经皮球囊肺动脉瓣成形术治疗婴儿和新生儿肺动脉瓣狭窄及闭锁

探讨经皮球囊肺动脉瓣成形（PBPV）术治疗婴儿和新生儿肺动脉瓣狭窄（PS）及室间隔完整型肺动脉瓣闭锁（PA/IVS）的安全性及有效性。方法　2006年1月至2009年4月,广东省心血管病研究所心儿科收治PS及PA/IVS婴儿和新生儿63例。其中危重新生儿20例（31.7％）。术前经超声心动图确诊为PS 56例,PA/IVS 7例。危重新生儿PS需先用直径2.5～4.0 mm小球囊预扩张肺动脉瓣,PA/IVS患儿需先行射频瓣膜打孔术。PBPV术所选球囊直径为肺动脉瓣环径（PVAD）1.2～1.3倍（新生儿所选球囊直径为PVAD 1.0～1.2倍）。结果　全组63例（100％）PBPV术成功。球囊径/瓣环径：1.2±0.1。右室压力明显下降[术前（104.3±32.7） mmHg（1 mmHg = 0.133kPa）,术后（52.0±10.5） mmHg,P < 0.001],跨肺动脉瓣压力阶差明显下降[术前（99.2±23.5） mmHg,术后（27.7±12.4）mmHg,P < 0.001]。平均手术时间（88.1±36.2） min,平均X线曝光时间（16.9±11.1） min。术中6例（9.5％）出现并发症,2例心包积液,2例低氧血症,1例三尖瓣腱索部分撕裂, 1例室上性心动过速。平均随访（19.0±12.3）个月,3例术后因残余中度或重度PS,3个月后再次行PBPV术。本组所有患儿肺动脉瓣仅轻度反流。结论　PBPV术治疗婴儿和新生儿PS及PA/IVS安全、有效。     

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目的探讨经皮球囊肺动脉瓣成形(PBPV)术治疗婴儿和新生儿肺动脉瓣狭窄(PS)及室间隔完整型肺动脉瓣闭锁(PA/IVS)的安全性及有效性。方法2006年1月至2009年4月,广东省心血管病研究所心儿科收治PS及PA/IVS婴儿和新生儿63例。其中危重新生儿20例(31.7%)。术前经超声心动图确诊为PS56例,PA/IVS7例。危重新生儿PS需先用直径2.5～4.0mm小球囊预扩张肺动脉瓣,PA/IVS患儿需先行射频瓣膜打孔术。PBPV术所选球囊直径为肺动脉瓣环径(PVAD)1.2～1.3倍(新生儿所选球囊直径为PVAD1.0～1.2倍)。结果全组63例(100%)PBPV术成功。球囊径/瓣环径:1.2±0.1。右室压力明显下降[术前(104.3±32.7)mmHg(1mmHg=0.133kPa),术后(52.0±10.5)mmHg,P<0.001],跨肺动脉瓣压力阶差明显下降[术前(99.2±23.5)mmHg,术后(27.7±12.4)mmHg,P<0.001]。平均手术时间(88.1±36.2)min,平均X线曝光时间(16.9±11.1)min。术中6例(9.5%)出现并发症,2例心包积液,2例低氧血...     

不同手术时机对危重肺动脉瓣狭窄球囊扩张术疗效的影响研究

目的 探讨不同手术时机对危重肺动脉瓣狭窄球囊扩张术疗效的影响.方法 书面知情同意下纳入2007年4月至2011年12月四川大学华西第二医院胎儿超声心动图产前诊断和(或)经出生后超声心动图证实的危重肺动脉瓣狭窄患儿21例进行病例对照研究,实施介入手术时年龄≤60 d,其中产前诊断者7例(产前组),院外转诊14例(分为产后A组和产后B组,产后A组:产后7～28 d转诊到我院的危重型肺动脉瓣狭窄病例,共6例.产后B组:产后29～ 60 d转诊到我院的危重型肺动脉瓣狭窄病例,共8例).产前诊断者,经一体化干预小组共同会诊制定干预方案,进行宫内诊治、围产期监护、出生后急诊经皮肺动脉瓣球囊成形术(PBPV);院外转诊者实施急诊PBPV.术前、术后1年内定期复查超声心动图评估右心室-肺动脉压差(PG)及右心室Tei指数.结果 产前组出生后持续静脉滴注前列地尔状态下经皮氧饱和度(SpO2) 82％ ～ 92％(86.57％±5.34％),无心功能不全患儿.出生后3～6d成功实施PBPV,术后SpO2 (97.33±1.15)％,术前右心室肺动脉有创压差(86.34+11.77) mm Hg(1 mm Hg =0.133 kPa),术后右心室肺动脉有创压差(31.43±8.46)mmHg,术前RV Tei指数0.68±0.05,术后迅速降低,术后1个月恢复正常.1例术后7个月因再狭窄再次实施PBPV.院外转诊14例,其中产前A组6例,术前心功能不全1例;产后B组8例,术前心功能不全3例.持续静脉滴注前列地尔状态下SP02(83％ ～91％),手术时间为出生后10 ～57 d,术后SpO2改善,术后心功能不全症状很快好转,甚至消失;术前右心室Tei指数明显增高,术后逐渐下降,产后A组术后12个月恢复正常,产后B组术后随访12个月,RV Tei指数为0.51±0.06,仍然较产前组及产后A组高(P＜0.05).1例术后9个月因再狭窄再次实施PBPV.产前组、产后A组、产后B组低氧暴露时间分别为(4.43±0.68)、(16.33±4.46)、(41.25±9.19)d,组间差异有统计学意义(P＜0.05).结论 产前明确诊断危重型肺动脉变狭窄、产后早期PBPV的患儿术前状况好、低氧暴露时间短、术后右心室功能恢复良好.“危重肺动脉瓣狭窄围生期一体化干预模式”能明显改善疾病预后及患儿生存质量.     

婴儿肺动脉瓣狭窄的介入治疗

目的　总结经皮球囊肺动脉瓣成形术（PBPV）治疗婴儿肺动脉瓣狭窄（PS）的经验,评价其必要性、安全性和有效性。方法　回顾性分析2009年1月至2018年12月青岛市妇女儿童医院心脏中心行PBPV治疗68例婴儿PS的临床资料。结果　患儿平均手术年龄为（6.34±3.6）个月,平均手术体重为（7.81±2.11） kg,术前体重/年龄Z值为-0.80±1.38,提示生长受限为22例（32.4%）,合并紫绀15例（22.1%）,呼吸急促23例（33.8%）。超声心动图评估右室长径/左室长径比值平均值为0.56±0.73,胸部正位片心胸比平均值为0.56±0.43,术前血氧饱和度平均值为94.74%±6.10%。术前右室/左室收缩压力比值平均值为0.68±0.26。球囊扩张后,肺动脉瓣平均压力梯度由（75.57±18.61） mmHg降至（22.37±5.21） mmHg,右心室收缩压平均值由（83.78±22.44） mmHg降至（54.16±20.81） mmHg。手术即刻成功率为100%。术中无严重心律失常、心腔穿孔、腱索断裂、血管损伤等并发症。术后早期患儿轻-中度肺动脉瓣反流比例为53.3%,无重度反流。随访3个月～10年间再狭窄率2.94%,32例随访5年以上患儿肺动脉瓣反流程度未进一步加重,无特殊处理。所有患者均未出现明显的右心室功能障碍。与接受PBPV治疗幼儿PS相比,婴儿PS临床症状明显,病情较重,术后早期及中远期随访手术效果满意。对本研究婴儿PS进行分组比较分析发现低体重、低龄儿可获得同样良好手术效果。结论　PBPV治疗婴儿肺动脉瓣狭窄十分必要,技术操作安全可行,随访结果持续有效。     

肺动脉瓣狭窄合并房间隔缺损的介入治疗效果分析

目的 探讨儿童先天性肺动脉瓣狭窄合并房间隔缺损的介入治疗方法和临床随访效果.方法 27例肺动脉瓣狭窄合并房间隔缺损的患儿,同时进行肺动脉瓣成形术和房间隔缺损封堵术,术后即刻、1周、3个月、6个月行超声心动图检查,随访其临床效果.结果 27例经皮球囊肺动脉瓣成形术均获得成功,术后即刻测量跨肺动脉瓣压力阶差由术前的(79±62)mmHg降至(49±36)mmHg,术后超声心动图随访过程中跨肺动脉瓣压力阶差持续下降.超声心动图测量房间隔缺损直径为3.4～24.6(11.7±6.9)mm,选择封堵器的直径为8～32(14.6±8.7)mm,27例中有24例成功进行封堵.结论 介入治疗肺动脉瓣狭窄并房间隔缺损是一种安全可靠的方法,严格掌握适应让,特别是房间隔缺损的适应证,可取得良好的临床效果.     

球囊成形术治疗婴幼儿重度肺动脉瓣狭窄临床效果

目的 探讨经皮球囊肺动脉瓣成形术(PBPV)治疗婴幼儿重度肺动脉瓣狭窄(PVS)的效果和安全性.方法 自2001年9月至2010年6月,共对44例2 ～ 35个月的重度PVS患儿进行PBPV治疗,并按年龄分为婴儿组( ＜ 12个月,n ＝ 18)、幼儿一组(12 ～ 23个月,n ＝ 13)、幼儿二组(24 ～ 35个月,n ＝ 13)比较不同年龄组患儿PBPV的治疗效果和并发症.结果所有患儿均成功实施PBPV,术后右心室压力和跨肺动脉瓣压力阶差显著下降,无死亡和严重并发症发生.术后出现肺动脉瓣关闭不全13例(29.5%),三尖瓣关闭不全5例(11.4%).婴儿组术前右心室收缩后和术后右心室收缩后、术后肺动脉收缩压、术后跨肺动脉瓣压力阶差与幼儿一组、二组比较差别无统计学意义(P均＞ 0.05).随访显示,患儿跨肺动脉瓣压力阶差基本保持稳定,仅1例患儿再次升高.结论 PBPV治疗重度婴幼儿PVS安全可靠,能有效解除肺动脉瓣狭窄.与幼儿一组、二组比较,婴儿PBPV在疗效和安全性方面差别无统计学意义.     

食管超声引导下经胸镶嵌治疗婴幼儿房间隔缺损合并肺动脉瓣狭窄

目的 探讨单纯食管超声(TEE)引导下经胸镶嵌治疗婴幼儿房间隔缺损合并肺动脉瓣狭窄的有效性和安全性.方法 回顾2014年3月至2016年9月我科收治的继发孔房间隔缺损合并中重度肺动脉瓣狭窄患儿29例,其中男19例,女10例,年龄(3～18)个月,平均7.5个月,体重(5.5～11.4)kg,平均8.2 kg.术前经胸超声检查及术中经食管超声检查明确房间隔缺损均为中央型,直径(9.6±1.4)mm,所选择封堵伞的型号为大于房缺最大径2～4mm的封堵伞.合并肺动脉瓣狭窄其跨瓣压差(78.6±9.4)mmHg,肺动脉瓣瓣环直径(11.1±1.6)mm,所选择球囊直径为肺动脉瓣环直径1.2～1.4倍,反复扩张2～3次.全身麻醉气管插管,经胸剑突小切口,单纯食管超声引导下同期行房间隔缺损封堵及肺动脉瓣球囊扩张.术后3个月门诊经胸超声随访.结果 全组29例房间隔缺损封堵及肺动脉瓣球囊扩张均成功,术中及术后随访未出现并发症.术后即刻测压(18.3±9.3)mmHg,术后较术前明显下降(P＜0.001).术后即刻超声提示房间隔水平未见分流.术后3个月随访,房间隔封堵器位置正常,无残余分流,肺动脉瓣跨瓣压差为(19.9±5.8)mmHg,与术前相比差异有统计学意义(P＜0.001).29例病例随访3～33个月,所有患儿生长发育良好,房间隔水平无分流,肺动脉瓣跨瓣压差平均为(17.4±6.9)mmHg,均在40mmHg以内,封堵器位置良好,其中微小量反流7例,少中量反流1例,余21例患儿未见肺动脉瓣反流.结论 食管超声引导下经剑突微创小切口镶嵌治疗婴幼儿心脏复合畸形近期安全有效,可达到传统体外循环手术的治疗效果,避免体外循环带来的创伤,但对其远期疗效的评估仍需进行长期随访.     

心电图在重度肺动脉瓣狭窄诊治中的价值

目的 探讨心电图在重度肺动脉瓣狭窄（PS）诊治中的应用价值。方法 结合心脏超声和心导管检查结果，对34例单纯性重度PS患儿（男21例，女13例；平均年龄3．5岁）经皮球囊肺动脉瓣成形术（PBPV）前后心电图进行分析；并与34例轻中度患儿心电图进行比较。结果 单纯重度PS的心电图电轴极度右偏、P波及VIR波振幅增高，与轻中度PS比较存在显著差异（P〈0．05）；V1T波振幅与重度PS的右心室压力、跨肺动脉瓣压力阶差呈直线正相关（r=0．81，0．82 Pα〈0．05）；PBPV不同疗效组间心电图比较无显著性差异（P〉0，05）；PBPV术后近期心电图随访显示，其在心电轴、V1R波的电压、V1T波异常方面均有显著好转（Pα〈0．05）。结论 心电图对PS严重程度具有一定的诊断价值；根据V1R波振幅可估测右心室压力和跨肺动脉瓣的压力阶差；心电图无法预测PBPV术的疗效，但对PBPV术后的随访有重要辅助作用。     

产前诊断对室间隔完整的肺动脉瓣闭锁和危重型肺动脉瓣狭窄新生儿治疗及预后影响

目的探讨产前诊断对室间隔完整的肺动脉瓣闭锁(PA/IVS)和危重型肺动脉瓣狭窄(CPS/IVS)新生儿早期治疗及近中期预后的影响。方法按病例对照研究方法,将28例有经皮球囊肺动脉瓣成形术(PBPV)手术指征的PA/IVS或CPS/IVS患儿根据有无产前诊断分为产前组(n=15)及产后组(n=13)。产前组于胎儿期明确诊断后即制定干预方案,产后组自外院转诊并明确诊断后制定干预方案。所有患儿均于血流动力学稳定后,在新生儿期接受PBPV,术后1个月、3个月、6个月、1年及2年时随访,比较两组患儿的临床情况、心脏彩色超声及介入术中的测量值。结果 28例患儿接受PBPV平均日龄为(7.53±3.18)d,平均体质量为(3 102.32±708.40)g,其中PA/IVS 9例,CPS/IVS 19例,随访时间(18.82±5.22)月,无死亡病例。产前组入院日龄及首次治疗日龄明显小于产后组,差异有统计学意义(P??0.05)。入院时产前组血流动力学较产后组稳定,产后组并发症发生率较高。随访过程中,两组再介入率、双心室循环情况及术后1年右室和肺动脉瓣的发育差异均无统计学意义(P??0.05)。结论产前诊断有助于对PA/IVS及CPS/IVS患儿的早期干预,并可减少术后并发症的发生。     

经皮球囊肺动脉瓣成形术治疗效果和远期随访

为评价经皮球囊肺动脉瓣成形术治疗单纯性肺动脉瓣狭窄患儿的近期和远期效果及影响疗效的因素 ,对经皮球囊肺动脉瓣成形术治疗的167例单纯性肺动脉瓣狭窄患儿进行随访 ,随访时间为术后 (0.6±0.3)年～(8.6±1.5)年 ,以超声心动图测得的右心室与肺动脉之间的跨瓣压差为随访指标。结果显示 ,167例中163例获得成功(成功率为97.6%) ,获成功者术前跨瓣压差为 (87.6±37.4)mmHg,术后立即降为 (35.9±18.5)mmHg,术后2年内跨瓣压差进一步下降 ,5年和8年随访结果表明其远期效果稳定。提示经皮球囊肺动脉瓣成形术安全可靠 ,术后即刻和远期效果好 ,未发生严重并发症     

Pulmonary hypertensive diseases

The commonest causes of pulmonary hypertension are secondary to endstage pulmonary disease or congenital heart disease (including structural abnormalities of the pulmonary veins). Less obvious causes include sleep disordered breathing due to obstructive sleep apnoea or neuromuscular disease, and occult interstitial lung disease. When these have been excluded, the primary pulmonary vascular diseases should be considered. These are primary pulmonary hypertension; pulmonary veno-occlusive disease; pulmonary embolic disease (thromboembolism, and non-thrombotic embolism) and invasive pulmonary capillary haemangiomatosis. The clinical signs and chest X-ray appearances are often non-specific. Echocardiography can often estimate pulmonary artery pressure and exclude congenital heart disease. Right heart catheterization is usually needed to confirm the diagnosis, estimate any reversibility of elevated pulmonary vascular resistance and exclude other causes. Precise diagnosis may require an open lung biopsy. For many of these conditions, treatment is difficult and the prognosis poor unless the child has a lung transplant.     

Total Anomalous Pulmonary Venous Return with the Circular Pulmonary Venous Connection: Outcome of Common Pulmonary Venous Agenesis

A rare case of total anomalous pulmonary venous return, in which the right and left peripheral pulmonary veins connected circularly and there was no central part of the pulmonary vein or the common pulmonary vein, is presented. To our knowledge, total anomalous pulmonary venous return with circular pulmonary venous connection has not been reported previously in the literature. It is thought that the complex connection between peripheral pulmonary veins with the absence of the central part of the pulmonary vein as well as the common pulmonary vein results from common pulmonary venous agenesis.     

Pulmonary actinomyces in a child

Abstract Pulmonary actinomycosis is rare in Australian children. A patient with the condition is described in whom a diagnosis was made by open lung biopsy. She was treated successfully with penicillin and tetracycline.     

辛伐他汀干预大鼠高肺血流所致肺动脉高压的机制

目的了解辛伐他汀干预高肺血流所致肺动脉高压（PH）的作用机制。方法对大鼠行腹主动脉-下腔静脉分流术建立高肺血流所致PH动物模型,予辛伐他汀片2mg/（kg.d）干预11周后行肺动脉压力测定、检测肺血管平滑肌细胞凋亡率和肺血管平滑肌细胞增殖率并与无分流组和分流组比较。结果辛伐他汀能较好的降低肺动脉压力,辛伐他汀组大鼠肺动脉压力明显低于分流组大鼠;辛伐他汀能很好的诱导肺血管平滑肌细胞凋亡和抑制肺血管平滑肌细胞增殖;辛伐他汀组大鼠肺血管平滑肌细胞凋亡率明显高于分流组和无分流组（Pa〈0.01）;而辛伐他汀组大鼠的肺血管平滑肌细胞增殖率明显低于分流组和无分流组（Pa〈0.01）。结论辛伐他汀通过诱导肺血管平滑肌细胞凋亡和抑制肺血管平滑肌细胞增殖对高肺血流导致的PH起较好的干预作用。     

Foreign-Body Pulmonary Embolism

The minimal incidence of pulmonary foreign-body embolism in a general pediatric pathology experience was ascertained by reviewing autopsy records, and the true incidence of pulmonary foreign-body embolism was determined by studying lung sections from 64 autopsies of patients who had undergone cardiac procedures. Seventeen cases of embolism were reported from 370 autopsies, an incidence of 4.6%. The true incidence of pulmonary foreign-body embolism was found to be 21.9% in the cardiac surgical autopsies and the minimal incidence was 5.1% in patients who had other types of surgery. No patients other than those having surgical procedures had embolism. Hair was found to be the embolic material in 35% of cases. Embolic lesions were characterized, staged, and correlated with clinical data. The pathogenesis of this condition is unclear, but it probably involves contamination of surgical materials with particulate matter.     

Morphometric Study on Pulmonary Arterial Thickness in Pulmonary Hypoplasia

This study addresses the question of whether pulmonary hypoplasia is associated with structural changes of the pulmonary arteries. Quantitative (medial thickness, external radius) and qualitative (airway level) parameters of pulmonary arteries were assessed in eight hypoplastic lungs and eight lungs from age-matched fetuses without underlying primary pulmonary disease. The gestational ages ranged from 16 to 40 weeks. Relative medial thickness of the pulmonary arteries was related to the external radius and to the type of accompanying airway. Medial thickness was significantly higher in pulmonary hypoplasia than in normal fetal lungs. In contrast to normal fetal lungs, muscular arteries extended to the intra-acinar level in pulmonary hypoplasia. These arteries had medial thickness values up to a maximum of 80%.     

Isolated Left Pulmonary Artery in Absent Pulmonary Valve Syndrome

A very rare combination including tetralogy of Fallot (TOF), absent pulmonary valve, and isolated left pulmonary artery is described. A 4-month-old girl presented with mild cyanosis and severe dyspnea. Echocardiography established the diagnosis of TOF and absent pulmonary valve with severe pulmonary regurgitation and moderate stenosis at the pulmonary valve site. A right ventricle angiogram showed the severe aneurismal dilation of the proximal right pulmonary artery and the remarkable pulmonary annular stenosis but failed to show the left pulmonary artery (LPA). An angiography after pulmonary vein wedge injection demonstrated the isolated and hypoplastic LPA.     

辛伐他汀对大鼠实验性高原性肺动脉高压肺血管重构的影响

目的研究辛伐他汀对高原性肺动脉高压（PH）大鼠肺血管重构的影响。方法健康雄性SD大鼠40只。体质量180～230g。随机分为4组：对照组（N）;高原环境低压低氧模型组（H＋P）;辛伐他汀低剂量干预组（H＋L）：2mg/（kg.d）;辛伐他汀高剂量干预组（H＋H）：20mg/（kg.d）;除N组外,其他组置于减压舱,模拟海拔5000m高原,23h/d,持续21d;N组在吊压环境中正常饲养3周。分别测定各组肺动脉压、右心室肥大指数;光镜观察肺小动脉管壁厚度百分比、肺非肌性小动脉肌化程度、肺小血管管壁细胞增殖度及新生内膜产生。结果1.除新生内膜指标外,H＋P组各项指标均高于余3组（Pa〈0.01）;2.H＋L与H＋H组各指标比较无统计学差异（Pa〉0.05）;3.各组均无新生内膜产生。结论辛伐他汀对低压缺氧所致高原性PH及肺血管重构有防治作用,低压缺氧不能造成新生内膜增生。     

Pulmonary Veins in Total Anomalous Pulmonary Venous Connection with Obstruction: Demonstration Using Silicone Rubber Casts

We studied the postmortem vascular anatomy of four patients with total anomalous pulmonary venous connection (TAPVC) with obstruction using a silicone rubber casting method. Two patients were studied clinically but died before therapeutic intervention, one died after corrective surgery, and another was not diagnosed before death. The morphologic types were supracardiac (2), infracardiac (1), and mixed (1). One of the supracardiac forms had a stenosis at the left vertical vein by a bronchoarterial vise, and a collateral channel to the right superior caval vein was also present. In the other supracardiac case, a left upper pulmonary vein was connected to the left vertical vein above the stenotic portion, resulting in postoperative pulmonary hemorrhage into the left upper lobe. The patient with infracardiac TAPVC had a tree-shaped descending vertical vein and small opening at the inferior caval vein. In the mixed type, a right upper pulmonary vein ran between the pulmonary artery and bronchus draining into the superior caval vein, and the rest of the drainage was to the portal vein.     

外源性二氧化硫对低氧性肺动脉高压大鼠肺动脉压力的影响

目的探讨外源性二氧化硫(SO2)对低氧性肺动脉高压大鼠肺动脉压力的影响。方法将大鼠分为对照组、低氧组和低氧加SO2组(予亚硫酸钠/亚硫酸氢钠)。对低氧和低氧加SO2组大鼠进行低氧处理21d。低氧处理后检测各组大鼠肺动脉平均压、收缩压及舒张压,分析外源性SO2对肺动脉压力的影响。结果与对照组比较,低氧组大鼠肺动脉平均压升高127.13%;与低氧组比较,低氧加SO2组大鼠肺动脉平均压降低24.0%。与对照组比较,低氧组大鼠肺动脉收缩压升高84.93%;与低氧组比较,低氧加SO2组大鼠的肺动脉收缩压降低23.10%。与对照组比较,低氧组大鼠肺动脉舒张压升高154.59%;与低氧组比较,低氧加SO2组大鼠的肺动脉舒张压下降不明显(P=0.065)。结论外源性SO2能显著降低低氧大鼠肺动脉平均压和肺动脉收缩压,而对肺动脉舒张压的影响不显著。