Epilogue: 40 years in paediatrics.
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Biomedical subjects
Publications and source records attributed to O C Ward.
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Between January 1976 and December 1987 42 children with Down syndrome and congenital heart disease underwent cardiac surgery. Four children had two operations. Age at the time of surgery ranged from 11 days to 14 years. The commonest operative procedure was repair of a patent ductus arteriosus. Four patients died post-operatively, two following repair of a complete atrio-ventricular canal defect (CAVD), one following correction of tetralogy of Fallot in association with a CAVD, and a fourth following closure of ventricular septal defect and atrial septal defect. The mortality for those who had open heart surgery was 13.3% and for the series as a whole the mortality was 6.6% over a period of follow-up ranging from two months to four years. A relatively conservative approach has been adopted with regard to surgery, based on the shorter natural expectation of life in Down syndrome, the complexity of many of the cardiac lesions involved and the recognition of the frequency of early intellectual deterioration in Down patients.
Rectal acetylsalicylic acid was given to 14 children who had undergone open heart surgery. The effect on their temperatures was similar whether 15--30 or 30--50 mg/kg was given. Either dose was more effective than no treatment. The greatest fall in temperature occurred after 4 or 5 hours. Rectal aspirin in a triglyceride base is effective in lowering postoperative temperature. It should also be of use in treating other fevers. A dose of 20--25 mg/kg is suggested.
A new and simple technique is described which clearly defines the aortic anatomy in infantile coarctation. The technique greatly helps to select those neonates whose lives can be salvaged by early surgery. There have been no serious adverse effects.
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The effect of bumetanide in infants with congenital heart disease presenting with cardiac failure was studied. The study was divided into acute (3 days) and long-term (mean 10.5 weeks) cases. A total of 12 male infants was included in the acute study and 13 cases were evaluated in the long-term study. The dose used in the acute study (0.015 mg/kg) was suboptimal; notwithstanding it was found to cause significant natriuresis and chloruresis. Bumetanide in doses varying in different infants from as little as 0.015 mg/kg on alternate days to as much as 0.10 mg/kg daily was shown to be an effective diuretic for long-term use. No side effects were observed in either study.
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Friedreich's disease occurring in 4 male siblings in a sibship of seven caused cardiac and neurological abnormalities and one twin of an affected member died in cardiac failure; at autopsy characteristic pathological features of Friedreich's disease were demonstrated in the nervous system and heart. One female sibling had a patent ductus arteriosus which was treated surgically and a continuous murmur was present in another sister but cardiac catheterization in childhood had been normal.