Heart surgery in infants with hemophilia.

We describe 2 infants with hemophilia A who had heart surgery under cardiopulmonary bypass with factor VIII replacement therapy, and we recommend a guideline for factor VIII support for cardiac surgery. One child had repair of total anomalous pulmonary venous connection. The second had cardiac catheterization followed by repair of ventricular septal defect and total anomalous pulmonary venous connection.

A case of long QT syndrome associated with familial occurrence of persistent patency of the arterial duct.

We describe an occurrence of long QT syndrome in association with persistent patency of the arterial duct in members of a family. Patency of the arterial duct was diagnosed in family members of 4 successive generations. The index case had long QT syndrome associated with such a patent duct.

New outpatient referrals to a tertiary paediatric cardiac centre: evidence of increasing workload and evolving patterns of referral.

Objectives: To assess the volume and range of diagnosis in new patients referred to paediatric cardiac outpatient clinics.

Methods: Data was collected prospectively, using a proforma completed at all outpatient clinics over a period of three months.

Results: There were 526 new referrals, representing an increase of almost one-fifth compared to 5 years ago.