Abstract

The introduction of the Fontan circulation has improved the long term survival of patients with Tricuspid Atresia (TA) in general. Neonates and infants with TA presenting reduced pulmonary blood flow (RPBF) are usually offered a systemic arterial-to-pulmonary artery shunt (SAPAS) in the hope that they will eventually achieve a Fontan circulation. There is evidence that palliative surgery may not improve survival in this subset of patients. Our aim was to determine whether palliative surgery improves survival in patients with TA and RPBF. An objective assessment of a treatment outcome necessitates a comparison of the treatment outcome with the natural history. We searched the literature for surgical outcome and natural history studies. The most external validity ones were used to create a composite survival graph to assess the efficacy of palliative surgery in patients with TA and RPBF. The data suggests that palliative cardiac surgery in patients with TA and RPBF is unlikely to improve the long-term survival compared to the natural history. There is also evidence that palliative cardiac surgery performed on patients with TA, who present for the first time after one year of age, is unlikely to improve long-term survival compared to the natural history. Palliative cardiac surgery should not be offered to patients with TA and RPBF. Palliative cardiac surgery probably should not be offered to any patient with TA who presents for the first time after 1 year of age.

Key words: Tricuspid atresia, surgery outcome.