Anesthetic Management for Multiple Family Members with Myotonic Dystrophy for Interventional Cardiac Procedures—A Case Series

Abstract

Myotonic muscular dystrophy (MMD) is a rare autosomal dominant disorder that can complicate anesthetic management of patients. MMD is characterized by progressively worsening muscle loss and weakness, cardiac conduction abnormalities, cardiomyopathy, restrictive lung disease, obstructive sleep apnea, and delayed gastric emptying. Patients presenting with MMD for any surgical procedure present a management challenge to the anesthesiologist. Several reports of airway loss due to medication-mediated respiratory depression, sudden death due to dysrhythmias, aspiration of stomach contents, and prolonged intubation have been reported. We present a case series of three family members with MMD type 1 who presented for electrophysiologic assessment of the cardiac conduction system and possible pacemaker insertion. While there are reports of anesthetic management of patients with myotonic dystrophy for various procedures, our report is unique in that we were able to demonstrate variations of anesthetic management based on the procedure and variation in disease phenotype—differing severity between family members.

A Normal Electrocardiogram Does Not Exclude Infra-Hisian Conduction Disease in Patients With Myotonic Dystrophy Type 1

“…The incidence of acute and long-term complications associated with implantation of a PPM is significant [19][20], and should therefore be reserved for selected patients who are likely to benefit. Furthermore, implantation of PPMs in patients with myotonic dystrophy might be more challenging because of associated respiratory muscle involvement, limiting the provision of sedation/anaesthesia [21]. Nonetheless, although the present study highlights the limited ability of ECG in identifying the presence of His-Purkinje conduction system disease, further research is necessary to clarify whether patients with normal HV interval on EPS and yet abnormal ECG are at low risk of life-threatening bradyarrhythmic events.…”

Dexmedetomidine infusion for sedation in a patient with myotonic dystrophy*

“…The current literature on anaesthetic management using dexmedetomidine in patients with myotonic dystrophy consists of a case series describing dexmedetomidine sedation for interventional cardiology procedures [6] and for sedation with neuraxial anaesthesia [7]. There have also been reports of the use of dexmedetomidine as an adjuvant to general anaesthesia in order to reduce opioid requirements in the peri‐operative period [8].…”