TY - JOUR
T1 - The Novel Familial ST-Depression Syndrome - Current Knowledge and Perspectives
AU - Christensen, Alex Hørby
AU - Bundgaard, Henning
N1 - Funding Information:
The AP Møller Foundation (H. Bundgaard), The Research Foundation at Rigshospitalet (H. Bundgaard), The Independent Research Fund Denmark (0134–00363B, A.H. Christensen), The Novo Nordisk Foundation Denmark (NNF20OC0065799, A.H. Christensen), and NordForsk supported this study.
PY - 2023/9
Y1 - 2023/9
N2 - Familial ST-depression syndrome represents a novel inherited disease characterized by nonischemic ST-segment depressions in multiple leads. The ECG phenotype appears to debut around puberty, while the typical onset of arrhythmias occurs around 50 years of age. Clinical manifestations include supraventricular arrhythmias, fast polymorphic ventricular tachycardia, sudden cardiac death, and left ventricular systolic dysfunction. The optimal treatment is unknown but asymptomatic individuals without red flags may not need treatment. In contrast, ICD implantation should be considered in patients with probable arrhythmic syncope and in those fulfilling general criteria for ICD treatment. Future research should focus on establishing the disease prevalence, optimizing risk stratification and treatment, and elucidating the underlying genetic etiology.
AB - Familial ST-depression syndrome represents a novel inherited disease characterized by nonischemic ST-segment depressions in multiple leads. The ECG phenotype appears to debut around puberty, while the typical onset of arrhythmias occurs around 50 years of age. Clinical manifestations include supraventricular arrhythmias, fast polymorphic ventricular tachycardia, sudden cardiac death, and left ventricular systolic dysfunction. The optimal treatment is unknown but asymptomatic individuals without red flags may not need treatment. In contrast, ICD implantation should be considered in patients with probable arrhythmic syncope and in those fulfilling general criteria for ICD treatment. Future research should focus on establishing the disease prevalence, optimizing risk stratification and treatment, and elucidating the underlying genetic etiology.
KW - Arrhythmia
KW - Atrial fibrillation
KW - Electrocardiography
KW - Polymorphic ventricular tachycardia
KW - Sudden cardiac death
UR - http://www.scopus.com/inward/record.url?scp=85162877659&partnerID=8YFLogxK
U2 - 10.1016/j.ccep.2023.04.008
DO - 10.1016/j.ccep.2023.04.008
M3 - Review
C2 - 37558304
AN - SCOPUS:85162877659
SN - 1877-9182
VL - 15
SP - 343
EP - 348
JO - Cardiac electrophysiology clinics
JF - Cardiac electrophysiology clinics
IS - 3
ER -