Wednesday, May 28, 2008


An otherwise healthy 20 year old presents with palpations on exercise. He was put on cardiac monitoring and this pattern was seen; it was short-lived and terminated spontaneously. On a further history, you find that a few of his family members have had sudden cardiac death. They follow an autosomal dominant inheritance. All other organ systems are negative. The patient is not taking any medications.

Challenge: What does the EKG show? What is the most likely cause?

Image is in the public domain.


Alex said...


long qt syndrome - familial? or some sort of channel-opathy

Craig said...

good job alex! sorry about late got a virus.

The EKG shows torsades de pointes (twisting of the points), a stereotypic arrhythmia associated with long QT syndrome. It is a polymorphic ventricular tachyarrhythmia, and can progress to ventricular fibrillation. The genetic inheritance suggests Romano-Ward syndrome (long QT). The other long QT syndrome is Jervell and Lange-Nielsen syndrome which is autosomal recessive and associated with sensorineural deafness. Torsades can also be precipitated by drugs that prolong the QT.

Sources: UpToDate; Wikipedia.