What are the possible cardiac complications of McCune-Albright syndrome (MAS)?

Updated: Jan 17, 2019
  • Author: Gabriel I Uwaifo, MD; Chief Editor: George T Griffing, MD  more...
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Answer

More severe presentations of MAS are clearly associated with sudden death. Although no arrhythmias have been detected in individuals with MAS, this is the presumed mechanism of sudden death. The stimulatory G protein (see Pathophysiology) is one of the primary intracellular signal transducers of the beta-adrenergic receptor. Constitutive activation of adrenergic signaling could result in a refractory and pathologic arrhythmia.

Of particular concern is the possibility that the tachycardia resulting from severe hyperthyroidism may complicate or trigger a cardiac event. Hyperthyroidism could increase the risk of such events in susceptible individuals.


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