1st Department of Cardiology, University of Medical Sciences, Poznań, Poland.
Cardiol J. 2011;18(2):119-27.
Forty years ago, Fontan and Baudet performed the first life-saving operation on a patient with a functionally single ventricle. This multi-stage procedure established the connection between systemic venous circulation and pulmonary arteries. As a consequence, the pulmonary circulation is supplied in a passive way, whereas the single ventricle pumps the blood into the systemic circulation only. Over the years, the technique of creating the abovementioned vascular connections has undergone several modifications. Due to the fundamental non-physiological hemodynamic relations between arterial pulmonary and systemic venous pressures, numerous complications can be observed in these patients including: supraventricular arrhythmias, thromboemboli, hepatic dysfunction, protein-losing enteropathy, heart failure, worsening cyanosis, systemic venous collateralization, and pulmonary arteriovenous malformations, as well as connective tissue lesions in bronchi. Although based on an ingenious concept, the operation remains of a palliative character. Occasionally, heart transplantation is the ultimate resolution. Pharmacological therapy, and surgical conversion, often appear to be ineffective. However, this procedure has enabled many patients to reach adulthood and enjoy their lives to the full. This fact poses a great challenge for cardiologists wishing to become more knowledgeable and experienced as regards such patients, if we are not to waste such fabulous surgical achievements.
四十年前，Fontan 和 Baudet 为一名功能性单心室患者进行了首次救生手术。这一多阶段手术建立了体循环静脉和肺动脉之间的连接。结果，肺循环被动供应，而单心室仅将血液泵入体循环。多年来，创建上述血管连接的技术已经经历了几次修改。由于肺循环和体循环静脉压力之间存在基本的非生理血流动力学关系，这些患者可能会出现多种并发症，包括：室上性心律失常、血栓栓塞、肝功能障碍、蛋白丢失性肠病、心力衰竭、发绀恶化、体循环侧支循环形成和肺动静脉畸形，以及支气管结缔组织病变。尽管该手术基于巧妙的理念，但仍具有姑息性。偶尔，心脏移植是最终的解决方案。药物治疗和手术转换往往似乎无效。然而，该手术使许多患者能够成年并充分享受生活。如果我们不想浪费如此出色的手术成果，这一事实对希望在这类患者方面变得更有知识和经验的心脏病专家提出了巨大挑战。
