Shaddy Robert E, Webb Gary
Division of Cardiology, The Children's Hospital of Philadelphia, and University of Pennsylvania School of Medicine, 34th Street and Civic Center Blvd, Philadelphia, PA 19104-4399, USA.
Expert Rev Cardiovasc Ther. 2008 Feb;6(2):165-74. doi: 10.1586/14779072.6.2.165.
There is a growing population of adult patients with congenital heart defects in the developed world. Most have been repaired, but few have been cured. Many have myocardial dysfunction. Most have exercise intolerance. Some have heart failure. As a group, they show neurohormonal activation similar to that seen in an adult heart failure population with acquired heart disease. Currently, the patients at greatest risk of heart failure are those without a systemic left ventricle, such as Mustard and Senning repairs of transposition of the great arteries (TGA), congenitally corrected TGA, and patients who have had a Fontan procedure. Exercise intolerance may predict hospitalization and death in such patients. For those patients with systemic left ventricles, it would seem reasonable to use the heart failure guidelines developed for patients with acquired heart disease. For those patients without a systemic left ventricle (e.g., a systemic right ventricle or single ventricle), there is currently no foundation for evidence-based therapy.
在发达国家，患有先天性心脏缺陷的成年患者数量日益增加。大多数患者已接受修复手术，但治愈的却寥寥无几。许多患者存在心肌功能障碍。大多数患者运动耐量下降。一些患者出现心力衰竭。总体而言，他们表现出与患有后天性心脏病的成年心力衰竭患者相似的神经激素激活。目前，心力衰竭风险最高的患者是那些没有体循环左心室的患者，如大动脉转位（TGA）的Mustard和Senning修复术、先天性矫正型TGA以及接受过Fontan手术的患者。运动耐量下降可能预示着此类患者会住院和死亡。对于那些有体循环左心室的患者，采用为患有后天性心脏病的患者制定的心力衰竭指南似乎是合理的。对于那些没有体循环左心室的患者（如体循环右心室或单心室），目前尚无循证治疗的依据。
