Holt-Oram syndrome in a Maltese family.

Introduction Holt & Oram (1960) linked various skeletal abnormalities with congenital heart disease in four generations of a family. In particular, malformations of the hands were associated with atrial septal defect (secundum type) and bizarre arrhythmias. In 1961, McKusick described a similar syndrome in a mother and daughter and suggested the term 'atrio-digital dysplasia'. Harris & Osborne (1966) related congenital anomalies of the radius with ventricular septal defect (ventriculo-radial dysplasia). We describe three members of a Maltese family (Fig. 1) with minor hand anomalies and atrial septal defect. A fourth member of the same family has finger-like thumbs and findings consistent with CHD.