新生儿持续肺动脉高压

新生儿持续肺动脉高压并不是一种单一的疾病,而是由多种因素所导致的临床综合征.不同病因及不同病理类型的患儿对临床治疗的反应有很大的差异.除传统的药物降低肺动脉压力外,机械通气、一氧化氮吸入等新的方法 均逐渐成为安全有效的治疗手段.     

新生儿持续肺动脉高压的诊断与鉴别诊断

新生儿持续肺动脉高压是新生儿期的危重症,正确的诊断和鉴别诊断对临床合理处理有重要意义.本文对新生儿持续肺动脉高压的临床表现与诊断的关系、相关的临床诊断程序、辅助检查方法及常见需要鉴别的疾病等进行介绍,重点对肺动脉高压的超声诊断方法进行了评价.     

新生儿持续肺动脉高压的诊断和治疗

新生儿持续肺动脉高压是新生儿期危重疾病及死亡原因之一.目前对其病因、诊断及治疗进行了不少研究,但总体仍缺乏可靠的研究结果,尤其在治疗方面缺乏循证医学的证据及多中心大样本的随机对照研究结果.     

新生儿持续肺动脉高压致差异性发绀1例

男，1h，生后皮肤发绀50min入院。足月儿，阴道分娩，无窒息。生后10min出现全身皮肤发绀，呻吟，吸氧后无好转而转入本院。查体：发育正常，反应较好，哭声响，发绀明显，呼吸80次／min，三凹征阳性，双肺呼吸音清，无啰音，心率168次／min，律齐，无杂音，肝脾不大。入院后予头罩吸氧、纠酸等治疗，经皮测动脉血氧饱和度右上肢85％、左上肢55％、双下肢30％～40％。     

20例新生儿肺动脉高压的临床分析

新生儿动脉高压症２０例,均由超声心动图证实。其中原发性肺动脉高压３例;继发性肺动脉高压１７例,１２例继发于先天性心脏病,５例继发于围产期窒息、吸入性肺炎及湿肺。４例先天性心脏病的患儿用硫酸镁治疗,临床有效。治疗后动脉血氧饱和度和动脉化毛细血管法测定氧分压明显升高,二氧化碳分压降低,吸入氧浓度减少,没有发现明显的药物副作用。本组死亡６例,１４例存活者中２例自愈率,１例手术治愈,６例药物治愈,２例仍存     

脑钠肽在新生儿持续肺动脉高压诊断和治疗中的意义

目的 探讨脑钠肽(BNP)在新生儿持续肺动脉高压(PPHN)诊断和治疗中的临床应用价值,明确BNP是否可以作为判别PPHN的生化定量指标.方法 采用前瞻性对照研究,对54例发绀患儿及时行心脏彩色超声Doppler检查,结合临床鉴别出PPHN组29例、non-PPHN组25例,以同期住院无发绀患儿12例作为对照组.应用免疫荧光分析测定66例患儿血清BNP水平,比较各组患儿间BNP水平差异,应用SPSS 13.0统计软件描绘ROC曲线,评价该方法的价值及寻找判断PPHN的最佳临界值.结果 PPHN组新生儿BNP水平[(1 937.40±1 392.36) ng·L-1]显著高于non-PPHN组新生儿[(537.00±731.49) ng·L-1]和对照组[(50.72±36.50) ng·L-1](F=19.42,P<0.001),而non-PPHN和对照组BNP水平比较无统计学意义(P>0.05);ROC曲线下面积为0.903,95%可信区间0.818～0.988;判别PPHN的最佳BNP水平为807 ng·L-1,以此为标准,判别PPHN的灵敏度为0.828,特异度为0.880.结论 PPHN新生儿血清BNP水平明显升高,而BNP可作为一种动态观察指标,在判别PPHN的发生及指导合理治疗、判断预后中有重要临床意义.     

新生儿持续肺动脉高压的高危因素

目的了解新生儿持续肺动脉高压（PPHN）发病的高危因素及胎龄、原发疾病与预后的关系。方法收集2003年1月-2007年6月复旦大学附属儿科医院NICU住院的146例PPHN患儿，统计各个患儿的性别、胎龄（早产儿和足月儿）、原发疾病（包括窒息、感染、肺部病变、先天性心脏病、膈疝等）和不同转归（痊愈、好转、无效、死亡）。分析新生儿PPHN的发生和转归与性别、胎龄及原发疾病的关系。结果PPHN 146例患儿。男90例，女56例；足月儿105例（71．9％），早产儿41例（28．1％）。原发于肺部病变者共74例（50．7％），包括湿肺（26例）、吸入性肺炎及胎粪吸入综合征（MAS）（26例）、新生儿呼吸窘迫综合征（RDS）（17例）、气胸（5例），围生期窒息缺氧43例（29．45％），先天性心脏病9例（6．16％），感染8例（5．48％），膈疝7例（4．79％），其他5例（3．42％）。治愈75例，好转34例，无效及死亡37例。足月儿组湿肺患儿23例，早产儿组3例；RDS患儿足月儿组8例，早产儿组9例。湿肺并PPHN未愈率7．7％，吸入性肺炎并PPHN未愈率15．4％，其他原发疾病并PPHN的未愈率均〉20％。结论新生儿PPHN多发生于男性足月儿，肺部病变和围生期窒息缺氧为最常见原因，湿肺、吸入性肺炎并PPHN预后较好，而窒息、RDS、感染及先天性心脏病、膈疝等预后较差。     

小剂量硝酸甘油联合酚妥拉明持续静脉滴注治疗新生儿肺动脉高压的疗效

目的 探讨小剂量硝酸甘油联合酚妥拉明持续静脉滴注治疗新生儿肺动脉高压(PAH)的疗效.方法 2000年6月-2006年12月经超声心动图检查证实存在PAH的新生儿178例为研究对象.随机分为对照组85例,治疗组93例.其中先天性心脏病或肺结构异常42例(对照组20例,治疗组22例);其他病因所致者136例(对照组65例,治疗组71例).患儿经皮测血氧饱和度(TcSaO2)均低于85%.对照组予综合治疗加酚妥拉明0.2～0.3 mg/(kg·min),按10μg(kg·min)的速度泵入,1次/d,连用3 d;治疗组在此基础上加用硝酸甘油,0.3～0.5 μg/(kg·min),连续泵入6 h,若无效每天递增0.3～0·5μg/(kg·min),最多应用7 d.所有患儿治疗前后进行临床表现、心率、呼吸、血压、TeSaO:及平均肺动脉压(MPAP)监测.结果 治疗组总有效率、MPAP及平均TeSaO2治疗前后与对照组比较差异均有显著性(Pa<0.05),其中二组其他病因患儿比较差异亦有非常显著性(Pa＜0.01).不同病因、不同MPAP治疗前后比较也有显著性差异(Pa<0.05).治疗组体循环血压均无明显变化.结论 小剂量硝酸甘油联合酚妥拉明持续静脉滴注治疗PAH新生儿疗效优于单独应用酚妥拉明,且无严重不良反应.     

新生儿窒息肺动脉压力变化的无创性动态监测

目的　研究新生儿窒息后肺动脉压力的变化特点及临床意义 ,提供简便无创的检测方法。方法　应用彩色脉冲多谱勒超声于生后 2 4h内、3、7及 12～ 14d分别检测了 30例窒息新生儿及 2 5例正常新生儿左 /右心室射血前期、左 /右心室射血时间及其比值的变化 ,并依此推算肺动脉压力。结果　窒息与正常新生儿肺动脉压力在 2 4h内分别为肺动脉舒张压 (5 5 1± 17 3)mmHg对 (15 0± 3 0 )mmHg ,肺动脉阻力 (14 2± 3 9)mmHg/(L·min·m2 )对 (5 3± 1 3)mmHg/(L·min·m2 ) ,肺循环阻力 /体循环阻力比值 (0 84± 0 4 7)对 (0 2 7± 0 2 0 ) ,差异极显著 ,P <0 0 1。至生后 1周末 ,两组婴儿上述各指标间均无明显差异。结论　肺动脉高压是新生儿窒息的重要病理生理变化 ,在生后 1周内应重视对窒息新生儿肺动脉压力的检测。多谱勒超声心动图简便、无创 ,适于观察新生儿肺动脉压力的变化     

Echocardiographic Evaluation of Ventricular Function in Children With Pulmonary Hypertension

Although described in adults, it remains unclear whether ventricular dysfunction exists in pediatric patients with pulmonary hypertension (PHN). The goal of this study was to identify differences in echocardiographic indices of ventricular function among pediatric PHN patients. From 2009 to 2011, pediatric PHN patients with normal intracardiac anatomy and age-matched controls (1:3 ratio) were enrolled in this retrospective case–control study. Diagnosis of PHN was based on tricuspid regurgitation velocity or septal position estimating right-ventricular (RV) pressure >50 % systemic. Measures of RV and left ventricular systolic and diastolic function, including tissue Doppler imaging (TDI) of the mitral annulus (MA) and tricuspid annulus (TA), were compared. Enrollees included 25 PHN patients and 75 age-matched controls (mean age 7.5 years [range 1 day to 19 years]). Parameters of RV systolic and diastolic function were worse in PHN patients. Compared with controls, PHN patients had significantly decreased tricuspid valve inflow ratio, decreased TA TDI early diastolic velocities, decreased systolic velocities, increased tricuspid E/E’ ratio (all p < 0.01) and increased myocardial performance index. In an age-stratified analysis, TDI measures in PHN patients <1 year of age were similar to controls, whereas differences in TA TDI velocities and MA TDI velocities were noted in patients ≥1 year of age. Abnormalities in Doppler echocardiographic indices of ventricular systolic and diastolic function were identified in pediatric PHN patients and were more prominent with older age. These indices are promising for serial noninvasive monitoring of disease severity, but further correlation with catheterization-derived measures is needed.     

Correlation of Echocardiographic Markers and Therapy in Persistent Pulmonary Hypertension of the Newborn

Background Persistent pulmonary hypertension of the newborn (PPHN) causes morbidity and mortality in neonates. High-frequency ventilation (HFV), inhaled nitric oxide (iNO), and extracorporeal membrane oxygenation (ECMO) are used when conventional treatment fails. This study aimed to identify echocardiographic predictors of progression to these therapies before clinical deterioration. Methods Echocardiographic parameters were compared for survival and need for ECMO, HFV, iNO, and prolonged mechanical ventilation (MV, ≥10 days). Results Of 63 neonates, 95% survived, with 14% requiring ECMO, 52% requiring HFV, 67% requiring iNO, and 35% requiring MV. The following echocardiographic indices reflecting left ventricular output were decreased in sicker infants: (1) A decreased ascending aortic velocity time integral indicated an increased likelihood of ECMO (p = 0.02), iNO (p = 0.01), or MV (p = 0.05), (2) Shorter transverse aortic arch antegrade ejection time indicated HFV (p < 0.01), iNO (p < 0.01), and MV (p = 0.03), (3) Absent or retrograde transverse aortic diastolic flow correlated with HFV (p = 0.01, iNO (p = 0.01), and MV (p < 0.01). These sicker patients were more likely to have smaller left ventricular end-diastolic areas (p < 0.03 for all) and right-to-left atrial shunting (ECMO, HFV, and MV). There were no differences in survival. Conclusions Decreased left ventricular size and output correlates with the need for advanced therapies in infants with PPHN. Early identification may allow more effective management and placement of neonates at risk.     

Pulmonary Hypertension with Coexisting Portal Hypertension

A 10-year-old boy who had portal hypertension secondary to portal fibrosis/intrahepatic biliary atresia developed syncopal episodes related to strenuous activity. A work-up excluded a metabolic or neurologic etiology and cardiac catheterization demonstrated significant pulmonary hypertension. Six months later he died and an autopsy revealed pulmonary plexogenic arteriopathy without microemboli. Previous reported cases of this symptom complex are reviewed. In addition, he had a history of nonspecific colitis, ulcerative stomatitis, and conjunctivitis responsive to steroid therapy. The possible relationship of these manifestations to the portal pulmonary pathology is discussed.     

Echocardiographic Reference Values for Right Atrial Size in Children with and without Atrial Septal Defects or Pulmonary Hypertension

Right atrial (RA) size may become a very useful, easily obtainable, echocardiographic variable in patients with congenital heart disease (CHD) with right-heart dysfunction; however, according studies in children are lacking. We investigated growth-related changes of RA dimensions in healthy children. Moreover, we determined the predictive value of RA variables in both children with secundum atrial septal defect (ASD) and children with pulmonary hypertension (PH) secondary to CHD (PH-CHD). This is a prospective study in 516 healthy children, in 80 children with a secundum ASD (>7 mm superior–inferior dimension), and in 42 children with PH-CHD. We determined three RA variables, i.e., end-systolic major-axis length, end-systolic minor-axis length, and end-systolic area, stratified by age, body weight, length, and surface area. RA end-systolic length and area z scores were increased in children with ASD and PH-CHD when compared to those variables in the healthy control population. Using the Youden Index to determine the best cutoff scores in sex- and age-specific RA dimensions, we observed a sensitivity and specificity up to 94 and 91 %, respectively, in ASD children and 98 and 94 %, respectively, in PH-CHD children. We provide normal values (z scores ?2 to +2) for RA size and area in a representative, large pediatric cohort. Enlarged RA variables with scores >+2 were predictive of secundum ASD and PH-CHD. Two-dimensional determination of RA size can identify enlarged RAs in the setting of high volume load (ASD) or pressure load (PH-CHD).     

雷公藤甲素与肺动脉高压

由各种因素引起的肺动脉高压(简称肺高压)的根本问题是肺血管重构,对儿童先天性心脏病所引起的肺高压的预防和治疗一直是儿童心血管领域研究的热点。近年来,国外学者已通过动物模型实验结果显示,中药雷公藤提取物雷公藤甲素可逆转实验性肺高压和右心心力衰竭的形成。通过对雷公藤甲素的免疫抑制、抗炎、抗肿瘤等生物活性对肺高压的作用机制进行探讨,提示可能与细胞增殖和凋亡,影响基质金属蛋白酶活性,抑制内皮细胞迁移和血管生成及抗炎有关。     

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。