Abstract

Long QT syndrome (LQTS) is a genetically determined ion channel disorder. It may be the reason of severe ventricular arrhythmias leading to sudden cardiac death. Early introduction of cardiopulmonary resuscitation may influence restoration of effi cient circulation in some cases, however brain death due to anoxia is possible. 19 year old female suffered from recurrent syncopal attacks. She was admitted to the hospital following cardiac arrest at home in a mechanism of ventricular fibrillation. During in-hospital treatment an episode of the pulseless electrical activity with a short episode of non-sustained polymorphic ventricular tachycardia was observed. Resting ECG showed sinus tachycardia 120 beats per minute, with QT interval corrected according to the Bazett's formula of 424 ms. Echocardiograph revealed generalized hypokinesia with normal heart valves' function. In the following period of observation patient deteriorated slowly. All brain stem refl exes disappeared except for residual respiratory drive, which persisted until 17th day. On 19th day patient was declared brain dead. Kidneys, corneas and aortal homograft were harvested. Heart was not transplanted due to suspicion of LQTS. Genetic studies confirmed polymorphism KCNH2 gene and whole family was included into the program of sudden cardiac death prevention. We presume that LQTS in brain death patients as a result of cardiac arrest might be unexpectedly frequent, especially in a group of younger donors.  Brain death may be the result of brain hypoxia after cardiac arrest in some cases and mechanical injuries due to syncopes in the others. In our opinion  transplantation of "LQTS hearts" with concomitant implantation of cardioverter - defibrillator may be justified due to increasing organ deficiency

Keywords: Heart Transplantation, Organ Procurement, case report