Zetina-Tun Hugo Jesús, Careaga-Reyna Guillermo, Galván-Díaz José, Sánchez-Uribe Magdalena
Clínica de Trasplante de Órganos Torácicos. Unidad Médica de Alta Especialidad, Hospital General Gaudencio González Garza, Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, Ciudad de México, México.
Dirección General. Unidad Médica de Alta Especialidad, Hospital General Gaudencio González Garza, Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, Ciudad de México, México.
Cir Cir. 2017 Nov-Dec;85(6):539-543. doi: 10.1016/j.circir.2016.09.001. Epub 2016 Oct 20.
Myocardial noncompaction of the left ventricle is a congenital cardiomyopathy characterised by left ventricular hypertrabeculation and prominent intertrabecular recesses. The incidence ranges from 0.15% to 2.2%. Clinical manifestations include heart failure, arrhythmias, and stroke. Prognosis is fatal in most cases. Heart transplantation is a therapeutic option for this cardiomyopathy, and few had been made worldwide.
The case is presented of a 20 year-old male with noncompacted myocardium of the left ventricle, who had clinical signs of heart failure. His functional class was IV on the New York Heart Association scale. He was successfully transplanted. Its survival to 15 months is optimal in class I New York Heart Association, and endomyocardial biopsies have been reported without evidence of acute rejection.
It is concluded that heart transplantation modified the natural history and improved survival in patients with this congenital heart disease.
左心室心肌致密化不全是一种先天性心肌病，其特征为左心室肌小梁增多和明显的小梁间隐窝。发病率在0.15%至2.2%之间。临床表现包括心力衰竭、心律失常和中风。大多数情况下预后不良。心脏移植是这种心肌病的一种治疗选择，全球范围内开展的此类手术较少。
报告了一名20岁患有左心室心肌致密化不全的男性病例，该患者有心力衰竭的临床体征。根据纽约心脏协会分级，其功能分级为IV级。他成功接受了心脏移植。术后15个月，其纽约心脏协会分级为I级，存活情况良好，心内膜活检报告无急性排斥反应迹象。
得出结论，心脏移植改变了这种先天性心脏病患者的自然病程并提高了生存率。
