Mitral atresia with a large left ventricle and an underdeveloped or absent right ventricular sinus: clinical profile, anatomic data and surgical considerations.

In mitral atresia with a large left ventricle, the tricuspid valve is either straddling and biventricular or entirely left ventricular. To learn how to assess the identity of the tricuspid valve in such cases 15 heart specimens were examined as well as the echocardiograms of 10 living patients. When the right ventricular sinus was underdeveloped (11 cases), a straddling tricuspid valve was present; when it was absent (14 cases), the tricuspid valve was entirely left ventricular.

The conduction system in patients with a prolonged QT interval.

The conduction system and the myocardium of five patients with Romano-Ward syndrome and one patient with the Jervell and Lange-Nielsen syndrome were studied to determine the cause of the prolonged QT interval. The patients were 9 and 15 months and 2, 5 and 19 years of age, respectively. All had a prolonged QTc interval.

Friedreich's ataxia with acute cardiomyopathy.

Friedreich's ataxia (FA) is a progressive, spinocerebellar degenerative disease. Onset is generally in the second decade of life, occurring as a neurologic degenerative process. Most, if not all, patients have an associated cardiomyopathy, which is frequently the cause of death.

Conduction system in two cases of sudden death two years after the Mustard procedure.

This report describes a serial section examination of the conduction system in two children who died suddenly 2 years following the Mustard procedure for complete transposition. The first child manifested sinus rhythm alternating with junctional rhythm in the last year of life. The second child, 2 months before death, had first-degree atrioventricular (AV) block which progressed to second-degree block with 2:1 conduction alternating with a junctional rhythm with AV dissociation.