Successful extravascular implantable cardioverter-defibrillator implantation in a patient with short QT syndrome: A case report.

UNLABELLED: Short QT syndrome (SQTS) can present with life-threatening ventricular fibrillation (VF), requiring timely resuscitation and secondary prevention. A male patient in his 20s was resuscitated from VF and diagnosed with SQTS. Genetic testing may identify rare variants, such as in SLC4A3, expanding the known genetic spectrum of SQTS. Tall T waves may cause subcutaneous implantable cardioverter-defibrillator (S-ICD) screening failure in some vectors, highlighting limitations of S-ICDs in certain SQTS patients. Extravascular ICD implantation can be a feasible and effective option for patients with SQTS when transvenous or subcutaneous ICDs are suboptimal.

LEARNING

OBJECTIVES

Short QT syndrome (SQTS) can present with life-threatening ventricular fibrillation, requiring timely resuscitation and secondary prevention.Genetic testing may identify rare variants, such as in SLC4A3, expanding the known genetic spectrum of SQTS.Tall T waves may cause subcutaneous implantable cardioverter-defibrillator (S-ICD) screening failure in some vectors, highlighting limitations of S-ICDs in certain SQTS patients.Extravascular ICD implantation can be a feasible and effective option for patients with SQTS when transvenous or subcutaneous ICDs are suboptimal.