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大动脉右位转位(D-TGA)的发病机制与外科治疗:第二部分。

Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II.

作者信息

Zubrzycki Marek, Schramm Rene, Costard-Jäckle Angelika, Morshuis Michiel, Gummert Jan F, Zubrzycka Maria

机构信息

Department of Surgery for Congenital Heart Defects, Heart and Diabetes Center NRW, University Hospital, Ruhr-University Bochum, Georgstr. 11, 32545 Bad Oeynhausen, Germany.

Clinic for Thoracic and Cardiovascular Surgery, Heart and Diabetes Center NRW, University Hospital, Ruhr-University Bochum, Georgstr. 11, 32545 Bad Oeynhausen, Germany.

出版信息

J Clin Med. 2024 Aug 15;13(16):4823. doi: 10.3390/jcm13164823.

DOI:10.3390/jcm13164823
PMID:39200964
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11355351/
Abstract

Dextro-transposition of the great arteries (D-TGA) is the second most common cyanotic heart disease, accounting for 5-7% of all congenital heart defects (CHDs). It is characterized by ventriculoarterial (VA) connection discordance, atrioventricular (AV) concordance, and a parallel relationship with D-TGA. As a result, the pulmonary and systemic circulations are separated [the morphological right ventricle (RV) is connected to the aorta and the morphological left ventricle (LV) is connected to the pulmonary artery]. This anomaly is included in the group of developmental disorders of embryonic heart conotruncal irregularities, and their pathogenesis is multifactorial. The anomaly's development is influenced by genetic, epigenetic, and environmental factors. It can occur either as an isolated anomaly, or in association with other cardiac defects. The typical concomitant cardiac anomalies that may occur in patients with D-TGA include ventriculoseptal defects, patent ductus arteriosus, left ventricular outflow tract obstruction (LVOTO), mitral and tricuspid valve abnormalities, and coronary artery variations. Correction of the defect during infancy is the preferred treatment for D-TGA. Balloon atrial septostomy (BAS) is necessary prior to the operation. The recommended surgical correction methods include arterial switch operation (ASO) and atrial switch operation (AtrSR), as well as the Rastelli and Nikaidoh procedures. The most common postoperative complications include coronary artery stenosis, neoaortic root dilation, neoaortic insufficiency and neopulmonic stenosis, right ventricular (RV) outflow tract obstruction (RVOTO), left ventricular (LV) dysfunction, arrhythmias, and heart failure. Early diagnosis and treatment of D-TGA is paramount to the prognosis of the patient. Improved surgical techniques have made it possible for patients with D-TGA to survive into adulthood.

摘要

大动脉右位转位(D-TGA)是第二常见的青紫型心脏病,占所有先天性心脏病(CHD)的5-7%。其特征为心室动脉(VA)连接不一致、房室(AV)一致,且与D-TGA呈平行关系。因此,肺循环和体循环分离[形态学右心室(RV)连接主动脉,形态学左心室(LV)连接肺动脉]。这种异常包含在胚胎心脏圆锥动脉干发育异常组中,其发病机制是多因素的。该异常的发生受遗传、表观遗传和环境因素影响。它可作为孤立异常出现,也可与其他心脏缺陷合并出现。D-TGA患者可能出现的典型合并心脏异常包括室间隔缺损、动脉导管未闭、左心室流出道梗阻(LVOTO)、二尖瓣和三尖瓣异常以及冠状动脉变异。婴儿期矫正该缺陷是D-TGA的首选治疗方法。手术前需要进行球囊房间隔造口术(BAS)。推荐的手术矫正方法包括动脉调转术(ASO)和心房调转术(AtrSR),以及Rastelli和Nikaidoh手术。最常见的术后并发症包括冠状动脉狭窄、新主动脉根部扩张、新主动脉瓣关闭不全和新肺动脉狭窄、右心室(RV)流出道梗阻(RVOTO)、左心室(LV)功能障碍、心律失常和心力衰竭。D-TGA的早期诊断和治疗对患者的预后至关重要。改进的手术技术使D-TGA患者能够存活至成年。

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