Disqualification of a haemodynamically functional heart with LQTS syndrome – unjustified organ loss?

Romuald Bohatyrewicz^1,2,3,4,5, Maciej Zukowski^1,2,3,4,5, Carl Ludwig Fisher-Froelich^1,2,3,4,5, Maria Wujtewicz^1,2,3,4,5, Radoslaw Owczuk^1,2,3,4,5, Joanna Pastuszka-Solek^1,2,3,4,5, Jowita Biernawska^1,2,3,4,5, Agnieszka Zienciuk^1,2,3,4,5, Antoni Ottowicz^1,2,3,4,5, Katarzyna Kotfis^1,2,3,4,5, Anna Milecka^1,2,3,4,5

¹Department of Anaesthesiology and Intensive Therapy, Pomeranian Medical University, Szczecin, Poland, ²Region Baden-Wuerttemberg , Deutsche Stiftung Organtransplanation, Stuttgart, Germany, ³Department of Anaesthesiology and Intensive Therapy, Medical University of Gdansk, Gdansk, Poland, ⁴Department of Cardiology and Electrotherapy, Medical University of Gdansk, Gdansk, Poland, ⁵Regional Transplant Coordination Center, Medical University of Gdansk, Gdansk, Poland

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. Early introduction of cardiopulmonary resuscitation may effect in a restoration of efficient circulation in some cases, however brain death due to anoxia is possible.

Case report

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 nonsustained polymorphic ventricular tachycardia was observed. All brain stem reflexes slowly disappeared except for residual respiratory drive, which persisted until day 17^th.  On 19^th day patient was declared brain dead. Predonation echocardiography showed normal heart contractility - EF 55%. She was heamodynamically stable, RR-120/80mmHg, 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]. LQTS obviously does not lead to intractable arrhythmias if earlier recognized and properly treated. So far, at least one case of unplanned but successful “LQTS heart” transplantation with subsequent ICD implantation has already been reported [2]. Probably many more were performed without such  knowledge. The authors of above mentioned publication would accept such heart for really sick patient [dr M. Shehata -  personal information]. 

 Conclusions

1. Transplantation of “LQTS hearts” with concomitant LCD  implantation may be considered after proper risk benefit assessment in special cases.
2. The clear opinion of the ISHLT concerning acceptance of “LQTS heart donors” is needed.