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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. ...

Early survival was achieved in 59% of patients in the first year of implementation of a protocol for surgery in infants with hypoplastic systemic ventricle and severe outflow obstruction. The construction of a neoaorta without the use of exogenous material may allow improved later growth of the neoaorta. Early cavopulmonary shunt can be performed safely and should reduce mid-term complications from cyanosis and systemic ventricular volume loading.

Tuberculosis was not suspected in 54% of children with MTL, and they posed diagnostic dilemma on admission. Bronchoscopy must be used as a diagnostic tool in children where tuberculosis cannot be excluded by radiology or specific skin tests. Thoracotomy and excision may be necessary to treat the obstructive symptoms.