OUTCOME OF 77 LIVE BORN CHILDREN WITH CON GENITAL HEART-DISEASE OR ARRHYTHMIAS DETECTED PRENATALLY

Seventy-seven live born children (September 1988 to May 1993) had pren atally detected morphological cardiac malformations (61 cases) and/or arrhythmias (17 cases). The outcome of these children was analysed wit h an average follow-up of 21.4 months (range : 1 day to 11 years). The parameters studied were age and condition of prenatal diagnosis, the term of pregnancy, the extracardiac malformations associated and the o utcome of the live born children. Fifty-two children survived with a m ean follow-up of 28.2 months (range : 5 months to 11 years) and 25 chi ldren died at a mean age of 3.3 months (range : 1 day to 32 months). T he cardiac malformations correlated with the postnatal diagnosis in 59 children (60 cardiac malformations). They included left or right outf low tract obstruction in 22%, septal defects or Tetralogy of Fallot wi th or without pulmonary atresia in 18.4%, arterial malposition in 12.9 % and arrhythmias or conduction defects in 20.7% of cases. The main re ason for the high mortality was the complexity of the cardiac malforma tions with, in particular, ventricular hypoplasia with right or left o utflow tract obstruction (41 % of mortality). The functional consequen ces of these abnormalities were only partially appreciated at prenatal diagnosis. The extracardiac malformations, more easily diagnosed, con tributed less to this mortality rate. The favourable prognosis of the arrhythmias or conduction defects diagnosed prenatally was confirmed b ut was, however, associated with a mortality rate of 12%. This retrosp ective study showed that one third of patients benefitted from repair of their cardiac malformations or from cure of their arrhythmia, one t hird of patients were on the waiting list for curative or palliative s urgery or had maintenance antiarrhythmic therapy; one third died short ly after birth. This poses the difficult problem of evaluating the qua lity of life of patients in whom a cardiac malformation is diagnosed p renatally and the eventual indication for therapeutic abortions in cas es with particularly serious conditions.