Familial polymorphic ventricular arrhythmias
A quarter century of successful medical treatment based on serial exercise-pharmacologic testing
John D. Fisher, MD, FACCa,
Dennis Krikler, MD, FRCP, FACCa and
Katherine A. Hallidie-Smith, MB, FRCP, FACCa
a Division of Cardiovascular Diseases, Royal Postgraduate Medical School, Hammersmith Hospital (now Imperial College School of Medicine, Hammersmith Campus), London, United Kingdom
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Figure 1 Family pedigree. Consistent with an autosonal dominant trait with variable penetrance. Asterisk indicates episodes that began by 1978 (age 30 years).
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Figure 2 Electrocardiogram of the propositus.
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Figure 3 Exercise test in the propositus, showing increasing numbers of extrasystoles with increasing work load. At 400 kilopond meters/min (KPM/min), there were up to four successive polymorphic extrasystoles. The QTc interval remains normal.
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Figure 4 Serial exercise-pharmacologic testing. Effect of medications on work load–heart rate relations in the propositus. The end point was fatigue or ventricular arrhythmias (bigeminal or more). I.V. = intravenous; P.O. = oral.
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Figure 5 Plasma catecholamines before (PRE), during (EX = exercise) and after exercise (POST). The propositus had catecholamine measurements with two exercise tests. Shaded areas represent the normal ranges.
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