Expert: Ronald Levy, M.D. - 3/8/2011

Question
I have mitochondrial myopathy and require surgery frequently for a subglotic stenosis.  Last time my anesthesiologist told me having surgery could be a problem  due to malignant hypothermia.  He stated I should tell my children to always let the Dr know I have the disease in case they could have a problem with anesthesia. I have been trying to find out exactly what the problem is on line without much success.  I would like an answer before I try to explain it to my children.  I have a pregnant daughter who could require surgery.  I am now worried.  Thank you for your answer.

Answer
Malignant HypERthermia (MH) is a disease whereby you ahve an abnormal response to certain anesthestic agents (or triggers), namely succinylcholine, all the inhalational agents and a few other minor drugs. It is a potentially life threatening complication and is very rare but is hereditary. You didn't say if you actually had an MH event or you are just susceptible. I have included a link to the MH hotline website which should be able to answer most of your questions.

http://patients.mhaus.org/

Hope this helps,

Ronald Levy, MD
Professor of Anesthesiology
UTMB-Galveston