婴幼儿室间隔缺损合并肺动脉高压的麻醉处理

目的探讨婴幼儿先天性心脏病室间隔缺损合并肺动脉高压行室缺修补术的麻醉处理要点。方法室间隔缺损伴肺动脉高压的婴幼儿86例,均在静吸复合全麻、低温体外循环下行室缺修补术。全组持续监测有创血压(ABP)、CVP、ECG、SpO2、体温、尿量、电解质、血气,术中加测右房压(RVP)、平均肺动脉压(PAP)。结果本组体外循环时间27min～114min,主动脉阻断时间15min～87min,74例自动复跳;12例电击除颤后复跳;除1例外,肺动脉压力均较术前下降;术后并发低心排综合征12例;术后出现心律失常2例;无1例出现肺动脉高压危象。结论麻醉前做好充分准备,提高患儿耐受能力;麻醉诱导平顺,配合术者尽快建立体外循环;麻醉深度适宜,维持血流动力学平稳,是保证肺动脉压不进一步增高的重要因素;合理应用血管活性药物,防治肺动脉高压危象鸦积极防范及处理术后并发症,可使患儿安全度过手术期。     

婴幼儿室间隔缺损合并肺动脉高压的外科治疗

目的 报道婴幼儿先天性室间隔缺损(VSD)伴肺动脉高压的手术修补结果及体会。方法 1998年1月-2002年12月,对86例VSD伴肺高压的婴幼儿采用中深低温体外循环行手术治疗。术中术后加强心肌保护和呼吸道管理。结果 治愈出院83例,死亡3例,死亡率为3．49％(3／86),均为肺高压临床4级病例。术后主要并发症为肺部感染、肺高压危象、低心排综合征。出院病人随访6月～5年,无死亡病例,心功能(NYHA)一级62例,二级18例,三级3例。结论 婴幼儿VSD合并肺动脉高压者,应积极早期手术。围术期的心肌保护和呼吸道管理是手术成败的关键。     

婴幼儿室间隔缺损合并肺动脉高压的外科治疗

目的 总结婴幼儿大型室间隔缺损（Ventricular Septal Defects,VSD）合并肺动脉高压的外科治疗经验.方法 2008年10月至2009年5月共收治婴幼儿大型VSD 98例,男56例,女42例,年龄4～24个月,体重5.1～15.3 kg;均为单发,其中合并房间隔缺损（Atrial Septal Defect,ASD）26例,合并三尖瓣关闭不全（Tricuspid Insufficiency,TI）18例,动脉导管未闭（Patent Ductus Arteriosus,PDA）12例.患儿均在全麻低温体外循环下实施一期矫治术.结果 治愈95例,死亡3例,术后并发症16例,其中低心排出量综合征6例,肺不张4例,肺炎3例,心律紊乱2例,气胸1例,术后早期复查彩超,除5例有少量残余分流外,其余患儿心功能及瓣膜关闭均正常,患儿均痊愈出院.结论 低体重、低年龄的大型室间隔缺损应尽早手术根治;术中尽量减轻体外循环损伤,保护脏器功能;术后加强呼吸道管理、预防低心排出量综合征和肺动脉高压危象是降低死亡率的关键.     

室间隔缺损合并肺动脉高压肺血管床功能状态评估指标的研究

目的探讨室间隔缺损合并肺动脉高压肺血管床功能状态的评估指标。方法对室间隔缺损合并重度肺动脉高压的患儿，于心导管术中应用酚妥拉明，将轻度全肺循环阻力（ＴＰＲ）增加的２７例与重度全肺循环阻力增加的１２例患儿的试验结果进行比较。结果两组患儿的单一肺动脉压降幅（Ｐｐ降幅）分别为２．３ｋＰａ（１７ｍｍＨｇ，１ｋＰａ＝７．５ｍｍＨｇ）和２．２ｋＰａ，Ｐ＞０．０５，差异无显著意义；而肺动脉压降幅（Ｐｐ降幅）／体循环压降幅（Ｐｓ降幅）的比值，试验前后肺动脉血氧饱和度变化值这二项指标的差异有非常显著意义（Ｐ＜０．０１）。Ｐｐ降幅／Ｐｓ降幅比值与全肺循环阻力的相关性良好［ｒ＝－０．８９９），Ｙ（ＴＰＲ）＝２６６８－１８９２Ｘ（Ｐｐ降幅／Ｐｓ降幅）］。结论用酚妥拉明作扩血管降压试验时，单一的肺动脉压降幅不能完全反映肺血管床的功能状态；而Ｐｐ降幅／Ｐｓ降幅比值，试验前后肺动脉血氧饱和度变化值这二项指标对室间隔缺损合并肺动脉高压患儿的肺血管床功能状态的评估，对手术适应证的选择具有一定指导意义。     

肺动脉压监测在幼婴室间隔缺损并中、重度肺高压修补术中的意义探讨

目的 探讨幼婴先天性心脏病间隔缺损（ＶＳＤ）合并肺动脉高压手术前,后肺动脉压力（ＰＡＰ）改变与预后的关系。方法 １９９４年１月－１９９８年１２月我科施行幼婴ＶＳＤ并中,重度肺动脉高压心内直视手术７０例,对比缺损修补前,后ＰＡＰ改变,持续监测其中３５例术后ＰＡＰ变化。结果 术前未作心志管检查者,术中测ＰＡＰ可初步判断其肺高压程度。     

室间隔缺损合并中重度肺动脉高压108例外科治疗

探讨先天性心脏病室间缺损合并中重度肺动脉高压的外科治疗及围术期处理。全组１０８例，年龄４－９６个月，体重４．７－２０ｋｇ。均有明显明显症状。其中３２例为双动脉干下型ＶＳＤ，其中４例伴有主动脉瓣脱垂和中主以上主动脉瓣返流。     

大型室间隔缺损合并肺动脉高压术后远期疗效的随访

先天性心脏病 (简称先心病 )左向右分流合并重度肺动脉高压术后可复性问题一直是临床关注的焦点。手术矫正心内畸形后 ,如肺动脉高压不能恢复 ,甚至加重则失去了外科治疗的意义。有关手术的疗效和手术适应证的选择 ,国内外均有些报道 ,但缺乏肺动脉高压血流动力学长期随访的资料。我们通过对 2 4例大型室间隔缺损合并重度肺动脉高压(全肺阻力增高 )患儿进行术前及术后长期血流动力学的随访 ,旨在了解手术对这些患儿肺动脉高压的远期疗效。对象本组患儿 2 4例 ,男 11例 ,女 13例 ;年龄 6个月～ 14岁 ,平均 (5 8± 4 7)岁。≤ 2岁者 11例 ,>2…     

先天性心脏病肺动脉高压手术前后右心功能变化和治疗

目的 了解先天性心脏病左向右分流导致的重度肺动脉高压对右心功能的影响以及手术矫治后右心功能的恢复状态。方法 对室间隔缺损(室缺)合并重度肺动脉高压(全肺循环阻力增加)的40例患儿,用心导管的方法进行术前、术后5～7年右心功能、肺循环的血流动力学随访测定。结果 术前右心心搏指数、作功指数、心排指数显著高于术后;术前右房压、右室收缩压及舒张压、肺动脉压力和阻力均增高,缺损修补后右室舒张压恢复正常,收缩压的降低与肺动脉压力下降有关;大型室缺左向右分流重度肺动脉高压右心功能不全系继发性右室高排出量心力衰竭,且伴有舒张功能障碍。结论 治疗心力衰竭不宜首选正性心肌收缩药物;降低肺动脉压力、减少左向右分流、根治心内畸形是合理的选择;术后右室收缩压持续不能恢复至正常水平,提示继发性肺血管梗阻性病变存在。     

婴儿大型室间隔缺损并发肺炎和呼吸衰竭的早期手术治疗

目的 探讨婴儿大型室间隔缺损（VSD）并发肺炎和呼吸衰竭早期手术治疗的可行性、手术时机和适应证以及术中和术后处理。方法 1999年10月～2004年1月，我院收治大型VSD并发肺炎和呼吸衰竭29例婴儿，19例进行带机早期手术治疗，10例行内科保守治疗。结果 手术病例中，18例婴儿手术成功、脱离呼吸机出院，术后主要并发症有肺不张、气胸、气管再插管，无全身感染、肺动脉高压危象以及严重低心排病例；1例婴儿死于气管狭窄，病死率5．3％。内科保守治疗病例中，8例在治疗途中因心肺衰竭死亡，病死率80Uo；2例好转撤离呼吸机出院。结论 把握手术时机和适应证，防治术中和术后肺损伤，带机早期手术治疗可提高和改善大型VSD并发肺炎和呼吸衰竭婴儿治疗效果和预后。     

5kg以下婴儿室间隔缺损的手术治疗

１９９４年３月～１９９８年６月,笔者对２３例５ｋｇ以下婴儿室间隔缺损（ＶＳＤ）伴肺动脉高压者施行手术治疗,占同期６２例１周岁以下婴儿ＶＳＤ修补术的３７．１％。临床资料本组２３例,男１３例,女１０例。年龄为３～１１个月（平均为６．７８±０．５３个月）,体重为３．２～５．０ｋｇ（平均为４．３８±０．２１ｋｇ）。术前反复呼吸道感染２３例,反复心衰１７例。１１例因难治性肺炎或心衰,经内科控制下急诊手术。全组均经Ｘ线胸片、心电图、超声心动图、心导管和心血管造影检查确诊。全组均伴有中度以上肺动脉高压,肺动脉…     

室间隔缺损并肺动脉高压的围术期治疗

目的总结小儿室间隔缺损(VSD)并肺动脉高压(PH)的围术期治疗经验。方法回顾性分析1995年4月~2004年10月18例VSD并PH患儿一般临床资料。术前1周静滴极化液、吸氧、人血丙种球蛋白提高患儿抵抗力,10例患儿用前列腺素E1(PGE1)。手术在体外循环下中度低温下矫治心内畸形。术后给予多巴胺、多巴酚丁胺3~10μg/(kg·min),硝酸甘油1~5μg(kg·min)。结果术后出院前腺动脉收缩压/主动脉收缩压比值(PP/PS)0.39±0.09与术前比较明显下降,平均动脉压(MPAP)较术前明显下降(P<0.05)。除1例死于低排综合征。余均治愈出院。结论完善的围术期治疗能使VSD并PH小儿取得良好效果。     

先天性心脏病并肺动脉高压患儿心脏直视手术的麻醉管理

目的探讨婴幼儿先天性心脏病(CHD)并肺动脉高压(PH)心脏直视手术的麻醉管理方法。方法对1999年7月~2002年9月CHD并PH手术婴幼儿87例,加强麻醉手术前准备、合理的选择麻醉药物及给药方法、术中严密监测循环及呼吸、应用血管活性药物,麻醉后密切观察和处理并发症。结果4例因感染和无法脱离体外循环机死亡,余均痊愈出院。结论CHD并PH的婴幼儿心脏直视手术的麻醉管理非常重要。     

A Rare Case of Left Lung Hypoplasia Associated With Congenital Pulmonary Artery Aneurysm and Ventricular Septal Defect

This report describes a 3-year-old boy who presented with hemoptysis and a history of congenital heart disease detected at the age of 3 months. Clinical and laboratory evaluation showed a large true congenital pulmonary artery aneurysm of the left main pulmonary artery occupying the entire left hemithorax and two small aneurysms in the right lung. In addition, a restricted ventricular septal defect was detected. It was evident on clinical and histologic grounds that the multiple aneurysms seen in this child stemmed from congenital weakness of the arterial wall in conjunction with increased pulmonary blood flow and elevated pulmonary artery pressure.     

Pulmonary Interstitial Glycogenosis Associated With Pulmonary Hypertension and Hypertrophic Cardiomyopathy

A neonate with pulmonary interstitial glycogenosis, pulmonary hypertension, and hypertrophic cardiomyopathy is described. The fatal outcome for this patient contrasts with the reported favorable prognosis associated with isolated pulmonary interstitial glycogenosis. To the authors’ knowledge, the association of pulmonary interstitial glycogenosis and hypertrophic cardiomyopathy has not been reported previously. The authors have broadened the phenotype of pulmonary interstitial glycogenosis and demonstrate the diagnostic value of lung biopsy in cases of unexplained neonatal pulmonary hypertension.     

Mechanisms of Exercise-Induced Pulmonary Hypertension in Patients With Cardiac Septal Defects

The objective of this study was to investigate mechanisms of exercise-induced pulmonary hypertension in patients with congenital cardiac septal defects. This was a randomized, placebo controlled, crossover drug trial in a single national pediatric cardiology centre that performs congenital cardiac defect surgery. There were 14 patients with cardiac septal defects and known exercise-induced pulmonary hypertension. The intervention consisted of 50 mg oral sildenafil versus placebo. Measurements included supine bicycle exercise echocardiography and oxygen uptake. The outcome measure was right-ventricular systolic pressure as estimated by Doppler tracings of tricuspid regurgitant jet as well as systolic and diastolic longitudinal myocardial velocities by color tissue Doppler echocardiography. Sildenafil did not change exercise right-ventricular systolic pressure during exercise; however, decreased systemic systolic pressure was seen. Enhanced biventricular longitudinal function with sildenafil compared with placebo was indicated by greater tissue Doppler velocities and displacement measurements during exercise. Finally, a less steep increase of right-ventricular pressure during exercise was associated with greater left-ventricular diastolic myocardial tissue Doppler velocity. Exercise-induced pulmonary hypertension in cardiac septal defects does not seem to have a pulmonary vasoconstrictive component, but it may be related to left-ventricular filling pressure. Furthermore, sildenafil improved biventricular systolic performance in this patient group, possibly related to decreased systemic afterload.     

Elevated Homocysteine and Asymmetric Dimethyl Arginine Levels in Pulmonary Hypertension Associated With Congenital Heart Disease

Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality among patients with congenital heart disease (CHD). This study was designed to determine biomarker levels in patients with PAH associated with CHD (PAH?CCHD) and CHD patients without PAH and to investigate the relationship of these potential biomarkers with hemodynamic findings. In this prospective single-center study, patients with CHD were analyzed according to the presence or absence of PAH and compared with healthy control subjects. Cardiac catheterization and echocardiographs were performed. Plasma homocysteine, asymmetric dimethyl arginine (ADMA), and nitric oxide (NO) levels were determined by enzyme-linked immunosorbent assay. Homocysteine and ADMA levels were higher in the PAH?CCHD group (n?=?30) than among CHD patients with left-to-right shunting but no PAH (n?=?20; P?<?0.001) and healthy control subjects (n?=?20; P?<?0.001). There was no difference in NO levels. Cyanotic PAH?CCHD patients had significantly higher homocysteine than acyanotic patients in the same group. No correlation was shown between echocardiographic/hemodynamic parameters and homocysteine, ADMA, and NO levels. Homocysteine and ADMA levels are increased in patients with PAH?CCHD. These parameters have the potential to be used as biomarkers in the diagnosis and follow-up evaluation of patients with PAH?CCHD. However, large, multicentered prospective studies are required to facilitate routine use of these biologic markers in the clinical setting.     

Congenital Subclavian Steal Syndrome Associated With Heterotaxy Syndrome and Atrioventricular Septal Defect

An infant under follow-up evaluation since the fetal stage was detected to have subclavian steal syndrome (SSS) associated with heterotaxy syndrome and atrioventricular septal defect. Such a complex association has not been reported previously. Congenital SSS occurs as a result of a subclavian artery originating from the main pulmonary artery through the ductus instead of the aortic arch. In rare cases, as in the reported case, it may occur due to proximal segment atresia of the left subclavian artery.