Abstract

Familiar Hypertrophic cardiomyopathy (FHC) is a rare hereditary cardiac disorder characterized by hypertrophy of the ventricular septum and variable degrees of subvalvular aortic stenosis. In this disease, the increase in myocardial contractility and reduction in peripheral vascular resistance can aggravate left ventricular outlet obstruction, leading to arrhythmias and cardiac ischemia. The objective of this report was to discuss the anesthetic management of cesarean section in a patient with FHC. A patient in the 33rd week of pregnancy and prior diagnosis of FHC presented, on the 24-hour Holter monitor, 22 episodes of non-sustained ventricular tachycardia (NSVT) and 2 episodes of sustained ventricular tachycardia (SVT). She complained of episodes of palpitation, dyspnea, and chest pain of short duration. The patient was medicated with atenolol, with control of symptoms and cardiac arrhythmias. Within 38 weeks and 5 days of gestation, the patient underwent elective cesarean section. Besides the usual monitoring, analysis of the ST segment and invasive blood pressure were also instituted. Anesthesia consisted of combined spinal-epidural technique with subarachnoidal administration of 5 microg of sufentanil followed by the administration of increasing doses of 0.375% bupivacaine until it reached the level of T6 (total of 16 mL). Metaraminol was used as a vasopressor. Perioperative maternal hypotension or other complications were not observed. General anesthesia is often used for cesarean sections in patients with FHC. Spinal-epidural anesthesia with slow installation of the blockade was a safe alternative. In those patients, one should avoid an increase in myocardial contractility and, if necessary, a a-agonist should be used to treat maternal hypotension.

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