原发性肺动脉高压的诊断和治疗

目的探讨儿童原发性肺动脉高压(PPH)的临床诊治特点。方法对收治15例PPH患儿的临床资料及治疗、转归进行分析。结果15例均有不同程度心功能不全症状、体征,心电图示右室肥厚,超声心动图示右房、右室大、肺动脉增宽。14例行心导管检查显示肺动脉压增高、肺小动脉楔压正常。PPH预后差,仅3例治疗后活动耐力增加。结论PPH症状无特异性,心导管检查在诊断中具有重要作用。PPH缺乏有效治疗,血管扩张剂和钙通道阻滞剂有一定疗效。     

Pulmonary Hypertension in Congenital Heart Disease: Irreversible Vascular Changes in Young Infants

Among 280 infants under 1 year of age with congenital heart disease autopsied at the University of Colorado Health Sciences Center between 1959 and 1978, there were six instances of grade IV¹ pulmonary artery hypertension. Five were patients with ventricular septal defect (four associated with other cardiovascular malformations). The sixth was a patient with atrioventricular canal. The youngest was 2¹/₂ months of age. Advanced degrees of pulmonary hypertensive arteriopathy (grade IV or more) have been said to be rare in infants, especially under the age of 1 year. The fact that all of these cases occurred within the last few years of the study suggests the possibility of improved supportive care leading to the prolonged survival of infants who might otherwise have died prior to developing severe disease. In addition, the role of altitude in accelerating the arteriopathy must be considered in the present series. In any case, this unexpected increase in the frequency of severe pulmonary hypertensive arteriopathy should stimulate consideration of early surgical correction of the underlying cardiovascular malformation, especially in areas of relatively high altitude.     

Exercise Testing in Children with Primary Pulmonary Hypertension

Cardiopulmonary exercise testing is a useful noninvasive tool to assess physiological changes associated with exercise. Developing noninvasive methods to assess the severity of cardiopulmonary disorders, as well as the response to therapeutic interventions, is useful in conditions, such as primary pulmonary hypertension, in which invasive procedures carry significant risks. The 6-minute walk test is a simple measure of exercise endurance. Exercise studies that measure both hemodynamic and ventilatory responses provide additional information regarding the interaction of the circulatory and pulmonary systems. Subtle changes in exercise capacity may suggest deterioration prior to clinical manifestations. This may lead to an earlier reevaluation, including repeat cardiac catheterization, and subsequently changes in medical and/or surgical therapy.     

Pulmonary Hypertension Associated with Portal Hypertension in a Child with Williams Syndrome—a case Report

A 14-year-old white female with Williams syndrome and portal hypertension presented in shock; at autopsy she was found to have grade II to VI vascular changes of pulmonary hypertension. This case demonstrates the association of portal hypertension and pulmonary hypertension in a pediatric patient.     

Complete Agenesis of the Right and Left Pulmonary Arteries, with Main Pulmonary Artery Originating from the Right Ventricle, Presenting as Primary Pulmonary Hypertension of the Newborn: Case Report

A female infant who had been misdiagnosed as having primary pulmonary hypertension of the newborn was found to have agenesis of the branch pulmonary arteries with collateral-dependent pulmonary circulation. The intracardiac anatomy was completely normal. Unifocalization of branch pulmonary artery was done, followed by stenting of the ductus arteriosus.     

肺动脉高压的内科治疗

肺动脉高压(PH)是以肺小血管收缩、重构和原位血栓形成为病理特征的临床血流动力学症候群,表现为肺动脉压力和肺血管阻力升高。内皮素、一氧化氮和前列环素通路失衡在PH病理发生中发挥重要作用。针对调节这3个通路的现代治疗方法与过去常规治疗方法综合应用可明显延长PH患者寿命,并提高其生活质量。     

混合性结缔组织病并肺动脉高压1例并文献复习

目的探讨混合性结缔组织病(MCTD)并肺动脉高压(PAH)的诊断与治疗,以提高对此病的认识。方法对MCTD并PAH 1例患儿的临床表现、既往史、辅助检查、诊疗过程进行分析,并复习相关文献。结果该患儿以间断晕厥为主要表现,超声显示重度PAH,抗核糖核酸蛋白抗体(U1-RNP)阳性,予免疫抑制及降低PAH治疗,病情明显好转。结论儿童MCTD并PAH起病隐匿,危害严重,诊断困难,应早期诊断,及时治疗,可改善预后。     

Congenital Misalignment of Pulmonary Veins with Alveolar Capillary Dysplasia Causing Persistent Neonatal Pulmonary Hypertension: Report of Two Affected Siblings

Misalignment of pulmonary vessels with alveolar capillary dysplasia is a rare cause of persistent pulmonary hypertension of the newborn. Most of the reported cases have been sporadic. We present two consecutive affected siblings with this disorder. This is the fifth reported family occurrence of this condition. In addition to the pulmonary abnormality, one of our cases had duodenal atresia. Received February 2, 1999; accepted June 28, 1999.     

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。     

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。     

高原肺动脉高压190例

目的研究高原肺动脉高压(PH)的临床特点,提高临床诊断的准确性。方法借鉴循证医学系统评价方法,对四川大学华西第二医院儿科1996~2005年出院诊断为高原PH的住院病例资料及通过中国期刊全文数据库检索的1994~2005年国内文献报告临床病例资料进行综合分析。结果共收集190例病例资料,其中17例来自四川大学第二医院儿科病历记录,173例来自国内文献报告。该病主要发生于移居高原的1岁以内小儿,男童较多见,种族差别明显。主要症状有呼吸困难发绀、咳嗽和充血性心力衰竭等;主要体征有右心衰和PH。结论高原PH临床表现复杂多样,超声心动图对该病诊断很有帮助,需加强认识,以提高临床诊断水平。     

原发性和继发性高血压患儿动态血压监测特点

目的 探讨儿童原发性高血压与继发性高血压的血压特点,提高对儿童高血压的病因识别.方法 选择2003年11月-2011年3月在首都医科大学附属北京安贞医院高血压科住院的高血压患儿为研究对象,按其病因分为原发性高血压和继发性高血压2组.患儿均常规测定身高、体质量、空腹血糖、血Cr、血尿酸、血脂及肾素、血管紧张素Ⅱ和醛固酮水平,并计算体质量指数(BMI).行手测血压、24 h动态血压监测,分析2组间日间、夜间收缩压(SBP)及舒张压(DBP)水平、日间、夜间SBP及DBP血压负荷等指标.结果 高血压患儿中男19例,女6例;年龄(12.4±2.5)岁.原发组患儿体质量和BMI均高于继发组患儿[(70.94±31.46)kg vs (45.93±14.62)kg; (24.98±6.96) kg·m-2 vs (19.13±4.89) kg·m-2],2组间差异均有统计学意义(P =0.013,0.031).与原发组患儿比较,继发组患儿的日间平均DBP水平[(91.14±10.67) mmHg vs (79.06±10.42) mmHg(1 mmHg=0.133kPa)]、夜间平均DBP水平明显升高[(81.43±12.71)mmHg vs (66.83±12.49) mmHg],日间SBP负荷[(79.46±18.17)％ vs(46.88±33.29)％],夜间SBP负荷[(89.02±15.74)％vs54.22 ±27.91)％],日间DBP血压负荷[(62.87±31.33)％ vs(30.94±27.36)％],差异均有统计学意义(Pa＜0.05).结论 原发性高血压患儿多伴有肥胖.继发性高血压患儿动态血压监测中日间和夜间DBP水平增高、日间和夜间SBP负荷、日间DBP负荷高.在儿童中动态监测血压水平,可帮助鉴别病因.     

波生坦治疗儿童肺动脉高压的研究进展

波生坦是一种非选择性的内皮素受体拮抗剂,在儿童肺动脉高压的治疗中具有重要作用。波生坦能改善肺动脉高压儿童的运动耐量、降低肺血管阻力、改善心功能,且不良反应较成人更少。与其他药物联用能改善患儿的生活质量。波生坦的远期效果、最佳治疗时程及药物联合应用仍有待于进一步研究。     

左向右分流致肺动脉高压对大鼠肺动脉胶原代谢的影响

目的建立左向右分流所致肺动脉高压大鼠模型,了解高肺血流量对肺血管胶原代谢的影响。方法对大鼠行腹主动脉下腔静脉分流术。术后11周以右心导管法测定肺动脉平均压(PAMP),采用免疫组织化学法检测大鼠肺动脉Ⅰ、Ⅲ型胶原蛋白的表达。结果分流11周后大鼠肺循环与体循环血流量之比分别为3.3∶1.0,为大量左向右分流。PAMP较对照组明显升高(P<0.01)。分流组大鼠肺中、小型肺动脉中Ⅰ、Ⅲ型胶原蛋白表达与对照组比较明显增加。结论高肺血流量可导致肺动脉高压,并促进肺动脉高压大鼠细胞外基质/胶原的堆积。     

西地那非治疗新生儿持续肺动脉高压比对研究(英文)

目的 探讨西地那非治疗新生儿持续肺动脉高压的疗效与安全性.方法 2005年1月至2008年10月收治持续肺动脉高压患儿45例,其中男25例,女20例;平均胎龄(39.3±2.4)周;平均出生体重(3 114.0±10.2)g;人院时平均年龄(13.0±0.8)h.病例随机分为西地那非、妥拉苏林和米力农治疗3组.应用超声心动仪检测肺动脉压力.结果 45例患儿中治愈30例,好转6例,无效9例,总有效率80.0%;西地那非、妥拉苏林和米力农3组患儿的治疗有效率比较,差异无统计学意义;3组患儿治疗前后平均肺动脉压力下降,差异有统计学意义;3组组间比较差异无统计学意义.所有患儿在治疗过程中均未见不良反应.结论 西地那非对新生儿持续肺动脉高压具有良好治疗效应,可有效降低平均肺动脉压力,改善心功能.     

Neonatal Pulmonary Artery Thrombosis Presenting as Persistent Pulmonary Hypertension of the Newborn

Pulmonary artery thrombosis in neonates occurs rarely. This report describes the case of a term infant with a pulmonary artery thrombosis presenting as persistent pulmonary hypertension of the newborn. The risk factors identified in the case included maternal diabetes and heterozygous factor V Leiden deficiency. The pulmonary thrombus was successfully treated with percutaneous catheter-based embolectomy.     

CONGENITAL ALVEOLAR CAPILLARY DYSPLASIA: Rare Cause of Persistent Pulmonary Hypertension

We report on a rare case of fatal congenital alveolar capillary dysplasia. The newborn boy of a 37 weeks' normal gestation suffered from persistent pulmonary hypertension without any cardiovascular malformation and died at the age of 4 weeks despite intensive treatment. The autopsy tissue was examined histologically, immunohistochemically, and ultrastructurally. Moreover, a three-dimensional tissue reconstruction based on serial sections was performed comparing the affected lung with normal lung tissue. We observed a unique pattern of pulmonary dysplasia: An extreme decrease of capillaries was localized centrally within thickened intra-acinar septa instead of capillaries intensely neighboring pneumocytes; ectatic veins normally running in the interlobular septa were found to accompany intralobular bronchovascular bundles, denying a clear distinction between pulmonary and bronchial veins; small muscular pulmonary arteries extended to the precapillary level and type 2 pneumocytes exceeded by far the type 1 pneumocytes, inverting the normal ratio. In summary, alveolar capillary dysplasia is assumed to be a primary capillary disorder of unknown origin, which possibly involves the regular differentiation of pneumocytes, according to the close alveolocapillary relationship during pulmonary ontogenesis. We consider the venous alterations as being part of the dysplasia, whereas the arterial phenomena might occur secondarily. Recent reports on affected siblings suggest a genetic component of pathogenesis.     

Childhood Pulmonary Blastoma: A Pleuropulmonary Variant of the Adult-Type Pulmonary Blastoma

Two fatal childhood cases of the rare pulmonary blastema are reported. One was associated with a congenital cystic adenomatoid malformation. Both neoplasms extended to involve visceral pleura and were entirely composed of blastemal and mesenchymal elements without recognizable neoplastic epithelial components. The mesenchymal component in both instances consisted of malignant rhabdomyoblasts, undifferentiated mesenchyme, and differentiated, apparently benign, cartilage. Review of the literature suggests that these features may be specific for the childhood forms of pulmonary blastoma. It is further suggested that pulmonary blastema, malignant mesenchymoma of the lung, and primary pulmonary rhabdomyosarcoma may have a common pathogenetic origin.     

通过微量渗透泵应用肾上腺髓质素对大鼠低氧性肺动脉高压的调节作用

目的研究肾上腺髓质素(ADM)对大鼠低氧性肺动脉高压的调节作用。方法24只雄性Wistar大鼠随机分为对照组、低氧组、低氧 ADM组,每组各8只。对于低氧 ADM组大鼠通过微量渗透泵皮下持续给予ADM(300ng/h)。低氧2周后,以右心导管法测定肺动脉收缩压(sPAP)和肺动脉平均压(mPAP),测量体循环平均压(mSAP),检测右心室与左心室加室间隔比值[RV/(LV S)]。结果低氧2周后大鼠sPAP、mPAP和RV/(LV S)较对照组明显增高(P均<0.01),低氧 ADM组大鼠sPAP、mPAP和RV/(LV S)较低氧组明显减低(P均<0.01),而mSAP在3组之间无明显差异。结论通过微量渗透泵皮下持续给予低氧大鼠ADM,缓解了低氧性肺动脉高压形成。