Journal of Rare Cardiovascular Diseases

We report a case of 22 years old woman with long QT syndrome type with the first cardiac incidents in the post partum period. We discuss the actual treatment recommendation in the long QT syndrome with special attention on indications for implantation of defibrillator‑cardioverter. JRCD 2014; 1 (6): 15–20

Long QT syndrome, ICD, risk stratification, β‑blocker

Kukla P, Filipecki A, Jastrzębski M, et al. Wrodzony zespół długiego QT

u młodej kobiety rozpoznany w okresie poporodowym. Kardiol. Pol. 2009; 7: 795–799.

Moss AJ, Zaręba W, Benhorin J, et al. ECG T wave patterns in genetically distinct forms of the hereditary long QT syndrome. Circulation 1995; 92: 2929–2934.

Odening KE, Choi BR, Liu GX, et al. Estradiol promotes sudden cardiac death in transgenic long QT type 2 rabbits while progesterone is protective. Heart Rhythm 2012; 9: 823–832.

Moss AJ. Sex hormones and ventricular tachyarrhythmias in LQTS: new insights regarding antiarrhythmic therapy. Heart Rhythm 2012; 9: 833–834.

Odening KE, Choi BR, Koren G. Sex hormones and cardiac arrest in long QT syndrome: Does progesterone represent a potential new antiarrhythmic therapy? Heart Rhythm 2012; 9: 1150–1152.

Rashba E, Zaręba W, Moss A, et al. Influence of pregnancy on the risk for cardiac events in patients with hereditary long QT syndrome. Circulation 1998; 97: 451–456.

Priori SG, Schwarz PJ, Napolitano C, et al. Risk stratification in Long QT. NEJM 2003; 348: 1866–1874.

Khositseth A, Tester DJ, Will ML, et al. Identification of a common genetic substrate underlying postpartum cardiac events in congenital long QT syndrome. Heart Rhythm 2004; 1: 65–66.

Seth R, Moss A, McNitt S, et al. Long QT syndrome and pregnancy. JACC 2007; 49: 1092–1098.

Zipes DP, Camm AJ, Borggrefe M, et al. ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death–executive summary: A report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop Guidelines for Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death) Developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Eur. Heart J. 2006; 27: 2099–140.

Moss AJ, Zareba W, Hall WJ, et al. Effectiveness and limitations of beta‑blocker therapy in congenital long‑QT syndrome. Circulation 2000; 101: 616–623.

Schwartz PJ, Spazzolini C, Priori SG, et al. Who are the long‑QT syndrome patients who receive an implantable cardioverter‑defibrillator and what happens to them?: data from the European Long‑QT Syndrome Implantable Cardioverter‑Defibrillator (LQTS ICD) Registry. Circulation 2010; 122: 1272–1282.