先天性心脏病合并中重度肺动脉高压82例

先天性心脏病合并中重度肺动脉高压,是先天性心脏病常见的严重并发症之一,手术治疗风险和难度较大,术前准备、术后管理非常重要.我院于2002-05至2009-04手术治疗先天性心脏病合并中重度肺动脉高压患者82例,临床近、中期疗效良好.     

先天性心脏病伴肺动脉高压65例的临床分析

目的:观察前列腺素E1对先天性心脏病合并肺动脉高压的血流动力学的影响.方法:将60例先天性心脏病肺动脉高压患者随机分为治疗组与对照组,对照组30例常规治疗,治疗组30例常规治疗基础上加前列腺素E1治疗.结果:治疗组临床症状、平均肺动脉压mPAP、动脉氧分压、动脉血氧饱和度有显著改善.结论:PGE1能显著改善临床症状,显著降低mPAP,提高动脉氧分压和动脉血氧饱和度,对先天性心脏病肺动脉高压治疗效果较好.     

高海拔地区小儿先心病合并肺动脉高压的围术期护理

目的：探讨高海拔地区小儿先天性心脏病合并肺动脉高压患儿的围术期护理措施和要点。方法：总结56例先天性心脏病合并肺动脉高压患儿手术后护理要点。结果：54例患儿均顺利撤离呼吸机,痊愈出院。2例患儿术后出现肺动脉高压危象,经处理后1例患儿好转出院,1例抢救无效死亡,无护理并发症发生。鳍论：高海拔地区先天性心脏病合并肺动脉高压患者围术期护理,术前要注重吸氧及肺动脉压力的降低;术后要特别加强呼吸道的护理、呼吸机的管理、心功能的维护,才能有效降低肺部并发症,提高手术成功率。     

高原先天性心脏病合并肺动脉高压的围术期处理

先天性心脏病合并肺动脉高压是影响预后和手术效果的严重并发症,通过积极的术前准备以及术后处理可明显减少肺动脉高压带来的严重并发症,降低死亡率.     

复杂先天性心脏病合并严重肺动脉高压婴幼儿术后呼吸系统的护理

复杂先天性心脏病患儿在新生儿或婴幼儿期即处于危重状态,如果不及时给予手术治疗,绝大部分因难以救治的肺炎合并心力衰竭或严重缺氧而导致死亡[1].肺动脉高压是左向右分流先天性心脏病常见的一种严重并发症,容易在术后早期诱发肺动脉高压危象,由于患儿低龄、低体重,术后病情变化快而复杂,呼吸系统的护理尤为重要,如处理不得当、不及时将会危及患儿生命.我院2005年6月～2008年12月共收治16例复杂先天性心脏病合并肺动脉高压的患儿,现将术后呼吸系统的护理要点总结如下.     

婴儿先天性心脏病合并肺动脉高压的外科治疗

目的:总结婴儿先天性心脏病合并肺动脉高压的外科治疗和围术期处理的经验。方法:2004年1月～2006年6月共手术治疗婴儿先天性心脏病合并肺动脉高压患者72例,男性45例,女性27例;年龄2个月～1岁,平均(7.3±2.8)月,体重3.5～9.2kg,平均(6.2±3.6)kg。术前肺动脉收缩压为47.4～89.2mmHg,平均(64.3±7.6)mmHg;动脉血氧饱和度81%～98%。结果:手术死亡3例(4.17%)。主要并发症有肺动脉高压危象、低心输出量综合症、肺不张、张力性气胸、呼吸机相关肺炎、血源性感染,呼吸衰竭等。死亡原因:肺动脉高压危象2例,低心输出量综合症1例。术后SaO287%～98%。随访6个月～2年,无晚期死亡,余患者心功能较术前改善。结论:肺动脉高压危象和低心输出量综合症是婴儿先天性心脏病合并肺动脉高压患者术后的主要并发症和死亡原因,早期手术及搞好围术期的处理是提高生存率的关键。     

婴儿先天性心脏病合并肺动脉高压术后监护21例

2006年8月-2010年2月,我科共收治先天性心脏病合并中高度肺动脉高压手术患儿21例。现对术后监护情况分析报告如下。     

先天性心脏病合并肺动脉高压患者的麻醉处理

肺动脉高压常见于肺血流增多的先天性心脏缺损,其发生率和严重程度与缺损的性质有关。总体而言,肺动脉高压是一类预后很差的综合征,尽管药物治疗有所发展,但至今仍以手术治疗为主。此类手术宜尽早在婴幼儿时期施行,以免病情随年龄增长而恶化。手术及麻醉过程中的很多因素可使肺血管阻力增高,如缺氧、手术刺激、炎性介质等,对于先天性心脏病合并肺动脉高压的患者选择合适的麻醉方式和药物维持循环稳定,防止肺循环阻力进一步增高至关重要。现就国内对先心病合并肺动脉高压患者的麻醉管理的研究进展作一综述。     

肺灌注显像在评定先天性心脏病合并肺动脉高压的临床应用

用放射性核素方法了解肺动脉高压的变化，不仅可以定性，还可以进行定量分析，同时作为一种无创性检查手段，具有简便、安全、可多次重复等优点，对于先天性心脏病合并肺动脉高压的术前判断和术后远期随诊，皆有明显的优点和实用性。     

肺灌注显像在评定先天性心脏病合并肺动脉高压的临床应用

用放射性核素方法了解肺动脉高压的变化，不仅可以定性，还可进行定量分析，同时作为一种无创性检查手段，具有简便、安全、可多次重复等优点，对于先天性心脏病合并肺动脉高压的术前判断和术后远期随诊，皆有明显的优点和实用性。     

Pulmonary arterial hypertension: a contemporary review

Pulmonary arterial hypertension has many causes, only some of which are well understood. The radiographic findings in pulmonary arterial hypertension are very similar regardless of the cause. Some radiographic features such as cardiac chamber enlargement, occur secondary to elevation of pulmonary pressures. In addition, sequential radiographs may demonstrate dramatic changes as the hypertension develops. This article presents examples of the various forms of pulmonary arterial hypertension, with pathologic correlation. Included are chronic pulmonary thromboembolism, plexogenic pulmonary arteriopathy, pulmonary venooclusive disease and persistent fetal circulation. Examples of lesions causing secondary pulmonary arterial hypertension: parenchymal lung disease, pulmonary venous hypertension, and congenital heart disease, are also illustrated.     

Rupture of a solitary,erosive peripheral pulmonary artery aneurysm

Aneurysms of the pulmonary arteries and their branches are rare. Although they may occur as isolated congenital anomalies, they more frequently are associated with congenital heart disease and pulmonary arterial hypertension. Less common predisposing factors include primary pulmonary hypertension, collagen vascular disease, trauma, and pulmonary banding. Inflammatory or infectious contributing factors include chronic granulomatous infection, infective endocarditis, pneumonia, and the Hughes-Stovin and Behçet's syndromes. Chronic pulmonary arterial hypertension, due to an underlying left-to-right shunt, as well as a superimposed focal bacterial pneumonia were the etiologic factors in the development of the segmental pulmonary artery aneurysm in our patient. The rarity of this constellation of predisposing factors and the diagnostic dilemma posed by the findings in our patient led us to report our case and review the pertinent literature.     

Pulmonary hypertension: the contribution of MDCT to the diagnosis of its different types

Pulmonary hypertension is characterized by progressive involvement of the pulmonary vessels that leads to increased vascular resistance and consequently to right ventricular failure. Vascular lesions are a common factor in a wide spectrum of diseases, and their result, pulmonary hypertension, is a severe clinical condition with a poor prognosis that worsens the normal course of the diseases to which it is associated (COPD, collagen disease, sarcoidosis, and congenital or acquired heart disease). It is important for pulmonary hypertension to be diagnosed as early as possible because nowadays drugs can reduce mortality and improve the quality of life; furthermore, some types of pulmonary hypertension (e.g., chronic thromboembolism and those associated with some congenital heart diseases like left-to-right shunt) can be treated surgically. In cases of suspected pulmonary hypertension, imaging methods can confirm the diagnosis, suggest a cause, help choose the most appropriate treatment, and monitor the response to treatment. This review describes the approach to pulmonary hypertension using different imaging techniques; special emphasis is given to the role of multidetector CT (MDCT), which makes it possible to study all the organs in the thorax in a single acquisition. We review the radiological signs of pulmonary hypertension and the current (Dana Point) radiological criteria for classifying the type of hypertension based on alterations in the lung parenchyma, mediastinum, pleural spaces, and pericardium, as well as on the study of the chambers of the heart.     

Echocardiographic diagnosis of pathologic processes of the central pulmonary vessels

Diseases of the central pulmonary arteries are difficult to diagnose. Echographic imaging of the pulmonary arteries can best be done using the suprasternal and transesophageal approach. In pulmonary arterial hypertension, the central pulmonary arteries increase in size, a fact that is used echographically to diagnose pulmonary hypertension. Even when there is volume overload (e.g. in congenital heart disease with a left-to-right shunt), characteristic changes of the pulmonary vessels are observed. Thromboemboli within the pulmonary arteries or thrombotic occlusion and a malignant process, which can lead to an obstruction or compression of the central pulmonary arteries, could be seen with echographic imaging techniques.     

Cardiac causes of pulmonary arterial hypertension: assessment with multidetector CT

The causes of pulmonary arterial hypertension (PAH) are diverse and include multiple congenital and acquired cardiac diseases as well as diseases primarily affecting the pulmonary vasculature, lung, pleura and chest wall. The traditional role of CT in evaluating PAH includes assessment of pulmonary vasculature and lung parenchyma with limited assessment of the heart. Advances in multidetector CT technology with improved spatial and temporal resolution now permit accurate delineation of cardiac morphology. CT pulmonary angiography (CTPA) is widely utilised in the workup of patients with suspected pulmonary vascular disease and can identify both pulmonary and cardiac causes. As the initial presentation for CTPA is often precipitated by nonspecific, unexplained symptoms and therefore undertaken by a general radiologist, it is important that a systematic approach to the interpretation of these studies, including cardiac evaluation, is routinely adopted. This paper reviews the CT evaluation in pulmonary hypertension with a particular focus on the cardiac causes, their subclassification into congenital systemic to pulmonary shunts and secondary to left heart disease, and their imaging features. It emphasises the use of a systematic approach to interpretation of CTPA examinations both in patients with known PAH and those with previously unsuspected disease.     

Assessment of pulmonary hypertension by CT and MR imaging

In the recent World Health Organization (WHO) classification the group of pulmonary arterial hypertension (PH) comprises the classic primary pulmonary hypertension and several conditions with definite or very high risk factors to develop pulmonary arterial hypertension. Therapeutic advances drive the need for a comprehensive pre-therapeutic evaluation for optimal treatment. Furthermore, follow-up examinations need to be performed to monitor changes in disease status and response to therapy. Up to now, the diagnostic imaging work-up of PH comprises mainly echocardiography, invasive right heart catheterization and ventilation/perfusion scintigraphy. Due to technical advances helical computed tomography (CT) and magnetic resonance imaging (MRI) became more important in the evaluation and for differential diagnosis of pulmonary arterial hypertension. Both modalities are reviewed and recommendations for clinical use are given.This work was supported by the German Research Council (DFG, FOR 474).     

Pulmonary hypertension: Work in progress

Pulmonary arterial hypertension is a rare disorder defined by mean pulmonary artery pressures that exceed 25 mm Hg at rest or are greater than 30 mm Hg with exercise. The mortality rate is high for untreated patients, mainly as a result of progressive right heart dysfunction. Pulmonary arterial hypertension has been historically classified as primary pulmonary hypertension or pulmonary hypertension resulting from an underlying disease process. Ongoing research in the nuclear medicine field holds great promise for understanding the pathophysiologic pathways for this condition, as well as the monitoring of the continually evolving therapeutic options.     

Untersuchungstechniken und Stellenwert der MRT bei der Diagnostik von Herzklappenerkrankungen

The main indications for cardiac magnetic resonance imaging (MRI) in the evaluation of valvular heart disease are pathologies of the aortic and pulmonary valve. For mitral and tricuspid valve pathologies MRI is not the first line modality as these are usually well visualized by echocardiography. The advantages of MRI in valvular heart disease are a high reliability in the evaluation of ventricular volumes and function as well as the assessment of the perivalvular arterial or atrial structures. This reliability and the limitless access to any imaging plane partially compensates for the lower temporal and spatial resolution in comparison to echocardiography. In patients with congenital heart disease, cardiac MRI is established as a valuable diagnostic tool in daily clinical management, especially for the evaluation of pulmonary valve defects. Nevertheless, echocardiography remains the first-line diagnostic imaging tool for the foreseeable future.     

Myocardial perfusion imaging in pediatric cardiology

Myocardial perfusion imaging (MPI) is an important procedure in pediatric cardiology in terms of evaluating myocardial ischemia, infarction and damage associated with various congenital or acquired heart diseases, such as Kawasaki disease, anomalous origin of the left coronary artery from the pulmonary artery and complete transposition of the great arteries after arterial switch surgery. This type of imaging can detect myocardial damage in the morphological right ventricle when it functions as a systemic pumping chamber in patients with complex congenital heart diseases after intra-cardiac repair. Myocardial perfusion imaging can also evaluate myocardial damage associated with primary or secondary cardiomyopathy in children. The magnitude of increased right ventricular uptake on MPI is a useful noninvasive means of estimating right ventricular pressure overload due to congenital heart or pulmonary diseases. This article reviews myocardial perfusion tracers and pharmacological stress tests used to diagnose heart conditions in children, and the current clinical roles of MPI in pediatric cardiology.