ABSTRACT
Pulmonary hypertension is a group of diseases, including pulmonary arterial hypertension associated with congenital heart disease (APAH-CHD), characterized by progressive deterioration in pulmonary hemodynamics associated with substantial morbidity and mortality risk. THALES is a national multicenter, prospective observational registry, providing data on patients with APAH-CHD. The study comprised APAH-CHD patients (>3 months of age) with confirmed diagnosis of right heart catheterization or echocardiographic findings. Initial and follow-up data were collected via regular hospital visits. Descriptive statistics are used for definitive purposes. Overall, 1034 patients aged 3 months-79 years (median 11.2 [Q1-Q3: 2.2-24.3] years) with APAH-CHD were enrolled at 61 centers, 50.3% being retrospectively enrolled. Most had either Eisenmenger's syndrome (49.2%) or systemic-to-pulmonary shunts (42.7%). Patients were mostly in functional class I-II at the time of diagnosis (46.6%). Mean 6-min walk distance (6MWD) was 369 ± 120 m. Mean pulmonary arterial pressure was 54.7 ± 22.2 mmHg for the whole group, and was highest in patients with Eisenmenger's syndrome. Targeted therapies were noted in 398 (38.5%) patients (monotherapy in 80.4%). Follow-up data were available in 506 patients. Survival at 140 months was 79% and was associated with baseline 6MWD >440 m (p = 0.009), brain natriuretic peptide level < 300 ng/L (p < 0.001). Follow-up 6MWD >165 m (p < 0.0001), brain natriuretic peptide level <300 ng/L (p = 0.031), and targeted therapies (p = 0.004) were also predictive of survival. THALES is the largest registry dedicated to APAH-CHD to date and provides important contributions on demographics, clinical characteristics, and gaps in disease management.
ABSTRACT
Atrial fibrillation (AF) is a very common cardiac arrhythmia with an increased mortality in patients with heart failure. Whether the best therapeutic approach to these patients is to restore sinus rhythm or to adequately control the ventricular rate is still controversial. The aim of this study was to compare both strategies in patients with AF and nonischemic heart failure. One hundred and fifty-four patients with AF duration greater than 48 hours and nonischemic left ventricular dysfunction were randomized either to a rhythm (n = 84) or rate (n = 74) control group. The composite end points of the study were embolism, death, and exercise capacity. The average age of the patients was 61 +/- 10 years in the rhythm control group and 58 +/- 12 years in the rate control group (P = NS). The average follow-up period was 35 +/- 21 months in the rhythm control group and 37 +/- 19 months in the rate control group (P = NS). In the first year of the study, exercise capacity and left ventricular ejection fraction (LVEF) were improved in the rhythm control group compared to the exercise capacity and LVEF of the rate control group (P < 0.0001 and P = 0.0005, respectively). There were no statistically significant differences in the embolic event rate between the two groups (P = NS). The mortality rate, especially for death due to pump failure, was significantly higher in the rate control group at the end of the study (P < 0.0001). Restoring and maintaining sinus rhythm had a beneficial effect on mortality and exercise capacity in patients with nonischemic heart failure and AF.
