Dysfunction of the systemic right ventricle is common after the atrial switch procedure for transposition of the great arteries. Cardiac transplantation remains the only long-term solution in terminal systemic right ventricular (RV) failure, but concomitant pulmonary hypertension (PHT) may preclude it. The increasing number of such patients, together with the concerns related to combined heart-lung transplantation (HLTx), urge us to consider other therapeutic options.
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