Aboulhosn Jamil, Child John S
Ahmanson/UCLA Adult Congenital Heart Disease Center, Division of Cardiology, David Geffen School of Medicine at UCLA, 650 Charles E. Young Drive South, BH-307, Los Angeles, CA 90095-1679, USA.
Curr Treat Options Cardiovasc Med. 2006 Dec;8(6):474-83. doi: 10.1007/s11936-006-0036-4.
The four characteristic findings in tetralogy of Fallot (TOF) include the following: 1) a malaligned ventricular septal defect; 2) right ventricular outflow and/or pulmonary valve/artery stenosis or atresia; 3) a dextraposed overriding aorta; and 4) right ventricular hypertrophy. This article focuses solely on the outcomes and treatment of surgically corrected patients born with TOF. In the modern era, early surgical repair consisting of ventricular septal defect closure and alleviation of right ventricular outflow obstruction have gained favor over early palliation with an aortopulmonary shunt followed by intracardiac repair. Surgical outcomes are excellent and dramatically improve prognosis. However, these patients are not "cured" and are at significant risk of developing subsequent electrical and hemodynamic problems. An operated patient with TOF should be evaluated at regular intervals by a cardiologist trained in congenital heart disease; any symptoms suggestive of hemodynamic or electrical compromise should spur further investigation. Advances in imaging, medical therapy, electrophysiology, device/resynchronization therapy, and percutaneous intervention provide the clinician with a number of therapeutic options. Surgical techniques have changed significantly since the early intracardiac repairs of the 1960s and 1970s. The deleterious hemodynamic and electrical effects of pulmonary regurgitation and ventriculotomy scars have spurred efforts to ensure pulmonary valvular competence and minimize the extent of ventricular incisions. Since Blalock and Taussig's first palliative shunt in 1945, the survival and quality of life for patients with TOF has improved dramatically; this is one of the great accomplishments for cardiovascular medicine in the 20th century. The 21st century promises further success with the advent of a myriad of technologic advancements.
法洛四联症（TOF）的四个特征性表现如下：1）心室间隔对位不良；2）右心室流出道和/或肺动脉瓣/动脉狭窄或闭锁；3）主动脉骑跨；4）右心室肥厚。本文仅关注患有TOF并接受手术矫正患者的治疗结果。在现代，早期手术修复，即关闭室间隔缺损并缓解右心室流出道梗阻，已比先进行体肺分流术姑息治疗随后再进行心内修复更受青睐。手术效果极佳，能显著改善预后。然而，这些患者并未“治愈”，仍有发生后续电学和血流动力学问题的重大风险。患有TOF的手术患者应由接受过先天性心脏病培训的心脏病专家定期评估；任何提示血流动力学或电学受损的症状都应促使进一步检查。影像学、药物治疗、电生理学、器械/再同步治疗以及经皮介入技术的进展为临床医生提供了多种治疗选择。自20世纪60年代和70年代早期进行心内修复以来，手术技术已发生显著变化。肺反流和心室切开瘢痕的有害血流动力学和电学影响促使人们努力确保肺动脉瓣功能正常并尽量减少心室切口范围。自1945年布莱洛克和陶西格首次进行姑息性分流术以来，TOF患者的生存率和生活质量已大幅提高；这是20世纪心血管医学的重大成就之一。随着众多技术进步的出现，21世纪有望取得更大成功。
