Eisenmenger sendromlu hastalarin ilk başvuru anindaki klinik ve hemodinamik özellikleri: Tersiyer merkez deneyimi.

Objective: In this study, patients admitted with the diagnosis of Eisenmenger syndrome (ES) in a tertiary referral center were analyzed. Methods: 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. Results: 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. Conclusion: 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. [ABSTRACT FROM AUTHOR]