The syndrome encompassing the combination of pericarditis, arthritis, and camptodactyly is a rarely described cause of pericardial constriction in children. It is likely that this association is being under-recognized. We report a new case in which the skeletal abnormalities were subtle. The syndrome should be included in the differential diagnosis of any child with persistent non-inflammatory pericardial effusion. A careful search at the bedside for the associated skeletal abnormalities should lead to the correct diagnosis. Pericardiectomy is the treatment of choice.

The majority of live births with severe congenital cardiac malformations that do not undergo intervention of some form die prematurely. The population of Malta provides an ideal closed cohort from which to monitor trends. We have observed a significant declining specific mortality from congenital heart disease, and circumstantial evidence relates this decline to the introduction of new techniques used in the diagnosis and treatment of these malformations.

MAGNETIC RESONANCE IMAGING OF THE heart started in the 1970's, over the subsequent 25 years, enormous advances have been made in both the hardware and software of magnetic resonance imaging machines so that we can now obtain fast, detailed and accurate images of the heart and great vessels. In chronological terms, therefore, we can say that cardiac magnetic resonance imaging has come of age. In this article we will highlight some of the clinical applications of different magnetic resonance imaging techniques, as well as some recent developments. We will demonstrate that, in clinical investigation of congenital heart disease, magnetic resonance imaging has truly come of age.

There has been increasing interest in the study of ventricular function in the patient with congenital heart disease. Numerous indexes have been derived for the assessment of ventricular function, suggesting that none is ideal. While the derivation of some measures of ventricular function have relied on advanced mathematical principles, it is still possible for the non-mathematician to obtain important insights into ventricular function from an assessment of the events which underpin the cardiac cycle. In this review, I use the mechanics of the cardiac cycle to introduce basic concepts of ventricular function for the non-expert. In this way, I analyse ventricular systolic and diastolic performance and describe the contribution of regional variability of function to overall performance. This approach also highlights the role of the ventricle in overall cardiovascular and metabolic homeostasis.

The cross-sectional and colour flow mapping echocardiographic appearances of extension of ductal tissue within the pulmonary artery in three infants referred for echocardiographic assessment of a cardiac murmur are reported. The ductal plaques described may be common but are not necessarily of clinical significance.

We describe a 39-year-old woman who was diagnosed as having an unusual atrioventricular septal defect with a communication from left atrium to right ventricle. A common atrioventricular junction, with partially separated right and left atrioventricular orifices, was found at transoesophageal ultrasonic examination. Both bridging leaflets were attached to the underside of the atrial septum, which was grossly malaligned relative to the ventricular septum. The shunt was exclusively from left atrium to right ventricle because of the overriding of the left atrioventricular valve, with the left component of the inferior bridging leaflet firmly fused to the ventricular septal crest.

Dick Rowe (Fig.) would be embarrased by this attention, as he was such a very modest man. Yet it is appropriate that the late Richard Desmond Rowe be included in Cardiology in the Young's Paediatric Cardiology Hall of Fame. As I consider the life and contributions of Dick Rowe, I am at first troubled, indeed saddened, by the realization that more than a decade has passed since his untimely and premature death just weeks before his planned retirement. I have his picture on the mantle of my office. Not a day goes by that I don't look at his countenance, however fleeting, reflecting on my good fortune to have worked with and considered Dick Rowe as mentor, colleague, teacher and friend. This sense of good fortune is shared by virtually all those touched by his warmth and generosity.

Improvements in diagnosis and surgical technique for correction have led to an increasing number of women with congenital heart disease reaching the child-bearing age. Pregnancy places considerable strain on the heart and circulation and necessitates marked cardiorespiratory adaptation. Today, with the exception of the Eisenmenger syndrome, there is no increased mortality associated with pregnancy in congenital heart disease. In contrast, there is still considerable morbidity, due to congestive heart failure, thromboembolic complications and disturbances of rhythm.

Fetal outcome is complicated by a high rate of spontaneous abortions (20–25%), retardation of fetal growth, and premature delivery (almost 100% in cyanotic mothers). Based on an extensive review of the literature, we discuss the specific risks in pregnancy depending on the hemodynamic situations produced by different heart defects. We also discuss the risks and advantages of different regimens for anticoagulation.

Counselling concerning contraception is frequently inadequate. The most important problems are thromboembolic complications with the use of hormonal contraception, and hyper- and dysmenorrhea in those using intrauterine devices. Finally, the genetic risks must be considered, differentiating between single gene defects and the sex of the parents suffering from congenital heart diseases.