西地那非治疗儿童肺动脉高压的现状与进展

肺动脉高压（PHTN）是由肺血管阻力逐渐增加导致以右心衰竭和过早死亡为特点的一种罕见疾病。通常肺动脉高压与一些基础条件相关，如先天性心脏病、结缔组织病、先天性膈疝等。当肺动脉高压同时存在这些疾病时，被称为继发性肺动脉高压（APAH）。特发性肺动脉高压（IPAH）以前被称为原发性肺动脉高压，为基础病因未知的肺动脉高压，     

肺动脉高压的一氧化氮临床治疗

肺动脉高压是一种慢性致命性疾病,以肺动脉压力和肺血管阻力不断升高为主要特征,最终导致右心衰竭而死亡。一氧化氮(NO)是血管内皮细胞产生的一种血管活性物质,具有舒张血管平滑肌细胞的作用。吸入NO能选择性作用于肺血管,降低肺动脉压力和肺血管阻力。近年来,NO已越来越多地应用于肺动脉高压的治疗,特别是对先天性心脏病合并肺动脉高压患者术中和术后以及新生儿持续肺动脉高压有显著作用。     

新生儿持续肺动脉高压与肺血管内皮细胞功能

新生儿持续肺动脉高压 (ｐｅｒｓｉｔｅｎｔｐｕｌｍｏｎａｒｙｈｙｐｅｒｔｅｎｓｉｏｎｏｆｎｅｗｂｏｒｎ ,ＰＰＨＮ)是指新生儿期肺血管阻力增高及肺血流速度增加而发生的临床综合征。其发生率为活产儿的 1‰ ,是新生儿多种疾病引起死亡的最终病理途径。本症多继发于先天性心脏病、慢性缺氧或急性呼吸衰竭 ,其中主要为肺实质疾病 ,特点为严重肺动脉高压 ,导致肺外右向左血液分流及低氧血症[1] 。1　ＰＰＨＮ病理生理改变1 1　肺血流速度增加　伴有肺内或心内分流的新生儿 ,随着肺血管阻力降低 ,肺血流速度 (Ｑｐ)常增加 ,使肺血管床扩张…     

儿童肺心病

慢性肺心病是由于某些疾病影响肺组织的结构和/或功能而导致的右心室肥厚,但须除外其原发原因的左心疾患或先天性心脏病。急性型因发病过程短暂,可无右心肥厚,但肺动脉高压仍为基本特点。右心室肥厚则在过了一定时间后才出现,因心脏负荷虽有增     

超声心动图评价先天性心脏病合并肺高压右心室功能新进展

肺动脉高压(pulmonary arterial hypertension,PAH)是一种以肺血管阻力进行性增加,最终导致右心功能衰竭甚至死亡的疾病[1]。在我国,先天性心脏病(congenital heart     

家族性肺动脉高压

家族性肺动脉高压(familial pulmonary arterial hypertension,FPAH)是一种以肺小动脉丛样病变为特点的常染色体显性遗传性疾病,可导致肺动脉压进行性升高、右心衰竭,甚至死亡[1].2003年前FPAH被划分在原发性肺动脉高压的范畴,2003年世界卫生组织在意大利威尼斯召开了第三次肺动脉高压专家工作组会议,对肺动脉高压进行了病因学分类,"原发性肺动脉高压"这一名称被取消,代之以"特发性肺动脉高压"(idiopathic pulmonary arterial hypertension,IPAH)和FPAH[2].     

钙通道在肺动脉高压发病机制中的研究进展

肺动脉高压是一类严重的进展性疾病,最终导致患者右心衰竭,甚至死亡。肺血管收缩、肺血管重构、血栓形成和血管硬化等诸多原因导致肺动脉高压的发生。众多研究表明,Ca~(2+)信号在维持血管张力和调控血管平滑肌细胞增殖、凋亡等过程中发挥了重要作用,而这一信号转导机制主要由钙通道来调节操纵。文章综述钙通道在肺动脉高压发病机制中的研究进展。     

内皮素在新生儿持续性肺动脉高压中的作用

新生儿持续性肺动脉高压（persistent pulmonary hypertension of the newborn，PPHN）是以肺血管阻力升高为特征的临床综合征，其导致卵圆孔及动脉导管水平的右向左分流及严重低氧血症，是多种引起新生儿死亡的疾病的最终病理途径。据报道，持续性肺动脉高压在新生儿中的发病率为0.43-6.8/1000。     

转化生长因子β/骨形成蛋白通路与肺动脉高压遗传学机制的研究进展

肺动脉高压由多种心、肺疾病所致,以动脉压力进行性升高、肺血管阻力逐渐增加为特征,最终导致右心衰竭和死亡,其发病机制至今尚未完全阐明.近期有研究发现,转化生长因子β/骨形成蛋白通路对肺动脉高压的发生发展可产生重要影响.针对转化生长因子β/骨形成蛋白通路的研究为肺动脉高压的防治提供了新的理论依据.该文就转化生长因子β/骨形成蛋白通路与肺动脉高压遗传学机制作一综述.     

低氧性新生儿持续肺动脉高压与肺血管重建发生机制

新生儿持续肺动脉高压(PPHN)为新生儿期的严重疾病,出生后肺动脉压力等于或超过体循环压力,出现动脉导管和(或)卵圆孔水平的右向左分流,导致明显的低氧血症.肺血管重建与PPHN的形成和发展过程有较强相关性,低氧引起的肺血管重建以血管壁的内膜、中膜和外膜细胞组成成分和调节机制紊乱,血管壁增厚为基本特征.该文从内皮细胞、平滑肌细胞和细胞外基质三方面来阐述低氧性PPHN与肺血管重建的关系及其可能机制.     

Hurler's syndrome with cor pulmonale secondary to obstructive sleep apnoea treated by continuous positive airway pressure

A 6-year-old boy with Hurler's syndrome presented with right heart failure and pulmonary hypertension secondary to severe obstructive sleep apnoea. Both his sleep apnoea and cor pulmonale were effectively controlled with continuous positive airway pressure therapy.     

Nesiritide for the Treatment of Pulmonary Hypertension and Cor Pulmonale in an Infant

Nesiritide is a synthetic form of B-type natriuretic peptide. It is approved for the treatment of acute exacerbations of congestive heart failure in hospitalized adult patients. It is currently under investigation for use in other settings and other patient populations. This article describes administration of nesiritide to an infant patient with severe pulmonary hypertension and cor pulmonale. No adverse reactions occurred during administration of the drug. Specifically, there was no hypertension, vomiting, arrhythmia, or changes in renal function. No changes in renal function occurred in the months subsequent to treatment. This case report illustrates that nesiritide can be safely administered to critically ill infants with pulmonary hypertension and cor pulmonale. Our patient experienced a decrease in pulmonary pressure and improved clinical condition during and after the infusion. However, further study is required to fully evaluate the safety and efficacy of nesiritide for these patients.     

Abnormalities of breathing control and airway maintenance in infants and children as a cause of cor pulmonale

Summary Respiratory control abnormalities may result in cor pulmonale. This report summarizes the clinical history, diagnostic evaluation, treatment, and outcome of 16 infants and children presenting with cor pulmonale subsequently found to be due to sleep-dependent hypoventilation. Eleven patients had cardiomegaly and electrocardiographic evidence of right ventricular hypertrophy (RVH) while 5 had only severe RVH or biventricular hypertrophy (BVH). Four infants with central hypoventilation syndrome (CHS)—absence of sleep-related ventilatory drive—had severe sleep-dependent asphyxia and resultant acute respiratory failure; all were ultimately treated with phrenic nerve pacing. One patient with alveolar hypoventilation syndrome (AHS)—a partial deficit in ventilatory drive during sleep—presented with severe pulmonary hypertension and ultimately died despite symptomatic relief with respiratory stimulants. Eleven patients presented with obstructive sleep apnea (OSA) and sleep-dependent asphyxia secondary to intermittent complete or to prolonged partial upper airway obstruction. Localized airway obstruction due to an anomalous innominate artery in 1 child was corrected by arteriopexy. Four children underwent adenotonsillectomy (T&A) with disappearance of symptoms in 1, clinical improvement in 2 and no clinical improvement in another. This unimproved patient and the 6 remaining OSA children improved dramatically after tracheostomy to bypass the sleep-dependent airway obstruction; none presently has evidence of cor pulmonale. In summary, early recognition and appropriate treatment of respiratory control disorders will improve sleep ventilation, eliminate asphyxia during sleep, and prevent the development of cor pulmonale.Supported in part by Children's Research Guild, Otho S. Sprague Foundation and NIH Grant RR-05475-14This paper was part of the Ray C. Anderson Symposium     

Studies on cor pulmonale in cystic fibrosis: I. Effects of diuresis.

The effects of acute diuresis in patients with cor pulmonale secondary to cystic fibrosis were studied. Both hemodynamic parameters and arterial blood gas changes were investigated. The major effect noted was a significant reduction of systemic venous pressure. Pulmonary artery and pulmonary artery wedge pressure either remained constant or declined slightly. No consistent changes were noted in cardiac output or arterial blood gases. Acute diuresis of moderate degree appears to be a safe and effective manner in which to treat the systemic venous congestion of cor pulmonale in this situation.     

Cor triatriatum masked by primary pulmonary hypertension

Summary In a 17-month-old boy with clinical signs of right heart failure, the diagnosis of primary pulmonary hypertension was made, based on cardiac catheterization findings with high pulmonary arterial pressure and normal pulmonary wedge pressure although two-dimensional echocardiography demonstrated a left atrial membrane. Postmortem examination confirmed a left atrial membrane (cor triatriatum), and the microscopic examination revealed the histological pattern of primary pulmonary hypertension. Due to reduced pulmonary blood flow, pulmonary venous obstruction was masked and could not be assessed by measuring pulmonary wedge pressure.     

小儿先心病术后机械通气时间延长的多因素分析及治疗策略

目的回顾性分析小儿先心病术后机械通气时间延长的相关因素,探讨相关治疗策略,从而优化拔管时机,提高先心病术后监护质量。方法 2013年5月至2016年5月我们收治188例先心病术后患儿,将可能影响机械通气时间的8项因素(年龄、体质量、体外循环时间、主动脉阻断时间、肺动脉高压、术后低心排、术后急性肺损伤、呼吸机相关性肺炎)进行多元逐步Logistic回归分析。并归纳机械通气时间延长相关因素的防治策略。结果体外循环时间、肺动脉高压、术后低心排综合征、急性肺损伤、呼吸机相关性肺炎5项因素与先心病术后机械通气时间延长有显著相关性。结论体外循环时间长、肺动脉高压、术后低心排综合征、急性肺损伤、呼吸机相关性肺炎是先心术后机械通气时间延长的主要影响因素。尽量缩短体外循环时间、积极控制肺动脉高压、防治低心排综合征、预防急性肺损伤和呼吸机相关性肺炎,可有效缩短机械通气时间,改善监护质量。     

Chronic cor pulmonale and refractory hypoxemia following pulmonary embolism in a six-month-old infant: surgical management by thromboendarterectomy]

A diagnosis of pulmonary embolism is uncommon in the infant and the child, and chronic cor pulmonale secondary to pulmonary embolism is an even rarer occurrence. CASE REPORT: In this study, a case of pulmonary embolism in a 6-month-old male infant has been reported. His past history included preterm birth, and severe bronchopulmonary dysplasia, with prolonged oxygen dependency. The positive diagnosis was based on cardiac ultrasound examination, with the direct imaging of a right pulmonary arterial thrombus. Surgical thromboendarterectomy was performed, with a long-term favorable outcome. CONCLUSION: After excluding from the diagnosis those hemostatic disorders known to be thrombogenic, the most likely hypothesis was retained, i.e., that it was catheter-related. A central venous catheter had been inserted during the neonatal period, and was probably responsible for the embolism. The clinical characteristics and the diagnostic and therapeutic aspects of chronic postembolic cor pulmonale have been discussed in the light of the present findings.     

Congenital heart disease in the Pierre Robin syndrome

Summary Congenital heart disease occurs in about 20 percent of patients with Pierre Robin syndrome. Ventricular septal defect, patent ductus arteriosus, and atrial septal defect are the most common congenital cardiac lesions in this syndrome. The associated upper airway obstruction can produce cor pulmonale, cardiomegaly, pulmonary edema, and cyanosis.     

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。