A Gerbode defect is a ventricular septal defect communicating directly between the left ventricle and right atrium. There has been some debate about the existence of such an entity. We present the case of a previously healthy 15-year-old boy who developed bacterial endocarditis following ear piercing and was subsequently found to have a defect between the left ventricle and right atrium, which was successfully repaired surgically.

Post-operative low cardiac output states remain a major cause of mortality following cardiac surgery in infants and children. Since 1979 we have used moderate induced whole-body hypothermia in the management of low-output states refractory to conventional modes of therapy. This is based not only upon the relationship between body temperature and oxygen consumption, but also on experimental work showing a beneficial effect of cooling upon myocardial contractility, particularly when there is pre-existing impairment of ventricular function. Between July 1986 and June 1990, 20 children with refractory low-output states were cooled by means of a thermostatically controlled water blanket to a rectal temperature of 32-33 degrees C. The median age was 12 months (1 week-11 years) with a median weight of 6 kg (3.5-33 kg). Ten children survived to leave hospital while a further two made a haemodynamic recovery. There was a marked reduction in heart rate (P < 0.001). The mean arterial pressure rose (P = 0.037) while there was a fall in mean atrial pressure (P < 0.001). There was a significant improvement in the urine output (P = 0.002). A fall in the platelet count (P < 0.001) was not accompanied by any change in the white cell count (P = 0.15). Although it is impossible to say whether cooling influenced the outcome in any of these children, it was usually effective in stabilising their clinical condition. The technique is simple and has a sound theoretical basis.(ABSTRACT TRUNCATED AT 250 WORDS)

The cases of 110 infants less than 1 year of age, who had surgical repair for coarctation of the aorta between June 1974 and February 1988, were analysed. Three groups of patients were identified. In group 1 there were 39 patients with isolated coarctation. In group 2 there were 25 infants with additional ventricular septal defects (VSD), while in group 3 there were 46 infants with other associated congenital cardiac defects. Repair was performed using the subclavian flap aortoplasty (SFA) procedure in 83 patients, resection with end-to-end anastomosis (EEA) in 23, patch aortoplasty in 3 and Goretex tube bypass in 1. Twenty-eight patients had simultaneous pulmonary artery banding and one concomitant closure of the VSD. The overall early mortality rate was 8.2% (5.1% in group 1, 0% in group 2, and 15.2% in group 3). Age at operation (under 1 month, p = 0.04) and other associated cardiac anomalies (p = 0.03) increased early mortality significantly. There were 11 late deaths (10.8%) among 101 patients followed from 1 to 15 years (mean 5.3 years). Twelve patients underwent further surgery for recoarctation, eight of them within 11 months. A further 11 patients currently have a Doppler gradient across their coarctation site of more than 20 mmHg, but have not undergone further surgery to the coarctation repair site.

Aortic root enlargement with a patch is sometimes indicated either to prevent aortic homograft valve distortion during implantation or to facilitate easy, tension-free closure of the aortotomy. Patches made of prosthetic material have been widely used for this purpose. The use of autogenous pericardium has recently been reported. Although dura mater has been shown to have great strength, low antigenicity, athrombogenicity, easy availability in large sizes and rapid bonding to most tissues, its use for patch enlargement of the aortic root has not been previously documented. From 1979 to 1983, 38 patients had dura mater aortic root gussets placed during aortic valve replacement at the Southampton General Hospital. In all cases, the patches were placed to facilitate aortic closure, or to prevent homograft valve distortion by enlarging the non-coronary sinus. Aortic homografts were implanted in 11 patients, Carpentier Edwards' Xenograft valves in 16, Björk-Shiley valves in 8 and Wessex Xenografts in 3 patients. All the patients survived and in a mean follow-up of 30 +/- 12.8 months (range 3 to 48 months) there has been no clinical evidence of patch failure due to leakage, rupture or aneurysm formation. These results suggest that glycerol-preserved dura mater is a satisfactory patch material for aortic root enlargement during aortic valve replacement.

The total of nearly 28,000 operations in 1995 is probably an underestimate, but it has been difficult to collect this data at all. Obviously many smaller countries have not been included, and some older patients undergoing operations such as atrial septal defect closure in adult units have been missed. The number of open heart operations per million vary in different countries and this presumably represents differing referral patterns. If their numbers are low in some countries, these results may be helpful in persuading their governments that more resources are needed for congenital heart surgery. It is also interesting to see the variation in the number of operations performed in the first year of life. Those countries with a lower rate may have a backlog of older patients who were previously palliated, or their surgeons may still prefer initial palliation and later correction in some conditions rather than early correction. It is hoped that with better prospective data collection, an assessment can be repeated in 2000.