Danon disease

  X-linked systemic disorder.

      Danon affects male patients earlier and more severely than female patients.

  Due to a deficiency of the lysosome-associated membrane protein 2 (
LAMP2)

  Characterized by
left ventricular hypertrophy, mental retardation, and skeletal myopathy.

  The final diagnosis is based on the genetic analysis of LAMP2 gene.

  There are no diagnostic ECG findings.

      Abnormal but nonspecific ECG findings are frequently seen.




ECG findings in Danon disease

  Wolff-Parkinson-White (WPW) pattern (68% of male, 27% of female patients) (common)

      WPW pattern is
NOT due to an accessory pathway.

      Seemingly delta wave is in fact due to
slurring of the initial part of QRS complex.

 
T wave inversion (common)

 
Voltage criteria for left ventricular hypertrophy (common)

  Bradycardia

  Atrioventricular block

  Paroxysmal atrial flutter and fibrillation

  Non-sustained ventricular tachycardia




References (including links to free full-text articles)

  J Community Hosp Intern Med Perspect 2017;7(2):107-114.

  Europace 2017;19(7):1204-1210.

  Tex Heart Inst J 2014;41(3):332-334.

  Int J Cardiol Heart Vessel 2013;2:1-7.

  J Hum Genet 2012;57(7):407-410.

  Am J Cardiol 2012;109(3):406-411.

  Genet Med 2011;13(6):563-568.

  JAMA 2009;301(12):1253-1259.





ECG 1. The ECG above belongs to a 21 years-old male with Danon disease.
WPW pattern (short PR interval,
slurred upstroke of the QRS complex mimicking delta wave),
increased QRS voltage (left ventricular hypertrophy pattern) and extensive T wave negativity are seen.

Drs. Torsten Konrad and Thomas Rostock have donated the above ECG to our website.

Click here for a more detailed ECG