PICU Management of Malignant Hyperthermia

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 MedicineDr. Rahul Damania: Cleveland Clinic Children’s HospitalIntroduction:Pediatric Intensive Care Unit (PICU) physicians passionate about medical education in the acute care pediatric settingEpisode focus: A case of a 23-month-old ex-28 week premie presenting with sudden high fever and rapidly rising ETCO2 during surgeryCase Presentation:Presented by Dr. Rahul Damania23-month-old ex-28 week premie intubated during hernia repair surgeryNoticed rapidly rising ETCO2, unprovoked tachycardia, and elevated temperatureTransferred to PICU, exhibiting rigidity, clenched jaw, metabolic acidosis, and elevated lactate.Consideration of Malignant Hyperthermia (MH) crisisKey Points:Elevated temperature, hypercapnia, metabolic acidosis, and unprovoked tachycardia raise concern for MHOrganized discussion on pathophysiology, clinical signs, symptoms, and managementMultiple Choice Question:Diagnosis of MH crisis during scoliosis repairCorrect Answer: D) Sarcoplasmic reticulumDantrolene acts on the sarcoplasmic reticulum to inhibit calcium release, crucial in MH managementClinical Presentation of MH Crisis:Tachycardia, acidosis, muscle stiffness, and hyperthermia are hallmark featuresPotential life-threatening complications underscore the urgency of recognition and treatmentTriggers and Pathophysiology of MH Crisis:Triggered by inhalational agents and depolarizing neuromuscular blocking agentsPathophysiology involves defective Ryanodine receptor leading to uncontrolled calcium releaseDifferential Diagnosis:Includes sepsis, thyroid storm, pheochromocytoma, and neuroleptic malignant syndromeDifferentiation from similar conditions crucial for accurate managementDiagnostic Approach:High clinical suspicionGenetic testing (ryanodine receptor gene sequencing) and Caffeine Halothane Contracture Test (CHCT) for diagnosisImmediate workup during crisis includes blood gas, lactate, CPK, CMP, and urine analysisGeneral Management Framework:MH crisis is a medical emergency requiring rapid interventionDantrolene Na administration, supportive measures, and continuous monitoring in PICUUtilization of Malignant Hyperthermia carts and involvement of specialized hotlinesClinical Pearls and Pitfalls:Early recognition is crucial.Proper administration of Dantrolene Na without delayExtended monitoring period in PICU to ensure stabilityConclusion:Importance of recognizing and managing MH crisisFeedback, subscription, and reviews encouragedWebsite picudoconcall.org for additional resourcesReferences:Fuhrman & Zimmerman - Textbook of Pediatric Critical Care ChapterMalignant Hyperthermia Association of the United StatesWhat 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.