外科と麻酔のジャーナル

概要

End-Stage Heart Failure in Becker’s Muscular Dystrophy: A Case Report of Perioperative Management for Oncological Surgery during COVID-19 Pandemic

Piovano Chiara, Savi M, Babbini M, Greco M, Monzani R, Cecconi M

Background: Becker’s Muscular Dystrophy (BMD) is an X-linked recessive disease caused by dystrophin deficiency and characterized by progressive skeletal muscle weakness and functional impairment especially involving proximal muscles of the lower limbs while resulting in several comorbidities, including restrictive respiratory insufficiency and dilated cardiomyopathy.

Materials and methods: We report a case of successful intraoperative management in a 63- year- old ASA IV patient affected by BMD associated with several comorbidities who presented with ingravescent dyspnea and dysphagia due to a supraglottic laryngeal carcinoma. He is also affected by a dilated cardiomyopathy with congestive heart failure (ACC/AHA stage C), chronic atrial fibrillation and pulmonary hypertension; he carries also a Cardiac-Resynchronization Therapy-Defibrillator (CRT-D).

Results: Total intravenous anaesthesia with ketamine allowed us to avoid propofol-induced cardiodepressant effects and spare perioperative high doses of opioids, granting hemodynamic stability and optimal pain control throughout the procedure. A challenging intraoperative management was implemented by using an intravenous strategy to solve the specific problems presented by this case.

Conclusion: This case highlights why it is useful for anesthetists to be familiar with multiple drugs in combination to prevent deleterious events on a potential critical patient who eventually did not require intensive care support after surgery, saving precious ICU resources especially scarce during COVID-19 surge.