Electrocardiographic Findings, Arrhythmias, and Left Ventricular Involvement in Familial ST-Depression Syndrome

Alex Hørby Christensen, Christoffer Rasmus Vissing, Adrian Pietersen, Jacob Tfelt-Hansen, Thomas Hartvig Lindkær Jensen, Steen Pehrson, Niels Christian Foldager Sandgaard, Finn Lund Henriksen, Kasper Karmark Iversen, Henrik Kjærulf Jensen, Morten Salling Olesen, Henning Bundgaard*

*Corresponding author for this work

Research output: Contribution to journalJournal articleResearchpeer-review

Abstract

BACKGROUND: Familial ST-depression syndrome is an inherited disease characterized by persistent, nonischemic ST-deviations, and risk of arrhythmias and heart failure. We aimed at further characterizing the ECG, arrhythmias, and structural characteristics associated with this novel syndrome. METHODS: Retrospective analysis of data from consecutive families with familial ST-depression Syndrome in Denmark. ECG features, prevalence and type of arrhythmias, occurrence of systolic dysfunction, and medium-term outcome were analyzed. RESULTS: Forty affected individuals (43% men; mean age at diagnosis 49.1 years) from 14 apparently unrelated families with ≥2 affected members were included. Autosomal dominant inheritance was observed in all families. The ECG phenotype seemed to develop in prepuberty and the ST-deviations were persistent and most pronounced in leads V4/V5/II, respectively. Serial ECG analyses showed stable to slow progression of the ECG phenotype. Exercise accentuated the ST-deviations with a maximum difference between rest/stress (mean) of -117 μV in lead V5. During a mean follow-up of 9.3±7.1 years 5 (13%) patients developed sustained ventricular arrhythmias or (aborted) sudden cardiac death, 10 (25%) developed atrial fibrillation, 2 (5%) other supraventricular arrhythmias, and 10 (25%) were diagnosed with left ventricular ejection fraction ≤50%. The ventricular arrhythmias were polymorphic with relatively short-coupled premature ventricular contractions at onset (300-360 ms); no QT prolongation was observed. Seven patients had at least one catheter ablation; 5 for supraventricular arrhythmias and 2 for ventricular arrhythmias. Males experienced more arrhythmic end points than females (P<0.01). CONCLUSIONS: The familial ST-depression ECG phenotype is stable to slowly progressive after medium-term follow-up. Clinically, both supra- and ventricular arrhythmias are common; as are some degree of left ventricular systolic dysfunction. Familial ST-depression represent a novel inherited cause of polymorphic ventricular tachycardia.

Original languageEnglish
Article numbere010688
JournalCirculation: Arrhythmia and Electrophysiology
Volume15
Issue number4
ISSN1941-3149
DOIs
Publication statusPublished - 1. Apr 2022

Keywords

  • atrial fibrillation
  • catheter ablation
  • heart failure
  • prognosis
  • syndrome
  • Ventricular Function, Left
  • Humans
  • Ventricular Premature Complexes
  • Depression
  • Male
  • Stroke Volume
  • Electrocardiography
  • Female
  • Retrospective Studies

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