Neill C A, Clark E B
Johns Hopkins Medical Institutions, Baltimore, Maryland 21287.
Tex Heart Inst J. 1994;21(4):272-9.
The chronicle of tetralogy of Fallot is part of a dramatic evolution in cardiology, cardiac surgery, and understanding of the developing heart. Many new tools and concepts have evolved since Steno of Denmark first described the defect in 1673, and since Fallot of Marseilles coined the term tetralogy in 1888. Four major eras of progress can be recognized. The 1st, the era of pathologic anatomy, culminated in the publication of Maude Abbott's Atlas of Congenital Cardiac Disease in 1936. The next, the era of clinicophysiology and surgery, was highlighted by the 1st Blalock-Taussig anastomosis in 1944, by open-heart surgery 10 years later, and by a new team approach to cardiology. The 3rd, or infant era, began in the mid 1970s with successful intracardiac repair in infants, the rise of echocardiography, and the introduction of prostaglandin therapy. The current era of cardiac development (beginning in the 1990s) gives hope for early understanding of the molecular basis of tetralogy. Tribute is due to the surgical and medical pioneers, and to the pioneer patients and their families, for revolutionary changes in diagnosis and treatment. The challenge of the next 100 years lies in increased understanding of the molecular biology of the defect and in preserving the blend of humanism, scholarship, and skill that have graced the advances of the past 3 centuries.
法洛四联症的发展历程是心脏病学、心脏外科以及对发育中心脏认识方面戏剧性演变的一部分。自1673年丹麦的斯滕诺首次描述该缺陷，以及1888年马赛的法洛创造“四联症”这一术语以来，许多新工具和概念不断涌现。可以识别出四个主要的进展阶段。第一个阶段是病理解剖学时代，1936年莫德·阿博特的《先天性心脏病图谱》出版标志着这一阶段达到顶峰。接下来是临床生理学和外科手术时代，1944年首次进行的布莱洛克 - 陶西格吻合术、10年后的心脏直视手术以及心脏病学新的团队协作方式成为这一阶段的亮点。第三个阶段，即婴儿时代，始于20世纪70年代中期，婴儿心脏内修复手术的成功、超声心动图的兴起以及前列腺素疗法的引入是其标志。当前的心脏发育时代（始于20世纪90年代）为早期了解法洛四联症的分子基础带来了希望。应当向外科和医学先驱们，以及先驱患者及其家属致敬，因为他们带来了诊断和治疗方面的革命性变革。未来100年的挑战在于加深对该缺陷分子生物学的理解，以及保持过去三个世纪进步中所展现的人文精神、学术素养和专业技能的融合。
