Clinical and hemodynamic features of Eisenmenger syndrome patients at the time of first admission: a tertiary referral-center experience

Abstract

In this study, patients admitted with the diagnosis of Eisenmenger syndrome (ES) in a tertiary referral center were analyzed. The data of 20 consecutive patients (mean age: 27.6+1.8 years, 7 male and mean follow-up time: 35.6 ± 9.1 months) with ES were retrospectively analyzed. Demographic characteristics, symptoms, physical examination, laboratory and hemodynamic parameters were analyzed at the time of first admission. The most frequent underlying heart diseases were ventricular septal defect (VSD) with complex congenital disease (n:8, 40%) and isolated VSD (n:7, 35%). 6-minute walking test distance was 347.9 ± 33.7 meters and 15 patients (75%) had a functional capacity of NYHA Class III, at the time of admission. ES was diagnosed with catheterization in all patients and mean systolic pulmonary arterial pressure measured by catheterization was 112 ± 6.8 mmHg. Pulmonary function tests, FVC (forced vital capacity), FEV1 (forced expiratory volume), FEV1/FVC values were respectively, 3.1 ± 0.4, 2.5 ± 0.4 L and 76.7 ± 3.3%. Metabolic tests were performed in all patients at the first visit. Mean VO2 max was 16.7 ± 1.0 ml / kg/min and VE/VCO2 rate was 53.9 ± 3.2%. Although PH and partial pressure of carbon dioxide levels were within normal range in blood gas analysis, oxygen saturation and partial pressure of oxygen levels were low. The most common underlying heart disease of ES patients is VSD. In this cases exercise capacity is restricted and this restriction is reflected in laboratory parameters.