Sinus node dysfunction requiring permanent pacemaker implantation in a young adult with Klinefelter syndrome

The American Journal of Case Reports
Ahmet KaragözOsman Bektaş

Abstract

Klinefelter syndrome is the most common genetic cause of male infertility and affects approximately 1 in 500 live births. Although accompanying cardiac disorder is not a specific feature of Klinefelter syndrome, rarely associated anomalies such as mitral valve prolapse, atrial septal defect, ventricular septal defect, tetralogy of Fallot, patent ductus arteriosus, and hypertrophic obstructive cardiomyopathy have been reported. A clear association between Klinefelter syndrome and arrhythmic disorders has not yet been demonstrated. We report a case of a sinus node dysfunction that required permanent pacemaker implantation in a young adult with Klinefelter syndrome. The patient was consulted to cardiology clinic due to bradycardia. On physical examination, no cardiac abnormality was detected except for bradycardia. Holter results showed sinus arrhythmia with a minimum heart rate of 33 bpm and maximum of 154 Bpm. There were 3612 ventricular premature beats, 30 ventricular pairs, 804 supraventricular premature beats, 7 supraventricular pairs, and 4 supraventricular runs, the longest of which was 5 beats. The patient had defined dizziness and nausea during Holter monitoring. Electrophysiological study (EPS) was planned because existi...Continue Reading

References

Feb 8, 2003·The Journal of Clinical Endocrinology and Metabolism·Anders BojesenClaus Højbjerg Gravholt
Nov 29, 2008·Congenital Heart Disease·Michael R HainstockBrent J Barber
Jun 19, 2012·Fertility and Sterility·Rebecca Z Sokol
Oct 25, 2012·International Journal of Cardiology·Daniela PasqualiAntonio Cittadini

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Citations

Mar 5, 2017·Journal of Endocrinological Investigation·A E CalogeroUNKNOWN Klinefelter ItaliaN Group (KING)
Feb 23, 2019·Expert Review of Endocrinology & Metabolism·Giacomo AccardoDaniela Pasquali
Feb 14, 2018·Endocrine Reviews·Claus H GravholtAnne Skakkebæk
Jul 28, 2016·The American Journal of Case Reports·Yong Tae ParkJae-Cheol Jo

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Methods Mentioned

BETA
X-ray
testosterone replacement therapy

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