Zengin Elvin, Sinning Christoph, Blaum Christopher, Blankenberg Stefan, Rickers Carsten, von Kodolitsch Yskert, Kirchhof Paulus, Drury Nigel E, Stoll Victoria M
Department of Cardiology, University Heart & Vascular Center Hamburg, Hamburg, Germany.
Adult Congenital Heart Disease Section, University Heart Center Hamburg, Hamburg, Germany.
Cardiovasc Diagn Ther. 2021 Apr;11(2):529-537. doi: 10.21037/cdt-20-632.
The number of adults with congenital heart disease (ACHD) has increased over the last decades due to advancements in medical care, including interventional and surgical therapies. We are therefore more frequently challenged by the long-term consequences of palliative or corrective surgery carried out during childhood. Although patients with ACHD may develop conditions related to general cardiovascular risk factors, such as coronary artery disease, the most common complications leading to morbidity and mortality are arrhythmias, heart failure and thromboembolic events. For the management of arrhythmias, current recommendations regarding ablation and device therapy must be considered, whilst also taking into account the anatomical limitations of their congenital heart defect or surgical pathways. Heart failure treatment in acute and chronic settings must also consider the particular anatomy present, including the nature of the systemic ventricle. Treatments strategies for ACHD are typically extrapolated from the respective guidelines in non-ACHD patients, despite a lack of evidence to support this strategy. Right heart failure can be especially challenging to manage in conditions where either a systemic right ventricle or shunt lesions resulting in volume and/or pressure loading of the right ventricle are present. All physicians and cardiologists in particular should be acquainted with the most common diseases in ACHD, their complications and management regime, especially with regards to heart failure as this is a common reason for acute presentation in the emergency department.
在过去几十年中,由于包括介入和外科治疗在内的医疗护理进步,患有先天性心脏病(ACHD)的成年人数量有所增加。因此,我们越来越频繁地面临儿童期进行姑息性或矫正性手术后的长期后果带来的挑战。虽然患有ACHD的患者可能会出现与一般心血管危险因素相关的病症,如冠状动脉疾病,但导致发病和死亡的最常见并发症是心律失常、心力衰竭和血栓栓塞事件。对于心律失常的管理,必须考虑当前关于消融和器械治疗的建议,同时还要考虑其先天性心脏缺陷或手术路径的解剖学限制。急性和慢性情况下的心力衰竭治疗也必须考虑到存在的特殊解剖结构,包括体循环心室的性质。尽管缺乏证据支持,但ACHD的治疗策略通常是从非ACHD患者的相应指南中推断出来的。在存在体循环右心室或导致右心室容量和/或压力负荷的分流病变的情况下,右心衰竭的管理可能特别具有挑战性。所有医生,尤其是心脏病专家,都应该熟悉ACHD中最常见的疾病、其并发症和管理方案,特别是关于心力衰竭的情况,因为这是急诊科急性就诊的常见原因。