Tetralogy of Fallot: Yesterday and Today

Tetralogy of Fallot: Yesterday and Today
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DOI:
10.1007/s00268-009-0296-8
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发表时间:
2010-04-01
影响因子:
2.6
通讯作者:
Starr, Joanne P.
Starr, Joanne P.
中科院分区:
医学3区
文献类型:
--
作者:
Starr, Joanne P.

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法洛四联症(TOF)是一种发绀型先天性心脏缺陷,1672年由斯顿森首次描述,后来以法洛命名。1888年,法洛将其描述为一种单一的病理过程,包括(1)肺动脉流出道梗阻,(2)室间隔缺损(VSD),(3)主动脉骑跨,以及(4)右心室肥厚。TOF的手术史始于1944年布莱洛克、陶西格和托马斯开发的体肺分流术(BT分流术)。10年后,利勒海利用交叉循环,基尔克林使用原始的心肺转流回路完成了TOF的根治手术。包括班森、埃伯特、马尔姆、特拉斯勒、巴拉特 - 博伊斯和卡斯塔涅达在内的其他几位外科医生的显著贡献将我们带入了现代手术时代。如今,TOF根治手术在6个月龄前进行,死亡率较低(<2%)。在某些特定病例中,改良版的BT分流术仍作为初始手术进行。长期生存率很高(85% - 90%)。患有TOF的成年幸存者人数不断增加,可能需要再次干预,包括手术干预或基于导管的干预。有前景的未来创新包括经皮肺动脉瓣置换术、组织工程自体瓣膜和管道以及基因操作。本文对TOF进行了综述,包括手术治疗的历史、当今的治疗方法以及长期预后。
Tetralogy of Fallot (TOF) is a cyanotic congenital cardiac defect that was first described by Stenson in 1672 and later named for Fallot, who in 1888 described it as a single pathological process responsible for (1) pulmonary outflow tract obstruction, (2) ventricular septal defect (VSD), (3) overriding aortic root, and (4) right ventricular hypertrophy. The surgical history of TOF began with the development of the systemic to pulmonary artery shunt (BT shunt) by Blalock, Taussig, and Thomas in 1944. Ten years later complete repair of TOF was performed by Lillehei using cross-circulation and by Kirklin with a primitive cardiopulmonary bypass circuit. Notable contributions by several other surgeons including Bahnson, Ebert, Malm, Trusler, Barratt-Boyes, and Castaneda would lead us into the modern era of surgery. Today, complete repair of TOF is performed before six months of age with low mortality (< 2%). In select cases a modified version of the BT shunt is still performed as the initial procedure. Long-term survival rates are excellent (85%-90%). Adult survivors with TOF are an ever-increasing population and may require reintervention, surgically or catheter based. Promising future innovations include percutaneous pulmonary valve replacement, tissue-engineered autologous valves and conduits, and genetic manipulation. This article presents a review of TOF, including the history of surgical treatment, present-day approaches, and long-term outcomes.