Patients with congenital heart disease often present unique challenges, especially in the electrophysiology laboratory. Here we present a case of a patient with medically refractory symptomatic atrial tachycardia, tricuspid atresia and a history of a modified Fontan procedure. The approach of an AV node ablation for palliation in our patient was met with challenges in identification of a His-bu...

Between 1968 and 1977, 32 patients who underwent the Fontan operation for tricuspid atresia at Bordeaux, France, or Leiden, The Netherlands, survived at least 1 year after surgery. These patients were evaluated clinically 7 to 16 years (mean 8.9 years) after surgery by review of clinical records, questionnaire, or direct examination. There were five deaths: one during arrhythmia, the second sud...

A girl with symmetrical intrauterine growth retardation and a birth weight of 2240 g was delivered at term by primary cesarean section to a 27-year-old primiparous woman. Fetal echocardiography had shown a nearly atretic tricuspid valve. Postnatally, the child stabilized rapidly but showed signs of heart failure with tachypnea and general weakness, as well as cyanosis (transcutaneous oxygen sat...

Nature of the problem Many congenital cardiac malformations result in a heart with only one effective ventricle. In most of these there is a second ventricle present, but it is too small to sustain cardiac output making construction of a biventricular circulation impossible. These hearts may be classified according to the atrioven-tricular connection. There are two possibilities: the atria are ...

Pentalogy of Cantrell is a rare entity of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. The complete pentalogy and its variants have been described in the literature. We report a 47-day-old girl with an incomplete pentalogy of Cantrell presenting with congenital absence of the sternum, congenital heart defects, and an epigastric hernia. Two even rar...

A case of congenital diverticulum of the left ventricle associated with a ventricular septal defect and severe hypoplasia of the right ventricle without tricuspid or pulmonary atresia is described and the relevant literature is reviewed. The distinction between true muscular diverticulae and congenital apical aneurysms is made, the latter term being preferred for similar abnormalities described...