Denborough and Lovell [1] first described malignant hyperthermia (MH) as an inherited syndrome in 1960. At present, it is generally accepted that MH is triggered by many anesthetics. Succinylcholine chloride (SCC) and volatile anesthetics have been especially implicated as important triggering drugs [2,3]. With these triggering drugs, induced hypermetabolism produces tachycardia, increased O2 consumption and CO2 production, premature ventricular contraction, hypotension and hypertension, cyanosis, tachypnea, muscle rigidity, and hyperthermia as the signs of MH. Also seen as complications of MH are electrolyte imbalances, myoglobinuria, hyperkalemia, creatine phosphokinase (CPK) elevation, impaired coagulation, renal failure, and severe metabolic and respiratory acidosis.

Clinical Classification and Incidence of Malignant Hyperthermia in Japan

The Vienna Malignant Hyperthermia (MH) Investigation Unit (which functions as the Austrian National MH Registry) was founded in 1976 by Karl Steinbereithner and Paul Sporn. The unit employs three anesthesiologists, one nurse, two technicians, and one secretary (all part-time) and is funded by the Austrian Government, Ministry of Health, the Ludwig-Boltzmann Society, and the University of Vienna Diagnostic testing of MH probands and family members has been performed since 1976. Initially, halothane and halothane-caffeine tests were done, but since the founding of the European MH Group in 1983, the European Protocol [1] has been used. In 1985 a program to provide safe anesthesia for MH patients was established [2]. A hot-line for MH-related problems has operated 24 hours a day since 1987. This paper summarizes the results of our studies on epidemiology and clinical course of MH in Austria. Only patients evaluated according to the European Protocol are included in this report.

Malignant Hyperthermia in Austria 1984-1993

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Malignant Hyperthermia in Austria 1984-1993