1、1 冠状动脉瘘 （Coronary Artery Fistulae）汇报题目：2疾病定义：冠状动脉瘘冠状动脉瘘( coronary artery fistulae( coronary artery fistulae，CAF) CAF) 是指冠状动脉与心腔、冠状静脉、肺动脉之间的异常连接，最早由德国解剖学家Wilhelm Krause 于1865年提出。CAFCAF 在普通人群中的发病率为0 .002 %, 占冠脉造影畸形的0 .13 %- 0 .22 %, 其中90 %以上与右心系统或心脏直接相连接的动、静脉血管如肺动脉、上腔静脉、冠状窦之间形成沟通, 流向相对低压的静脉系统, 本质上产生左向
2、右分流的血流动力学效应病因分类：1.先天性先天性CAF：2.2.获得性获得性CAFCAF：胎儿心血管系统发育时局部心肌发育停止，心肌肌小梁间的窦状间隙无法退化，从而形成CAF。常伴随其他心脏结构畸形，如法洛四联症、单室心、动脉导管未闭等。外伤、心脏外科手术、介入手术等。3临床表现：症状：体征：1.通常无明显症状。2.老年患者中可能会出现呼吸困难、心绞痛，偶尔会有心律失常。3.左向右分流大的如冠状动脉-左室瘘容易导致左心室容量超负荷, 出现充血性心力衰竭的症状。1.通常无明显体征。2.有体征的患者表现为心前区连续性杂音。4W诊断：非侵入性检查：侵入性检查： 1.X线，ECG通常无特异性表现。2.
3、心超：二维超声心动图显示有瘘的那支冠状动脉明显扩张。 : 无论哪支冠状动脉瘘至哪个心腔均显示左心房、左心室和主动脉根部内径增大 ：彩色多普勒血流显像（CDFI）在瘘的心腔或肺动脉内显示异常血流束信号3.冠脉CTA: 表现为异常的冠状动脉及其分支增粗，迂曲，严重者可呈瘤样扩张，往往在瘘口周围明显彭大，通过异常的通道，血流可分流入不同的心腔及大血管。冠状动脉造影：是CAF诊断的金标准，可显示CAF 的起源、走行、分布、瘘口位置及大小、瘤样扩张及窃血现象等信息。5治疗：1.CAF 是否需要治疗取决于其对血流动力学的影响2.通常认为对分流甚小、血流动力学影响不大、且无临床症状的孤立的CAF 无需治疗。
4、3.对于血流动力学显著异常、存在临床症状的或暂时虽无血流动力学影响, 但远期可能产生严重并发症的需积极给与治疗。治疗人群：治疗方法：1.保守治疗：感染性心内膜炎的预防和对症药物治疗;2.瘘道封堵：介入治疗方式包括可控弹簧圈栓塞、支架植入、自 膨胀伞 ，状封堵器、新型Amplat zer血管塞治疗等;3.外科手术：方式有结扎或/和补片、人工血管转流或移植等6Case1:A 72-year-old woman presented with episodes of extreme exhaustion and fatigue occurring at rest. A continuous murmu
5、r (never before documented) was heard widely over the precordium.The effort electrocardiogram and echocardiogram were normal.7At cardiac catheterization, a left-to-right shunt of 1.29:1 (Qp:Qs) was found.Coronary angiography (A and B) showed one fistula (F) arising in the right coronary artery (RCA)
6、 and ending in the pulmonary artery (PA),A second fistula arising in the left anterior descending artery (LAD), also terminating in the pulmonary artery.8Multislice computed tomographic angiography (C and D) showed the two fistulas (F1 and F2) entering the pulmonary artery separately.An attempt at c
7、oil embolization of the right coronary artery fistula failed and the patient was referred for surgical ligation of the fistulas. Post-operatively her symptoms have disappeared.9Case2:A 36-years-old healthy athlete.ECG showed a typical postero-septal accessory pathway with left ventricular pre-excita
8、tion at warm-up.The ECG alteration disappeared during the exercise in the absence of symptoms and other abnormalities. Physical examination was normal . Family history was unremarkable for heart disease. 10The athlete underwent two-dimensional trans-thoracic echocardiography to exclude the underlyin
9、g cardiac diseases; Colour-Doppler examination revealed an anomalous diastolic jet of flow directed into the main pulmonary artery trunk on the left sideA coronary artery fistula was suspected even though left-to-right shunt was not significant (Qp/Qs ratio 1.2) and there were no signs of pulmonary
10、or systemicoverload.11Cardiac computed tomography (CCT) was performed. It showed a complex fistula originating from all the proximal coronaries and draining into the main pulmonary artery, fistulizing into the pulmonary artery trunk(C; arrowhead) and surrounding the main pulmonary artery. （D; arrowh
11、eads）The fistula showed a main body right on top of the proximal segment of the main pulmonary artery (DF; asterisk) and also connection with bronchial arteries (E, F; arrowheads）12ADD TITLEConventional coronary angiography confirmed the findings (GI; arrowheads).In view of the lack of symptoms and signs of ventricular overload, the athlete was considered eligible for competitive sport but require to be monitored with ECG+echocardiography every 6 months.13THANK YOU
