Clinical features of adult patients with Eisenmenger syndrome associated with different types of congenital heart disease

Abstract

To explore the clinical features and hemodynamics of adult patients with Eisenmenger syndrome in different types of congenital heart diseases (CHD). Patients with Eisenmenger syndrome with different types of CHD diagnosed by right heart catheterization were enrolled from 31 clinical centers in China during the period from May 2007 to October 2010. Age, gender, body mass index (BMI), symptoms and signs, World Health Organization functional class (WHO-FC) of pulmonary hypertension, six-minute walk distance (6MWD) and hemodynamics were recorded. All the above indices were analyzed and compared. A total of 224 patients with Eisenmenger syndrome with 3 kinds of CHD were enrolled, including atrial septal defect (n = 67), ventricular septal defect (n = 104) and patent ductus arteriosus (n = 53). Among them, there were 67 males (29.9%) and 157 females (70.1%) with a mean age of (29.6 ± 9.9) years (range: 15-63). Mean BMI was (19.9 ± 4.0) kg/m(2) and mean 6MWD (371 ± 75) m. The majority of patients were in WHO-FC II (n = 158, 70.5%) and III (n = 64, 28.6%). Electrocardiogram of 77.2% of them indicated hypertrophic right ventricle. Mean right atrial pressure was (8.9 ± 5.7) mm Hg (1 mm Hg = 0.133 kPa), mean pulmonary arterial pressure (mPAP) (77.2 ± 19.1) mm Hg, cardiac index (3.03 ± 1.35) L·min(-1) · m(-2) and pulmonary vascular resistance (PVR) (1621 ± 887) dyn · s · cm(-5). The majority of patients with Eisenmenger syndrome with different types of CHD are young females and ventricular septal defect is the most frequent underlying cause. The deterioration of heart function in patients with Eisenmenger syndrome is non-parallel to mPAP and PVR in CHD.