It is rare to find congenital cardiac disease in association with familial primary hypertrophic osteoarthropathy. We have now encountered three siblings, two of whom had digital clubbing, patent arterial ducts and delayed closure of the cranial fontanels. The third infant was unusual in that there was no clubbing, or cranial abnormality, despite a small ventricular septal defect. To the best of our knowledge, this association has not previously been observed.

We describe two patients with right isomerism, corrected with a fenestrated Fontan operation, who suffered severe progressive cyanosis. Cardiac catheterisation in both revealed a massive right-to-left shunt from the inferior caval vein, through the liver, to a hepatic vein draining directly to the left side of the intra-atrial baffle. The anomalous vein was successfully ligated in both patients.