The short QT syndrome (SQTS) is a primary cardiac electrical disease characterized by abnormally short QT intervals and an increased propensity to develop arrhythmias. It is a relatively recent addition to the list of inherited channelopathies responsible for sudden cardiac death (SCD). Gussak, et al. [1] first described SQTS in 2000, and about 70 cases are reported till now.

A 10-year-old boy, resident of Delhi, suddenly fell unconscious, was not responding, not breathing and quickly turned blue and cold at home. His uncle, an officer with city police, was trained to carry out cardio-pulmonary resuscitation (CPR). He immediately started CPR, and approximately after 7 cycles of CPR, child was revived. At arrival to our hospital, he was stable with normal vital signs and consciousness. ECG done revealed QT/QTc interval of 280ms/305ms. His electrolytes, magnetic resonance imaging brain, electro-encephalography and thyroid profile were normal. Repeat ECG at heart rate of 60 revealed QT interval 280ms and J point to T-wave peak interval of 200ms (Fig. 1). Family history was negative for SCD. We placed an implantable Cardio-defibrillator (ICD).

Fig. 1 ECG done at HR-60/min showing QT interval of 280ms.

A short QT interval is usually considered if QTc is <340ms. However, there is an overlapping range of QT intervals between affected individuals and apparently healthy subjects. Gollob, et al. [2] proposed a diagnostic criterion for SQTS. SQTS is mostly seen in males and common presentation is aborted SCD (24-32%), arrhythmias and syncope [3]. As the risk of SCD is high in SQTS, ICD placement is strongly recommended for secondary prevention. However, role of ICD in primary prevention is not well defined. Information regarding pharmacological therapy for SQTS is fairly limited, and quinidine has been suggested as one of the therapies. SQTS is considered a rare electrical abnormality associated with SCD in individuals with structurally normal heart. Timely diagnosis and optimal treatment can significantly improve the overall prognosis of the patient and family members. There is a scarcity of data about SQTS in terms of its clinical presentation, diagnosis, genotype-phenotype correlation, risk-stratification and treatment. This case aptly highlights the importance of bystander CPR in saving life in such disorders. Basic life support education should be promoted widely to save many more lives.

1. Gussak I, Brugada P, Brugada J, Wright RS, Kopecky SL, Chaitman BR, et al. Idiopathic short QT interval: a new clinical syndrome? Cardiology. 2000;94:99 -102.

2. Gollob MH, Redpath CJ, Roberts JD. The short QT syndrome: Proposed diagnostic criteria. J Am Coll Cardiol. 2011;57:802-12.