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. 2024 Sep 20;40(6):1519-1522.
doi: 10.1002/joa3.13150. eCollection 2024 Dec.

Atrial standstill in a young patient with ischemic stroke associated with inheritance of a novel HCN4 mutation

Abhinav B Anand  1 , Bhavik S Shah  1 , Girish R Sabnis  1 , Ajay U Mahajan  1
Affiliations

Atrial standstill in a young patient with ischemic stroke associated with inheritance of a novel HCN4 mutation

Abhinav B Anand et al. J Arrhythm. .

Abstract

We present a case of HCN4 gene mutation presenting with atrial standstill and stroke in the young.

Keywords: HCN4 gene; SCN5A gene; atrial standstill; familial sick sinus syndrome; young stroke.

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Conflict of interest statement

The authors have no conflict of interest to declare.

Figures

FIGURE 1
FIGURE 1
Twelve lead electrocardiogram shows heart rate of 45 beats per minute, junctional rhythm without retrograde conduction during the junctional beats. There are no visible p waves/fibrillatory wave preceding the QRS complex. Lower panel shows MV Doppler inflow velocities (left) and septal annulus TDI (right). There was presence of E wave without A wave indicating loss of atrial contractility. Similar findings were obtained at TV inflow and lateral mitral annulus TDI (not shown).
FIGURE 2
FIGURE 2
Intracardiac electrograms. There is absence of atrial electrograms on both coronary sinus and right atrium. HV interval is 48 ms. Pacing at maximum output from right atrium and coronary sinus did not lead to atrial capture/ presence of p waves on surface ECG.
FIGURE 3
FIGURE 3
Presence of biatrial late gadolinium enhancement on cardiac MRI shown in apical 4 chamber view.
FIGURE 4
FIGURE 4
Family pedigree of the patient. The paternal aunt underwent pacemaker implantation at the age of 48 years (further details not available).
See this image and copyright information in PMC

References

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