先天性心脏病介入治疗1016例临床疗效分析

目的探讨先天性心脏病介入治疗的临床疗效。方法回顾性分析1016例行先天性心脏病介入治疗的先天性心脏病患者的临床资料,着重分析手术方法和结果。其中动脉导管未闭（PDA）364例,房间隔缺损（ASD）199例,室间隔缺损（VSD）270例,肺动脉瓣狭窄（PS）107例,房间隔缺损合并动脉导管未闭22例,室间隔缺损合并动脉导管未闭11例,肺动脉瓣狭窄合并动脉导管未闭8例,房间隔缺损合并肺动脉瓣狭窄27例,房间隔缺损合并室间隔缺损3例,法洛四联症合并侧支5例。结果技术成功率98．6％（1002／1016）,全组无死亡。随访1～6个月,封堵器位置固定,无移位或破损。结论先天性心脏病介入治疗具有成功率高、创伤小、并发症低,操作简单,疗效确切,恢复快等特点,是治疗先天性心脏病的理想手段之一。     

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。     

经皮介入治疗常见先天性心脏病500例临床疗效和安全性分析

【】目的 回顾性分析500例使用Amplazter封堵器介入治疗先天性心脏病的临床疗效和安全性进行分析 。方法 回顾性分析我院2003年1月-2015年5月500例先天性心脏病患者的临床资料,男208 例,女292 例,年龄2-65岁(平均15.5岁),室间隔缺损88 例,房间隔缺损300 例,动脉导管未闭106 例,肺动脉瓣狭窄6例,其中室间隔缺损合并房间隔缺损3 例,房间隔缺损合并动脉导管未闭3 例,室间隔缺损合并动脉导管未闭 2例S,所有患者均经临床心电图、经胸超声心动图进行术前的筛选,术中监测和术后随访,所有患者均采用国产封堵器及输送系统。结果 总成功率96.8％,室间隔缺损 4例失败,房间隔缺损8 例失败,动脉导管未闭4 例失败,住院时间3-7天,全组介入治疗患者门诊行心彩超、心电图、胸部X片随访1-3年,均无并发症发生。结论 只要严格掌握手术适应症,经皮导管介入治疗先天性心脏病操作简单、创伤小、安全、可靠、成功率高、并发症低、恢复快、疗效确切,部分患者可替代外科手术,同时国产封堵器价格低,封堵效果好。     

同期介入治疗先天性心脏病多发畸形疗效分析

目的:探讨经导管同期介入治疗先天性心脏病多发畸形的可行性、安全性和疗效,并分析其治疗策略。方法:选择136例先天性心脏病多发畸形患者,其中房间隔缺损(ASD)合并肺动脉瓣狭窄(PS)46例,ASD合并室间隔缺损(VSD)32例,ASD合并动脉导管未闭(PDA)20例,VSD合并PDA 20例,PDA合并PS 12例,VSD合并PS 4例,PDA合并PS及ASD 1例,VSD合并PS及ASD 1例,所有患者均在导管室行同期介入治疗,治疗顺序为:肺动脉瓣狭窄球囊扩张术、室间隔缺损封堵术、动脉导管未闭封堵术、房间隔缺损封堵术。术后连续心电监护5天,并定期随访心电图(ECG)及经胸超声心动图(TTE)检查。结果:136例患者介入治疗均获成功,术后心导管造影及TTE提示封堵器固定好,未见残余分流。定期随访无严重并发症发生。结论:严格掌握适应证,正确选择手术操作顺序与方法,经导管同期介入治疗先天性心脏病多发畸形成功率高,疗效可靠。     

房水平单向活瓣式补片在先天性心脏病并发重度肺动脉高压术中的应用

目的:探讨并发重度肺动脉高压的先天性心脏病患者的外科手术方式。方法: 采用房水平单向活瓣补片对48例本病患者进行手术治疗。其中,室间隔缺损30例,房间隔缺损3例,室间隔缺损并发房间隔缺损8例,室间隔缺损伴动脉导管未闭7例。结果: 术后1例（2%）死于低心排综合征。术后早期均有明显低氧血症。术后1周仍出现跨单向活瓣补片分流40例,术后3个月活瓣仍开放10例,术后26个月提示活瓣均关闭,分流消失,经皮血氧均为95%以上,肺动脉压均明显下降。结论: 房水平单向活瓣式补片治疗先天性心脏病并发肺动脉高压,使患者度过术后危险期,提高手术成功率。     

新生儿和小婴儿危重先天性心脏病急诊手术

目的探讨新生儿和小婴儿危重先天性心脏病急诊手术的必要性及提高手术疗效的措施。方法收集新生儿和小婴儿危重先天性心脏病55例的急诊手术资料，男性35例，女性20例。患儿术前有反复肺炎心衰史或持续缺氧发作，其中动脉导管未闭（PDA）合并肺动脉高压（PH）3例、室间隔缺损（VSD）伴肺动脉高压34例、室间隔缺损（VSD）合并房间隔缺损（ASD）8例、室间隔缺损合并动脉导管未闭4例、严重肺动脉狭窄（PS）合并房间隔缺损4例、法洛四联症（TOF）2例，均行急诊手术治疗。结果术后并发气胸3例、呼吸机相关性肺炎2例、拔管后再插管3例、低心排2例、术后早期严重心功能不全合并有毛细血管渗漏综合征3例，常规治疗下仍少尿或无尿，持续3-5h，肺内渗出增多，中心静脉压进行性上升，均行腹膜透析治疗。全组病例51例痊愈出院，死亡4例。结论新生儿和小婴儿危重先天性心脏病急诊手术效果较好。合理的围手术期管理、合适的体外循环方法及手术方案是提高手术成功率的关键。     

组织多普勒速度成像评价先天性心脏病合并肺动脉高压患者左心室功能的研究

目的:探讨组织多普勒速度成像技术(TVI)评价先天性心脏病(先心病)合并肺动脉高压(PAH)患者左心功能的价值。方法:选取先心病合并肺动脉高压患者30例分为3组,轻度肺动脉高压12例,中度10例,重度8例,其中4例临床诊断为艾森曼格综合征失去手术机会,畸形为室间隔缺损20例,动脉导管未闭8例,房间隔缺损合并室间隔缺损2例。全部病例均为在我院确诊为先心病并在我院拟行手术治疗的患者。分别对以上病例进行常规超声测量及进行TVI成像模式下参数测定。结果:3组患者相比:重度肺动脉高压组收缩期室壁前向运动速度较轻中度肺动脉高压组明显减低,舒张早期运动速度(Ve)减低,二尖瓣环运动速度降低。结论:TVI组织速度成像技术可无创有效的评估肺动脉高压患者左心功能的变化。     

基层医院开展体外循环心内直视手术25例体会

我院与北京阜外医院协作,自1990年5月至1991年3月,在体外循环下行心内直视手术25例,现重点讨论基层医院开展此类手术的体会如下: 临床资料本组25例中,男性14例、女性11例,年龄4～41岁,体重12.5～50kg。先天性心脏病21例,其中房间隔缺损1例,房间隔缺损并右肺静脉异位引流1例,室间隔缺损13例(伴有肺动脉高压者2例),肺动脉瓣狭窄2例,动脉导管未闭合并肺动脉高压2例,法乐氏四联症2例。风湿性心脏病二尖瓣损害4例。     

先天性心脏病合并重度肺动脉高压患者围手术期麻醉管理

对49例先天性心脏病合并重度肺动脉高压患者,行房间隔缺损修补术9例,室间隔缺损修补术26例,动脉导管心内直视修补术3例,部分性心内膜垫修补术9例,全肺静脉异位引流纠治术2例,同时行严格的围手术期麻醉管理.结果麻醉过程平稳,术后治愈出院47例,死亡2例.认为对先天性心脏病合并重度肺动脉高压患者围手术期的麻醉处理,只有遵循保持患者血流动力学稳定,降低肺动脉压、肺循环阻力,维护心功能,避免肺动脉压危象发生的原则,才能提高手术成功率、降低手术死亡率.     

Congenital unilateral absence of a pulmonary artery: a report of two adult cases

Congenital unilateral absence of a pulmonary artery (UAPA) is a rare anomaly that is generally diagnosed in childhood and frequently associated with other cardiovascular abnormalities. Two patients with unilateral absence of a pulmonary artery first diagnosed as adults are reported. Our two cases demonstrate that presentation of UAPA may range from an incidental finding to chronic, life-threatening symptoms. Because UAPA may require urgent intervention, physicians should be alerted to the possibility of this entity presenting later in life.     

单纯性单侧肺动脉缺如的临床特点及回顾性分析

目的:提高对单纯性单侧肺动脉缺如(UAPA)的认识和早期诊治水平。方法:结合我院2009年经肺血管造影检查明确诊断的1例单纯性单侧肺动脉缺如的临床资料,和国内1999年至2009年文献检索到的13例单纯性单侧肺动脉缺如病例的详细资料,对UAPA的临床特点进行回顾性总结分析。结果:我院患者以胸闷憋气症状就诊,曾于外院误诊为"肺栓塞",行多排CT肺动脉造影(CTPA)、X线肺血管造影(CPA)最终确诊单纯性UAPA。总结14例病例后发现单纯性UAPA病例以成年人多见(10/14),女性8例,男性6例,左侧缺如6例,右侧缺如8例,主要症状为:咳嗽、咳痰、反复肺部感染(9/14),活动后胸闷、气促、喘息(9/14),咯血(3/14),最终以选择性肺血管造影检查和多排螺旋CT肺血管造影确诊病例为最多,分别为6例,5例。在8例有治疗资料中,对症治疗4例(4/14),手术治疗4例(4/14)。结论:UAPA是一种罕见的先天性肺血管畸形,单纯性UAPA更为少见,症状以咳嗽、咳痰、咯血、反复肺部感染、胸闷及气促为主。目前X线肺血管造影仍然是诊断UAPA的金标准。近几年多排螺旋CT肺血管造影、核磁共振(MRI)肺血管造影正逐渐成为诊断UAPA的重要手段之一。目前单纯UAPA的治疗方法主要以对症治疗为主,外科治疗可根据病情选择患侧肺动脉重建、选择性肺侧支血管栓塞和结扎、患侧全肺或肺叶切除术。     

Isolated unilateral absence of a pulmonary artery: a case report and review of the literature

OBJECTIVE: The purpose of the present study was to determine the symptomatology, diagnostic procedures, and therapeutic strategies of patients with an isolated unilateral absence of a pulmonary artery (UAPA). BACKGROUND: Isolated UAPA is a rare anomaly. Some case reports exist, but the best diagnostic and therapeutic approaches to these patients remain unclear. METHODS: A retrospective analysis was made of 108 cases reported between 1978 and 2000. The database of the National Library of Medicine (MEDLINE) was used to identify cases that were published in any language from 1978 onward. RESULTS: Of the 108 patients identified, 14 were asymptomatic. The median age was 14 years (range, 0.1 to 58 years). Most patients had symptoms such as frequent pulmonary infections (37%), dyspnea or limited exercise tolerance (40%), or hemoptysis (20%). Pulmonary hypertension was present in 44% of the patients. Surgical procedures were performed in 17% of patients, and the overall mortality rate was 7%. CONCLUSION: Only a few patients with isolated UAPA remain asymptomatic during follow-up. The diagnosis can be made by chest radiograph, echocardiography, CT scan, and MRI. Hilar arteries can be shown by cardiac catheterization and pulmonary venous wedge angiography. This is important since revascularization may improve pulmonary hypertension. The avoidance of high altitudes and pregnancy may further improve outcomes.     

Outcomes of Percutaneous Closure of Patent Ductus Arteriosus Accompanied With Unilateral Absence of Pulmonary Artery

Background

Limited data have reported the outcomes of percutaneous closure of patent ductus arteriosus (PDA) in patients with unilateral absence of pulmonary artery (UAPA). This study aimed to evaluate the symptomatology, diagnosis and therapy, especially the transcatheter closure of PDA in patients with PDA associated with UAPA.

Absence of the fetal right pulmonary artery complicated with coarctation of the aorta: Prenatal and postnatal diagnosis

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital cardiovascular malformation that can present as an isolated lesion or may be associated with other congenital heart malformations. Several studies have reported UAPA after birth. To our knowledge, the absence of the right pulmonary artery in the fetus has not been reported. Here, we report a rare case of fetal right pulmonary artery absence with aortic coarctation, which was confirmed by postpartum ultrasound and computed tomography angiography (CTA). Our case demonstrates that fetal echocardiography, especially the three‐vessel view, is beneficial for the prenatal diagnosis of pulmonary artery malformations.     

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.     

Clinical, echocardiographic and evolutive aspects of right atrial thrombosis

The clinical and echocardiographic features of right atrial thrombi were examined in 9 patients, 5 men and 4 women aged 16 to 86 years. The 2D echocardiographic diagnosis was confirmed at autopsy (4 cases) or by the association of severe recurrent pulmonary embolism (5 cases). Three patients had associated ischaemic heart disease and on patient had dilated cardiomyopathy. The clinical presentation was: acute cor pulmonale (5 cases including 2 patients which biventricular myocardial infarction), chronic post-embolic cor pulmonale (1 case), tricuspid valve obstruction (1 case), general ill health with pyrexia (1 case) and heparin-induced thrombocytopenia (1 case). Predisposing factors included: absence of anticoagulent therapy (7 cases), previous supraventricular arrhythmias (2 cases) and right ventricular failure (6 cases, including 2 of right ventricular infarction). In 2 patients the thrombi were relatively immobile and had a wide base of implantation on the interatrial septum; in 1 patient, multiple thrombi were observed lining the right heart cavities from the inferior vena cava to the pulmonary infundibulum. In the other 6 patients, the thrombi were very mobile with a visible pedicule of implantation (2 cases) or totally free (4 cases). The variable polylobulated appearances, completely irregular whirling motion and intermittent prolapse into the tricuspid valve were characteristic features of the latter 4 cases. They disappeared spontaneously (2 cases) or after fibrinolytic therapy (2 cases) in under 36 hours. Three patients were operated with one postoperative death. The global hospital mortality was 22%. The present occasional detection of right atrial thrombosis will certainly become more common if patients with pulmonary embolism, right ventricular infarction or deep venous thrombosis are systematically examined by 2D echocardiography in the acute phase of their illness.     

Clinical and angiocardiographic observations on the so-called absence of a branch of the pulmonary artery

We present 5 cases with angiographically absent pulmonary artery: 4 with absence of right pulmonary artery and 1 with absence of the left. We excluded cases of atresia of pulmonary artery, ventricular septal defect and major aorto-pulmonary collaterals. We excluded also cases of anomalous origin of a pulmonary artery from the aorta. Surgical inspection in 3 patients demonstrated total absence of pulmonary main branch (1 case), with intraoperative death, and presence of the hilar portion of the pulmonary artery (2 cases), with successful palliative procedure. It is supportive of efforts to identify as early in life as is feasible the absent vessel, for surgical purposes. In fact the early surgical procedure will ensure development of the vessel and normal lung growth, and will decrease the risk of the following intracardiac repair, when needful.     

Reactive haemophagocytic syndrome in adult-onset Still's disease: a report of six patients and a review of the literature

OBJECTIVE: To examine the prevalence and characteristics of patients with reactive haemophagocytic syndrome (RHS) complicating adult-onset Still's disease (AOSD). METHODS: Of 50 patients with AOSD fulfilling Yamaguchi and Fautrel criteria followed in our department, clinical and laboratory data, course and treatment of six patients with histologically proven RHS and without any obvious cause other than AOSD were retrospectively recorded. RESULTS: RHS led to AOSD in two cases, whereas it appeared after a mean duration of 3.5 years from onset of AOSD in the other cases. The main symptoms were fever (n = 6), polyarthralgias or myalgias (n = 4), lymphadenopathy or splenomegaly (n = 3), pharyngitis (n = 3), rash (n = 3), pleuritis (n = 3), hepatomegaly (n = 1), normal or low leucocyte count (n = 4), anaemia (n = 6), lymphocytopenia (n = 6), thrombocytopenia (n = 4), hyperbasophilic lymphocytes (n = 2), abnormal liver function tests (n = 6) and increased serum triglyceride level (n = 6). Serum ferritin concentration was constantly increased (>10,000 microg/l in five cases, with <5-35% in glycosylated form). Two patients presented with coagulopathy. Treatment comprised corticosteroids (n = 4) and intravenous immunoglobulins (n = 3), whereas prednisone was unchanged in one case. One death due to pneumonia occurred 15 days after RHS. With a follow-up ranging from 2 to 7.5 years, the other patients were in remission with prednisone plus etanercept (n = 1), prednisone plus methotrexate (n = 1), low-dose prednisone (n = 2) or without treatment (n = 1). CONCLUSION: RHS is not uncommon in AOSD. It should be evoked in a patient with AOSD in the absence of hyperleucocytosis, thrombocytopenia, lymphopenia and coagulopathy, or in the presence of high serum ferritin and triglyceride levels.