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Background: There is evidence that "inappropriate hypertrophy" of the single left ventricle, which occurs as a result of acute preload reduction, leads to adverse consequences on ventricular function. However, a systematic study of the capability of tissue Doppler imaging (TDI) to assess systolic and diastolic ventricular functions after the Fontan procedure is still missing., Methods: Twenty-four postoperative patients aged 12-33 years were prospectively evaluated with two-dimensional echocardiography equipped with TDI capabilities. Nineteen age-matched normal subjects were selected as controls. Good-quality echoes for the measurement of ejection fractions were available in 21 patients. Ten patients (group 1) had systolic dysfunction (ejection fraction < 50%), and 11 patients (group 2) had normal systolic function. Peak systolic and diastolic wall velocities were acquired from the two-chamber view in the myocardia and mitral annulus., Results: Compared with controls, the Fontan patients had a significantly reduced peak systolic velocity at wall and annulus sites. A linear correlation existed between ejection fraction and systolic myocardial velocity from the annular sites. Group 1 patients had lower wall velocities and lower annulus velocities both in systole and diastole. Group 2 patients had preserved systolic velocities but decreased regional and annular early diastolic velocities, suggesting impaired filling. Multiple correlation analysis showed a relation between peak early diastolic mitral velocity and ventricular ejection fraction, mean mitral annular motion at systole, mass/volume ratio, and the number of years post Fontan revision., Conclusions: Myocardial velocities recorded after the Fontan operation give insight into systolic and diastolic ventricular functions. The peak systolic mitral annular velocity correlated well with the ventricular ejection fraction. The peak early diastolic velocity and the ratio between the early and late diastolic mitral annular velocity are reduced and reflect diastolic dysfunction even in the presence of normal systolic ejection fraction.

Intrauterine echocardiography is changing our knowledge of congenital heart disease; cardiac defects diagnosed in utero have distinctive features of both prevalence and morphology when compared with those observed just after birth. We reviewed a series of 171 fetal heart conditions: 148 were diagnosed at intrauterine echocardiography, the diagnosis being verified at autopsy in 41, and 23 were observed at the postmortem only. Peculiarities of prevalence consisted in an excess of various defects, such as hypoplastic left heart syndrome, atrial isomerism, pulmonary atresia, and atrioventricular and atrial septal defects, and in a reduced number of completely different conditions, such as transposition of great arteries and aortic coarctation. Differences in prevalence have been attributed to difficulties in diagnosing some particular anomalies in utero, to the selection of pregnancies undergoing screening, and to the special intrauterine evidence of some heart defects. Peculiarities in morphology result from the coexistence with extracardiac malformations, from the changes in shape conditioned by fetal hemodynamics, and from the intrauterine evolution of the morphology of some malformations. We concluded that the knowledge of these characteristic traits was helpful to cardiac pathologists, pediatric cardiologists, and obstetricians, and allowed the re-evaluation of the role of hemodynamic factors in remodeling the malformed cardiovascular appara-tus.

This study consists of a prevalence congenital heart disease (CHD) registered in the Pediatric Cardiology Department of Umberto I General Hospital in Rome between January 1st 1992 and December 31-th 1993. Cases recorded in this period have been taken part of a larger study called Italian Multicentric Study for recording and follow-up of congenital heart disease (IMS-CHD); the purpose is to determine the prevalence of CHD in Italy and discover the outcome of affected children. In this duration, 187 new cases have been recorded, in which 63.6% had a single defect while 36.4% had multiple defects. These isolated defects were most frequently occurred (51.9%) following the stenosis of the pulmonary artery (15.5% and the defects of the interatrial septum (15%).

Permanent mechanical ablation of an accessory atrioventricular pathway was observed in an infant during intracavitary electrophysiological mapping. The persistent lack of preexcitation was confirmed during a 15-month follow-up period.

Background: Fetal echocardiography has achieved high sensibility and specificity rates and it has become a reliable tool to detect intra-uterine congenital heart defects., Objectives: The purpose of the present study was to clarify the potential of anatomo-clinical correlation in fetal echocardiography not only for diagnosing heart defects, but also for understanding and following their morphogenesis and natural history in utero., Methods: Nine hundred pregnancies, referred by several first-level centers, have been followed up from the sixteenth week to birth, and the newborn baby has been studied, too. In case of either voluntary abortion, or intra-uterine or neonatal death, a post-mortem examination was performed whenever possible., Results: Since 1982, 110 fetal heart defects have been detected: 94 were diagnosed by means of fetal echocardiography (in 21/94 an autopsy was carried out) and 16 were directly observed at the post-mortem (spontaneous or voluntary abortions not previously referred). The heart defects most commonly observed were the atrioventricular (14.5%), ventricular (9.0%), and atrial (7.2%) septal defects, the absent left atrioventricular connection (8.1%), and atrial isomerism (6.3%). The congenital heart defects diagnosed in utero vary from those observed in the newborn and infants in terms of distribution (because of difficulties in diagnosis, selection of pregnancies, and differences in manifestation) and morphology (because of the coexistence with other extracardiac malformations, the changes due to altered fetal hemodynamics, and the intrauterine evolution of the morphology of cardiac defects)., Conclusions: Heart defects observed in utero have a peculiar prevalence and a different morphology in respect to their infantile counterparts and can be followed in their morphologic evolution.