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

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Presentation transcript:

Expanding the electrical phenotype of NKX2-5 mutations: Ventricular tachycardia, atrial fibrillation, and complete heart block within one family Simone Jhaveri, MD, Peter F. Aziz, MD, FHRS, Elizabeth Saarel, MD, FHRS HeartRhythm Case Reports Volume 4, Issue 11, Pages 530-533 (November 2018) DOI: 10.1016/j.hrcr.2018.08.001 Copyright © 2018 Heart Rhythm Society Terms and Conditions

Figure 1 Pacemaker device interrogation for patient 1 showing atrial flutter at a cycle length of 160 ms, persistent despite antitachycardia pacing (arrow). HeartRhythm Case Reports 2018 4, 530-533DOI: (10.1016/j.hrcr.2018.08.001) Copyright © 2018 Heart Rhythm Society Terms and Conditions

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. HeartRhythm Case Reports 2018 4, 530-533DOI: (10.1016/j.hrcr.2018.08.001) Copyright © 2018 Heart Rhythm Society Terms and Conditions

Figure 3 Holter monitor for patient 2 demonstrating a run of ventricular tachycardia at 180 beats/min. HeartRhythm Case Reports 2018 4, 530-533DOI: (10.1016/j.hrcr.2018.08.001) Copyright © 2018 Heart Rhythm Society Terms and Conditions

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. HeartRhythm Case Reports 2018 4, 530-533DOI: (10.1016/j.hrcr.2018.08.001) Copyright © 2018 Heart Rhythm Society Terms and Conditions