmenger syndrome?


A prospective, registry-based study was performed. Patients ages >18 years with Eisenmenger syndrome were divided into those with simple atrial septal defects (ASDs), those with ventricular septal defect (VSD) or patent ductus arteriosus (PDA), and those with combined lesions (atrioventricular septal defect [AVSD]). Patients with complex lesions were excluded. Outcomes included all-cause mortality, transplantation, and hospitalization for cardiopulmonary etiologies. RV function was evaluated using TAPSE.


A total of 58 patients (mean age 35.1 ± 13.2 years) were studied, of which 60% had Down syndrome. Of the total cohort, 21 (36%) were receiving specific pulmonary arterial hypertension therapy, including 100% of patients with ASD, 35% of patients with VSD or PDA, and 13% of patients with AVSD. During a mean follow-up of 3.2 years, 9 patients (15.5%) died, 2 (3.4%) underwent transplantation, and 18 (31%) were hospitalized for cardiopulmonary causes. On multivariate analysis, only TAPSE was related to adverse outcomes (hazard ratio, 0.82; 95% confidence interval, 0.708-0.95; p = 0.008). Lower TAPSE was related to the presence of pulmonary artery thrombosis (R = -0.378; p = 0.006).


The authors concluded that in patients with Eisenmenger syndrome, RV dysfunction evaluated using TAPSE is associated with worse outcomes.


Previous studies have demonstrated high risk features for patients with Eisenmenger physiology to include Down syndrome, lower oxygen saturation, history of syncope, iron deficiency anemia, elevated uric acid, poor functional capacity, and history of arrhythmia. This study adds RV function, as measured by TAPSE. Interestingly, the authors did not include a subjective evaluation of RV function by an experienced echocardiographer as a measure in this study. Although this technique has obvious limitations, it is probably the most commonly used means of assessment of RV function. Cardiac magnetic resonance imaging has become an important tool in the assessment of RV function, although it has significant limitations in this patient population. Given the altered AV valve anatomy in patients with AVSD, the validity of TAPSE in the patient population is unknown. This study suggests that decreased RV function as measured by TAPSE can be a helpful tool in the assessment of adults with Eisenmenger physiology as a result of noncomplex lesions.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Congenital Heart Disease, CHD and Pediatrics and Prevention, Pulmonary Hypertension, Hypertension

Keywords: Tricuspid Valve, Down Syndrome, Hypertension, Pulmonary, Ventricular Function, Hypertension, Heart Septal Defects

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