31 December 1979 performed at the University Hospital Gasthuisberg over a three-year period, the anatomy of the thymus was carefully examined as a routine. Ten patients with agenesis or hypoplasia of the thymus were found. Stress involution was differentiated from hypoplasia on the basis of the symmetrical aspect of the shrinkage and the persistence of the normal bilobed anatomy. In hypoplasia at least one lobe of the thymus is completely absent. Thymic aplasia was defined as the absence of thymic tissue in the upper mediastinum. In nine of the 10 patients the tissue of the anterosuperior mediastinum and neck, which had been removed en bloc, was serially sectioned at 5 V. and every 10th section was stained and histologically examined. All 10 patients had presented to the departments of paediatric cardiology or neonatology with cardiac symptoms and signs. Ventricular septal defects were classified after Goor and Lillehei4 and truncus arteriosus after Collett and Edwards.5 Two types of interrupted aortic arch6 were distinguished: interruption distal to the left subclavian artery (type A) and interruption distal to the left common carotid artery (type B). This terminology was also applied, 452

The arterial blood supply of the A V node and main bundle has been studied in I0 normal hearts. In each heart i5o to 200

With the aim of defining the clinical profile of cardiac tumours in childhood and of promoting their early recognition, a study of these relatively rare conditions was undertaken as ajoint project by the members of the Association of European Paediatric Cardiologists. Within 5 years, 29 cases were collected from I5 centres. This report is limited to 22 primary cardiac tumours that were histologically verified and classified as 'benign'. There were 6 rhabdomyomas, 6 teratomas, 5 fibromas, 3 myxomas, and 2 haemangiomas. No patient with a well-documented primary malignant tumour was encountered. Though classified as 'benign', such tumours caused death in iI patients by encroachment upon the cardiac cavities or compression of the conducting tissues. The salient clinical features and the macroscopical and histological findings are briefly reviewed. Thirteen cases were correctly diagnosed during life and before operation. Of the II children operated upon, 8 made a complete recovery: 4 had a teratoma, 3 a fibroma, and I a myxoma.In I965 at the annual meeting of the Association of European Paediatric Cardiologists in St Andrews, Scotland, it was proposed to start a joint research project on the incidence and clinical profile of cardiac tumours in childhood. Within 5 years, 29 cases (Table I) were collected from I5 centres: Amsterdam,

The cardiac findings in two sibs with Friedreich's ataxia are described. The clinical signs were suggestive of hypertrophic obstructive cardiomyopathy. During left heart catheterization a systolic pressure gradient across the left ventricular outflow tract could be provoked by an infusion of isoprenaline. Left ventricular angiocardiograms and echocardiograms showed gross thickening of the interventricular septum. In one patient a systolic anterior movement of the anterior leaflet of the mitral valve was seen. The importance of serial echocardiographic examination for patients with Friedreich's ataxia is emphasized.

A case of fibroma of the right ventricle in a child of 16 months is reported. The clinical and hemodynamic findings were those of severe tricuspid stenosis. Angiocardiography proved the obstruction to be due to a cardiac neoplasm, localized within the right ventricle. An attempt at surgical removal failed because the tumor, almost completely filling the cavity of the right ventricle, was too extensive and could not be distinctly delineated from the healthy myocardium.