Malignant Hyperthermia Takes Another Young Life

In early May 2006 I received a phone call from the Medical Examiner’s office in New York City.  The reason for the call was a death of a young man a day or so previously in after surgery in an ambulatory surgery center on the upper East Side of Manhattan.  A 21 year old healthy young man (aside from controlled diabetes) had a procedure done under general anesthesia to correct a penile deformity and went into cardiac arrest at the end of the anesthetic. The young man was rushed to a nearby hospital emergency room where he was in cardiac arrest, with CPR in progress. What was remarkable was that his body temperature was over 107 degrees and his muscles were rigid.  Blood tests showed a severe acidosis.  However, there was no response to further resuscitative efforts and the patient was declared dead. 

That began a several year adventure that finally led to a settlement of a legal proceeding against the ambulatory center, the anesthesiologist, the company that employed the anesthesiologist among others. 

When I receive a call such as this, which fortunately, is rather uncommon, I hesitate to jump to the conclusion that malignant hyperthermia caused the problem. That is because information is frequently limited and often there is more to the story than appears superficially.  So, one of the first things I wanted to determine was whether the anesthetic was indeed a general anesthetic and if so what drugs were used.  I found out that the anesthesia consisted of propofol for induction of anesthesia, succinylcholine, to help place the breathing tube and isoflurane, one of the MH trigger anesthetics.  There was apparently no unusual reaction to the succinylcholine. (In many MH susceptibles, succinylcholine causes muscle rigidity rather than relaxation.)  I also want to know if the patient has other medical problems, previous history of anesthesia exposure or problems with anesthesia in other members of the family. All were negative. 

Well, I opined, that indeed this might represent a case of MH, but I still needed to know more and needed to see the anesthesia records and other medical information. However, I stated that there may be a possibility of determining whether the event was related to MH by sending a tissue sample or blood sample to the University of Pittsburgh DNA diagnostic laboratory or to Prevention Genetics a private company that performs genetic testing. Both places had been established, with partial support of MHAUS a year or two prior to the event. 

Because there are several deaths each year in ambulatory surgery centers, the medical examiner and indeed the state health department are very interested in understanding the cause of such bad events.  An autopsy was performed and aside from the muscle rigidity and hyperthermia, there were only minor other issues noted. 

Some time later I was contacted by a lawyer representing the family who wanted to know if I would be available to help them sort out what happened.  This meant that I would review the material and depositions if that were necessary. 

Over the next few years the picture of what happened became clearer, but would never truly explain how a case of MH was either undetected for a long while or proceeded at such lightning speed as to cause the cardiac arrest without time to begin treatment.

More details emerged from the depositions. According to the anesthesia record, there was no elevation of exhaled carbon dioxide, no increase in heart rate, no rigidity or major change in vital signs until the cardiac arrest took place. Importantly, body temperature was NOT being monitored. 

At the end of the three hour case, the patient was extubated (breathing tube removed), but because he was not responding he was reintubated and shortly thereafter went into cardiac arrest. There was no problem in reintubating the patient according to the records. The patient was shocked, although it was not clear if he was experiencing ventricular fibrillation at that moment.  Emergency response 911 was called while external chest compressions were being done. When the emergency medical technicians arrived, there was a slight delay as the personnel at the reception area were hesitant to let them access the ORs. However, very quickly they found the OR and took over the resuscitation. They found that the patient was hot and the muscles, particularly the jaw muscles were rigid. Wasting no time they moved the patient to the Emergency Department a few blocks away. Despite an hour of resuscitative efforts and dantrolene administration, the patient failed to respond. It should be noted that the surgery center did have dantrolene present and a protocol (very outdated however), but none was administered.   In addition, the ED physician felt that the anesthesia provider did not have a clear understanding of MH and its treatment. 

Within a few weeks, the DNA report arrived indicating that the patient had a well described mutation in the ryanodine receptor gene (c6617C>T, with an amino acid change p.Thr2206Met) known to be causal for MH. 

This past summer after multiple depositions and reports all parties agreed to a settlement of the legal case. This was small consolation for bereaved parents, family and friends. 

What happened here? This is difficult to know since nothing in the anesthesia record indicated something amiss until the very end of the case. This is very unlikely because for a patient’s temperature to reach 107 degrees, at least 20 to 30 minutes must elapse, in my opinion. In addition, prior to the temperature elevation there is always an increase in heart rate and in exhaled carbon dioxide. The muscle rigidity was clearly documented by the emergency medical technicians and the emergency medicine physicians and such rigidity also takes time to occur. 

If the patient’s body temperature were being monitored, perhaps this would have been a clue to the onset of malignant hyperthermia. Body temperature is a vital sign and is a de facto standard of care in anesthesia. 

 There definitely seemed to be a lack of understanding of MH on the part of those responsible for the anesthetic care of the patient and certainly this led to a delay in treatment. For example, there was no MHAUS wall chart in the ORs of the facility.

Such a blatant case of MH is tragic and very disturbing. MHAUS over the past 27 years has provided information, advice to all physicians and personnel involved directly or indirectly in management of patients undergoing surgery and anesthesia. Fortunately this, along with educational efforts by many others, has resulted in a great diminution of mortality from MH. We need to and will continue to do more. We have increased the number of patient/provider conferences. We are planning to introduce guidelines for training to prepare for an MH crisis. We will soon be introducing a “transfer of care” guideline developed in association for with the Ambulatory Surgery Foundation for those situations where patients experience MH in an ambulatory setting and need to be moved to a hospital. We are constantly revising and updating our educational material. Our experts are speaking on MH at many different venues throughout the US. Many of them write articles and chapters in medical and nursing textbooks. We are beginning to translate material into other languages.  

To me, the only way to insure that every anesthesia provider and every nurse are aware of MH and how best to recognize and treat the syndrome is to require annual MH preparedness drills at every site where anesthesia is delivered.   

 Another aspect of this case that bears mention is the ability to pinpoint the cause of death in this case because of the ability to perform DNA testing.  

The Board of Directors of MHAUS, the Professional Advisory Council, the hotline consultants, the staff and volunteers who work with us are dedicated to eliminating the threat of MH and mortality from the syndrome that preys on young and healthy people.  

For those who are interested in reading more about this case by accessing publicly available data, please contact the MHAUS office.        

            The mission of MHAUS is to promote optimum care and scientific understanding of MH and related disorders