Ciancarella Paolo, Fazzari Fabio, Montano Valentina, Guglielmo Marco, Pontone Gianluca
Department of Radiology and Bioimaging, Bambino Gesù Children Hospital-IRCCS, Piazza di Sant'Onofrio 4, 00165 Rome, Italy.
Division of Cardiology II, Department of Internal Medicine and Cardiovascular Diseases, University Hospital "Paolo Giaccone", Via del vespro 129, 90127 Palermo, ItalybItaly.
J Saudi Heart Assoc. 2018 Apr;30(2):143-146. doi: 10.1016/j.jsha.2017.09.004. Epub 2017 Oct 5.
A 21 year old male with no relevant medical history presented to our Institution for further assessments of a right paracardiac mass founded on a chest X-ray. Chest computed tomography revealed a wide median defect of the diaphragm at the level of xiphoid process of the sternum, with the herniation of omental fat tissue in the mediastinum. Cardiac magnetic resonance confirmed the presence of a huge hernia originating from the foramen of Morgagni (sterno-costal hiatus), displacing the heart leftwards and posteriorly and compressing the right ventricle (RV), giving to it a tubular shape.The signal characteristics were typical of fat tissue, with hyperintense signal in T1 and T2 weighted black blood images and homogeneus signal suppression on STIR T2 black blood images. Short axis real time cine images, performed during deep inspiration, showed an early diastolic ventricular septal bounce, with flattening of the interventricular septum during mid-late diastole: they represented signs of diastolic dysfunction of the right ventricle, resembling a sort of "pseudo-constrictive" pathophysiological model. The patient was thus referred to surgical repair of the diaphragmatic defect.
一名21岁无相关病史的男性因胸部X线检查发现右心旁肿块前来我院进一步评估。胸部计算机断层扫描显示胸骨剑突水平膈肌有较宽的正中缺损,网膜脂肪组织疝入纵隔。心脏磁共振成像证实存在一个起源于莫尔加尼孔(胸骨-肋间隙)的巨大疝,心脏向左后方移位并压迫右心室(RV),使其呈管状。信号特征为典型的脂肪组织,在T1加权和T2加权黑血图像中呈高信号,在STIR T2黑血图像上信号均匀抑制。在深吸气时进行的短轴实时电影图像显示舒张早期室间隔弹跳,舒张中晚期室间隔扁平:这些表现为右心室舒张功能障碍的征象,类似于某种“假性缩窄”病理生理模型。因此,该患者被转诊进行膈肌缺损的手术修复。