1. Transfer Guidelines for Malignant Hyperthermia
Marilyn Green Larach M.D.
Senior Research Associate
The North American
Malignant Hyperthermia Registry of MHAUS
Because successful management of an MH crisis demands fast reactions for successful outcomes, we’ve developed transfer guidelines.
Contact me by at if you wish an annotated copy of this lecture. Please do not share electronically.

2. Disclosure Statement Dr. Larach received an MHAUS honorarium
To support guideline development
Both MHAUS and the ASF sell transfer of care posters
No financial benefit to Dr. Larach
MHAUS = Malignant Hyperthermia Association of the United States
ASF = Ambulatory Surgery Foundation, affiliated with the Ambulatory Surgery Center (ASC) Association

3. Goals of Talk Introduce Transfer of Care Guidelines
Discuss Need for Guidelines
Provide Overview of Content
Review MH Presentation & Treatment
Goals of talk: These are guidelines and not protocols. Also, to share with you clinically relevant results of Registry study concerning MH presentation and treatment.
Three references: Transfer of Care poster, 2008 MH Treatment poster, 2010 Anesthesia and Analgesia Registry publication.

4. Assumes an ASC using MH Triggers has Available:
Anesthesia Care Provider
36 Vials of Dantrolene
MHAUS Emergency Therapy Poster
MH Crisis Drills
Please do not share electronically.
Photographic credit for MH drill picture to Outpatient Surgery Magazine (February 2010, p. 30)
Credit for Emergency Therapy Poster to MHAUS

5. Key elements to successful MH treatment: team, MH cart with dantrolene, appropriate monitors
Photographic credit to Outpatient Surgery Magazine Online, (Fielder C. Managing malignant hyperthermia. 2010; 9:30)

6. Actual Transfer of Care Poster--Please do not share electronically.

7. Development of Guidelines for Emergent MH Transfers
Joint Consensus Document
ASF
MHAUS
13 Panel Members
Anesthesiologists
CRNA
Emergency Medicine Physician
Emergency Medical Technician
ASC nurse/administrator
ASF nurse/administrator
Transfers between ASC and Acute Care Hospital
2 year process under the direction of Drs. Sharon Dirksen, Henry Rosenberg, and me.
8 Anesthesiologists included physicians with expertise in MH, pediatric and adult anesthesia, in-patient and ASC anesthesia and a SAMBA representative.

8. Guideline Goals
Assist ASC to prepare own individual emergent MH transfer plan predicated on the facilities and capabilities of the:
ASC
Emergency transport services
Receiving hospital
Transfer of Care poster
Develop a transfer plan specific for MH that allows for continuation of care for these critically ill patients.

9. Guidelines and Not Protocol
ASC Locations Vary
Staff Resources
Lab Resources
Distance to Receiving Hospital
Is your ASC close to or far away from the receiving hospital?
Illustration of the variability of ASC’s even within one state. The upper Ambulatory Surgery Center is embedded in a large new outpatient facility of the University of Pennsylvania. The lower ASC is free-standing and located next to a corn field, but it is also less than a mile away from its receiving teaching hospital, Penn State Hershey Medical Center.
Photographic credit: Hospital of the University of Pennsylvania and Penn State Hershey Medical Center (Hershey Outpatient Surgery Center)

10. Guidelines and Not Protocol
Emergency Transport Services Vary
Availability
Weather
Distance to Receiving Hospital
Severity of Patient Condition
Photographic credit for air ambulance: Penn State Hershey Medical Center
Photographic credit for ground ambulance: Wikipedia

11. Guidelines and Not Protocol
Receiving Hospitals Vary
Facilities
Personnel
Hospitals come in different sizes with different facilities and services
Photographic credit: Wikipedia Commons: University Hospitals of Cleveland
Photographic credit: Steve Rasmussen, Rural Roads, Summer 2010 (National Rural Health Association) [Kiowa County Memorial Hospital]

12. HIPAA allowable
What are the first signs of an MH reaction
Photography credit to the Post and Courier (Charleston, SC)

13. Recognition of Suspected MH
First signs
Hypercarbia
Sinus tachycardia
Masseter spasm
Temperature abnormalities may be early MH sign
Most common pattern
Respiratory acidosis and muscular abnormalities
Increased temperature or rapidly increasing temperature first to third MH sign in 64% of patients with median temperature maximum of 39.1C
Respiratory acidotic pattern: inappropriate hypercarbia, tachypnea, ETCO2>55 torr, arterial pCO2>60 torr, or arterial pH<7.25
Muscular pattern: masseter spasm, generalized muscular rigidity, cola-colored urine, peak CK >10,000 U/L or peak K+>6.0 mEq/L
MH Clinical Presentation Paper: Larach et al., Clinical presentation, treatment, and complications of malignant hyperthermia in North America from 1987 to 2006, Anesth Analg 2010; 100:

14. How do we treat an MH crisis?
Photographic credit to the Post and Courier (Charleston, SC)

15. Begin Treatment Declare MH Emergency Discontinue Triggering Agents
100% Oxygen at High Flow
Give Dantrolene
2.5 mg/kg IV push
Titrate to effect
Initiate Transfer Plan
Whenever possible, don’t move unless
clinician judges patient to be stable
Transfer of Care Poster
Dantrolene: 36 vials of dantrolene sodium for injection must be available wherever MH trigger agents are used
MH Clinical Presentation Paper Data:
Dantrolene: Initial dantrolene dose for 229 MH Events was a median of 2.4 (1st quartile 1.9, 3rd quartile 2.8, range ) mg/kg
*Transfer of Care Poster: *If possible, the patient should be moved when, according to the clinician’s judgment, the patient is stable.”

16. Key Patient Stability Indicators
ETCO2 is declining or normal
HR is stable or decreasing
No ominous dysrhythmias
Temperature is declining
Generalized muscular rigidity is resolving (if present)
IV dantrolene administration has begun
Transfer of Care Poster

17. MH Morbidity and Mortality
Consciousness Level Change/Coma
Cardiac Dysfunction
Pulmonary Edema
Renal Dysfunction
Disseminated Intravascular Coagulation
Hepatic Dysfunction
Other
Relapse
Death
MH Clinical Presentation Paper Data:
In a Registry study of 181 MH events, 35% of all MH cases had complications including 19% relapse rate (new clinical sign of MH>120 minutes after initial presentation). There were 8 cardiac arrests and 4 deaths.
Higher maximum temperatures significantly increase the likelihood of all MH complications including DIC that is associated with a 50-fold increased likelihood of cardiac arrest and and an 89-fold increased likelihood of death

18. Factors Increasing MH Complication Likelihood
Increased time 1st sign to 1st dantrolene
For every 30 minute increase in the interval between 1st MH sign and 1st dantrolene dose, the complication likelihood increased 1.6 times.
Increased maximal temperature
For every 2C increase in maximal temperature, the complication likelihood increased 2.9 times.
MH Clinical Presentation Paper Data
To avoid MH complications, you need to give dantrolene early and you need to control temperature.

19. Transport Team Type varies with scenario & transport time Capabilities
Ventilatory support
Cardiopulmonary & temperature monitoring
Fluid resuscitation
Medication administration
Life support
Phone communication
May require ASC anesthesia staff
Transfer of Care poster
Medication administration includes but not limited to: IV dantrolene, non-depolarizing muscle relaxants, sedatives/hypnotics
Phone communication: transport command center and MH Hotline (1-800-MH-Hyper or )

20. Receiving Health Care Facility
Existing transfer agreement
Inpatient capabilities
Adult/Pediatric Critical Care
Continuous temperature and cardiopulmonary monitoring
Non-invasive/invasive cooling
Continuous sedation
Dantrolene
Dysrhythmia treatment
Hemodialysis
Transfer of Care poster
Transfer agreements must be arranged in advance with a facility that meets state, federal and accreditation requirements

21. Receiving Health Care Facility Consultant Availabilities
Anesthesiology
Critical Care
Hematology
Surgery
Nephrology
Medical Toxicology
Transfer of Care poster:
Hematology: MH complicated by DIC in 13% of cases (MH clinical presentation paper)
Surgery: Complete aborted surgery, treat compartment syndrome
Nephrology: Renal dysfunction (due to ATN) complicated 7% of MH cases
Toxicology: not MH but drug/recreational drug interactions

22. Report Data from ASC Cardiovascular signs Temperature and site
Minute ventilation with ETCO2
Dantrolene amount given & response
Muscular rigidity status
Electrolytes
I.V. site
Urinary catheter & urine color
Transfer of Care paper

23. Communication Coordination
Direct communication concerning patient status & admission location between
Anesthesia care provider at ASC
AND
Physicians accepting care at Receiving Hospital
Transfer of Care poster

24. Transfer Decisions by On-Site ASC Health Care Professional
Timing of Transfer
Choice of Transfer Team
Choice of Receiving Hospital
Factor In:
Transport time
Bed availability
Clinical stability
Transfer of Care Poster
May require ASC personnel and equipment to safely transfer MH patient
(Personal Opinion: In most cases, health care professional should be the person with the greatest amount of anesthesia expertise)

25. Implementation of Transfer Decision
Don’t delay transfer pending specific personnel or equipment availability if emergent transfer is mandatory
**Accompany patient with appropriate medications and equipment if needed to serve the best interests of the patient
**Personal Recommendation
Transfer of Care protocol
May require ASC personnel and equipment to safely transfer MH patient
**(Personal recommendation: In most cases, the health care professional should be the person with the greatest amount of anesthesia expertise. Work out command issues within the transport vehicle prior to an MH event.)
*Not a quote from the transfer guidelines)

26. Photographic credit to the Post and Courier (Charleston, SC)

27. Photographic credit to the Post and Courier (Charleston, SC)

28. Create Your Own ASC MH Transfer Plan
Start with Guidelines
Research available transport teams
Consult with physicians at referral hospitals
Research your interaction with the transport team if you want to treat your patient in the transport vehicle.

30. Clinical Characteristics
24.1% Emergency
Sux 3.8 times more often
Orthopedic, ENT, General Surgery
Sux 1.9 times more often
Temperature Monitoring (n=259)
14% skin liquid crystal sole probe
In 10 patients, skin liquid crystal didn’t trend with core temp probe
MH Clinical Presentation Paper
99.3% pulse ox
96.2% capnography
90.9% temp monitoring
For skin liquid crystal temperature probe non-trenders: Tmax was 40.0 deg C
26 with no temp probes: no sign’t difference in induction-1st adverse sign. However, for 5/26 patients there was a rapidly inreasing temperature or increased temperature with median max temp of 39.1 deg C

31. Anesthetic Triggers (n=284)
MH Clinical Presentation Paper
Type of anesthetic agent administered prior to 284 malignant hyperthermia events. In two patients with missing data, we know that a volatile anesthetic was discontinued but whether succinylcholine was also used was not documented.
0.7%=2 cases
0.4%=1 case The no sux, no volatile patient was 2 years old with a suspected family history of malignant hyperthermia susceptibility undergoing a dental procedure.
All but one administration of succinylcholine was intravenous.
Volatile anesthetics included: halothane (15.6%), enflurane (2.8%), isoflurane (57.8%), desflurane (12.1%), and sevoflurane (20.6%).

32. Presentation 99% Respiratory Acidosis 26% Metabolic Acidosis
80% Muscular Abnormalities
MH Clinical Presentation Paper
All but one of the metabolic acidosis cases had respiratory acidosis as well. The majority did not have metabolic acidosis.

33. Clinical Presentation Pattern (n=196)%
+Respiratory +Metabolic +Muscular
20.4
+Respiratory +Metabolic –Muscular
5.1
+Respiratory –Metabolic +Muscular
58.2
–Respiratory +Metabolic +Muscular
0.5
+Respiratory –Metabolic –Muscular
15.3
–Respiratory –Metabolic +Muscular
MH Clinical Presentation Paper
This Table lists the distribution of the 196 malignant hyperthermia events with data permitting evaluation for the presence () or absence () of respiratory acidosis, metabolic acidosis, and/or muscular presentations.
Presentation type not associated with varying combinations of volatile anesthetics and succinylcholine.

34. Dantrolene Dosage (n=229)
Dose
Median
1st Q
3rd Q
Range
Initial (mg/kg)
2.4
1.9
2.8
Initial (vials) 8 3 11
1 - 58
Total (mg/kg)
5.9
3.0
10.0
Total (vials) 17 7 36
MH Clinical Presentation Paper
This Table contains data on dantrolene doses used during 229 malignant hyperthermia events.
Initial doses are reported for 228 events because there was 1 unreported initial dose.
Initial number of vials=unreported data in paper
Total dose: in 25% of patients more than 36 dantrolene vials were required
Dantrolene is supplied in 20 mg/vial of lyophilized crystals that are mixed with sterile water prior to use.

35. Adjunctive Treatment (n=284)%
Hyperventilation with FiO2=1 87
IV fluid loading 77
Active cooling 70
Bicarbonate 54
Anesthesia circuit change 48
Mannitol 34
Furosemide 32
Glucose and insulin 14
MH Clinical Presentation Paper
Time from 1st sign to volatile anesthetic discontinuation was 10 minutes with 1st quartile 2, 3rd quartile 30 minutes.
Less frequent but more than 1 subject and in order of decreasing frequency: CPR, calcium, lidocaine, procainamide, epinephrine, vasopressors, dopamine, defibrillation, norepinephrine, atropine, bretylium, hemodialysis, phenylephrine (see Table 5).
Treatment frequency correlated with the severity of abnormal findings. (See Appendix B).