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CASE REPORT

Surgical treatment of long QT syndrome: the cardiac autotransplantation technique with preservation of the conduction system

Miguel Barbero-MarcialI; Eduardo SosaI; Nana Miura IkariI; Arlindo A RisoI; Paulo R CamargoI; Fúlvio PileggiI; Adib D JateneI

DOI: 10.1590/S0102-76381995000200006

ABSTRACT

The long QT syndrome is characterized by intermittent or permanent prolongation of the QT interval, ventricular tachyarrhythmias and frequent sudden death. There is three previous reports in the literature, two unsuccessful. In all these patients a postoperative pacemaker was implanted. In our Institute, in October 1993 to May 1994, three patients with this syndrome, one female and two male aged 10,11 and 13 years were submitted to total denervation of the heart through autotransplantation. Despite full dosis of propranolol these patients were severaly symptomatic, with recurrent ventricular tachycardia. The technique was orientated to avoid lesion of the conduction system. The superior and inferior vena cava, the aortic and the pulmonary trunk were transected. Only the posterior wall of the left atrium between the pulmonary veins was incised. After surgery the patients remained in sinus rhythm without episodes of ventricular tachycardia even during serial ergometric test. Considering these preliminary results we recommend the described technique for patients with the long QT syndrome refractarles to adequate medication.

RESUMO

A síndrome do QT longo é caracterizada por prolongação intermitente ou permanente do intervalo QT, taquicardias paroxísticas ventriculares (TVR) e morte súbita. Existem três casos publicados na literatura tratados cirurgicamente com a técnica do autotransplante cardíaco, com 1 paciente sobrevivente. Nos 3 casos foi necessária, após a operação, a utilização de marcapasso átrio-atrial ou atrioventricular. No InCór, entre outubro de 1993 e maio de 1994, 3 pacientes portadores desta síndrome, 1 do sexo feminino e 2 do masculino, com idades, respectivamente, de 10, 11 e 13 anos, foram submetidos a desnervação por autotransplante. No pré-operatório, apesar de dose máxima de ß-bloqueadores, os freqüentes episódios de taquicardia ventricular não foram evitados. Com carga mínima o teste ergométrico provocava TVR. A retirada do órgão foi feita seccionando-se as veias cavas, aorta, tronco pulmonar e as veias pulmonares, com remanescente de átrio esquerdo. Assim, o sistema de condução permaneceu íntegro e o ritmo, sinusal. No pós-operatório os testes ergométricos realizados mostraram ausência de taquicardia ventricular. Considerando os resultados imediatos, julgamos que a técnica relatada de autotransplante cardíaco como método de completa desnervação pode ser empregada em pacientes portadores da síndrome do QT longo, com baixo risco cirúrgico e preservação do sistema de condução.
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REFERENCES

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