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pREVIOUS STUDIES in our laboratories haveshown that patients with the systolic click syndrome exhibit a characteristic disorder of left ventricular contraction, with impaired shortening of the inflow tract and, in many instances, systolic expansion of the region of myocardium surrounding the mitral valve ring.1 The presence of a segmental myocardial abnormality appears to provide a plausible explanation for the occurrence of regional ECG abnormalities, as well as arrhythmias commonly observed in these patients in the absence of evidence of significant mitral valve dysfunction, and may also account for the late systolic timing of mitral incompetence in patients without obvious mitral leaflet abnormality. The possibility was considered that this segmental myocardial contraction disorder, in turn, might be related to an abnormality of the coronary circulation. Accordingly, the anatomy of the coronary circulation was examined by means of selective coronary arteriography

Right ventricular dilatation is an infrequent finding at fetal echocardiography. Previous studies have documented an association with aortic coarctation. However, there are associations with other congenital abnormalities. We reviewed our experience of fetal right heart dilatation in order to recognize concurrent anomalies and to assess the outcome of the affected fetuses. We studied all fetuses with right ventricular dilatation over a 5-year period. We documented associated cardiac and noncardiac lesions, and outcome data were assessed in terms of the number of fetuses that were born live and the number surviving to 2 months of age. Forty-three fetuses with right heart dilatation were seen. Fifteen had associated cardiac abnormalities: most commonly coarctation (n = 4) and VSD (n = 4). Seven had associated noncardiac abnormalities. There were seven fetuses who also had chromosomal abnormalities. In total, there were three terminations of pregnancy, four intrauterine deaths, one stillbirth and 35 live-births. Twenty-eight were alive at 2 months of age (70% of the nonterminated pregnancies). Fetal right heart dilatation is frequently associated with both cardiac and noncardiac congenital abnormalities. Our outcome figures suggest a guarded prognosis be given during counseling of parents of fetuses with right heart dilatation.

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Four cases ofjuxtaposition of the atrial appendages diagnosed during cardiac catheterization and angiocardiocopyright.