Korean Circ J.  2013 Dec;43(12):830-833. 10.4070/kcj.2013.43.12.830.

Repeated Aborted Sudden Cardiac Death with Long QT Syndrome in a Patient with Anomalous Origin of the Right Coronary Artery from the Left Coronary Cusp

• Park YM
• Kim SJ
• Park CH
• Kang WC
• Shin MS
• Koh KK
• Choi IS

• KMID: 2224778
• DOI: http://doi.org/10.4070/kcj.2013.43.12.830

Abstract

A 15-year-old female with a prior history of aborted cardiac death and surgical correction of anomalous origin of the right coronary artery (RCA) presented with polymorphic ventricular tachycardia. Her electrocardiogram after defibrillation was suggestive of congenital long QT syndrome (LQTS). The patient was treated with a beta-blocker and remained free from ventricular arrhythmia during the follow-up of more than 6 months. Here, we present the case of a young female with repeated aborted cardiac death accompanied by anomalous origin of the RCA and congenital LQTS for the first time.

Keyword

Long QT syndrome; Tachycardia, ventricular; Coronary vessel anomalies

MeSH Terms

Adolescent
Arrhythmias, Cardiac
Coronary Vessel Anomalies
Coronary Vessels*
Death
Death, Sudden, Cardiac*
Electrocardiography
Female
Follow-Up Studies
Humans
Long QT Syndrome*
Tachycardia, Ventricular

Figure

• 

  Fig. 1 A 15-year-old female was referred to the cardiology department due to repeated aborted sudden cardiac death. Initial electrocardiogram showed polymorphic ventricular tachycardia.

• 

  Fig. 2 Cardiac computed tomography revealed anomalous origin of the RCA from the left coronary cusp coursing between the pulmonary artery and the aorta (white arrows). Ao: aorta, RCA: right coronary artery, PA: pulmonary artery.

• 

  Fig. 3 Her follow-up electrocardiogram after stabilization showed a sinus rhythm with an inverted T wave and a prolonged QT interval: 470 ms, and QTc: 531 ms, which was suggestive of congenital long QT syndrome.

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