成人单纯性单侧肺动脉缺如1例并文献复习

单侧肺动脉缺如(unilateral absence of pulmona-ry artery,UAPA )是一种罕见的肺血管先天性畸形,多合并其他的先天性疾病,如法洛四联症等.而单纯性单侧肺动脉缺如(isolated unilateral absence of pulmonary artery,IUAPA)更为罕见,...     

单纯性左侧肺动脉缺如一例报道及文献复习

目的 提高对单纯性单侧肺动脉缺如(UAPA)的临床表现、病理生理、诊断和治疗的认识.方法 报道上海市静安区中心医院呼吸科诊治的1例单纯性左侧肺动脉缺如患者的相关资料,并结合从多个中文期刊数据库检索到的关于单纯性UAPA的病例资料进行回顾性研究.结果 44例患者平均31.5岁(3个月～69岁),主要症状有反复肺部感染(47.4％)、咯血(52.6％)、气喘或活动后气促(57.9％)、胸闷(31.6％)、心悸(18.4％)、胸痛(7.9％)等,有1例患者没有症状.43.2％(19/44)的患者存在肺动脉高压.CT肺动脉造影(CTPA)、MRI、电子束CT (EBCT)、血管造影(DSA)均有确诊价值.20.5％的患者施行了手术治疗.结论 单纯性UAPA是一种非常罕见的先天性畸形,其症状无明显特异性,易漏诊、误诊.可通过CTPA、MRI、EBCT、DSA等确诊.最理想的手术方法是患侧肺动脉重建术,但在我国还无法实行.     

单纯性左侧肺动脉缺如1例

<正>患者女性,68岁,因"反复咳嗽、咳痰、咯血42年,加重1个月余",于2011年3月9日入院。患者42年前曾因大咯血住院抢救,之后反复咳嗽、咳黄脓痰,伴间断少量咯血,一直诊断"支气管扩张症"。近10余年,出现渐进性活动后气     

一侧肺动脉缺如1例报道及文献复习

本文报告1例经心血管造影和计算机断层扫描（CT）、磁共振扫描（MRI）诊断的先天性右肺动脉缺如，并进行文献复习，主要分析了肺动脉缺如的病理生理改变、临床表现以及诊断和鉴别诊断。     

单侧肺动脉缺如患者心电图分析（附1例报告和相关中文文献分析）

单侧肺动脉缺如（UAPA）是一种少见的疾病,该病的临床和心电图表现均缺少系统的描述,往往因认识不足而导致误诊。现报道1例UAPA并对有关UAPA中文文献进行复习,分析该病的临床和心电图表现。     

肺动脉起源于动脉导管远端的影像学特点分析

目的:总结肺动脉起源于动脉导管远端的先天性心脏病影像学特点并探讨其形成机制。方法:回顾16例符合肺动脉起源于动脉导管远端的先天性心脏病患者的影像学资料结合胚胎学理论,总结、分析其形态学特点。结果:一侧肺动脉起自主肺动脉,对侧肺动脉缺如5例;一侧肺动脉起自主肺动脉,对侧肺动脉起源于动脉导管远端2例;一侧肺动脉起源于动脉导管远端,对侧肺组织由粗大体肺侧枝供应2例;双侧肺动脉分别起源于同侧动脉导管远端5例;一侧肺动脉起源于动脉导管远端,对侧肺动脉缺如1例;一侧肺动脉起源于动脉导管远端,对侧肺动脉连接分流管道1例。结论:肺动脉起源于动脉导管的先天性心脏病的影像学表现多样,其病理解剖特点与第六对主动脉弓发育异常的范围、出生后导管是否闭合及合并畸形相关。     

先天性单侧肺动脉缺如25例影像学诊断

目的:评价先天性一侧肺动脉缺如(UAPA)多排螺旋CTA的影像学诊断价值。方法:回顾分析25例我院明确诊断为先天性一侧肺动脉缺如的胸X线片、经胸超声心动图检查、胸部多排螺旋CTA及心血管造影表现。结果:15(15/25)例为右肺动脉缺如,10(10/25)例为左肺动脉缺如。单发UAPA 4例,均为右肺动脉缺如。UAPA合并其他心血管畸形21例,其中合并简单心血管畸形9例,合并复杂畸形12例,简单畸形中包括动脉导管未闭、房间隔缺损、室间隔缺损等,复杂畸形中包括肺动脉闭锁、法洛四联症各4例,肺动脉狭窄3例,另有大动脉转位等。9例经外科手术证实,12例经心血管造影证实。结论:对于一侧肺动脉缺如的诊断,多排螺旋CTA检查作为一种无创的检查方法与心血管造影同样具有明确诊断价值,CTA所显示的客观的解剖结构,为治疗方案的选择提供依据。     

右肺动脉缺如行肺静脉楔入造影1例

肺静脉楔入造影是用来显示在常规左右心常规造影不能满意提供肺总动脉及左右分支解剖资料的造影方法,为发绀型先天性心脏病患者手术前进一步确定肺动脉解剖的必要诊断手段,国内报告尚少.自2019年1月海南省开始实施新生儿先天性心脏病筛查项目,发现了不少危重先天性心脏病并及时实施治疗,其中1例经过筛查发现并最终确诊为右肺动脉缺如的...     

支架置入术治疗大动脉炎所致右肺动脉狭窄伴左肺动脉缺如一例

先天性一侧肺动脉缺如(unilateral absence of a pulmonary artery,UAPA)为一种罕见的心血管畸形,多与其他先天性心脏病并存。大动脉炎(takayasu’s arteritis,TA)为主动脉及其主要分支以及肺动脉或冠状动脉的慢性进行性非特异性炎症,引起血管的狭窄、堵塞或扩张。头臂动脉型、胸腹主动脉型、混合型均可合并肺动脉受累,单纯肺动脉受     

72例单侧肺动脉起源于升主动脉的外科治疗

目的 探讨单侧肺动脉起源于升主动脉的手术治疗经验及早中期预后。方法 2009年1月至2019年12月于上海交通大学医学院附属上海儿童医学中心共收治78例单侧肺动脉起源于升主动脉。6例患儿因无手术指征或行姑息手术,未纳入本研究。根据手术方式的不同,分为4组：直接吻合组（A组）、心包补片扩大组（B组）、大动脉flap肺动脉成形组（C组）和内隧道补片分隔组（D组）。结果 体外循环时间48.0~260.0 min,中位时间91.0 min;术中主动脉阻断时间27.0~151.0 min,中位时间54.0 min。院内死亡3例。术后反应性肺高压危象6例。64例术后规律随访,随访时间为2.4月~9.4年,中位时间为1.4年。无远期死亡发生。术后1、2、5年免于肺动脉梗阻的概率分别为70.0%、66.9%、62.8%。4种手术方式术后肺动脉梗阻的时间分布有差异（?2 = 7.904,P = 0.048）,且A组较B组（P = 0.046）和C组（P = 0.024）术后肺动脉梗阻发生更少。B组与C组,D组与A、B、C组术后肺动脉梗阻情况无统计学异。结论 单侧肺动脉异常起源于升主动脉术后预后较好。对于大年龄患儿,术后肺高压危象时有发生。单侧肺动脉梗阻是主要的术后并发症,需要密切随访术后肺动脉的情况。在没有吻合张力情况下,直接吻合是最好的再植方法。     

Case report of isolated congenital absence of right pulmonary artery with collaterals from coronary circulation

Isolated unilateral absence of a proximal pulmonary main artery is a rare congenital lesion which is often associated with other cardiovascular abnormalities and a diverse clinical presentation. It is usually diagnosed in childhood. Patients who survive into adulthood is uncommon. We report a case of 46 year old hypertensive and obese female who presented with progressive dyspnea. She had features of pulmonary hypertension. The diagnosis was confirmed by CT pulmonary angiography which showed absence of right pulmonary artery and conventional pulmonary angiography which showed ipsilateral lung receiving collaterals from Right coronary artery and its branches. The purpose of this report is to highlight the fact that UAPA, although a rare entity, should be kept in mind in patients with unexplained PAH and prolonged respiratory symptoms unresponsive to routine treatment modalities.     

Pathological features of the pulmonary artery in Takayasu arteritis

Little attention has been paid to the pathological features of the pulmonary artery in Takayasu arteritis. Autopsy specimens of 6 cases of this disease were studied. Lesions were found in the aortic arch and its brachiocephalic branches in all cases and in both the aortic arch and thoracoabdominal aorta in 5 cases. The pathohistologic characters of the pulmonary artery were very similar to those of the systemic artery. Stenosis-recanalization, so-called blood vessels-in-blood vessels, of the pulmonary elastic arteries were found in four cases. These lesions were not observed in the systemic arteries, and most of the newly formed channels in them seemed to be branches of bronchial arteries. Luminal obstruction of pulmonary muscular arteries was observed in 4 cases, cellular arteritis of muscular arteries in 2 cases, and angiomatoid dilatation of small blood vessels in 2 cases. Thus in this study we found peculiar stenosis-recanalization lesions of the pulmonary elastic arteries, and also showed that the pulmonary elastic and muscular arteries are frequently involved in Takayasu arteritis. These findings suggest that pulmonary hypertension could influence morbidity and long-term mortality in this disease.     

一侧肺动脉缺如合并先天性心脏病的介入治疗分析

目的:总结经导管介入治疗一侧肺动脉缺如合并先天性心脏病的疗效。方法:我科自2005年1月至2009年4月诊断5例一侧肺动脉缺如合并先天性心脏畸形,其中合并动脉导管未闭4例,合并房间隔缺损1例;左肺动脉缺如1例,右肺动脉缺如4例;本组男性1例,女性4例,年龄1.5～11岁,3例肺部反复感染、1例咯血及1例发绀。均通过临床体检、X线胸片、心电图、经胸超声心动图检查及多排CT或造影确诊。结果:4例合并动脉导管通过介入治疗成功封堵。术后平均3d出院,未出现并发症,随诊3个月～4年心脏均恢复正常大小。1例肺动脉高压为极重度,合并房间隔缺损,出现发绀,不能除外原发肺动脉高压因素,放弃介入治疗,予口服扩血管药物治疗,出院随诊。结论:一侧肺动脉缺如合并先天性心脏病临床常表现为中、重度肺动脉高压多伴一侧肺发育不良,手术风险大。应用介入治疗显示出创伤小、并发症少,恢复快等优势,挽救了一些外科也难以处理的高风险重症患儿。     

老年慢性肺原性心脏病合并冠心病临床分析

目的：探讨老年慢性肺心病合并冠心病的的临床特点。方法：回顾分析福建省老年医院2002年1月至2006年12月期间82例老年慢性肺心病合并冠心病患者（A组）和195例老年单纯慢性肺心病患者（B组）的临床资料，比较分析两组患者的临床特点。结果：两组比较是易患因素高血压、糖尿病和肥胖比例有显著差别（P〈0．05），临床表现陈旧性心肌梗塞史、完全性左束枝传导阻滞（LBBB）、心界向左或向左下扩大、持续缺血的ST—T改变、Ⅱ～Ⅲ度房室传导阻滞、电轴右偏和重度右心衰竭有显著差别（P〈0．05）。结论：慢性肺心病患者若有陈旧性心肌梗塞史、高血压、糖尿病、肥胖或LBBB、心界向左或左下扩大应考虑合并冠心病。     

Surgical correction of isolated unilateral absence of right pulmonary artery

A 2-year-old girl with isolated unilateral absence of right pulmonary artery is described. Catheterization at 5 months demonstrated hypoplastic right pulmonary artery by pulmonary venous wedge angiography, and the patient underwent right Blalock-Taussig shunt and angioplasty of right pulmonary artery with autologous pericardial roll as an initial step. At 2 years, she underwent anastomosis of right pulmonary artery to main pulmonary artery with an autologous pericardial tube. Postoperative computed tomography showed a patent reconstructed right pulmonary artery.     

Huge pulmonary artery aneurysm

Pulmonary artery aneurysms (PAAs) are uncommon entities. PAAs are caused mostly by trauma (often iatrogenic), infections and Behcet’s disease (BD). Less common causes are pulmonary hypertension, congenital heart disease and neoplasm. BD is a multisystem disorder presenting with recurrent oral and genital ulcerations, as well as ocular involvement, and PAA is one of its rare complications. A case of huge PAA, in which the usual criteria for the clinical diagnosis of BD were present, is described. Transcatheter embolization resulted in clinical improvement.     

原发性肺动脉肉瘤误诊误治原因分析

目的 提高对原发性肺动脉肉瘤的认识,减少误诊、误治.方法 结合北京安贞医院2001年10月至2009年3月手术病理证实的4例原发性肺动脉肉瘤患者的的临床资料和国外有关文献,对原发性肺动脉肉瘤的误诊、误治原因进行分析.结果 原发性肺动脉肉瘤的临床表现和肺血栓栓塞症相似,主要有活动性呼吸困难/气促、胸闷、胸痛、咳嗽、咯血、晕厥等,无特异性;彩色多普勒超声心动图显示右心扩大,三尖瓣返流.肺动脉高压,右室流出道及主肺动脉内团块样异常回声;多普勒血管超声显示下肢静脉正常;CT肺动脉造影显示肺动脉扩张,管腔狭窄,主肺动脉及左、右肺动脉内大块充盈缺损等.4例患者术前全部误诊为肺栓塞,给予溶栓抗凝治疗,疗效不佳.手术后死亡1例,3例存活者病情缓解,无一例辅助放疗和(或)化疗.结论 原发性肺动脉肉瘤临床上少见,易误诊为肺栓塞给予溶栓抗凝治疗,确诊需手术,手术为主的综合治疗是治疗此病的有效手段.