Cardiac manifestations of malignant hyperthermia susceptibility

• Published in: Circulation (New York, N.Y.) N.Y.), 1978-11, Vol.58 (5), p.916-925
• Main Authors: Huckell, V F, Staniloff, H M, Britt, B A, Waxman, M B, Morch, J E
• Format: Article
• Language: English
• Subjects: Adult; Cardiac Catheterization; Electric Stimulation; Female; Heart - diagnostic imaging; Heart - physiopathology; Humans; Male; Malignant Hyperthermia - diagnosis; Malignant Hyperthermia - pathology; Malignant Hyperthermia - physiopathology; Muscle Contraction; Muscles - pathology; Muscles - physiopathology; Pain; Physical Exertion; Radionuclide Imaging; Tachycardia - complications; Thorax; Ventricular Fibrillation - complications
• ISSN: 0009-7322; 1524-4539
• DOI: 10.1161/01.CIR.58.5.916

Malignant hyperthermia is a disease resulting from defective cellular membranes, usually presenting as drug-induced pyrexic crises. We describe four patients with life threatening ventricular arrhythmias or chest pain in the absence of pyrexic crises. Three presented with life threatening arrhythmias and the fourth with severe atypical chest pain. Two patients had a family history of multiple sudden deaths. Resting CKs were elevated in three patients while CK-MB was elevated in one. Resting ECGs were abnormal in three. Three patients had recurrent ventricular tachycardia, two had recurrent ventricular fibrillation and multiple cardiac arrests. Cardiac catheterization showed abnormal left ventricular wall motion in two and minimal mitral valve prolapse in one while all had normal coronary arteries. Thallium-201 myocardial imaging demonstrated large perfusion defects in the patient with electrocardiographic Q waves and normal coronary arteries. Myocardial involvement has been demonstrated by clinical, electrocardiographic, hemodynamic, angiographic and myocardial imaging abnormalities. Malignant arrhythmias occurred in these patients in the absence of pyrexic crises or drug admininstration. Abnormal calcium release in the myocardium, as documented in skeletal muscle membranes, may be a unifying concept for the various manifestations described.