No abstract

The clinical, physiologic, and anatomic features of a special group of cases of total anomalous, pulmonary venous connection are reviewed. The occurrence of severe pulmonary venous obstruction in total anomalous pulmonary venous connection produces a characteristic syndrome: 1. Very early onset of dyspnea and heart failure. 2. Considerably more cyanosis than the usual case without obstruction. 3. Typical x-ray showing diffuse hazy lung fields with reticulated appearance without cardiac enlargement. 4. Electrocardiographic evidence of severe right ventricular hypertrophy with a tall R or qR pattern in lead V 1 and reversal of the R/S ratio over the precordium. 5. Physiologic findings of practically normal pulmonary flow, right ventricular and pulmonary arterial pressures greater than systemic, and a marked gradient between the pulmonary arterial "wedge" and right atrial pressures. 6. Rapid deterioration and death in the first weeks or months of life.

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. A sixth patient was a 16 year old girl who died suddenly; several members of her family had a prolonged QT interval. The only common finding in the conduction system in all cases was marked fatty infiltration in the approaches to the atrioventricular (AV) node. In four, the AV bundle was lobulated, with loop formation in one. In four, the AV bundle and bundle branches showed fibrosis. The ventricular myocardium in all cases was chronically inflamed. In two cases, the AV node was partially embedded in the central fibrous body. It is not clear how these changes are related to the disturbance in the repolarization process and the prolongation of the QT interval. However, it is interesting that all patients had an abnormality in the conduction system.