脓毒性肺血栓栓塞在青少年法洛氏四联症.pdf

Images in Cardiovascular Medicine Septic Pulmonary Thromboemboli in an Adolescent With Tetralogy of Fallot Patrick Farahmand MD Alban Redheuil MD PhD Sylvain Chauvaud MD Je rome Jouan MD Amine Jemel MD Jean Noel Fabiani MD A 14 year old adolescent man from the Republic of Congo with no personal medical history presented with progressive shortness of breath and exercise limitation On clinical examination he had a 3 6 systolic murmur and regular cardiac rhythm and cyanotic fingers and clubbing as well Pulmonary auscultation was normal No recent episode of fever or shivering or peripheral sign of endocarditis were reported Pulse oxymetry showed oxygen saturation at 64 The diagnosis of Tetralogy of Fallot was subsequently estab lished Laboratory results showed a normal white blood cell count with no abnormalities The initial echocardiogram showed a large perimembra nous septal defect of 1 5 cm diameter an aortic override a predominant right to left ventricular shunt a severe infundib ular pulmonary stenosis and right ventricular hypertrophy Left ventricular diameter was 39 mm at end diastole and the left ventricular ejection fraction was normal A 4 mm mobile element was unexpectedly visualized at the level of the pulmonary valve An ECG gated multidetector pulmonary computed tomog raphy angiogram with iodinated contrast injection was then performed to explore this unusual finding Preoperative transesophageal echocardiography was not considered as appropriate for this young poorly cooperating adolescent patient It revealed a 28 14 mm hypodense intraluminal filling defect attached to the pulmonary valve Figures 1 and 2 This mass suggesting a thrombus or vegetation nearly occluded the pulmonary trunk In addition multiple bilateral pulmonary emboli were seen with images suggestive of mycotic aneurysms Figure 3 The right inferior lobar artery was occluded There were also pulmonary nodules in relation to distal pulmonary arteries with peripheral ground glass opacities indicative of peripheral pulmonary abscesses Fig ure 4 Bronchial arterial collaterals were well developed Pulmonary arterial pressure measurement by catheter was avoided because of the major risk of material migration Surgery was indicated to remove and analyze the mass from the pulmonary arterial trunk and depending on the resultant pulmonary arterial pressure to repair the Tetralogy of Fallot Intraoperative transesophageal echocardiography was per formed to confirm the diagnosis Movie I in the online only Data Supplement The intervention was performed through a median sternotomy and pulmonary arterial pressure was isosystemic 105 64 by direct measurement Hypothermic cardiopulmonary bypass with antegrade cold blood cardio plegia was used Incision of the pulmonary artery showed the mass which seemed thrombotic attached to a bicuspid pulmonary valve All of the abnormal material was excised from the main pulmonary artery without removing the pul monary valve and sent for pathological and bacterial exami nation Figure 5 After closure of the main pulmonary artery and weaning from cardiopulmonary bypass the pulmonary arterial pres sure remained isosystemic 108 67 Infundibular pulmonary stenosis protected this patient from pulmonary overcircula tion as we can see in Eisenmenger syndrome but multiple distal thromboemboli led to irreversible secondary pulmonary Figure 1 Computed tomography angiography axial view Large near obstructive fi lling defect of the pulmonary trunk corre sponding to a septic thrombus attached to the pulmonary valve arrow From the University of Paris Rene Descartes France P F A R S C J J A J J N F and Departments of Cardiovascular Surgery P F S C J J A J J N F and Cardiovascular Radiology A R Georges Pompidou European Hospital Paris France The online only Data Supplement is available with this article at http circ ahajournals org cgi content full 123 19 2164 DC1 Correspondence to Patrick Farahmand MD Department of Cardiovascular Surgery Georges Pompidou European Hospital 20 rue Leblanc 75908 Paris Cedex 15 France E mail patfarahmand Circulation 2011 123 2164 2166 2011 American Heart Association Inc Circulation is available at http circ ahajournals orgDOI 10 1161 CIRCULATIONAHA 110 991257 2164 at CHONBUK NATIONAL UNIVERSITY on May 16 2011 circ ahajournals orgDownloaded from arterial hypertension and thus curative treatment of the Tetralogy of Fallot could not reasonably be performed However the postoperative course was uncomplicated with the use of nitric oxide sildenafil and norepinephrine for 48 hours Pathological and bacterial analysis of the mass showed the presence of a recent fibrinocruoric thrombus with numerous colonies of Gram positive coccoidal bacteria Abiotrophia defectiva 1a nutritionally variant streptococcus was identi fied with complementary tests therefore antimicrobial ther apy with Ampicillin was used for 6 weeks to treat this endocarditis Anticoagulants were started immediately after the intervention and continued on a long term basis because of the major risk of lethal recurrent massive thromboemboli and despite the risk of intracranial bleeding facilitated by the Tetralogy of Fallot The patient was discharged from the hospital at day 5 after surgical intervention At 3 months after surgery there had been no worsening of his symptoms Pulmonary arterial thromboendarterectomy was not considered because of the septic nature of the distal lesions of the pulmonary arteries The only surgical therapy feasible for this patient would have been a cardiopulmonary transplantation which was not cho sen at this time because of social considerations Tetralogy of Fallot was not diagnosed in this patient in his early years and he was recently sent to France from Central Africa by the Chain of Hope because of the late appearance of clinical signs Although no clinical sign of infection was noticed initially computed tomography angiography images Figure 2 Computed tomography angiography transverse view Large near obstructive fi lling defect of the pulmonary trunk cor responding to a septic thrombus arrow Figure 3 Computed tomography angiography Association of images evoking multiple mycotic aneurysms dark arrow right inferior segmental pulmonary occlusion light arrow due to mas sive emboli and proximal and distal periarterial pulmonary nod ules with peripheral ground glass opacities evoking pulmonary abscesses checkered arrows Figure 4 Computed tomography angiography Left inferior pul monary artery dark arrow surrounded by peripheral ground glass opacities evoking pulmonary abscesses light arrow Figure 5 Macroscopic view of the thrombus attached to the pulmonary valve Farahmand et alUnusual Complication of Tetralogy of Fallot2165 at CHONBUK NATIONAL UNIVERSITY on May 16 2011 circ ahajournals orgDownloaded from were able to demonstrate severe signs of pulmonary infection with multiple pulmonary mycotic aneurysms pulmonary peripheral abscesses and pulmonary arterial occlusions Those lesions were secondary to pulmonary valve endocar ditis a rare complication of Tetralogy of Fallot 2Multiple septic emboli from the initial septic thrombus were respon sible for distal pulmonary occlusions therefore severe sec ondary isosystemic pulmonary hypertension precluded surgi cal curative treatment Disclosures None References 1 Sharaf MA Shaikh N Abiotrophia endocarditis case report and review of the literature Can J Cardiol 2005 21 1309 1311 2 Svane S Primary thrombosis of pulmonary artery in a child with tetralogy of Fallot Br Heart J 1977 39 815 819 2166CirculationMay 17 2011 at CHONBUK NATIONAL UNIVERSITY on May 16 2011 circ ahajournals orgDownloaded from 少年法洛氏四联症的脓毒性肺栓塞 少年法洛氏四联症的脓毒性肺栓塞 Patrick Farahmand MD Alban Redheuil MD PhD Sylvain Chauvaud MD Je rome Jouan MD Amine Jemel MD Jean Noel Fabiani MD 一位来自刚果共和国没有既往史的 14 岁少年表现为进行性加重的气短和活动受 限 体格检查发现心脏 3 6 收缩期杂音 心律整齐 指端青紫和杵状指 肺部听诊正 常 近期无发热 寒战或心内膜炎的外周症状 脉氧饱和度 64 随后确立法洛氏四联 症的诊断 实验室检查显示白细胞计数和形态正常 初始超声心动图显示一个大的膜周室间隔缺损直径 1 5cm 主动脉骑跨 显著的右 向左分流和严重的肺动脉漏斗状狭窄 以及右心室肥厚 左心室直径在舒张末期为 39mm 左心室射血分数正常 意外发现肺动脉瓣水平一个 4mm 活动团块 因为术前食管超声心动图不适合这样合作欠佳的青少年患者 使用碘造影剂心动 图门控多排计算机断层肺血管造影进一步检查 发现 一个 28 14mm 低密度管腔内充 盈缺损附着于肺动脉瓣 图 1 和 2 这个我们考虑为血栓或赘生物的团块几乎闭塞肺 动脉干 另外 在图像上发现多处双侧肺栓塞 提示霉菌性动脉瘤 图 3 右下叶肺 动脉闭塞 在远端肺动脉灌注区域也有肺结节伴外周磨玻璃影 提示外周肺脓肿 图 4 各级支气管动脉正常 为避免感染迁移风险未进行导管肺动脉压力测量 拟行手术切除肺动脉干肿块并 分析其性质 根据肺动脉压根治法洛氏四联症 术中施行经食管超声心动图证实诊 断 胸骨中线劈开后开始手术 直接测量肺动脉压同体循环动脉压相等 105 64 使用低温体外循环顺行冷血心脏停搏 切开肺动脉显示肿块 外观似血栓形成 附着 于二叶肺动脉瓣 从主肺动脉切除所有异物送病理学和细菌学检查 图 5 关闭肺动脉干后中断体外循环 肺动脉压仍与体循环等 108 67 漏斗状肺动 脉狭窄保护患者不至于因过度肺循环致艾森曼格综合征 但多发远端肺栓塞导致不可 逆的继发肺动脉高压 因此有效治疗法洛