A large experience with fetal congenital heart disease allows the spectrum of disease to be described with accuracy and compared with that in infancy. Knowledge of the natural history of heart malformations when they present in the fetus allows accurate counseling to be offered to the parents. If the trend in parental decisions found in this series continues, a smaller number of infants and children with complex cardiac lesions will present in postnatal life.
Objective-To establish identifiable prenatal factors in fetal heart block which might predict death in utero, the need for intervention, or the probability of pacemaker requirement. Setting-Tertiary referral unit for fetal echocardiography. Subjects-36 fetuses with congenital complete heart block and structurally normal hearts identified between 1980 and 1993. Methods-Maternal anti-Ro antibody status was documented. Prenatal variables examined included absolute heart (ventricular) rate, change in rate, and development of hydrops fetalis. Postnatally, heart rate, need for pacing, and the indications for pacing were detailed. Results-Of the total of 36 patients, there are 24 survivors; 11 are paced. Of those fetuses which died, two were electively aborted for severe hydrops, seven died in utero, two were immediate postnatal deaths, and one was an unrelated infant death. The trend was for the heart rate to decrease during fetal life and postnatally. Fetuses with deteriorating cardiac function did not always show the lowest heart rates. Bradycardia of less than 55 beats/min in early pregnancy or rapid decrease in heart rate prenatally were poor prognostic signs. Hydrops was also associated with bad outcome, 10 out of the 12 hydropic fetuses dying (83%). Of 10 fetuses presenting with a heart rate above 60/min, nine survived of whom three required pacing. Of seven presenting with heart rates of S0/min or less, only three survived and two of these required pacing. Of the two fetuses with negative maternal anti-Ro antibody status one died in utero and one required heart transplantation after pacemaker insertion. Conclusions-Isolated complete heart block identified in fetal life does not always have a good prognosis. An individual heart rate does not accurately predict the outcome in utero or the need for postnatal pacing. Regular, careful monitoring during pregnancy is required in order to optimise care and timing of any interventions.
We conclude that salbutamol can be effective in the treatment of fetal complete heart block and should be considered in patients with this condition where there is evidence of deteriorating cardiac function.
Objective-To investigate the echocardiographic, morphological, and histological appearances of aorto-left ventricular tunnel observed in four fetal hearts and compare the findings with those reported in older patients with the malformation. Background-Previous studies have concentrated on clinical features of the malformation from birth to adult life and have speculated on either its embryological formation or its acquisition during late intrauterine life. The presentation of a large series of cases in fetal life is a unique opportunity to study the malformation at an early stage in its natural course. Methods-A retrospective study was performed of four cases of aorto-left ventricular tunnel discovered among 872 cases of congenital abnormalities diagnosed at a tertiary centre for fetal echocardiography. Detailed echocardiographic and anatomical observations were made of the malformation as identified during fetal life. The precise anatomical arrangement was determined and compared with previous descriptions found in journals published in English. Results-In fetal life, as after birth, the malformation is characterised by enlargement and hypertrophy of the left ventricle, enlargement of the aortic root, and free regurgitation at the level of the aortic valve. Anatomical abnormalities are found at the aortic ventriculoarterial and sinutubular junctions as well as in the intervening aortic wall. These are unrelated to necrosis, ischaemia, or the presence of mucopolysaccharides. Conclusions-The lesion is a developmental abnormality that should be reliably diagnosed by fetal echocardiography combined with colour flow Doppler echocardiography during the midtrimester. The exact anatomical relations clarified by this study are pertinent to diagnosis and subsequent surgical correction. (Br Heart _ 1995;74:443-448)
Since 1980, 11 examples of cardiac tumour have been detected in the fetus out ofa total of 794 congenital cardiac malformations. Patients were referred because of fetal hydrops in two, a family history oftuberous sclerosis in two, and because of the detection of a tumour mass during a scan at the local hospital in seven. The gestational age range at presentation was from 20-34 weeks. Of eight fetuses where death occurred, the histological type was rhabdomyoma in seven and teratoma in one. In seven cases, the lesion appeared single and in four there were multiple tumours. In two ofthe cases ofrhabdomyoma, other family members had evidence oftuberous sclerosis. Termination ofpregnancy took place in four cases; of seven continuing pregnancies, spontaneous intrauterine death occurred in four, and three children are still alive. Two of the three survivors has the clinical picture of tuberous sclerosis. The last case is as yet only 1 month old.In summary, even where the lesion is single, the most likely diagnosis in fetal cardiac tumour is rhabdomyoma, with associated tuberous sclerosis. However, the characteristic features of this latter condition may not become evident until some months after birth. examples of cardiac tumours in fetal life. We have studied the presentation, the type, and outcome of these cases.
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