D'Alto Michele, Dimopoulos Konstantinos, Budts Werner, Diller Gerhard-Paul, Di Salvo Giovanni, Dellegrottaglie Santo, Festa Pierluigi, Scognamiglio Giancarlo, Rea Gaetano, Ait Ali Lamia, Li Wei, Gatzoulis Michael A
Department of Cardiology, Second University of Naples, Monaldi Hospital, Naples, Italy.
Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.
Heart. 2016 Jun 15;102(12):910-8. doi: 10.1136/heartjnl-2015-308903. Epub 2016 Mar 24.
Pulmonary arterial hypertension (PAH) in adult patients with congenital heart disease (CHD) is associated with increased morbidity and mortality. The present review aims to discuss the clinical applications of invasive and non-invasive diagnostic modalities and to describe the strengths and weaknesses of each technique. Chest radiograph is an inexpensive investigation providing information on pulmonary arterial and hilar dilatation, pruning of peripheral pulmonary arteries and cardiomegaly. Transthoracic two-dimensional and Doppler echocardiography is the most widely used imaging tool. It provides information on cardiac anatomy and an estimate of haemodynamics and biventricular remodelling and function. In addition, echocardiography is valuable in assessing prognosis and monitoring the efficacy of therapy. Structural and functional changes associated with CHD-PAH, mainly affecting the right ventricle and pulmonary circulation, may represent an ideal target for evaluation with cardiac magnetic resonance. This non-invasive imaging modality has a low biological impact. CT plays an important role for patients with limited echocardiographic windows and those who are unable to undergo MRI (claustrophobia, poor compliance, presence of a pacemaker/implantable cardioverter defibrillator). It is the modality of choice for detailed assessment of pulmonary vessel obstruction or thrombosis. Finally, heart catheterisation remains the gold standard for diagnosing and confirming PAH in patients with CHD and for shunt evaluation. The diagnostic assessment of CHD-PAH requires great expertise and a deep knowledge of both CHD and PAH pathophysiology and should take place in a tertiary centre, where multiple data can be appropriately integrated and applied clinically.
先天性心脏病（CHD）成年患者的肺动脉高压（PAH）与发病率和死亡率增加相关。本综述旨在讨论有创和无创诊断方法的临床应用，并描述每种技术的优缺点。胸部X线片是一种廉价的检查方法，可提供有关肺动脉和肺门扩张、外周肺动脉变细以及心脏扩大的信息。经胸二维和多普勒超声心动图是使用最广泛的成像工具。它可提供心脏解剖结构信息，并估计血流动力学以及双心室重塑和功能。此外，超声心动图在评估预后和监测治疗效果方面很有价值。与CHD-PAH相关的结构和功能变化主要影响右心室和肺循环，可能是心脏磁共振评估的理想靶点。这种无创成像方式对生物的影响较小。对于超声心动图检查窗口有限以及无法进行磁共振成像（幽闭恐惧症、依从性差、存在起搏器/植入式心脏复律除颤器）的患者，CT发挥着重要作用。它是详细评估肺血管阻塞或血栓形成的首选方式。最后，心导管检查仍然是诊断和确认CHD患者PAH以及评估分流的金标准。CHD-PAH的诊断评估需要专业知识以及对CHD和PAH病理生理学的深入了解，并且应该在三级中心进行，在那里可以对多种数据进行适当整合并应用于临床。
