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.     

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...     

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).     

Fate of bicuspid aortic valves in patients undergoing aortic root repair or replacement for aortic root enlargement

OBJECTIVE: To better understand the outcome of the bicuspid aortic valve (BAV) after valve-sparing root replacement, we evaluated BAV function after its preservation at the time of the ascending aorta (AA) repair. PATIENTS AND METHODS: From January 1, 1994, through March 31, 2002, BAVs were preserved during repair of AA aneurysms in 21 patients (mean +/- SD age, 45+/-12 years; 13 males). We reviewed the clinical and surgical records of these patients for baseline characteristics, operative procedures, and data at latest follow-up. RESULTS: The primary indication for operation was AA enlargement (median diameter, 55 mm; range, 43-65 mm) in 18 patients and severe aortic regurgitation (AR) in 3 patients. Preoperatively, the BAV exhibited mild or moderate stenosis in 5 patients (mean gradient, 25 mm Hg; range, 19-34 mm Hg), moderate AR in 2, and severe AR in 3. Aortic valve repair was performed in 11 patients. Seventeen patients had excision and graft replacement of the enlarged AA, and 4 patients had primary AA repair. There were no perioperative deaths. Moderate or severe AR was relieved in all patients early postoperatively and during follow-up (median, 2.5 years; maximum, 7.6 years). One patient required aortic valve replacement at 4.8 years for AR. An additional patient had recurrent, severe AR but was asymptomatic and has not undergone reoperation. CONCLUSION: Repair of diseased BAVs or preservation of functionally normal BAVs during surgery for AA aneurysms has a low perioperative risk, and early results are encouraging. Additional observation is necessary to determine whether this strategy has real advantages over early aortic valve replacement.