肺动脉闭锁合并动脉导管未闭的形态学分析

目的:分析肺动脉闭锁(PA)患者中动脉导管未闭(PDA)的起源、形态及肺动脉发育情况。方法:回顾41例PA合并PDA患者的影像学资料结合胚胎学理论,分析PDA及肺动脉的形态学特点。结果:动脉导管连接肺动脉融合部35例,其中起源于主动脉弓降部32例,起源于锁骨下动无脉或无名动脉3例;双侧PDA例,导管分别起源于主动脉弓降部及无名动脉或锁骨下动脉根部,连接同侧肺门部肺动脉,无肺动脉融合部;动脉导管连接同侧肺门部肺动脉,另一侧肺由体肺侧枝供应肺血2例。结论:PA合并的PDA起源、形态及供血范围可出现较多变异;连接肺动脉融合部时动脉导管多合并不同程度的狭窄,连接一侧肺动脉时动脉导管多无明显狭窄甚至出现不同程度的脉动脉高压。     

介入治疗心脏复合畸形疗效及安全性研究

目的评估介入治疗心脏复合畸形的可行性,安全性及疗效。方法心脏复合畸形患者10（男6,女4）例,年龄5～53岁。其中房间隔缺损（ASD）并发肺动脉瓣狭窄（PS）2例、室间隔缺损（VSD）2例和动脉导管未闭（PDA）1例,PDA并发主动脉瓣关闭不全和PS各1例;VSD修补术后遗留ASD2例,ASD修补术后遗留PDA1例和刀刺伤致左室前侧术后遗留VSD1例。ASD或PDA并发PS者,先扩张肺动脉瓣,再行ASD或PDA封堵治疗。ASD并发VSD者,先封堵VSD,再封堵ASD。结果10例患者介入手术均1次成功。5例ASD封堵器的直径为8～38mm,2例膜部对称性VSD封堵器的直径为14mm和6mm,1例用10mm肌部VSD封堵器。2例PDA均用弹簧圈封堵。封堵PDA或VSD后造影无残余分流。封堵ASD后超声心动图示无残余分流。3例并发PS者,肺动脉瓣扩张术后即刻肺动脉跨瓣压差明显下降,由术前50、38、40mmHg分别降低为20、15和21mmHg。有1例PDA封堵弹簧圈脱落至肺动脉远端未能取出。随访1年肺功能正常,无肺不张。全部患者随访3个月～1年,无任何并发症发生。结论经导管介入治疗心脏复合畸形安全可行,近期疗效肯定。     

动脉导管未闭介入治疗的护理

动脉导管未闭(PDA)是指主动脉和肺动脉之间的一种先天性的异常通道,多位于主动脉狭部和左肺动脉根部之间,发病女性多于男性[1]。Amplater伞介入疗法是安全性高、疗效好、并发症少的心脏介入治疗方式。我科自2002年8月～2005年7月对6例动脉导管未闭患者开展Amplater伞的介入治疗     

室间隔封堵器在成人细小动脉导管未闭介入治疗中的疗效和安全性

目的:探讨细小型成年动脉导管未闭(PDA)患者的造影、介入治疗特点,观察应用室间隔缺损(VSD)封堵器关闭此类动脉导管未闭的疗效、安全性和意义。方法:选择成年PDA患者25例,造影观察PDA的形态、大小,将其分为长管型10例(A组),迂曲不规则型9例(B组),动脉瘤型6例(C组)。UCG显示直径1.2~2.8mm,造影显示最小直径1.2mm,最大直径4.3mm。PDA长度7.9~11.9mm,平均(9.9±1.3)mm。结果:选择对称型室间隔封堵器和肌部室间隔封堵器进行介入封堵治疗,治疗前后肺动脉压力无明显差异,但造影与TTE测量最窄处直径差异有统计学意义(P0.05)。总体成功率100%。随访无明显降主动脉缩窄、左肺动脉狭窄及其他并发症。结论:室间隔封堵器关闭细小型PDA疗效肯定、安全。     

经导管介入治疗婴幼儿动脉导管未闭临床疗效分析

目的:探讨经导管介入治疗婴幼儿动脉导管未闭(PDA)的治疗意义、临床经验、操作技术及安全性。方法:回顾性分析经导管介入治疗婴幼儿PDA 348例临床资料。男性226例,女性122例。年龄:4个月～3岁,平均1岁7个月,体质量:4.5～18 kg,平均10.2 kg。常规右心导管检查,主动脉弓降部造影,置入封堵器。术后24 h,1个月、3个月、6个月及12个月行超声心动图(UCG)检查。结果:PDA最窄处内径2.5～8.2 mm(平均3.97 mm),QP/QS 1.1～17.6(平均3.54)。肺动脉高压252例(72.4%),术后肺动脉压力均有显著下降。封堵后29例(8.3%)存在微量至少量残余分流,均于封堵术后6个月内完全消失。345例经导管介入治疗成功,技术成功率99.14%。3例封堵器移位,转外科手术治疗。结论:经导管介入治疗婴幼儿动脉导管未闭,具有安全、有效、操作简便及适应证广等优点,可作为治疗大多数婴幼儿动脉导管未闭的首选方法。     

经导管介入治疗婴幼儿大型动脉导管未闭临床疗效

目的 探讨应用导管介入治疗直径≥5 mm婴幼儿大型动脉导管未闭(patent ductus arteriosus,PDA)的临床疗效.方法 回顾性分析60例大型PDA(直径≥5 mm)患儿的临床资料,着重分析手术方法、临床疗效和随访结果.结果 56例PDA采用Amplatzer或国产先健动脉导管封堵器,3例采用膜部室间隔缺损封堵器,1例采用肌部室间隔缺损封堵器.封堵成功率为98.3%(59/60),术后1 d超声心动图显示15%(9/60)微量至少量残余分流,术后3月超声心动图复查未见残余分流;3例采用室间隔缺损封堵器患儿术后12月超声心动图示左肺动脉血流速度增快.结论 应用导管介入治疗直径≥5 mm婴幼儿大型PDA是安全、有效的方法.     

经导管法治疗动脉导管未闭941例临床分析

目的探讨介入治疗动脉导管未闭(PDA)的临床应用体会。方法1998年3月至2007年8月在我科介入治疗PDA 941例,其中男259例,女682例;年龄3个月~74岁(平均13.7岁);体重3.5~101 kg(平均31.2 kg)。结果主动脉造影按Krichenko分类,A型641例(68.1%),B型11例(1.1%),C型202例(21.5%),D型8例(0.9%),E型79例(8.4%)。PDA最窄内径1.2~15(4.6±2.9)mm,主动脉端内径2.0~32.7(11.1±5.7)mm,长度2~42.6(6.7±3.3)mm;平均肺动脉压9.7~106(34.7±19.4)mm Hg。采用进口AGA蘑菇伞146例,AGA房间隔缺损封堵伞2例,国产PDA蘑菇伞720例,国产膜部室间隔缺损封堵伞18例,国产肌部室间隔封堵伞6例,国产偏心PDA封堵伞18例和德国pfm弹簧圈31例。7例未能完全封堵,治愈率达99.3%。结论绝大多数患者可用介入方法达到治愈,正确掌握PDA介入治疗的适应证范围,准确娴熟的操作技术能减少并发症的发生,慎重处理婴幼儿合并重度肺动脉高压的PDA患者,正确评价肺动脉高压的性质是介入治疗指征的关键。伴有重度肺动脉高压的PDA患者远期疗效尚需积累经验。     

6kg以下小婴儿主动脉缩窄合并室间隔缺损的外科矫治

目的 :探讨新生儿、小婴儿主动脉缩窄 (CoA)合并室间隔缺损 (VSD)的外科治疗方法。方法 :1998年 12月至 2 0 0 3年 7月共收治 9例 6kg以下CoA合并VSD患儿 ,其中新生儿 3例。一期手术 3例 ,分期手术 3例 ,仅做主动脉缩窄成形术 3例 ,其中 1例加做肺动脉环缩术。 6例采用人造血管补片主动脉成形术 ,3例采用左锁骨下动脉翻转主动脉成形术。结果 :全组患儿无死亡 ,随访 8～ 36个月 ,心功能改善明显 ,超声心动图检查提示无主动脉瘤样扩张或主动脉再狭窄。结论 :大多数患儿均可采用体外循环下一期根治CoA和VSD ,但是对于VSD为限制性及不能耐受体外循环的患儿可采用二期手术。一期手术选择左外侧和正中双切口方法安全有效。     

改良的Blalock-Taussig分流术

尽管婴幼儿先天性心脏病根治手术的进展很快,但体-肺分流术仍具有重要地位。自从 Blalook-Taussig(锁骨下动脉-肺动脉)手术后,又有 Potts(降主动脉-左肺动脉)手术、Waterston(升主动脉-右肺动脉)手术、以及用人造血管作主-肺动脉分流     

室间隔缺损并发心脏畸形及室间隔缺损术后残余分流的介入治疗

目的 研究儿童室间隔缺损（VSD）并发其他心脏畸形及先心术后VSD残余分流介入治疗的安全性和有效性。 方法 回顾性分析上海交通大学医学院附属上海儿童医学中心2016年1月～2020年12月期间收治的VSD并发其他先天性心脏病（CHD）同时介入治疗及复杂型CHD术后VSD残余分流介入治疗患者的临床资料。 结果 62例VSD并发其他CHD患儿中,房间隔缺损（ASD）37例、动脉导管未闭（PDA）25例。其中男21例,女41例,年龄36 (23,180) 月,体质量15 (11,48) (kg);11例复杂型CHD术后出现VSD残余分流,男6例,女5例,最短外科术后时间为14 d,最长术后132月,中位外科术后时间56月,体质量18.2（11,50）kg。所有病例均完成VSD介入封堵,其中1例心尖部肌部多发VSD通过动脉端逆行释放封堵器,1例肌部VSD经过颈内静脉途径完成封堵。2例术后存在少许残余分流,无术后房室传导阻滞,无显著加重的房室瓣或主动脉瓣反流。 结论 儿童VSD并发其他心脏畸形的介入治疗安全有效,手术方式及操作程序需要根据不同的患者制定个体化方案。     

Membranous subaortic stenosis and patent ductus arteriosus.

Six children (five of them girls) each initially had a large patent ductus arteriosus with auscultatory, radiologic, and electrocardiographic findings typical of that lesion. After surgery for the lesion, an ejection-type basal systolic murmur led to detection of discrete membranous subaortic stenosis, which became worse in late childhood. The murmur became louder, the systolic pressure gradient increased from slight to significant, and hypertrophic subaortic stenosis or aortic insufficiency (or both) developed in the older children. Surgical excision of the membrane afforded improvement, except in one patient with the most severe involvement. Because of the unexpected finding of discrete membranous subaortic stenosis in these infants and young children who had undergone surgery for a large patent ductus arteriosus and because of the treacherous worsening of the effects of the discrete membranous subaortic stenosis as childhood progressed, it is important that those patients with a persistent systolic murmur after ductal ligation not be discharged from cardiac follow-up as cured. Serial cardiac catheterization during the growing years appears to be the most accurate way of detecting worsening discrete membranous subaortic stenosis, so that the membrane can be excised before severe complications occur.     

Post-infarction peripheral cysts of the lung in pediatric patients: a possible cause of idiopathic spontaneous pneumothorax

Occlusion of the main pulmonary artery to the right lung in a newborn infant produced peripheral (subpleural) infarction of the lung and cyst formation. Two older infants were found at autopsy to have subpleural cysts of the upper lobes. One had had surgery for repair of an atrial septal defect and a patent ductus arteriosus eight months prior to death, while the second had died suddenly of carbon monoxide intoxication. The authors suggest that pulmonary arterial occlusion may have been responsible for cyst formation in all three cases and may produce cysts in other infants that lead to idiopathic spontaneous pneumothorax in older children and young adults. The presence of bronchopulmonary arteries in fetuses and newborn infants may provide the mechanism for the production of subpleural cysts following pulmonary arterial occlusion.     

Usefulness of magnetic resonance angiography in the evaluation of complex congenital heart disease in newborns and infants

This study evaluated the quality of the visualization of extracardiac thoracic vessels by magnetic resonance angiography (MRA) in young infants with congenital heart disease. Echocardiography is often sufficient in evaluating CHD in young infants. Cardiac catheterization is needed in some instances to evaluate extracardiac thoracic vessels. Extracardiac thoracic vessels can be accurately evaluated using MRA in adults and older children, but image quality in small infants may be limited. Twenty-nine magnetic resonance angiographic scans were performed at a single institution on 28 infants aged <3 months (median 6 days, range 1 to 90 days) with complex CHD in whom imaging was inconclusive by echocardiography. A blinded observer at a different institution graded (from 0 to 3) the quality of the visualization of the main, branch, lobar, and second-generation pulmonary arteries; lobar pulmonary veins; aortopulmonary collaterals; vena cavae; thoracic aorta and its branches; patent ductus arteriosus; and visceral sidedness. The results of MRA were compared with those of x-ray angiography and surgical inspection, when available. The mean image quality grade was >2 for all structures except the second-generation pulmonary arterial branches, for which it was 2. The median total scan duration was 9 minutes (range 3 to 46). Findings were concordant with surgical inspection (n = 25) and cardiac catheterization (n = 8) in all subjects. There were no complications. In conclusion, MRA is excellent for the visualization of extracardiac thoracic vessels in young infants with CHD and can be used as an alternative to cardiac catheterization when echocardiography is inconclusive.     

The efficacy and safety of the Amplatzer ductal occluder in young children and infants

Background: We have used the Amplatzer ductal occluder for transcatheter closure of large persistently patent arterial ducts, and used our experience to assess the safety and efficacy of the device in young children and infants. Methods and patients: We used the Amplatzer ductal occluder prospectively in 43 patients with large patent arterial ducts, reviewing our experience to identify any problems or complications. Results: The procedure proved successful in 42 of the patients. We achieved complete occlusion of the duct in 33 (78.5 per cent) of the patients on the day of insertion. In 6 additional patients, complete occlusion occurred 1 week to 6 months after the procedure. Trivial leaks persisted in 2 patients, while one had a significant residual leak. Problems were encountered in 7 patients. The procedure failed in one, a device was wasted in 2, pulled through in 3, while we experienced kinking of the long Mullins sheath, being unable to retrieve the device, in one patient. Minor complications occurred in 6 patients, finding flow at a peak velocity of 2.2 metres per second in the descending aorta in 2 patients, and at 2.5 metres per second in 2 further patients, and flow at 2.5 metres per second in the pulmonary arteries of two patients. One patient experienced a major complication due to excessive bleeding. Out of the 14 patients suffering adverse events, 13 weighed less than 10 kilograms. This rate of problems and complication in these patients weighing less than 10 kilograms was significantly higher than in the patients weighing more than 10 kilograms. Conclusion: Transcatheter occlusion of moderate to large patent arterial ducts with the Amplatzer ductal occluder device is safe and effective, with a high rate of complete occlusion. Problems and minor complications may be encountered in children weighing less than 10 kilograms. If the device is to be deployed completely in the ductal ampulla, and to avoid descending aortic obstruction, the size of the retention flanges of the occluder should not exceed the largest diameter of the patent arterial duct.     

潮气呼吸肺功能测定在婴幼儿喘息性疾病中的应用及意义

儿童喘息疾病是一种综合性呼吸疾病,患儿通常患有变应性鼻炎、变应性皮炎等一些过敏性症状,同时还可能有家族遗传史的倾向,具有气道高反应性.潮气呼吸方法测定肺功能在儿科临床主要应用于婴幼儿,目前已成为临床测定婴幼儿肺功能的首选方法.本文对潮气呼吸肺功能各参数的临床意义及潮气呼吸肺功能在婴幼儿喘息性疾病中的应用作一综述,为其在婴幼儿喘息性疾病中的临床应用提供参考.     

Assessment of the ductus arteriosus in preterm infants utilizing suprasternal two-dimensional/Doppler echocardiography

Evaluation for patent ductus arteriosus by both Doppler examination and direct two-dimensional echocardiographic visualization has been reported in infants and children. However, visualization of a patent ductus arteriosus in preterm infants with lung disease has been difficult. Using a recently developed 7.5 MHz mechanical scanner with interfaced two-dimensional directed pulsed Doppler ultrasonography, 36 examinations were performed from a suprasternal approach in 27 patients (age range 1 day to 3 months, mean 18 days; weight range 490 to 2,500 g, mean 1,260). Complete imaging for evaluation of patency of the ductus arteriosus was successful in 33 (92%) of 36 examinations, and imaging of the pulmonary end of the ductus arteriosus was successful in all. In 18 examinations, the ductus arteriosus was closed by both two-dimensional echocardiography and Doppler examination. In four cases the ductus arteriosus was widely patent by both two-dimensional echocardiography and Doppler examination. Eleven echocardiographic examinations revealed a narrowed ductus arteriosus, and of these, 10 (91%) showed Doppler findings of patent ductus arteriosus. It is concluded that combined two-dimensional/Doppler echocardiographic assessment allows confident detection of both a large unrestrictive and a small, stenotic patent ductus arteriosus in preterm infants with lung disease.     

Parathyroid hormone responses to marked hypocalcemia in infants and young children undergoing repair of congenital heart disease.

OBJECTIVES. The integrity of the parathyroid axis was tested in 18 infants and young children undergoing repair of congenital heart disease with cardiopulmonary bypass. BACKGROUND. Infants are believed to have an immature parathyroid hormone response to hypocalcemia. Whereas adults are known to respond appropriately to hypocalcemia during cardiopulmonary bypass, children have not been studied carefully. METHODS. Calcium, magnesium, parathyroid hormone, phosphate and total protein were measured in blood samples withdrawn at defined times before, during and after cardiopulmonary bypass. RESULTS. At the initiation of cardiopulmonary bypass, ionized calcium decreased markedly in 12 infants less than or equal to 24 months old (mean +/- SEM 1.11 +/- 0.04 to 0.29 +/- 0.05 mM) and decreased significantly in 6 young children greater than 24 months old (1.19 +/- 0.02 to 0.42 +/- 0.12 mM). In response to hypocalcemia, parathyroid hormone concentration increased significantly in both the infants (from 42 +/- 8 to 103 +/- 29 and 85 +/- 22 pg/ml) and the young children (from 39 +/- 8 to 44 +/- 20 and 92 +/- 30 pg/ml). Before separation from cardiopulmonary bypass, increased parathyroid hormone concentration restored ionized calcium concentration to 0.75 +/- 0.03 mM in the infants and to 0.92 +/- 0.07 mM in the young children. There was no significant influence of either age or the use of deep hypothermia and circulatory arrest on either calcium or parathyroid hormone responses. Total magnesium and total protein concentrations decreased on initiation of cardiopulmonary bypass and thereafter remained stable. Phosphate concentrations were unchanged during the study. CONCLUSIONS. In infants and young children undergoing cardiac surgery, the parathyroid hormone response to both hypocalcemia and to rising ionized calcium concentrations was at least as great as that of adults. Thus, the calcium-parathyroid-vitamin D axis functions in infants and young children as it does in adults.     

经导管介入治疗婴幼儿大型动脉导管未闭临床疗效

目的探讨应用导管介入治疗直径≥5mm婴幼儿大型动脉导管未闭（patent ductus arteriosus．PDA）的临床疗效。方法回顾性分析60例大型PDA（直径≥5mm）患儿的临床资料,着重分析手术方法、临床疗效和随访结果。结果56例PDA采用Amplatzer或国产先健动脉导管封堵器．3例采用膜部室间隔缺损封堵器,1例采用肌部室间隔缺损封堵器。封堵成功率为98．3％（59／60）,术后1d超声心动图显示15％（9／60）微量至少量残余分流,术后3月超声心动图复查未见残余分流：3例采用室间隔缺损封堵器患儿术后12月超声心动图示左肺动脉血流速度增快。结论应用导管介入治疗直径≥5mm婴幼儿大型PDA是安全、有效的方法。     

Associations between age,respiratory comorbidities,and dysphagia in infants with down syndrome

Objectives: Children with Down syndrome (DS) have a high risk of dysphagia and the pediatric pulmonologist may be involved in diagnosis and management. The objective of this study is to evaluate the associations between age, dysphagia, and medical comorbidities in young children with DS. We hypothesized that swallow study findings are more likely to change in younger infants and that medical comorbidities may be associated with dysphagia. Study design: Results of videofluoroscopic swallow studies (VFSS) and fiberoptic endoscopic evaluation of swallowing (FEES) from 2010 to 2016 were collected retrospectively in children with DS with initial swallow study at less than 12 months of age. Results were analyzed for findings and change based on age at initial study, reason for referral, and medical comorbidities. Results: One hundred eleven infants with 247 VFSS and 14 FEES were included. Deep laryngeal penetration and/or aspiration were found in 31.9% of infants less than 6 months and 51.3% of infants 6 to 12 months. Children with initial swallow study performed at greater than or equal to 6 months of age were more likely (80.0%) to have unchanged findings on follow‐up study compared to children imaged at less than 6 months (35.3%). Laryngomalacia, pulmonary hypertension, pneumonia, and congenital cardiac disease were associated with dysphagia. Conclusion: We confirmed that dysphagia is common in infants with DS and comorbidities and provided preliminary evidence that swallow study findings may be more likely to change in children tested under 6 months of age. Providers should consider that results for instrumental swallow studies may change, particularly if the test was completed on a young infant.     

Medium-term follow-up and modes of failure following epicardial pacemaker implantation in young children.

AIMS: Young children suffering from congenital or post-operative AV-block require life-long pacemaker stimulation. Due to the anatomical prerequisites initially epicardial electrodes are implanted and the generator is placed in the upper abdominal wall. The following study investigated modes of failure leading to reoperation in this group of technically challenging patients. METHODS AND RESULTS: Between October 2000 and May 2005, a total of 21 infants (age 3 days to 5 years) underwent pacemaker implantation using a subxyphoidal incision for newborns (and a partial lower or complete median sternotomy for older children). Nine patients had previous cardiac surgery for complex congenital defects. The remaining 12 young children suffered from congenital AV-Block (CAVB). Twenty-one bipolar epicardial electrodes (Medtronic Capsure epi) were fixed to the right ventricle, 15 had additional implantation of a bipolar atrial lead. The pacemaker generator (Medtronic Kappa 701) was implanted into the right upper abdominal wall. Indications for revision were recorded. No mortality was observed; pacing and sensing parameters remained stable up to a 5-year follow-up. A total of four reoperations occurred. Three of the four revisions were caused by ventricular electrode fracture. At revision, two electrodes were broken at the crossing between the pericardial cavity and the abdominal wall, one bipolar lead at the Y-division into the two tip electrodes. One reoperation was due to a pacemaker recall. All revisions were performed without complications. CONCLUSION: In our institute epicardial pacing in young children was associated with a satisfactory clinical outcome, but also a significant number of failures leading to reoperation, mainly due to electrode fracture caused by the muscular activity of this patient group. Reoperations were performed at a low risk.