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Septic arthritis developed in a neonate after an infection of her hand. Despite medical and surgical treatment endocarditis of her aortic valve developed and the resultant regurgitation required emergency surgery. At operation a new valve cusp was fashioned from preserved calf pericardium. Nine years later she was well and had full exercise tolerance with minimal aortic regurgitation.

A case of pulmonary atresia with intact ventricular septum is reported in which total surgical correction was carried out successfully at 32 hours of age. Cardiac catheterisation at 17 months has revealed virtually normal haemodynamic and angiographic findings. Surgical correction of this condition in the neonatal period is discussed.

A review of 126 cases of coarctation of the aorta confirms the need for surgical resection in infants with intractable congestive cardiac failure. The high association with additional cardiovascular abnormalities in patients presenting in early infancy contributes significantly to the mortality. Patients with large ventricular septal defects and coarctation of the aorta are at risk and may require pulmonary artery banding at the time of resection of the aortic coarctation. Long-term complications included restenosis (18 cases) and persistent hypertension (10 cases). In order to prevent persistent hypertension, it is suggested that elective resection of the coarctation be done at 1 year of age.

In 42 children with congenital heart disease coagulation factor levels were studied serially during the first 20 hours following cardiopulmonary bypass surgery. The acyanotic patients, and also cyanotic patients who survived the operation, showed a progressive improvement in their coagulation profile from initial low postoperative levels. In 12 cyanotic patients who died within 72 hours, however, the coagulation factor levels either remained low, or fell further, until death. Fresh frozen plasma was administered to eight of these patients without apparent benefit. The abnormal coagulation profile correlated significantly with low skin temperature and increased blood loss and was considered to represent excess intravascular coagulation secondary to low cardiac output and poor tissue perfusion.

Since 1960 we have performed 80 colonic interpositions on 79 children. We present a retrospective review of their progress. Sixty-nine interpositions were for long-gap oesophageal atresia, while the remainder followed oesophagectomies, eight for strictures and two others. Overall, the transthoracic route was used in 69 per cent of cases, while 29 per cent were retrosternally placed and one was retropleural. The mean follow-up was 11.08 years. The mortality rate was 12 per cent, with one-third of deaths unrelated to the operation. Graft failure, which occurred on eight occasions (10 per cent), was due to ischaemia in four, intractable stricture in two and stomal ulceration in two. Leakage from the proximal anastomosis occurred in 31 per cent of cases and stricture formation in 27 per cent. Thirty per cent of patients complained of acid reflux, of whom one-third developed stomal ulceration. We present data concerning other complications, including long-term recurrent respiratory infections, malabsorption, gastrointestinal haemorrhage, diarrhoea, intestinal obstruction and redundancy of the graft. Staging the procedure did not affect the outcome, however the higher incidence of serious complications encountered following retrosternal interposition finally led us to abandon this procedure in favour of the transthoracic route. There has been little improvement in the growth rate, but in terms of swallowing ability the outcome was satisfactory in 94 per cent of cases.

Thank you Ladies and Gentlemen, and particularly the organisers of this conference for giving me this opportunity to speak. You understand I am more or less a local boy, and one of us had to say something.

Cardiac pacing in children has increased in recent years partly because of technologic advances in pacing wires and generators and partly because more elaborate procedures are being carried out for complex congenital heart disease, which are not uncommonly, and sometimes inevitably, complicated by postoperative heart block.

Surgical repair of the mitral valve was undertaken in two young female patients during the active phase of bacterial endocarditis to eradicate persistent infection. Operation resulted in rapid resolution of infection with good haemodynamic results in both patients. A mitral valve prosthesis has the disadvantage for children of needing replacement because of growth, and for young women of leading to problems in pregnancy because of the need for lifelong anticoagulant treatment. Thus repair rather than replacement of the mitral valve should be considered in patients, especially young women, presenting with mitral regurgitation in these circumstances.

Since 1984 five patients who had previously had cavopulmonary shunts for cyanotic congenital heart disease have returned to the Birmingham Childrens' Hospital with increasing breathlessness and cyanosis. Two had had a classic Glenn (unidirectional) cavopulmonary shunt and three, a bidirectional shunt; all shunts had been performed 5 to 18 years earlier. Each patient was reinvestigated and underwent creation of a brachial artery-basilic vein fistula. This procedure is known to have good patency rates when used in other situations (for example, for hemodialysis), but it has not been previously described for use in this context. Of the five patients, four had a considerable and sustained symptomatic improvement on follow-up of 12 to 49 months (mean 31 months), but the condition of one continued to deteriorate; that child has subsequently undergone a Fontan procedure, also with no improvement. Only one patient complained of coldness of the arm, and there were no other complications. We believe a brachial artery-basilic vein fistula has considerable advantages in terms of ease of operation and postoperative complications when compared with an axillary arteriovenous fistula, used as a supplement to an inadequate cavopulmonary shunt.

Prior to 1970, most patients with tricuspid atresia (TA) and reduced pulmonary blood flow were palliated by means of a systemic to pulmonary artery shunt (SPAS). In Birmingham between 1967 and 1988, 19 patients with TA (aged 16 days to 23 years, mean 3.8 years) have undergone side-to-side cavopulmonary anastomosis (CPA) with preservation of pulmonary artery continuity. One had undergone a previous right Blalock-Taussig shunt and in 1 patient with left and right SVCs, bilateral CPAs were performed. Nine patients were less than 1 year old at the time of operation. There were no operative deaths. One late death occurred due to pneumonia. Immediate clinical improvement occurred in all cases. Six symptomatic patients subsequently required further surgery (mean interval between operations 8.9 years, range 4.6-14.2 years). The remaining 12 patients have recently been reviewed. Mean follow-up was 9.5 years (range 5 weeks to 21 years). Exercise tolerance remains good (9 in NY-HA Class 1 or 2) and resting transcutaneous oxygen saturations are higher (mean 85%, range 70%-89%) compared with preoperative values (P less than 0.01). In small children, CPA avoids the complications of SPAS and, in some patients, may itself achieve adequate long-term definitive palliation.

M-mode and cross-sectional echocardiography performed in a patient with acute pulmonary embolism showed a sausage shaped, mobile mass in the right ventricular cavity highly suggestive of a right ventricular myxoma. Emergency thoracotomy 24 hours later showed the right ventricle to be free of tumour but both pulmonary arteries contained embolised venous thrombi, one or more of which were thought to have given rise to the false echocardiographic diagnosis of a right ventricular tumour.

For the past 25 years an emergency pulmonary embolectomy service has been offered to the hospitals serving a conurbation of 1.5 million. Fifty-five of these procedures have been performed during a short period of normothermic circulatory standstill produced by clamping the superior and inferior venae cavae. Of 36 patients who underwent pulmonary embolectomy without an episode of asystole or ventricular fibrillation, 35 survived the operation (97.2%). However, there were seven deaths during the postoperative period, three related to pulmonary embolism and four to other causes (mortality 20%). Conversely, in a group of 19 patients who had an episode of cardiac arrest, 14 died during or after the operation of pulmonary embolism and two of unrelated causes (mortality 73.7%). In properly selected patients this technique achieves a satisfactory measure of success. It can be used in hospitals that do not have cardiac surgical facilities and, because of its simplicity, it can be performed during the early period after pulmonary embolism when the risk of death is greatest.

A 60-year-old woman developed subacute bacterial endocarditis following a minor operation. She presented the unusual picture of breathlessness with cyanosis related to posture, confirmed by laboratory measurement. She had an atrial septal defect with right-to-left shunt, and had sustained multiple pulmonary infarcts, resulting in the obliteration of a large section of her pulmonary vascular bed. The puzzling absence of pulmonary hypertension was explained by tricuspid incompetence, although there was no clinical evidence of this, and on two occasions the right atrial pressure tracing was normal. The use of a balloon catheter to predict the results of operation is described. At operation the severely damaged tricuspid valve was replaced, and the septal defect was closed. There was an immediate improvement in the arterial oxygen tension after operation. She subsequently survived an episode of ventricular fibrillation, left hospital 11 months after her original admission and is now leading a normal life.

Nine cases of congenital cystic adenomatoid malformation of the lung are described. One was stillborn: two presented in the newborn period (one of them surviving after a lobectomy), and the remaining six were older children all of whom survived after lobectomy. There have been only three cases previously reported in children outside the newborn period. The pathological, clinical, and radiological features are discussed and compared with previously reported cases.