The characteristic echocardiographic findings for congenital absence of the pulmonary valve with tetralogy of Fallot were described in three patients, whose diagnoses were confirmed by operation and/or autopsy. The abnormal linear echo, which was thought to be derived from the rudimentary pulmonary valve tissue, was recorded anteriorly to the dense echo from the subpulmonary muscle mass. The ec...

A genetic analysis of 126 correlative cases of Fallot's tetralogy has been performed. The isolated cases showed male predominance and a higher risk for the sibs of female cases, suggesting a multifactorial causative mechanism. Dermatoglyphs were typical, with a high number of whorls on the fingers and a tendency to distal t triradius. There were 13 cases belonging to polymalformative syndromes,...

OBJECTIVE The aim of this study was to examine the prognostic significance of ventricular arrhythmia on the ambulatory electrocardiogram (ECG) after repair of tetralogy of Fallot. BACKGROUND Ventricular arrhythmia is common after repair of tetralogy of Fallot and has been proposed as the basis for late sudden death. The prognostic significance of ventricular arrhythmia on ambulatory ECG and t...

BACKGROUND Patients with repaired tetralogy of Fallot experience variable outcomes for reasons that are incompletely understood. We hypothesize that genetic variants contribute to this variability. We sought to investigate the association of 22q11.2 deletion status with clinical outcome in patients with repaired tetralogy of Fallot. METHODS AND RESULTS We performed a cross-sectional study of ...

OBJECTIVE To evaluate the results of pulmonary valve replacement in patients with severe pulmonary regurgitation after tetralogy of Fallot repair in Hong Kong. DESIGN Retrospective review. SETTING University teaching hospital, Hong Kong. PATIENTS Consecutive patients undergoing pulmonary valve replacement after repair of tetralogy of Fallot between August 2002 and December 2008. MAIN OU...

OBJECTIVES We have compared mortality and risk factors for late deaths in patients with tetralogy of Fallot undergoing surgical repair in 1972 and 1982 in a Japanese multicentric study, examining in particular the impact of time of repair. BACKGROUND There is limited information on the effect that time of repair, and our constantly changing approach to it, has on late outcome in repaired tetr...

BACKGROUND Although aortic root pathology has been described in patients with tetralogy of Fallot, the scope of the problem remains poorly defined. We sought to determine the prevalence and predictors of aortic root dilatation in adults with repaired tetralogy of Fallot. METHODS AND RESULTS A multicenter cross-sectional study was conducted with standardized reassessment of echocardiographic p...

OBJECTIVE To examine autonomic function as assessed by heart rate variability in patients 10 or more years after repair of tetralogy of Fallot, and to relate this to cardiac structure, function, and electrocardiographic indices. METHODS Heart rate variability was measured by standard time domain techniques on a 24 hour Holter ECG in 28 patients, aged 12 to 34 years (mean 19.5), who had underg...

5. Dushane JW, Kirklin JW: Late results of the repair of ventricular septal defect on pulmonary vascular disease. In Advances in Cardiovascular Surgery, edited by Kirklin JW: New York, Grune and Stratton, 1973, p 9 6. Jarmakani MM, Graham TP Jr, Canent RV Jr., Spach MS, Capp MP: Effect of site of shunt on left heart volume characteristics in children with ventricular septal defect and patent du...

BACKGROUND Congenital heart disease (CHD) is the most common human birth defect, and clinicians need to understand the anatomy to effectively care for patients with CHD. However, standard two-dimensional (2D) display methods do not adequately carry the critical spatial information to reflect CHD anatomy. Three-dimensional (3D) models may be useful in improving the understanding of CHD, without ...