This reviw presents the cardiac and non-cardiac malformations in 6o cases with asplenia and polysplenia with special reference to distinguishing factors which may be helpful in the clinical recognition of these syndromes.The asplenia cases were predominantly male and presented with cyanosis. Theyfrequently had transposttion of the great arteries (72%) with pulmonary stenosis or atresia (88%) and total anomalous pulmonary venous drainage (72%). Deaths were caused by cardiacfailure and anoxia in 57 per cent of cases. Most of the patients died in thefirstyear of life (79%), but longer survival is possible in the asplenia syndrome.The polysplenia cases were predominantly female and survived longer. The characteristic clinical findings were the relatively more benign presenting signs and the leftward or superiorly orientated P wave axis on the electrocardiogram. Conotrumcal abnormalities were less comnon and total anomalous pulmonary venous drainage did not occur. On angiography the inferior vena caval drainage via the azygos system was clearly identified and this was present in all cases at surgery.Our study indicated that the cardiac anomalies in polysplenia were less severe than they were in asplenia and therefore the prognosis in the former syndrome is likely to be more favourable. Three families had two affected sibs but no single genetic factor could be identified. The aetiology of these syndromes remains undetermined.

No abstract

Conduction disturbances have been documented after correction of ventricular septal defects by the ventricular route. Recently, repair of the ventricular septal defect has been through the right atrium to overcome damage to the conduction system and a right ventriculotomy. Thirty-nine children with ventricular septal defects under the age of 5 years were operated upon by the atrial route (group 1). The incidence of conduction disturbances in this group was compared with that occurring in 19 children of comparable age with a ventricular septal defect repaired via a right ventriculotomy (group 2). Complete right bundle-branch block developed in 13 of 39 children (33.3%) in group 1, compared with 15 of 19 children (78.9%) in group 2. This was a statistically significant reduction in complete right bundle-branch block in group 1. The incidence of left axis deviation occurring with complete right bundle-branch block was similarly statistically reduced. Transient complete heart block and arrhythmias were not statistically different in the two groups. The atrial approach to the repair of the ventricular septal defect significantly reduced the incidence of complete right bundle-branch block alone and occurring with left axis deviation.

From May 1963 to December 1985, 329 infants and children underwent repair of simple transposition of the great arteries (TGA) using Mustard's technique. To assess improvement, the patients were separated into two groups by date of operation: Group I, May 1963 to December 1973 (N = 106), and Group II, January 1974 to December 1985 (N = 223). The operative mortality was 11 (10.4%) in Group I and two (0.9%) in Group II. The 10-year actuarial survival rate was 73.4% in Group I and 93.7% in Group II. Baffle complications, similar in both groups, were identified in 81 patients; 19 were major, causing death or requiring reoperation. By latest electrocardiogram, 21 of 45 (46.7%) of Group I patients and 129 of 180 (71.7%) of Group II patients were in normal sinus rhythm. Late ambulatory electrocardiography, however, revealed that a majority of patients had sinus node dysfunction or other dysrhythmias. Right ventricular (RV) angiography revealed definite diminution of RV contractility in 14 (11%) of 126 children. At late follow-up, 113 of 148 (76%) children were in New York Heart Association (NYHA) Class I and 35 of 148 (24%) were in NYHA Class II. Thirty-five (21%) patients were on cardiac medication chiefly for dysrhythmia management. There has been significant improvement in the early and late mortality with the Mustard operation, and serious baffle complications are infrequent. Dysrhythmias continue to be a major problem but the surviving patients are clinically well and relatively few have significant RV dysfunction.

No abstract