Early neonatal lethality and cardiac dysfunction in Ex5 mice. A) Kaplan-Meier survival curve shows that Ex5 mice die within 14 d of birth, indicating that SUR2 and SUR2-55 are necessary for survival. B) Histological analysis shows that Ex5 mice had enlarged hearts compared to WT littermate controls (LV, left ventricle; RV, right ventricle). C) Ex5 mice have an increased heart weight to tibia length ratio compared to WT (5.6±0.3 vs. 4.4±0.2 mg/mm, P<0.05). D) Echocardiography was performed on neonatal mice from postnatal d 6 to 11. Ex5 mice exhibited a progressive decrease in ejection fraction compared to littermate controls during this time period. E) Representative M-mode echocardiograms from d 11 WT and Ex5 neonatal mice demonstrate a decline in cardiac fractional shortening (39.3±2.8 vs. 59.7±1.0%, P<0.05).