PECULIARITIES OF PREVALENCE AND MORPHOLOGY OF CONGENITAL HEART-DISEASE DETECTED IN-UTERO

Intrauterine echocardiography is changing our knowledge of congenital heart disease; cardiac defects diagnosed in utero have distinctive fea tures of both prevalence and morphology when compared with those obser ved just after birth. We reviewed a series of 171 fetal heart conditio ns: 148 were diagnosed at intrauterine echocardiography, the diagnosis being verified at autopsy in 41, and 23 were observed at the postmort em only. Peculiarities of prevalence consisted in an excess of various defects, such as hypoplastic left heart syndrome, atrial isomerism, p ulmonary atresia, and atrioventricular and atrial septal defects, and in a reduced number of completely different conditions, such as transp osition of great arteries and aortic coarctation. Differences in preva lence have been attributed to difficulties in diagnosing some particul ar anomalies in utero, to the selection of pregnancies undergoing scre ening, and to the special intrauterine evidence of some heart defects. Peculiarities in morphology result from the coexistence with extracar diac malformations, from the changes in shape conditioned by fetal hem odynamics, and from the intrauterine evolution of the morphology of so me malformations. We concluded that the knowledge of these characteris tic traits was helpful to cardiac pathologists, pediatric cardiologist s, and obstetricians, and allowed the reevaluation of the role of hemo dynamic factors in remodeling the malformed cardiovascular apparatus. (C) 1998 by Elsevier Science Inc.