Exercise capacity and biventricular function in adult patients with repaired tetralogy of Fallot.

BACKGROUND: Adult patients with repaired tetralogy of Fallot (rTOF) often have diminished exercise capacity. The primary objective of this study was to examine whether abnormalities of biventricular function play a role in exercise limitation in patients with rTOF. METHODS: This was a retrospective review of 99 adult patients with rTOF. Right ventricular (RV) and left ventricular (LV) function were assessed echocardiographically using the myocardial performance index (MPI). Maximal oxygen consumption (VO(2) Max) was measured during a level 1 cardiopulmonary exercise test. RESULTS: The mean age of the cohort was 34 +/- 11 years (50% females). Although most of the patients reported good functional capacity, the peak Vo(2)max was decreased at 22 +/- 6 mL/kg per minute (66% +/- 13% predicted Vo(2)max for age and sex). The mean RV and LV MPI were 0.30 +/- 0.07 and 0.42 +/- 0.09, respectively. In the multivariate model, higher RV MPI (P = .04) and LV MPI (P = .005) values, representing impaired ventricular function, were associated with diminished Vo(2)max. There was a significant correlation between the RV and LV MPI (r = 0.54, P = .001). CONCLUSIONS: Impairment of RV and LV function, as measured by MPI, is associated with diminished exercise capacity in patients with repaired tetralogy of Fallot. Furthermore, there is a linear relationship between the RV and LV function suggesting that ventricular interactions are contributing to the limited exercise capacity in this group of patients. Strategies aimed at preserving biventricular function or improving adverse ventricular interactions could help to improve functional capacity in these patients.

Cardiac outcomes after pregnancy in women with congenital heart disease.

OBJECTIVE: Women with congenital heart disease (CHD) are at risk for adverse cardiac events during pregnancy; however, the risk of events late after pregnancy (late cardiac events; LCE) has not been well studied. A study was undertaken to examine the frequency and determinants of LCE in a large cohort of women with CHD. DESIGN: Baseline characteristics and pregnancy were prospectively recorded. LCE (>6months after delivery) were determined by chart review. Survival analysis was used to determine the risk factors for LCE. SETTING: A tertiary care referral hospital. PATIENTS: The outcomes of 405 pregnancies were studied (318 women; median follow-up 2.6 years). MAIN OUTCOME MEASURES: LCE included cardiac death/arrest, pulmonary oedema, arrhythmia or stroke. RESULTS: LCE occurred after 12% (50/405) of pregnancies. The 5-year rate of LCE was higher in women with adverse cardiac events during pregnancy than in those without (27±9% vs 15±3%, HR 2.2, p=0.02). Women at highest risk for LCE were those with functional limitations/cyanosis (HR 3.9, 95% CI 1.2 to 13.0), subaortic ventricular dysfunction (HR 3.0, 95% CI 1.4 to 6.6), subpulmonary ventricular dysfunction and/or significant pulmonary regurgitation (HR 3.2, 95% CI 1.6 to 6.6), left heart obstruction (HR 2.6, 95% CI 1.2 to 5.2) and cardiac events before or during pregnancy (HR 2.6, 95% CI 1.3 to 4.9). In women with 0, 1 or >1 risk predictors the 5-year rate of LCE was 7±2%, 23±5% and 44±10%, respectively (p<0.001). CONCLUSIONS: In women with CHD, pre-pregnancy maternal characteristics can help to identify women at increased risk for LCE. Adverse cardiac events during pregnancy are important and are associated with an increased risk of LCE.

Mid-term outcomes in adults with ebstein anomaly and cavopulmonary shunts.

BACKGROUND: In patients with Ebstein anomaly and poorly functioning right ventricles, a cavopulmonary shunt (CPS) can be created to reduce the preload on the right ventricle. The purpose of this study was to examine the early and mid-term outcomes in adults with Ebstein anomaly who have undergone tricuspid valve repair or replacement with or without a concomitant CPS. METHODS: We examined the outcomes of 40 consecutive patients seen at our center with Ebstein anomaly who had undergone tricuspid valve repair or replacement with (n = 23) or without (n = 17) concomitant CPS. Follow-up data were obtained by either chart review or contacting the referring cardiologist. Mid-term survival was examined using Kaplan-Meier curves. RESULTS: The mean age at surgery was similar in patients with and without CPS (42 +/- 12 versus 39 +/- 19 years; p = 0.63). There were 2 early postoperative deaths owing to refractory right-sided heart failure. Mid-term follow-up data were available in 95% of patients. The mean follow-up time was 6.7 +/- 4.8 years. Patients who received a CPS more commonly had preoperative heart failure or cyanosis (p = 0.04) and had worse preoperative functional status (p = 0.09). In both groups, arrhythmias were the most common late complication. There were 5 late deaths, 3 of which occurred in patients with CPS. Five-year survival with or without CPS was comparable (83% +/- 9% versus 86% +/- 10%; p = 0.85). CONCLUSIONS: Adolescent and adult patients with Ebstein anomaly undergoing tricuspid valve replacement or repair and concomitant CPS are at risk for early and mid-term complications. However, Ebstein surgery along with CPS appears to be a reasonable surgical strategy in patients not thought to be suitable for tricuspid valve surgery alone.

Pregnancy outcomes in women with dilated cardiomyopathy.

OBJECTIVES: The objectives of this study were to determine adverse outcomes during pregnancy in women with dilated cardiomyopathy (DCM) and to compare their cardiac outcomes with those of nonpregnant women with DCM. BACKGROUND: Women with DCM are at risk for complications during pregnancy, but few studies have examined outcomes in this specific population. METHODS: This was a substudy of a larger prospective cohort study of outcomes in women with heart disease. Maternal cardiac, obstetric, and fetal outcomes in pregnancy in women with DCM were examined. For comparison, cardiac outcomes in nonpregnant women with DCM (n = 18) matched by age and left ventricular (LV) systolic function were examined. A matched-pair survival analysis was used to compare groups. RESULTS: Thirty-six pregnancies in 32 women with DCM were included. Thirty-nine percent (14 of 36) of the pregnancies were complicated by at least 1 maternal cardiac event. In the multivariate analysis, moderate or severe LV dysfunction and/or New York Heart Association functional class III or IV (p = 0.003) were the main determinants of adverse maternal cardiac outcomes during pregnancy. In the subset of women with moderate/severe LV dysfunction, 16-month event-free survival was worse in pregnant women compared with nonpregnant women (28 +/- 11% vs. 83 +/- 10%, p = 0.02). The adverse neonatal event rate was highest among women with obstetric and cardiac risk factors (43%). CONCLUSIONS: In pregnant women with DCM the risk of adverse cardiac events is considerable, and pre-pregnancy characteristics can identify women at the highest risk. Pregnancy seems to have a short-term negative impact on the clinical course in women with DCM.