Pulmonary Artery Sarcoma Masquerading as Chronic Pulmonary Thromboembolism

We describe the case of a 60-year-old woman who presented with pulmonary artery sarcoma, a very rare tumor of the cardiovascular system. Her tumor was initially misdiagnosed as chronic pulmonary thromboembolism, and she underwent pulmonary endarterectomy.Early diagnosis of primary pulmonary artery sarcoma is crucial. That alternative should always be considered before settling on a diagnosis of pulmonary embolism. Suspicion should be aroused by the failure of anticoagulant treatment to alleviate pulmonary perfusion abnormalities and systemic symptoms. Surgical resection of the tumor—preferably by pulmonary endarterectomy, followed by reconstruction as needed—is currently the most promising treatment for pulmonary artery sarcoma.     

The Diagnosis and Treatment of Primary Pulmonary Artery Sarcoma: Two Case Reports

Pulmonary artery sarcoma (PAS) is considered a very rare tumor with a poor prognosis. We herein report two cases of PAS that were diagnosed by positron emission tomography (PET)/computed tomography (CT). In both cases, PET was an effective option for diagnosing tumors, and surgical resection was a valid treatment for these diseases. If a pulmonary artery tumor is suspected, PET/CT is useful for diagnosing PAS and very helpful for choosing the surgical treatment strategy.     

Pedunculated Pulmonary Artery Sarcoma Suggested by Transthoracic Echocardiography

Pulmonary artery sarcoma (PAS) is an extremely rare malignancy. It is usually found after it grows large enough to occupy almost the entire lumen of the pulmonary artery and causes serious clinical symptoms. Thus, it is usually difficult to distinguish PAS from pulmonary thromboembolism based on imaging examinations. Few case reports had shown the attachment of PAS to pulmonary artery, a key characteristic for diagnosis, and differential diagnosis of PAS. In this case, we found a PAS, which did not cause local obstruction and some tumor emboli, which obstructed the branches of the pulmonary arteries and caused pulmonary hypertension and clinical symptoms. Transthoracic echocardiography (TTE) revealed a part of the tumor attached to the intima of the main pulmonary artery with a peduncle and had obvious mobility, which was suggestive of PAS and differentiated it from the pulmonary thromboembolism. To our knowledge, this is the first case report of a pedunculated PAS suggested by TTE. Combined with pulmonary artery computed tomography angiography, the diagnosis of PAS is strongly suggested before the operation. This case indicates that TTE could reveal the attachment and mobility of PAS in the main pulmonary and may provide useful information for the diagnosis and differential diagnosis of PAS, especially a pedunculated PAS.     

肺动脉栓塞在多层螺旋CT强化及后处理中的影像表现及诊断评价

目的观察肺动脉栓塞（PAE）在多层螺旋CT（MSCT）强化下扫描及后处理图象中的特征,评价其对肺动脉栓塞的诊断价值。方法35例可疑PAE病人均行MSCT强化扫描,并进行多平面重建（MPR）、最大密度投影（MIP）、容积再现（VR）及仿真内窥镜（VE）后处理。结果35例受检者主干肺动脉至段肺动脉的显示率为100％;对亚段及5级肺动脉的显示率为80．23％。在35例中32例可见到不同部位、不同类型和形态的充盈缺损,并有不同种类的间接征象。3例未见肺动脉内充盈缺损,其中2例各有1种间接征象。结论多层螺旋CT扫描及重建后处理是肺动脉栓塞的快速、有效、安全、简便可行的诊断方法。     

急性肺动脉血栓栓塞症核素肺灌注显像与电子束计算机断层摄影术的对比研究

目的通过对急性肺栓塞患者的核素肺灌注显像与电子束计算机断层摄影术(EBCT)的对比分析,初步探讨两者之间存在差异的原因和规律,以及肺动脉血管阻塞与血流灌注变化之间的对应关系。方法20例急性肺栓塞患者,24h内先后接受核素肺灌注显像和EBCT肺血管增强造影。逐个肺段对比EBCT所显示的肺动脉阻塞与肺灌注显像所显示的肺血流灌注的情况。结果多数(81.7%)肺动脉完全闭塞型病变对应相应肺段的灌注完全缺失,血流灌注分布与非完全闭塞型病变相比有显著性差异(P<0.01)。在非完全闭塞型病变中,多级肺动脉病变对应灌注完全缺失的比例较高(60.0%),多级肺动脉病变与单级肺动脉病变相比,血流灌注亦有明显的差异(P<0.05)。而不同类型的非完全闭塞型病变所对应的血流灌注没有明显差异(P>0.05)。结论不同形式的血管阻塞所造成的血流灌注变化的不同,是EBCT与肺灌注显像存在差别的主要原因之一,肺栓塞的血管阻塞和灌注受损是相互联系又有所不同的两个方面,肺灌注显像和EBCT结合应用能够使肺栓塞的诊断和疗效评价更加全面准确。     

Catheter-Based Therapies for Massive Pulmonary Embolism

Massive pulmonary embolism carries a high mortality rate as a result of right ventricular failure. In addition to anticoagulation, systemic thrombolysis is the standard first line of therapy for patients with life-threatening massive pulmonary embolism. Surgical embolectomy is often considered in patients with contraindications to receiving systemic thrombolysis or when thrombolysis has failed. Surgical embolectomy is not without inherent risk and limitations.Although there is a paucity of large clinical trials, available data suggests catheter-based treatment of massive pulmonary embolism restores hemodynamic stability and thus is an alternative to surgical therapy.     

Echocardiographic and Doppler Findings in Primary Sarcoma of the Pulmonary Artery

Primary sarcoma of the pulmonary artery is a rare heart tumor. In the reported case, the clinical findings were nonspecific, and were characterized by progressive dyspnea, fever, and a systolic murmur in the pulmonary area. Echocardiographic examination showed an echogenic mass partially obstructing the pulmonary artery trunk, dilation of the right cardiac chambers, and a pressure overload pattern. Doppler and color Doppler demonstrated a high-velocity systolic flow jet in the pulmonary artery due to obstruction of the vessel by the tumor, as well as severe high-velocity tricuspid regurgitation. The patient died suddenly soon thereafter. The autopsy confirmed almost total occlusion of the pulmonary artery by a neoplastic mass. Histopathological diagnosis was primary vascular leiomyosarcoma of the pulmonary artery. In addition, a large tumor embolus had occluded the right pulmonary artery. Doppler echocardiography proved useful in noninvasively recognizing the sarcoma of the pulmonary artery, and explaining the clinical picture and hemodynamic derangements produced by this tumor. This could, in other patients, allow an early diagnosis and timely surgical intervention.     

多层螺旋CT肺动脉造影在诊断周围型动脉栓塞中的应用

目的探讨多层螺旋CT肺动脉造影(MSCTPA)对周围型肺栓塞(PPE)的诊断价值。方法回顾性分析417例MSCTPA的患者资料,结合多层面曲面重组成像(CPR)、最大密度投影(MPR)、曲面重组(MIP)等图像后处理手段,分析PPE的MSCTPA的表现。结果检出的188例周围型动脉肺栓塞患者中,孤立性PPE 1例,PPE合并中心型肺动脉栓塞187例。188例患者共检出PPE 1583支,其中,累及亚段1012支,亚亚段PE 473支,亚亚段以远分支PE 98支。结论 MSCTPA能够准确显示PPE的范围及影像表现特征,对诊断PPE及病情评估具有重要的影像学参考价值,是诊断PPE的首选影像学检查手段。     

Intimal Sarcoma in the Aortic Arch Partially Obstructing the Aorta with Metastasis to the Brain

Primary tumors of the aorta are rare entities. We report the unusual manifestation of an aortic intimal sarcoma that presented as a brain metastasis in a 56-year-old, otherwise healthy woman. After the brain mass had been resected, multiple imaging methods revealed pseudocoarctation and the primary tumor in the aortic arch. To our knowledge, this is the first report of the diagnosis of an aortic intimal sarcoma with use of real-time, 3-dimensional transesophageal echocardiography.     

Isolated Spontaneous Celiac Artery Dissection in a 47-Year-Old Man with von Willebrand Disease

Isolated spontaneous dissection of the celiac artery is rare, and its occurrence without aortic dissection is even rarer. The typical symptom of this dissection is acute-onset abdominal pain. Complications of the condition include aneurysm formation, rupture, and abdominal-organ ischemia or infarction, especially in the liver or spleen. We report the case of a 47-year-old man with von Willebrand disease who had an isolated spontaneous dissection of the celiac artery. We used computed tomography and computed tomographic angiography in the diagnosis and characterization of the dissection. To our knowledge, this is the first report of celiac artery dissection in a patient with von Willebrand disease.     

Q Fever Endocarditis Presenting with Superior Mesenteric Artery Embolism and Renal Infarction

Q fever is a zoonotic disease with a reservoir in mammals, birds, and ticks. Acute cases in human beings can be asymptomatic, or they can present with a flu-like illness, pneumonia, or hepatitis. Approximately 5% of cases progress to chronic Q fever. Endocarditis, the most typical manifestation of chronic Q fever, is usually associated with small vegetations that occur in patients who have had prior valvular damage or who are immunocompromised. We present what we think is the first reported case of superior mesenteric artery embolism from Q fever endocarditis of the aortic valve, in a 39-year-old woman who needed surgical embolectomy and subsequent aortic valve replacement.     

原发性肺动脉肉瘤误诊为肺血栓栓塞症原因分析

目的提高对原发性肺动脉肉瘤（PAS）的认识和早期诊治率。方法结合3例肺动脉肉瘤患者的的临床资料和国外有关文献,对肺动脉肉瘤及肺血栓栓塞症（PTE）的临床特点进行分析。结果肺动脉肉瘤的临床表现和肺血栓栓塞症相似,但前者多有发热、体重下降,无下肢深静脉血栓形成,胸部增强CT显示肺动脉扩张,主肺动脉及左、右肺动脉内大量肿块阴影,管腔外肿块阴影,溶栓抗凝治疗无效等,确诊需手术。结论肺动脉肉瘤易误诊为肺血栓栓塞症,临床医师应提高认识。     

Endometrial Stromal Sarcoma Metastatic from the Uterus to the Inferior Vena Cava and Right Atrium

Endometrial stromal sarcoma metastases usually occur within the pelvis and rarely involve the great vessels or the heart. We present the case of a 55-year-old woman who was referred for endovascular therapy to treat presumed thrombosis of the inferior vena cava. The suspected thrombus was recalcitrant to endovascular removal with use of an AngioVac venous drainage device. Results of an intraprocedural transvenous biopsy revealed the mass to be the intravascular extension of an endometrial stromal sarcoma. The patient underwent surgical excision of the tumor, and, shortly thereafter, a hysterectomy and salpingo-oophorectomy. This complex case highlights both the rarity of malignancy masquerading as caval thrombus and the importance of multispecialty collaboration.     

Transthoracic Echocardiographic Assessment of Spindle Cell Sarcoma of the Pulmonary Artery in a Child

In this report, we present a case of spindle cell sarcoma of the pulmonary artery diagnosed by transthoracic echocardiography. To the best of our knowledge, this case is the youngest reported case of pulmonary artery sarcoma (PAS) to date. PAS is frequently confused for pulmonary embolism; in this case, echocardiographic findings allowed for differentiation between pulmonary embolism and solid tumor.     

Massive Pulmonary Artery Aneurysm Causing Left Main Coronary Artery Compression in the Absence of Pulmonary Hypertension

We report the case of a 62-year-old woman who presented with classic symptoms of stable angina. Cardiac images and catheterization results revealed absent pulmonary valve syndrome and compression of the left main coronary artery by a massively dilated pulmonary artery aneurysm. The patient''s anginal symptoms were relieved after pulmonary arterioplasty.Others have described proximal left main coronary artery compression in the presence of a dilated and hypertensive pulmonary artery. To our knowledge, this is the first case in which a pulmonary artery aneurysm caused left main coronary insufficiency in the absence of pulmonary hypertension—a clinically important complication of congenital pulmonary valve-related pulmonary arteriopathy.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Diagnoses and Surgical Results in 12 Pediatric Patients

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital condition. It responds well to early diagnosis and treatment, but otherwise the prognosis is poor. We present our case series of 12 patients (mean age, 2 ± 2.58 yr; age range, 2 mo–8 yr), emphasizing the diagnostic process and discussing our surgical results. The diagnosis of ALCAPA should be suspected in infants who have dilated cardiomyopathy with electrocardiographic changes that suggest ischemia, and in older children who have isolated mitral regurgitation. When clinical suspicion is high, the results of 2-dimensional echocardiography combined with color-flow Doppler studies in expert hands can establish the diagnosis, thus avoiding angiography in critically ill infants. The treatment of choice in our patients was transfer and reimplantation of the left coronary artery onto the ascending aorta. There were 2 deaths: both were infants in extremis who underwent emergency surgery. An older child with severe ventricular dysfunction was given mechanical ventricular assistance and then heart transplantation. As of this report, all 10 survivors remained well and asymptomatic.     

中国肺动脉栓塞误诊近四年文献分析

目的研究中国目前阶段肺动脉栓塞（PE）误诊状况,旨在调查我国目前PE误诊状况发生的变化。方法通过中国期刊网中国医院知识仓库（CNKI-CHKD）检索2001-2004年发表与PE误诊有关的病例研究报告,对被误诊患者一般情况、治疗及转归、死亡原因、发表文献医院级别、误诊疾病等进行了回顾性调查研究,与1980-2000年的误诊情况作了比较,并调查了1994-2004年每年发表有关PE所有文献与误诊文献篇数变化的趋势。结果（1）经检索后共筛选出误诊文献110篇,累计报告PE误诊患者1540例,误诊时间0．5小时～16年,平均1．86年,涉及临床、诊断技术多个科室。（2）首诊误诊经确诊后进行有效干预（抗凝、溶栓）组,有效率高于误诊后未干预（内科保守治疗）组（OR为11．67,95％CI5．861-23．249）,病死率低于未干预组（OR为0．19,95％C10．084-0．412）。未干预组死亡原因主要为猝死、休克难以纠正等,干预组死亡原因为猝死、脑出血、休克等。（3）省级医院、地市级医院、县级医院、乡镇厂矿医院等报告误诊文献篇数与误诊病例数量分别为37篇547例（33．6％、35．5％）、43篇671例（39．1％、43．6％）、23篇265例（20．9％、17．2％）、3篇34例（2．7％、2．2％）。（4）1994--2004年发表有关PE的文献与误诊文献篇数逐年增多,平均每年递增分别为26．6％、9．1％。（5）被误诊疾病多达70余种,前4位依次是：冠心病449例次（26．8％）、肺炎217例次（12．9％）、充血性心力衰竭142例次（8．5％）、胸膜炎114例次（6．8％）。结论（1）误诊原因与首诊医师对病情不能充分认识有关,被误诊的患者若不能尽快确诊而进行有效干预则严重威胁其健康。（2）与1980-2000年比较,PE误诊疾病次序已有所变化,但冠心病与肺炎仍占据误诊疾病的前两位,应加强PE与两种疾病问的鉴别诊断能力。（3）中国医生对PE及其误诊的重视程度已有明显提高,但基层医院医师的认识水平有待进一步加强,应加强我国临床医师对肺栓塞知识继续教育普及的力度．我国防治PE的仟备仍仟萤而诺沅．     

Primary Pulmonary Valve Leiomyosarcoma in a 35-Year-Old Woman

Primary cardiac leiomyosarcomas are rare, with a high incidence of local recurrence. Herein, we report the case of a 35-year-old woman who was admitted with right ventricular failure and suspected pulmonary embolism. Upon echocardiography, we detected a mass in the pulmonary trunk that involved the pulmonary valve and led to valvular stenosis.The optimal protocol for treating these tumors is as yet unclear. Complete resection can rarely be achieved. However, palliative surgery is usually undertaken because many patients present with mechanical obstruction, such as significant pulmonary stenosis.