Successful Percutaneous Ablation of Ventricular Tachycardia in Congenitally Corrected Transposition of the Great Arteries, a Case Report

Ventricular tachycardia (VT) is rarely recognized in patients with congenitally corrected transposition of the great arteries (CCTGA). We describe a 48-year-old woman with CCTGA, systemic atrioventricular valve replacement for Ebsteinoid malformation of the valve, ventricular dysfunction and ventricular tachycardia related to the previous surgical scar. The patient had successful non-contact mapping and radiofrequency ablation of the offending tachycardia substrate.     

Angiotensin-converting enzyme inhibitors in adults after the Mustard procedure

Angiotensin-converting enzyme inhibitors had no significant effect on cardiopulmonary exercise function in 14 patients who had undergone a Mustard operation for transposition of the great arteries. In some patients aerobic capacity improved and maximum systolic blood pressure decreased.     

Outcomes of pregnancy in women with tetralogy of Fallot

OBJECTIVES: We sought to determine pregnancy outcomes in patients with tetralogy of Fallot (TOF). BACKGROUND: Pregnancy outcomes in patients with TOF are incompletely defined. METHODS: Clinical, hemodynamic, and obstetric data were reviewed for women with TOF and prior pregnancy. RESULTS: Of 72 respondents, 43 (mean age, 26 years) had 112 pregnancies (range, 1 to 5); 82 pregnancies were successful. Eight women had unrepaired TOF at the time of their 20 successful pregnancies. At first assessment (age > or =18 years), six patients had pulmonary hypertension, three had moderate or severe right ventricular (RV) systolic dysfunction, and 13 had severe RV dilation due to pulmonic regurgitation. Sixteen patients had 30 miscarriages (27%) and one term stillbirth. Mean overall birth weight was 3.2 kg (range, 2.1 to 4.2 kg). Unrepaired TOF (p = 0.05) and morphologic pulmonary artery abnormality (p = 0.03) were independently predictive of infant birth weight. Six patients had cardiovascular complications during pregnancy: supraventricular tachycardia in two, heart failure in two, pulmonary embolism in a patient with pulmonary hypertension, and progressive RV dilation in a patient with severe pulmonic regurgitation. Five infants (6%) had congenital anomalies. CONCLUSIONS: Patients with TOF have an increased risk of fetal loss, and their offspring are more likely to have congenital anomalies than offspring in the general population. Adverse maternal events, although rare, may be associated with left ventricular dysfunction, severe pulmonary hypertension, and severe pulmonic regurgitation with RV dysfunction.     

Atrial function in the Fontan circulation: comparison with invasively assessed systemic ventricular filling pressure

The International Journal of Cardiovascular Imaging - Abnormal atrial mechanics in biventricular circulations have been associated with elevated left heart filling pressures. Similar associations...     

Illness understanding in children and adolescents with heart disease

AIMS—To evaluate illness knowledge and understanding in children and adolescents with congenital and acquired heart disease, and whether the degree of understanding is related to age, sex, or complexity of the heart disease.
DESIGN—Prospective cohort study.
SETTING—Tertiary paediatric cardiac centre.
METHODS—Patients' understanding of their congenital heart disease was assessed in a representative sample of volunteers aged between 7-18 years using semistructured interviews based upon Leventhal's illness representation model.
RESULTS—63 of 69 interviews were suitable for analysis. There were similar numbers of boys and girls and a wide distribution of heart defects. Only 30% of patients had a good understanding of their illness; 77% did not know the medical name of their condition, and 33% had a wrong or poor understanding of their illness. Understanding was unrelated to age, sex, or the nature of the heart disease. Understanding of illness duration was significantly related to age, but not to sex or to the nature of the disease.
CONCLUSIONS—Illness understanding is poor in children and adolescents with heart disease, and many have an entirely wrong concept of their disease. Intensified efforts to ensure better patient and parental understanding are needed.


Keywords: understanding illness; children; heart disease     

A hybrid form of cardiac resynchronisation therapy in patients with failing systemic right ventricles

INTRODUCTION: Late systemic right ventricular (RV) dysfunction after atrial redirection surgery is common. Patients may require cardiac transplantation in early adulthood. METHODS: We undertook cardiac resynchronisation (CRT)/defibrillator therapy in two patients as a bridge to transplantation. RESULTS: Two males (aged 24, 110 kg and 26 years, 106 kg); having undergone a Mustard procedure for dextro-transposition of the great arteries at 7 and 6 months of age respectively, presented with impaired systemic RV function and New York Heart Association III symptoms. Both patients had dual chamber pacemakers in-situ for sinus bradycardia. Upgrade to CRT was performed by conserving the existing endocardial leads and placement of epicardial electrodes. One demonstrated sustained improvement over a 24 month follow-up period. CONCLUSION: A hybrid CRT strategy is feasible in patients with failing systemic RVs and pre-existent endocardial dual chamber pacemakers. Appropriate patient selection criteria and optimum lead placement, however, still needs further evaluation in this population.     

Aerobic capacity in adults with tetralogy of Fallot

BACKGROUND: We investigated the aerobic capacity of 168 adult patients who had undergone successful surgical repair of retralogy of Fallot at the University of Toronto Congenital Cardiac Centre for Adults. METHODS: We compared values of peak uptake of oxygen, peak heart rate, forced vital capacity, and forced expiratory volume in 1 second to predicted values for their age groups. RESULTS: The patients who had undergone surgical repair of tetralogy of Fallot demonstrated an overall diminished peak uptake of oxygen, at 51%,and peak heart rate, at 79%, compared to predicted values. No difference in peak aerobic capacity was found according to the initial surgical strategy of palliation or repair. CONCLUSIONS: Adult patients who have undergone surgical repair of tetralogy of Fallot have lower peak uptake of oxygen, and peak heart rate, compared to predicted values. The reduction in the peak heart rate may affect their exercise capacity. The peak uptake of oxygen also decreased with increasing age at the time of testing, and the age at surgical repair.     

Determinants of outcome in isolated ventricular noncompaction in childhood

Isolated ventricular noncompaction is now recognized as an important cause of pediatric cardiomyopathy; however, predictors of outcome have yet to be detailed. Echocardiographic features at presentation, including increased noncompacted to compacted segment ratio and enlarged left ventricular dimension, were found to be associated with poor outcomes (heart transplantation, transplant listing, or death).