Spadotto Veronica, Voges Inga, Kilner Philip J, Yacoub Magdi H, Ernst Sabine, Ho Siew Yen, Babu-Narayan Sonya V
NIHR Cardiovascular Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College, London, UK.
Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy.
Glob Cardiol Sci Pract. 2016 Jun 30;2016(2):e201619. doi: 10.21542/gcsp.2016.19.
Juxtaposition of atrial appendages is a rare cardiac congenital anomaly, usually associated with other cardiac malformations. Until now, it has not been linked to any significant clinical implications. We report cardiovascular magnetic resonance (CMR) findings of two adult patients who underwent atriopulmonary Fontan operation in the setting of left juxtaposition of the atrial appendages. The patients were in sinus rhythm at the time of the CMR study. Both patients had episodes of sustained atrial tachyarrhythmia requiring electrical cardioversion and were anticoagulated with warfarin with target INR 2-3. CMR images showed a thrombus located in the enlarged and juxtaposed right appendage in both patients. Blood flow frequently appears slow or sluggish in the dilated right atrium following atriopulmonary Fontan surgery. In addition, cine CMR suggested that blood flow reaches very low velocities in the massively dilated juxtaposed right atrial appendage cul-de-sac, thus potentially creating a substrate for clot formation. These findings propose that juxtaposed atrial appendages in atriopulmonary Fontan is an additional risk factor for clot formation, specifically in the dilated right atrial appendage on the left side juxtaposed with the left atrial appendage and that prophylactic anticoagulation is highly justified in these patients.
心房附件并列是一种罕见的心脏先天性异常,通常与其他心脏畸形相关。到目前为止,它尚未与任何重大临床意义相关联。我们报告了两名成年患者的心血管磁共振(CMR)检查结果,这两名患者在心房附件左侧并列的情况下接受了心房肺动脉Fontan手术。在进行CMR研究时,患者处于窦性心律。两名患者均有持续性房性快速心律失常发作,需要进行电复律,并使用华法林抗凝,目标国际标准化比值(INR)为2-3。CMR图像显示两名患者扩大并列的右心房附件中均有血栓。在心房肺动脉Fontan手术后,扩张的右心房内血流常常显得缓慢或淤滞。此外,电影CMR显示,在极度扩张的并列右心房附件盲端,血流速度极低,因此可能形成血栓形成的基础。这些发现表明,心房肺动脉Fontan中的并列心房附件是血栓形成的一个额外危险因素,特别是在与左心房附件并列的左侧扩张右心房附件中,并且在这些患者中预防性抗凝是非常合理的。