Palliative surgery for congenital heart disease in small children - abstract

Ten small children with severe congenital heart disease have had palliative surgical procedures performed with good clinical results and without mortality at the University Hospital of the West Indies. Seven of these were cases of Tetralogy of Fallot and 3 were cases of large ventricular septal defects. It is planned to do a curative surgical operation for all these patients when they are old enough to permit the safe use of cardiopulmonary bypass. The 7 cases of Tetralogy of Fallot were aged from 11 months to 5 years. A Blalock-Taussig shunt was performed in the first 3 cases, and a shunt between the ascending aorta and right pulmonary atery was carried out in the last 4 cases. The indications for surgery were: (a) severe cyanosis with gross exercise intolerance (5 cases), and (b) severe paroxysmal cyanotic attacks not responding to "conservative" measures (2 ases). One patient, aged 4 years, had not been able to stand or walk. Another had a history of hypoxic convulsions. There were two post-operative complications - post-intubation laryngeal swelling, and left iliac vein thrombosis secondary to a previous saphenous vein exposure in the groin for intravenous infusion. The follow-up period ranged from 3 months to 31/2 years. All the patients did well. Their effort tolerance was markedly improved and cyanosis was absent or nearly so. The 3 cases of venticular septal defects were aged 3 months, 11 months, and 1 year. They had banding of the pulmonary artery performed for severe congestive cardiac failure which did not respond to intensive conservative therapy with diuretics and digoxin. In one case the diagnosis was confirmed pre-operatively by cardiac catherization which demonstrated a large left to right shunt with a pulmonary: systemic flow ratio of 7:1. In the other 2 cases the diagnosis was made on clinical radiological and electrocardiographic evidence. At surgery, a patient ductus arteriosus was excluded and the presence of a typical thrill over the anterior surface of the right ventricle and a large pulmonary artery were demonstrated. There were no post-operative complications directly attributable to the surgery. All 3 patients are still attending regularly for follow-up which ranged from 3 to 7 months in January, 1972. Two have shown marked improvement but the third is only moderately improved (AU)

Tetralogy of fallot in three siblings

Although the etiology of congenital heart disease is unknown in most instances, there is increasing evidence that heredity plays a significant role. Campbell and other workers have found that the incidence of congenital cardiac malformations in families of children with congenital heart disease is six times higher than the general population. Many workers have also shown that when two siblings are affected, the lesion is usually the same. Extra-cardia abnormalities are not significantly higher in cardiac patients when known syndromes are omitted. A family is described in which three siblings are afflicted with tetralogy of fallot. In one tetralogy is the only lesion. In another, there is also cleft lip. In the third, there is associated cleft lip and cleft palate. The mother had 15 pregnancies with 12 children alive. Three children died in infancy of diarrhoea or pneumonia. Pertinent clinical details, chest x-rays and electrocardiograms will be discussed (AU)