Key result
Individualized hydroquinidine or ICD therapy shows relatively benign long-term outcomes in Short QT syndrome.
Why the study?
Short QT syndrome is a rare inheritable disease linked to sudden cardiac death, but data regarding long-term outcomes in affected families are limited.
Cohort (n=17)
Long-term follow-up of patients with Short QT syndrome demonstrates that despite a high familial risk of sudden cardiac death, individualized treatment with ICDs and/or hydroquinidine results in relatively benign outcomes.
May support individualized therapy in Short QT syndrome; leaves open need for larger prospective validation.
Background Short QT syndrome ( SQTS ) is a rare inheritable disease associated with sudden cardiac death. Data on long-term outcomes of families with SQTS are limited. Methods and Results Seventeen patients with SQTS in 7 independent families (48% men; median age, 42.4 years; corrected QT interval, 324.9±40.8 ms) were followed up for 13.5±2.5 years. A history of sudden cardiac death was documented in 71% of families. A large number of them showed sudden cardiac deaths at a younger age, with a predominance of men (67%). Five patients had syncope (29%) and 9 (53%) had atrial fibrillation or atrial flutter. An SQTS -related gene was found in 76% of the patients as follows: KCNH 2 ( SQTS 1) in 4, CACNA 1C ( SQTS 4) in 3, and CACN b2 ( SQTS 5) in 6. Five patients (29%) received an implantable cardioverter-defibrillator and 5 patients received long-term prophylaxis with hydroquinidine. During follow-up, 1 patient received an appropriate implantable cardioverter-defibrillator shock attributable to ventricular fibrillation. The patient received no further implantable cardioverter-defibrillator shocks after treatment with hydroquinidine. Conclusions The risk of sudden cardiac death in SQTS families is high. However, after appropriate risk assessment and individualized treatment options (hydroquinidine and/or implantable cardioverter-defibrillator), the long-term outcome is relatively benign when patients are seen at a reference center.
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El‐Battrawy et al. (2018) conducted a cohort in Short QT syndrome (n=17). Hydroquinidine and/or implantable cardioverter-defibrillator was evaluated on Long-term clinical outcomes including sudden cardiac death and appropriate ICD shocks. Long-term follow-up of 17 patients with Short QT syndrome showed that individualized treatment with hydroquinidine or an ICD yielded a relatively benign outcome, with 1 appropriate ICD shock.
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