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PICU Management of Malignant Hyperthermia
Episode 84 • 14th April 2024 • PICU Doc On Call • Dr. Pradip Kamat, Dr. Rahul Damania
00:00:00 00:29:31

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Welcome to PICU Doc On Call, A Podcast Dedicated to Current and Aspiring Intensivists.

  • Hosts:
  • Dr. Pradip Kamat: Children’s Healthcare of Atlanta/Emory University School of Medicine
  • Dr. Rahul Damania: Cleveland Clinic Children’s Hospital

Introduction:

  • Pediatric Intensive Care Unit (PICU) physicians passionate about medical education in the acute care pediatric setting
  • Episode focus: A case of a 23-month-old ex-28 week premie presenting with sudden high fever and rapidly rising ETCO2 during surgery

Case Presentation:

  • Presented by Dr. Rahul Damania
  • 23-month-old ex-28 week premie intubated during hernia repair surgery
  • Noticed rapidly rising ETCO2, unprovoked tachycardia, and elevated temperature
  • Transferred to PICU, exhibiting rigidity, clenched jaw, metabolic acidosis, and elevated lactate.
  • Consideration of Malignant Hyperthermia (MH) crisis

Key Points:

  • Elevated temperature, hypercapnia, metabolic acidosis, and unprovoked tachycardia raise concern for MH
  • Organized discussion on pathophysiology, clinical signs, symptoms, and management

Multiple Choice Question:

  • Diagnosis of MH crisis during scoliosis repair
  • Correct Answer: D) Sarcoplasmic reticulum
  • Dantrolene acts on the sarcoplasmic reticulum to inhibit calcium release, crucial in MH management

Clinical Presentation of MH Crisis:

  • Tachycardia, acidosis, muscle stiffness, and hyperthermia are hallmark features
  • Potential life-threatening complications underscore the urgency of recognition and treatment

Triggers and Pathophysiology of MH Crisis:

  • Triggered by inhalational agents and depolarizing neuromuscular blocking agents
  • Pathophysiology involves defective Ryanodine receptor leading to uncontrolled calcium release

Differential Diagnosis:

  • Includes sepsis, thyroid storm, pheochromocytoma, and neuroleptic malignant syndrome
  • Differentiation from similar conditions crucial for accurate management

Diagnostic Approach:

  • High clinical suspicion
  • Genetic testing (ryanodine receptor gene sequencing) and Caffeine Halothane Contracture Test (CHCT) for diagnosis
  • Immediate workup during crisis includes blood gas, lactate, CPK, CMP, and urine analysis

General Management Framework:

  • MH crisis is a medical emergency requiring rapid intervention
  • Dantrolene Na administration, supportive measures, and continuous monitoring in PICU
  • Utilization of Malignant Hyperthermia carts and involvement of specialized hotlines

Clinical Pearls and Pitfalls:

  • Early recognition is crucial.
  • Proper administration of Dantrolene Na without delay
  • Extended monitoring period in PICU to ensure stability

Conclusion:

  • Importance of recognizing and managing MH crisis
  • Feedback, subscription, and reviews encouraged
  • Website picudoconcall.org for additional resources

References:

  • Fuhrman & Zimmerman - Textbook of Pediatric Critical Care Chapter
  • Malignant Hyperthermia Association of the United States
  • What is MH?
  • [Managing a crisis](https://www.mhaus.org/ healthcare-professionals/managing-a-crisis/)
  • Rosenbaum HK, Rosenberg H. UpToDate: Malignant hyperthermia: diagnosis and management of acute crisis.

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