Consequences of a selective approach toward pulmonary valve replacement in adult patients with tetralogy of Fallot and pulmonary regurgitation.

Objective: The aim of the study was to assess the long-term results of a selective policy toward pulmonary valve replacement in adult patients with repaired tetralogy of Fallot and severe pulmonary regurgitation.

Methods: Sixty-seven patients with tetralogy of Fallot were followed up from 15 +/- 3 years until 27 +/- 3 years after surgery.

Results: Twenty-two patients had mild-to-moderate pulmonary regurgitation.

A new syndrome with noncompaction cardiomyopathy, bradycardia, pulmonary stenosis, atrial septal defect and heterotaxy with suggestive linkage to chromosome 6p.

We report a three-generation family with nine patients affected by a combination of cardiac abnormalities and left isomerism which, to our knowledge, has not been described before. The cardiac anomalies include non-compaction of the ventricular myocardium, bradycardia, pulmonary valve stenosis, and secundum atrial septal defect. The laterality sequence anomalies include left bronchial isomerism, azygous continuation of the inferior vena cava, polysplenia and intestinal malrotation, all compatible with left isomerism.