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成人先天性心脏病:早年修复的心脏缺陷的残留、后遗症及并发症

[Congenital heart disease in adults: residua, sequelae, and complications of cardiac defects repaired at an early age].

作者信息

Oliver Ruiz José María

机构信息

Unidad de Cardiopatías Congénitas del Adulto, Hospital Universitario La Paz, Madrid, Spain.

出版信息

Rev Esp Cardiol. 2003 Jan;56(1):73-88.

Abstract

Nowadays, it is estimated that 85% of the infants born with congenital heart disease (CHD) will survive to adulthood, thanks mainly to surgical or therapeutic procedures performed during infancy or childhood. The clinical profile and disease pattern of adults with CHD is changing. The prevalence of certain adult CHDs, such as tetralogy of Fallot, transposition of the great arteries or univentricular heart, is rising, but these conditions have practically become new diseases as a result of therapy. Most surviving patients present residua, sequelae, or complications, which can progress during adult life. These disorders can present electrophysiological disturbances, valvular disease, persistent shunts, myocardial dysfunction, pulmonary or systemic vascular disease, problems caused by prosthetic materials, infectious complications, thromboembolic events, or extravascular disorders involving multiple organs or systems. In tetralogy of Fallot, the most striking problems that affect long-term prognosis are pulmonary valve regurgitation, right ventricle dysfunction, and atrial or ventricular arrhythmias. The main problems appearing after physiological atrial repair of transposition of the great arteries are related to right ventricular function, since it is structurally unprepared for systemic circulation, and atrial arrhythmias. Surgical repair of univentricular heart using Fontan techniques should be considered a palliative procedure that does not modify the underlying structural disorder and exposes the postoperative patient to severe complications and problems. The increase in the number of patients with CHD who will reach adulthood in the coming decades makes it necessary to carefully consider the new healthcare demands that are being generated, who should be responsible for them, and how and where solutions can be found.

摘要

如今,据估计,患有先天性心脏病(CHD)的婴儿中85%能够存活至成年,这主要归功于在婴儿期或儿童期进行的手术或治疗程序。患有CHD的成年人的临床特征和疾病模式正在发生变化。某些成人CHD,如法洛四联症、大动脉转位或单心室心脏的患病率正在上升,但由于治疗,这些病症实际上已成为新的疾病。大多数存活患者存在残留病变、后遗症或并发症,这些可能在成年期进展。这些病症可表现为电生理紊乱、瓣膜疾病、持续性分流、心肌功能障碍、肺或体循环血管疾病、由人工材料引起的问题、感染性并发症、血栓栓塞事件或涉及多个器官或系统的血管外病症。在法洛四联症中,影响长期预后的最显著问题是肺动脉瓣反流、右心室功能障碍以及房性或室性心律失常。大动脉转位生理性心房修复后出现的主要问题与右心室功能有关,因为其结构上未为体循环做好准备,以及房性心律失常。使用Fontan技术对单心室心脏进行手术修复应被视为一种姑息性手术,它不会改变潜在的结构紊乱,并且使术后患者面临严重的并发症和问题。在未来几十年中,将成年的CHD患者数量的增加使得有必要仔细考虑正在产生的新的医疗保健需求、谁应该对此负责以及如何以及在何处找到解决方案。

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