[Background] It is quite frequently to vacillate between the one & one half repair and the Fontan-type repair in case of a choice of surgery in patients(pts) with hypoplastic right heart. To settle this issue, we addressed the comparison of the long–term outcome of the one & and one half physiology and the Fontan physiology in pts with hypoplastic right heart.

[Subjects and methods] We reviewed twenty-one pts with hypoplastic right heart who had undergone functionally physiological corrections in our institute. Ten pts with one & one half physiology and eleven age-matched pts with perfect Fontan physiology were enrolled. Both the groups had been treated with warfarin potassium, aspirin, and ACE-I/ARB/βblocker for cardiovascular protection.

[Results] Data were shown in the table.

[Discussion] The one & one half physiology is inferior to the Fontan physiology in the cardiac index and the plasma natriuretic peptide levels. Furthermore, it is suggested that the exercise tolerance is lower in the one & one half physiology than in the Fontan physiology. On the other hand, the former is superior to the latter in the mean IVC pressure and the serum AST and γGTP levels. However, there was no significant difference of coagulability between the two physiologies under the treatment with warfarin potassium, aspirin, and ACEI/ARB/βblocker. In conclusions, the one & one half physiology might be inferior to the Fontan physiology in the long-term outcome in pts with hypoplastic right heart.