Novel insight into the natural history of short QT syndrome

Andrea Mazzanti, Ajita Kanthan, Nicola Monteforte, Mirella Memmi, Raffaella Bloise, Valeria Novelli, Carlotta Miceli, Sean O'Rourke, Gianluca Borio, Agnieszka Zienciuk-Krajka, Antonio Curcio, Andreea Elena Surducan, Mario Colombo, Carlo Napolitano, Silvia G. Priori

Research output: Contribution to journalArticlepeer-review


Objectives This study intends to gain further insights into the natural history, the yield of familial and genetic screening, and the arrhythmogenic mechanisms in the largest cohort of short QT syndrome (SQTS) patients described so far. Background SQTS is a rare genetic disorder associated with life-threatening arrhythmias, and its natural history is incompletely ascertained. Methods Seventy-three SQTS patients (84% male; age, 26 ± 15 years; corrected QT interval, 329 ± 22 ms) were studied, and 62 were followed for 60 ± 41 months (median, 56 months). Results Cardiac arrest (CA) was the most frequent presenting symptom (40% of probands; range,

Original languageEnglish
Pages (from-to)1300-1308
Number of pages9
JournalJournal of the American College of Cardiology
Issue number13
Publication statusPublished - Apr 8 2014


  • genetics
  • short QT syndrome
  • sudden cardiac death
  • ventricular arrhythmias

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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