Disqualification of Hemodynamically Functional Heart With LQTS Syndrome - Would it be Still Justified?

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Long QT syndrome (LQTS) is a genetically determined ion channel disorder. It might be the reason of severe ventricular arrhythmias leading to sudden cardiac death. Immediate cardiopulmonary resuscitation may effect in a restoration of efficient circulation in some cases, however brain death due to anoxia is possible.Case report19 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. In following period of observation patient deteriorated slowly. All brain stem reflexes disappeared except the residual respiratory drive, which persisted until day 17th. On 19th day patient was declared brain dead. Predonation echocardiography showed normal heart contractility - EF 55%. She was heamodynamically stable, RR-120/80 mmHg, HR-106/min, without catecholamine infusion. Kidneys, corneas and aortal homograft were harvested. Heart was not transplanted due to suspicion of LQTS. Genetic studies, completed later, confirmed polymorphism KCNH2 gene and all family was included to program of sudden cardiac death prevention.We presume that LQTS in brain dead 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.Current guidelines of the ISHLT do not recommend to transplant hearts with intractable ventricular arrhythmias [1], but LQTS obviously does not lead to intractable arrhythmias if earlier recognized and properly treated.So far, only one case of unplanned but successful “LQTS heart” transplantation with subsequent ICD implantation has been reported [2]. According to our knowledge it was not confirmed by genetic studies. Another one, not published yet, was done in Department of Cardiac Surgery of Medical University of Wien (personal information from prof. Andreas Zuckermann). Probably many more “LQTS hearts” were transplanted without such knowledge.Conclusions1. Transplantation of “LQTS hearts” with concomitant LCD implantation may be considered after proper risk benefit assessment.2. The clear opinion of the ISHLT concerning acceptance of “LQTS heart donors” is needed.References1. Costanzo M.R. et al. The International Society of Heart and Lungs Transplantation Guidelines of the Care of Heart Transplantation Recipients. J Heart Lung Transplant 2010;29:914–956.2. Liu T. et al. Prolonged QT and cardiac arrest after heart transplantation: inherited or acquired? J Electrocardiol 2011 44(3):350–2.

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