Wolff-Parkinson-White Syndrome (cont.)
What other heart conditions is Wolff-Parkinson-White syndrome associated with?
Wolff-Parkinson-White syndrome often occurs with other structural abnormalities of the heart or underlying heart disease. The
most common heart defect associated with the condition is Ebstein anomaly, which affects the valve that allows blood to flow from the right atrium to the right ventricle (the tricuspid valve). Additionally, Wolff-Parkinson-White syndrome can be a component of several other genetic syndromes, including hypokalemic periodic paralysis (a condition
that causes episodes of extreme muscle weakness), Pompe disease (a disorder characterized by the storage of excess glycogen), and tuberous sclerosis (a condition that results in the growth of noncancerous tumors in many parts of the body).
How common is Wolff-Parkinson-White syndrome?
Wolff-Parkinson-White syndrome affects 1 to 3 in 1,000 people worldwide. Only a small fraction of these cases appear to run in families.
Wolff-Parkinson-White syndrome is a common cause of an arrhythmia known as paroxysmal supraventricular tachycardia. Wolff-Parkinson-White syndrome is the most frequent cause of this abnormal heart rhythm in the Chinese population, where it is responsible for more than 70 percent of cases.
What genes are related to Wolff-Parkinson-White syndrome?
Mutations in the PRKAG2 gene cause Wolff-Parkinson-White syndrome.
A small percentage of all cases of Wolff-Parkinson-White syndrome are caused by mutations in the PRKAG2 gene. Some people with these mutations also have features of hypertrophic cardiomyopathy, a form of heart disease that enlarges and weakens the heart (cardiac) muscle. The PRKAG2 gene provides instructions for making a protein that is part of an enzyme called AMP-activated protein kinase (AMPK).
This enzyme helps sense and respond to energy demands within cells. It is likely
involved in the development of the heart before birth, although its role in this
process is unknown.
Researchers are uncertain how PRKAG2 mutations lead
to the development of Wolff-Parkinson-White syndrome and related heart
abnormalities. Research suggests that these mutations alter the activity of
AMP-activated protein kinase in the heart, although it is unclear whether the
genetic changes overactivate the enzyme or reduce its activity. Studies indicate
that changes in AMP-activated protein kinase activity allow a complex sugar called glycogen to build up abnormally within cardiac muscle
cells. Other studies have found that altered AMP-activated protein kinase
activity is related to changes in the regulation of certain ion channels in the
heart. These channels, which transport positively charged atoms (ions) into and
out of cardiac muscle cells, play critical roles in maintaining the heart's
normal rhythm.
In most cases, the cause of Wolff-Parkinson-White syndrome is unknown.
Next: How do people inherit Wolff-Parkinson-White syndrome? »
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