Outcome late after repair of teratology of Fallot

An overview is given on congenital heart disease in general and on tetralogy of Fallot (TOF) more specifically. Etiology and birth prevalences will be discussed, as well as treatment options and long-term outcome in TOF patients with regard to ventricular function and clinical condition. Since the right ventricle (RV) is the most affected ventricle in patients after TOF repair, more detailed information is given on RV anatomy, physiology, and function. Imaging techniques to assess systolic and diastolic function, in particular magnetic resonance imaging (MRI), wil

Exercise capacity and ventricular function in patients treated for isolated pulmonary valve stenosis or tetralogy of Fallot

BACKGROUND We hypothesized 1) that long-term ventricular outcome and exercise capacity would be better in patients with isolated pulmonary valve stenosis (PS) treated with balloon pulmonary valvuloplasty (BPV) than in patients operated for tetralogy of Fallot (TOF), and 2) that ventricular outcome and exercise capacity would not be different in PS patients and healthy controls. METHODS We included 21 PS patients after BPV (16.2 ± 5.2 years) and 21 patients operated for TOF (16.6 ± 5.6 years), matching them for gender, age at treatment, and age at study. Patients underwent cardiovascular magnetic resonance (CMR) imaging, exercise testing, 12-lead ECG and 24-hour Holter monitoring for assessment of right ventricular (RV) size and function, pulmonary regurgitation (PR), exercise capacity and electrocardiographic status. Healthy controls for CMR imaging and exercise testing were matched for gender and age at study. RESULTS RV volumes and PR percentage were significantly larger in TOF patients than in PS patients; biventricular ejection fraction (EF) was not different. PR was mild in most PS patients. RV end-systolic volume was significantly larger in PS patients than in healthy controls; RVEF was significantly lower. Both patient groups had similar exercise test results. Peak workload and VO(2) max. were significantly lower in PS patients than in healthy controls. CONCLUSIONS Longstanding mild PR in PS patients can lead to an enlarged RV, reduced RV function and reduced exercise capacity. Despite more PR and larger RV volumes in TOF patients, exercise capacity and biventricular function are similar in both patient groups

Safety and observer variability of cardiac magnetic resonance imaging combined with low-dose dobutamine stress-testing in patients with complex congenital heart disease

Item does not contain fulltextBACKGROUND: In patients with complex congenital heart disease (CHD) abnormal ventricular stress responses have been reported with dobutamine stress cardiovascular magnetic resonance (DCMR). These abnormal stress responses are potential indicators of long-term outcome. However, safety and reproducibility of this technique has not been reported in a larger study. The aim of this study was to report our experiences regarding safety and intra-observer and inter-observer variability of low-dose DCMR in complex CHD. METHODS: In 91 patients, 110 low-dose DCMR studies were performed with acquisition of a short axis set at rest, and during dobutamine administration (7.5 mug/kg/min maximum). We assessed biventricular end-diastolic volumes, end-systolic volumes, stroke volumes, ejection fraction and ventricular mass. Intra- and inter-observer variability for all variables was assessed by calculating the coefficient of variation (%), i.e. the standard deviation of the difference divided by the mean of 2 measurements multiplied by 100%. RESULTS: In 3 patients minor side effects occurred (vertigo, headache, and bigeminy). Ten patients experienced an increase in heart rate of >150% from baseline, although well tolerated. For all variables, intra-observer variability was <10% at rest and during stress. At rest, inter-observer variability was 10.5% maximal. With stress-testing, only the variability of biventricular end-systolic volumes (ESV) exceeded 10%. CONCLUSIONS: In patients with complex CHD low-dose DCMR is feasible, and safe. Intra-observer variability is low for rest and stress measurements. Inter-observer variability of biventricular ESV is high with stress-testing. Whether this limits the potential usefulness of DCMR for risk assessment during follow-up has to be assessed

Abnormal right atrial and right ventricular diastolic function relate to impaired clinical condition in patients operated for tetralogy of Fallot

Cardiovascular Aspects of Radiolog

5-year serial follow-up of clinical condition and ventricular function in patients after repair of tetralogy of Fallot

Cardiolog

Ventricular response to dobutamine stress relates to the change in peak oxygen uptake during the 5-year follow-up in young patients with repaired tetralogy of Fallot

Cardiolog

Ventricular response to dobutamine stress cardiac magnetic resonance imaging is associated with adverse outcome during 8-year follow-up in patients with repaired Tetralogy of Fallot

AIMS: The aim of this study was to evaluate the possible value of dobutamine stress cardiac magnetic resonance imaging (CMR) to predict adverse outcome in Tetralogy of Fallot (TOF) patients. METHODS AND RESULTS: In previous prospective multicentre studies, TOF patients underwent low-dose dobutamine stress CMR (7.5 µg/kg/min). Subsequently, during regular-care patient follow-up, patients were assessed for reaching the composite endpoint (cardiac death, arrhythmia-related hospitalization, or cardioversion/ablation, VO2 max ≤65% of predicted). A normal stress response was defined as a decrease in end-systolic volume (ESV) and increase in ejection fraction. The relative parameter change during stress was calculated as relative parameter change = [(parameterstress - parameterrest)/parameterrest] * 100. The predictive value of dobutamine stress CMR for the composite endpoint was determined using time-to-event analyses (Kaplan-Meier) and Cox proportional hazard analysis. We studied 100 patients [67 (67%) male, median age at baseline CMR 17.8 years (interquartile range 13.5-34.0), age at TOF repair 0.9 years (0.6-2.1)]. After a median follow-up of 8.6 years (6.7-14.1), 10 patients reached the composite endpoint. An abnormal stress response (30% vs. 4.4%, P = 0.021) was more frequently observed in composite endpoint patients. Also in endpoint patients, the relative decrease in right ventricular ESV decreased less during stress compared with the patients without an endpoint (-17 ± 15 vs. -26 ± 13 %, P = 0.045). Multivariable analyses identified an abnormal stress response (hazard ratio 10.4; 95% confidence interval 2.5-43.7; P = 0.001) as predictor for the composite endpoint. CONCLUSION: An abnormal ventricular response to dobutamine stress is associated with adverse outcome in patients with repaired TOF