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

Karagöz A, Dikbaş O, Teker E, Vural A, Günaydın ZY, Bektaş O - Am J Case Rep (2015)

Bottom Line: Holter results showed sinus arrhythmia with a minimum heart rate of 33 bpm and maximum of 154 Bpm.Electrophysiological study (EPS) was planned because existing findings indicated risk of cardiac syncope.Findings of EPS were interpreted as sinus node dysfunction.

View Article: PubMed Central - PubMed

Affiliation: Department of Cardiology, Giresun University, Giresun, Turkey.

ABSTRACT

Background: 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.

Case report: 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 existing findings indicated risk of cardiac syncope. Findings of EPS were interpreted as sinus node dysfunction. A permanent pacemaker implantation was performed and the patient has been free of symptoms since.

Conclusions: This concomitance should be kept in mind when examining patients with Klinefelter syndrome with bradycardia and/or syncope. It is easily mistaken for epilepsy, which is a commonly encountered abnormality in Klinefelter syndrome.

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Electrophysiological study indicated sinus node dysfunction.
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f3-amjcaserep-16-136: Electrophysiological study indicated sinus node dysfunction.

Mentions: The patient had a history of epilepsy, but he had been free of seizures for a long time and the characteristics of syncope attack depicted by him were not compatible with a seizure. The patient underwent EPS. BCL (basal cycle length), AH, and HV intervals were 1010 msec, 89 msec, and HV 59 msec, respectively (Figure 3). Sinus node recovery time (SNRT) and corrected sinus node recovery time (cSNRT) were 1400–1600 msec and 390–590 msec, respectively. Atrioventricular Wenckebach interval was 520 msec (Figure 4). After administration of atropine, BCL, SNRT, and AVW intervals were 800 msec, 880 msec, and 380 msec, respectively.


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

Karagöz A, Dikbaş O, Teker E, Vural A, Günaydın ZY, Bektaş O - Am J Case Rep (2015)

Electrophysiological study indicated sinus node dysfunction.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4362339&req=5

f3-amjcaserep-16-136: Electrophysiological study indicated sinus node dysfunction.
Mentions: The patient had a history of epilepsy, but he had been free of seizures for a long time and the characteristics of syncope attack depicted by him were not compatible with a seizure. The patient underwent EPS. BCL (basal cycle length), AH, and HV intervals were 1010 msec, 89 msec, and HV 59 msec, respectively (Figure 3). Sinus node recovery time (SNRT) and corrected sinus node recovery time (cSNRT) were 1400–1600 msec and 390–590 msec, respectively. Atrioventricular Wenckebach interval was 520 msec (Figure 4). After administration of atropine, BCL, SNRT, and AVW intervals were 800 msec, 880 msec, and 380 msec, respectively.

Bottom Line: Holter results showed sinus arrhythmia with a minimum heart rate of 33 bpm and maximum of 154 Bpm.Electrophysiological study (EPS) was planned because existing findings indicated risk of cardiac syncope.Findings of EPS were interpreted as sinus node dysfunction.

View Article: PubMed Central - PubMed

Affiliation: Department of Cardiology, Giresun University, Giresun, Turkey.

ABSTRACT

Background: 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.

Case report: 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 existing findings indicated risk of cardiac syncope. Findings of EPS were interpreted as sinus node dysfunction. A permanent pacemaker implantation was performed and the patient has been free of symptoms since.

Conclusions: This concomitance should be kept in mind when examining patients with Klinefelter syndrome with bradycardia and/or syncope. It is easily mistaken for epilepsy, which is a commonly encountered abnormality in Klinefelter syndrome.

Show MeSH
Related in: MedlinePlus