Navarro-Aguilar V, Flors L, Calvillo P, Merlos P, Buendía F, Igual B, Melero-Ferrer J, Soriano J Rueda, Leiva-Salinas C
Department of Radiology, Hospital Universitario y Politécnico La Fe, Bulevar sur s/n, Valencia 46026, Spain.
Department of Radiology, Hospital Universitario y Politécnico La Fe, Bulevar sur s/n, Valencia 46026, Spain; Department of Radiology and Medical Imaging, University of Virginia Health System, 1215 Lee Street, Charlottesville 22908, Virginia, USA.
Clin Radiol. 2015 Mar;70(3):295-303. doi: 10.1016/j.crad.2014.10.005. Epub 2014 Nov 14.
Univentricular congenital heart diseases include a range of entities that result in a functionally single ventricular chamber. Although the only curative therapy is cardiac transplantation, there are several palliative surgical techniques that prevent ventricular volume overload, diverting part or all the systemic venous circulation into the pulmonary arteries. The modern Fontan procedure, which consists of anastomosing both the superior (SVC) and inferior vena cava (IVC) to the right pulmonary artery (RPA), is nowadays the last step before transplantation. The importance of imaging in these entities lies not only in the understanding of the new circuit established after surgical correction, but also in the early detection of the wide spectrum of cardiac and extracardiac complications that can occur due to the new physiological condition. Due to the increased survival of these patients, long-term complications are becoming more common. The main cardiac complications are atrial enlargement, ventricular dysfunction, and stenosis or thrombosis of the conduit. Pulmonary artery stenosis, pulmonary arteriovenous fistulae (PAVF), systemic-pulmonary veno venous shunts (VVS), hepatic congestion, cardiac cirrhosis, and protein-losing enteropathy are potential extracardiac complications.
单心室先天性心脏病包括一系列导致功能上单一心室腔的病症。虽然唯一的治愈性疗法是心脏移植，但有几种姑息性手术技术可防止心室容量过载，将部分或全部体循环静脉血引流至肺动脉。现代Fontan手术包括将上腔静脉（SVC）和下腔静脉（IVC）都吻合到右肺动脉（RPA），如今是移植前的最后一步。影像学在这些病症中的重要性不仅在于理解手术矫正后建立的新循环，还在于早期发现由于新的生理状况可能出现的广泛的心脏和心外并发症。由于这些患者的生存率提高，长期并发症变得更加常见。主要的心脏并发症是心房扩大、心室功能障碍以及管道狭窄或血栓形成。肺动脉狭窄、肺动静脉瘘（PAVF）、体肺静脉分流（VVS）、肝淤血、心源性肝硬化和蛋白丢失性肠病是潜在的心外并发症。
