Hypotensive anesthesia for a patient with Friedreich's ataxia and cardiomyopathy

Abstract

Friedreich's ataxia is an autosomal recessive, inherited condition resulting in progressive limb and truncal ataxia. Cardiomyopathy is present in 90% of cases and kyphoscoliosis, causing steady deterioration of pulmonary function, in up to 80% of the cases. Insertion of Harrington rods and spinal fusion may slow the progress of the pulmonary dysfunction, but perioperative cardiopulmonary failure is a risk. Surgery is associated with marked blood loss which may be reduced by hypotensive anesthesia. We report a case of Friedreich's ataxia with cardiomyopathy who underwent hypotensive anesthesia for insertion of Harrington rods and spinal fusion. Sodium nitroprusside infusion resulted in a marked decrease in cardiac output associated with supraventricular tachycardia. Administration of isoflurane produced satisfactory hypotension with considerable reduction of intraoperative bleeding.