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Case Reports
. 2018 Aug 10;4(11):530-533.
doi: 10.1016/j.hrcr.2018.08.001. eCollection 2018 Nov.

Expanding the electrical phenotype of NKX2-5 mutations: Ventricular tachycardia, atrial fibrillation, and complete heart block within one family

Simone Jhaveri  1 , Peter F Aziz  1 , Elizabeth Saarel  1
Affiliations
Case Reports

Expanding the electrical phenotype of NKX2-5 mutations: Ventricular tachycardia, atrial fibrillation, and complete heart block within one family

Simone Jhaveri et al. HeartRhythm Case Rep. .
No abstract available

Keywords: Atrial fibrillation; Genotype-phenotype; Heart block; ICD; NKX2-5; Pacemaker; Sudden death; Ventricular arrhythmia.

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Figures

Figure 1
Figure 1
Pacemaker device interrogation for patient 1 showing atrial flutter at a cycle length of 160 ms, persistent despite antitachycardia pacing (arrow).
Figure 2
Figure 2
Progression of atrioventricular (AV) nodal disease in patient 2. A: Electrocardiogram (ECG) at 17 years showing sinus bradycardia and first-degree AV block with PR interval of 400 ms. B: ECG at 18 years showing complete heart block with AV dissociation and a narrow complex escape rhythm.
Figure 3
Figure 3
Holter monitor for patient 2 demonstrating a run of ventricular tachycardia at 180 beats/min.
Supplemental Figure
Supplemental Figure
Pacemaker device interrogation for patient 2. A: Demonstrating an episode of atrial fibrillation/flutter at a cycle length of 120 ms. B: Demonstrating 3.1% of total time in atrial tachycardia.
See this image and copyright information in PMC

References

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