We evaluate the systemic right ventricle (SRV) failure of congenitally corrected transposition of the great arteries using the scintigraphic studies in a case with 32-year-old male who improved the heart failure by the use of beta-adrenergic blockers. The myocardial perfusion sympathetic nervous system activity mismatch with preserved coronary flow and wall thickness indicates a distinctive feature as SRV cardiomyopathy, suggesting the importance of recovery of SRV sympathetic dysfunction for improving the prognosis.

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