The relative amounts of cardiac myosin heavy-chain isoforms (MyHC) in right atrial tissue (RA) of 16 neonates and children suffering from congenital heart disease have been investigated. Quantification of MyHC was based on one-dimensional gel electrophoresis and on histometrical evaluation of cyro-sections stained with monoclonal antibodies against alpha- and beta-MyHC. The mean right atrial pressures ranged from 2 to 14 Hg. The RA load was normal in eight patients (5.1 +/- 1.3 mm Hg) and overloaded in eight cases (10 +/- 2.5 mm Hg). The arterial oxygen saturation was normal in 12 and ranged between 85% and 89% in four cases. In all patients a large proportion of atrial myocytes coexpressed alpha- and beta-MyHC. However, in the cases with pressure overloaded RA the amount of beta-MyHC was found to be 1.6 times higher than in the cases with normal pressure. This indicates an adaptational response to overload, as was previously described for the adult human heart. In light of this finding, it seems important to conserve as much as possible of the trained right atrial wall during a Fontan type of operation.