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The modified Norwood procedure for first-stage palliation of hypoplastic left heart syndrome is possible in the majority of patients without the use of exogenous materials and does not result in an increased incidence of neoaortic arch obstruction. Repair of the aorta without patch supplementation may improve the potential for long-term growth of the new aorta.

Intrinsic obstruction (endocardial sclerosis or thickening) is associated with worse prognosis and earlier re-intervention than extrinsic (anatomical) obstruction. We advocate an early, aggressive approach to the management of patients with TAPVC, especially in the presence of PVS. This complication is most appropriately managed by a combination of re-operation and repeated balloon dilation.

Objectives-To assess the current results and outcome of surgery in infants and children with right atrial isomerism and complex congenital heart disease. Setting-Tertiary referral centre. Methods-20 consecutive children with right atrial isomerism and complex congenital heart disease underwent surgery over a 6 year period between August 1987 and July 1993. The results and outcome were analysed according to age, presentation, and surgical procedures. Results-Patients were divided into two groups depending on age at presentation and initial surgery: group A comprised 11 patients who required surgical intervention in the first month of life (mean age 5 days); and group B comprised nine patients who required initial surgical intervention after the first month of life (mean age 6-8 months). Seven (64%) of the 11 patients in group A had obstructed pulmonary venous drainage and ten (91%) had pulmonary atresia. There were seven early deaths (64%), including the five patients who required systemic to pulmonary artery shunt and simultaneous repair of obstructed pulmonary veins. The long-term survival rate in this group was 18% (two of 11). Pulmonary venous obstruction was present in two (22%) of the nine patients in group B and four (44%) Right atrial isomerism is associated with complex congenital heart disease. The characteristic spectrum of significant cardiac lesions includes anomalous pulmonary venous drainage which is often obstructed, a common atrium, complete atrioventricular septal defect, ventriculoarterial discordance, and severe pulmonary outflow tract obstruction or atresia. Other associated lesions are visceral heterotaxia, asplenia, and congenital malformations mainly involving the genitourinary and gastrointestinal system. '-3 The prevalence of atrial isomerism (both right and left) in the New England regional infant cardiac program has been reported to be 4% of all infants with congenital heart malformations.4 The prevalence of right atrial isomerism compared with that of left atrial isomerism is higher in necropsy series3 than in surgical series.5 This is related, in part, to the increased severity and complexity of congenital heart disease in right atrial isomerism and in part, to the increased risk of sepsis in the absence of a functioning spleen.6 The natural history of patients with right atrial isomerism and complex congenital heart disease is death of more than one third of untreated patients within the first week of life. One year survival was only 21 % in the necropsy series reported by Rose et al.3 Cardiac failure and anoxia were the predominant causes of death (54%), followed by surgery (20%) and infection (10%).Previous studies have documented poor surgical results in infants and children with right atrial isomerism and complex congenital heart disease.578 When considering the recent advances in surgical results in the treatment of infants and children with other forms of complex congenital heart disease9 one might also expect improved results in children with right atrial isomerism. ...

In truncus arteriosus repair, RV to PA continuity established by a direct anastomosis was associated with a low incidence of surgical RVOT re-intervention. This technique has the potential for RVOT growth and may be a useful alternative when an appropriate allograft is unavailable, particularly in the neonate where the risk of pulmonary hypertension are lower.

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To compare echocardiography with clinical examination, radiography, and electrocardiography for the detection of congenital heart defects (CHD) a four year prospective study was carried out in 166 neonates with selected congenital gastrointestinal malformations (anorectal anomaly, tracheo-oesophageal fistula, duodenal atresia, exomphalos, and gastroschisis). Routine examination and investigation detected CHD in 16 neonates. Using echocardiography CHD was diagnosed in 38 (23%) neonates of whom five had two gastrointestinal malformations: in 22/57 (39%) with a tracheo-oesophageal fistula, 10/67 (15%) with an anorectal anomaly, 4/20 (20%) with exomphalos, 6/20 (30%) with duodenal atresia, and 1/7 with gastroschisis.A significantly higher incidence of CHD in neonates with gastrointestinal malformations was diagnosed using echocardiography (23%) compared with routine examination and investigation (9%). Early diagnosis of CHD allowed a unified approach to be presented to the family. (Arch Dis Child 1994; 70: F206-F208) tions requiring surgery were entered into this study. They underwent clinical examination, electrocardiography, chest radiography, and were referred for a cardiac opinion. Examination by a cardiologist and echocardiography were performed at the earliest possible date. Comparison was made between the clinical diagnosis and that suggested by the investigations. Information was stored on a database and coded.As it is recognised that all neonates have a patent foramen ovale, an atrial septal defect was only diagnosed when there was unrestricted left to right shunting on colour flow Doppler through an atrial communication. Likewise, babies were only diagnosed as having a persistent ductus arteriosus if one was present at 1 month of age. STATISTICAL METHODSThe comparison between the observed rate of diagnosis of CHD using clinical and electrocardiographic with radiographic examination and that using echocardiography was performed by a paired proportions t test. The comparison between our observations and those in the literature were performed using a two sample t test of proportions.