Tricuspid atresia--profile and outcome

Tricuspid atresia (TA) is the third most common cyanotic congenital cardiac lesion, with a mortality rate of 90% before the age of 10 years. Surgical intervention has reduced the mortality, but with unfavourable anatomy the mortality remains high. The aim of this study was to look at the profile of a cohort of children with TA and to evaluate treatment modalities and outcome. Twenty-seven children were included in this retrospective descriptive study. Twenty-three were referred with central cyanosis and 25% with cardiac failure. All patients had a left superior QRS axis on electrocardiogram (ECG). Eleven patients died, 8 before surgical intervention and 3 postoperatively. Outcome was mainly influenced by the anatomy of the pulmonary arteries especially when the pulmonary arteries were extremely hypoplastic. Patients with increased pulmonary blood flow did better than those with decreased pulmonary blood flow. There was a statistically significant difference in survival between the surgical and non-surgical groups. Few of our patients qualified for the Fontan procedure mainly because of increased pulmonary pressures in the high pulmonary blood flow group and severe hypoplastic pulmonary arteries in the low pulmonary blood flow group. Early referral is essential for palliation so that more definitive surgery can be done to achieve better results.