Congenital heart disease
Identifying High Risk in Adults With Congenital Heart Disease and Atrial Arrhythmias

https://doi.org/10.1016/j.amjcard.2011.04.021Get rights and content

Atrial arrhythmias are associated with an increased mortality risk in adults with congenital heart disease (CHD). However, little is known about risk stratification in the specific group of adult patients with CHD and atrial arrhythmias. We sought to identify predictors of mortality in adult with CHD and atrial arrhythmias and to establish a risk score. The study involved 378 adult patients with CHD (mean age 39 ± 13 years) and atrial arrhythmias who had serial follow-up in a tertiary referral center from 1999 through 2009. During a median follow-up of 5.2 years, there were 40 deaths (11%). Overall mortality rate was 2.0% per patient-year. Common modes of death included heart failure–related death (35%), sudden cardiac death (20%), and perioperative death (18%). Independent predictors of mortality were poor functional class (hazard ratio 3.69, 95% confidence interval [CI] 1.69 to 8.03, p = 0.001), single-ventricle physiology (hazard ratio 3.33, 95% CI 1.51 to 7.35, p = 0.003), pulmonary hypertension (hazard ratio 2.96, 95% CI 1.41 to 6.19, p = 0.004), and valvular heart disease (hazard ratio 2.73, 95% CI 1.33 to 5.59, p = 0.006). A risk score was constructed using these predictors in which patients were assigned 1 point for the presence of each risk factor. Mortality rates in the low-risk (no risk factor), moderate-risk (1 risk factor), and high-risk (>1 risk factor) groups were 0.5%, 1.9%, and 6.5% per patient-year, respectively (log-rank p <0.001). In conclusion, in adult with CHD and atrial arrhythmias specific clinical variables identify patients at high risk for death. Importantly, the absence of any of these risk factors is associated with an excellent survival despite the presence of atrial arrhythmias.

Section snippets

Methods

All adult patients with CHD and atrial arrhythmia who were followed serially from January 1999 through December 2009 in the Toronto Congenital Cardiac Centre for Adults were included. We chose these dates to minimize missing data and provide contemporary data. Patients were identified by a search of the institutional database. The study group included all patients with a clinical presentation of documented sustained intra-atrial reentrant tachycardia or atrial fibrillation. Patients who had

Results

In total 378 adult patients with CHD and sustained atrial arrhythmias were included. Baseline characteristics including types of arrhythmias are presented in Table 1. Of all study patients 212 patients (56%) had a history of emergency room visit or hospital admission for sustained atrial arrhythmia. Two hundred fifty-one patients (66%) had paroxysmal atrial arrhythmias; the remainder (n = 127, 34%) had persistent/permanent atrial arrhythmias. Of patients with paroxysmal atrial arrhythmia most

Discussion

The present study provides an estimate of the mortality risk in adult patients with CHD and atrial arrhythmias. In this heterogenous group of adults we found that mortality rates were not uniform. Adults with functional limitation and specific high-risk congenital cardiac lesions (i.e., valvular heart disease, single-ventricle physiology, and pulmonary hypertension) were at increased risk of death. Based on those variables, a risk score was derived to help identify the high-risk adult patient

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