二尖瓣病变并肺动脉高压围术期低氧血症分析

目的总结二尖瓣病变并肺动脉高压患者围术期低氧血症的治疗方法。方法选择二尖瓣病变并肺动脉高压围术期发生低氧血症、低心排血量患者76例,给予呼吸机辅助呼吸、硝酸甘油0.5~1.0μg/（kg.min）持续泵入、米力农0.3~0.7μg/（kg.min）泵入。结果死亡3例,均因严重低氧血症、心衰,经治疗低氧血症等无改善,于术后7~10 d因继发多器官功能衰竭死亡;其余患者经治疗后好转出院,心功能Ⅱ、Ⅲ级。随访5月~5年,心功能Ⅰ级17例、Ⅱ级58例,术后3年死亡1例,该患者因置入生物瓣合并高血压死于脑出血。结论二尖瓣病变并肺动脉高压患者围术期易发生低氧血症,严重的低氧血症可通过扩张肺动脉降低肺动脉压并配合心功能支持药物及呼吸机治疗矫正。硝酸甘油、米力农等有效剂量泵入是降低肺动脉压改善低氧血症的有效方法。     

Respiratory dysfunction and pulmonary disease in cirrhosis and other hepatic disorders

End-stage liver disease and its complications are a leading cause of death among adults in the United States, and thousands of patients await liver transplantation. The liver plays a central role in health and homeostasis and thus the diseased liver leads to many deleterious effects on multiple organ systems, including the pulmonary system. We review the general effects of cirrhosis on the respiratory system, including mild hypoxemia, atelectasis, and hepatic hydrothorax. Cirrhosis is associated with 2 unique entities that affect the pulmonary vasculature: hepatopulmonary syndrome and portopulmonary hypertension. Hepatopulmonary syndrome, which is found in approximately 20% of patients awaiting liver transplantation, refers to the triad of hepatic dysfunction, hypoxemia, and intrapulmonary vascular dilations, and responds well to liver transplantation. In portopulmonary hypertension, cirrhosis and portal hypertension lead to pulmonary arterial hypertension, and portopulmonary hypertension has been considered a contraindication for transplantation. Currently, patients must have mild to moderate pulmonary hypertension to be considered for transplantation, and may still require long-term therapy with vasodilators to prevent right-ventricular failure and, consequently, failure of the newly transplanted liver allograft.     

心肺联合超声在低氧血症新生儿中的应用价值

目的探讨心肺联合超声在低氧血症新生儿病因诊断、病情监测及预后判断中的价值。 方法选取2018年1月至2018年8月杭州市第一人民医院新生儿科收治的低氧血症新生儿45例为实验组,选取同期住院的非低氧血症新生儿20例作为对照组。所有患儿均进行超声心动图联合肺部超声检查。应用超声心动图诊断结构性疾病并评估心功能,同时使用肺部超声评分法评估肺部情况,对超声检查结果及临床病因诊断结果进行综合分析。 结果45例低氧血症患儿临床病因有多种,包括呼吸窘迫综合征、先天性心脏病、胎粪吸入综合征、暂时性呼吸增快症、窒息后复苏等。45例低氧血症患儿中,超声心动图提示左心功能下降8例,右心功能下降1例,肺动脉高压33例;肺部超声表现为不同程度B线增多,肺局部实性变,双侧肺部评分0~30分,以13~24分者居多,单侧肺部最高评分18分。20例对照组新生儿超声心动图及肺部超声表现均未见明显异常。45例低氧血症患儿中,8例左心功能下降的患儿肺部均出现了弥漫性肺泡-间质综合征,1例右心功能下降患儿,肺部超声表现为以A线为主,单侧肺部评分2分。 结论心肺联合超声可为低氧血症新生儿的临床病因诊断提供线索和依据,对疾病进展进行监测及预判,且具有无辐射、方便快捷、费用低廉等优势,值得临床推广应用。     

Recognition and management of pulmonary hypertension

Pulmonary hypertension is a common manifestation of many diverse diseases. In most cases, the cause of elevated pulmonary arterial pressure is apparent on the basis of history, physical examination, chest x-ray, and ancillary noninvasive tests such as blood gases, pulmonary function tests, or echocardiography. Occasionally, more invasive tests such as cardiac catheterization and pulmonary angiography may be necessary. In those patients in whom no cause can be found, the pulmonary hypertension is labeled as primary or idiopathic. Effective therapy exists for alleviating pulmonary hypertension in most cases of secondary pulmonary hypertension. The prognosis of patients with primary pulmonary hypertension is dismal, although encouraging reports are beginning to appear regarding the use of vasodilators.     

牛心包补片修补心脏缺损并雾化吸入伊洛前列环素降低肺动脉高压

背景：对于先天性心脏病合并肺动脉高压的治疗,传统降低肺动脉高压的药物效果确实,但存在不易监测、停药后反跳等现象;传统的心脏涤纶补片较易引起吻合口变形、血栓、栓塞、溶血和感染等并发症。目的：观察应用戊二醛处理牛心包补片和雾化吸入伊洛前列环素治疗先天性心脏病合并肺动脉高压的效果。方法：选择90例先天性心脏病合并肺动脉高压患者,术中采用戊二醛处理的牛心包补片修补心脏间隔缺损,术后雾化吸入伊洛前列环素30 ng/（min·kg）,日间12 h按1次/4 h给药,夜间12 h按1次/6 h给药,连续给药2 d。记录每次吸入药物前、吸入后即刻、吸入30 min后平均动脉压、平均肺动脉压、体循环阻力指数与肺循环阻力指数,于随访期观察有无心包材料相关并发症和心功能情况。结果与结论：90例患者复查超声心动图见活瓣均已关闭,无分流,心脏间隔缺损无回声中断,心脏收缩功能正常,未发现与牛心包补片相关的不良反应。所有患者不同时间点平均动脉压、体循环阻力指数比较差异无显著性意义。患者吸入药物后即刻平均肺动脉压、肺循环阻力指数明显低于吸入药物前（P 〈0.01）,吸入30 min后平均肺动脉压、肺循环阻力指数亦明显低于吸入药物前（P 〈0.05）。表明应用戊二醛处理的牛心包补片和雾化吸入伊洛前列环素治疗先天性心脏病合并肺动脉高压安全、有效。     

先天性心脏病伴重度肺动脉高压术后早期并发症分析

目的 探讨先天性心脏病（CHD）伴重度肺动脉高压（PH）体外循环（CPB）术后早期并发症及处理。方法对本科2003年6月至2009年6月行CPB手术的45例CHD伴重度PH患者进行回顾分析,讨论术后早期主要并发症和死亡原因。结果术后早期主要并发症有严重低氧血症（13．33％）、缺氧发作（11．11％）、低心排（6．67％）。住院死亡率6．67％,死亡原因均为低心排,主要为右心室功能失代偿。多因素分析显示,低温CPB（OR=0．56;95％CI0．35-0．89）是严重低氧血症的一个危险因素。结论术后早期并发症主要由术后PH为基础的心肺功能不全所导致,对术后高危患者动态监测肺动脉压、右心功能和延长呼吸机支持,有助于防治并发症。     

Pulmonary hypertension in patients with interstitial lung diseases

Pulmonary hypertension (PH) in patients with interstitial lung diseases (ILDs) is not well recognized and can occur in the absence of advanced pulmonary dysfunction or hypoxemia. To address this topic, we identified relevant studies in the English language by searching the MEDLINE database (1966 to November 2006) and by individually reviewing the references of identified articles. Connective tissue disease-related ILD, sarcoidosis, idiopathic pulmonary fibrosis, and pulmonary Langerhans cell histiocytosis are the ILDs most commonly associated with PH. Pulmonary hypertension is an underrecognized complication in patients with ILDs and can adversely affect symptoms, functional capacity, and survival. Pulmonary hypertension can arise in patients with ILDs through various mechanisms, Including pulmonary vasoconstriction and vascular remodeling, vascular destruction associated with progressive parenchymal fibrosis, vascular inflammation, perivascular fibrosis, and thrombotic angiopathy. Diagnosis of PH in these patients requires a high index of suspicion because the clinical presentation tends to be nonspecific, particularly in the presence of an underlying parenchymal lung disease. Doppler echocardiography is an essential tool in the evaluation of suspected PH and allows ready recognition of cardiac causes. Right heart catheterization is needed to confirm the presence of PH, assess its severity, and guide therapy. Management of PH in patients with ILDs is guided by identification of the underlying mechanism and the clinical context. An increasing number of available pharmacologic agents in the treatment of PH allow possible treatment of PH in some patients with ILDs. Whether specific treatment of PH in these patients favorably alters functional capacity or outcome needs to be determined.     

How to manage secondary pulmonary hypertension

Establishing an underlying cause and treatment plan for patients with pulmonary hypertension presents a significant challenge to practicing physicians. Doppler echocardiography is a simple, cost-effective tool for detecting pulmonary hypertension and evaluating right ventricular function. Nonspecific therapy (use of digoxin and diuretics, anticoagulation) for pulmonary hypertension and right ventricular failure achieves a degree of symptomatic improvement and should be considered in patients with moderate to severe disease. CTEPH should be considered in patients with dyspnea. Because severs forms of pulmonary hypertension usually are not discovered until late in the disease course, a high level of suspicion is required when evaluating symptoms and risk factors consistent with pulmonary vascular disease. Pulmonary hypertension is classified as idiopathic, or primary, when no secondary cause can be identified. Primary pulmonary hypertension is a devastating disease that largely affects young women. Significant advances in treatment have been made and will be discussed in detail in part 2 of this article.     

肝肺综合征与气体信号分子

肝肺综合征是在慢性肝病、门静脉高压症的基础上,由多种因素共同参与、相互作用而导致低氧血症,肺血管扩张的一种疾病。肝肺综合征的发病机制较复杂且不完全明确,但气体信号分子参与了肺血管张力调节,并导致肺血管扩张。肝肺综合征预后差,严重威胁患者身体健康,积极有效地研究和防治肝肺综合征具有重要意义。本文就气体信号分子在肝肺综合征发病机制中的作用作一综述。     

Secondary pulmonary hypertension: a review of the cardiac causes

Pulmonary hypertension (PAH) is defined as mean pulmonary artery pressure greater than 25 mm Hg at rest, or greater than 30 mm Hg with exercise. While there are a myriad of etiologies for PAH, many times a cardiac etiology may be present. This is particularly important from both diagnostic and therapeutic standpoints, as many lesions are readily repairable with corrective surgery. Often, PAH may be drastically reduced, or even returned to baseline, after surgical correction. This article focuses on these cardiac etiologies of secondary PAH. Primary PAH, and PAH secondary to disease of the lung parenchyma, will not be discussed. Pulmonary hypertension is generated by increased flow through the pulmonary arteries, increased resistance to flow, or venous obstruction at the entrance to the left atrium. There is no upper limit of PAH that definitively precludes surgery, thus all patients with surgically approachable lesions should be referred for evaluation.     

Persistent pulmonary hypertension of the newborn]

Persistent pulmonary hypertension of the newborn (PPHN) is a cardiopulmonary disorder characterized by systemic arterial hypoxemia secondary to elevated pulmonary vascular resistance with resultant shunting of pulmonary blood flow to the systemic circulation. This disorder can be classified into four forms dependent on the etiology of the pulmonary hypertension: (1) Hypoplastic lung associated with congenital diaphragmatic hernia and oligohydramnions. (2) Primary PPHN (without known causative factor), such as preterm PPHN. (3) Secondary PPHN (with known causative factor), such as meconium aspiration syndrome, birth asphyxia or respiratory distress syndrome. (4) Relative PPHN associated with heart failure of hydrops fetalis or ischemic myocardial dysfunction. Inhalation of nitric oxide, which previously known as endothelial-derived-relaxation-factor, has been studied intensively as therapy for PPHN.     

Diagnosis of secondary hypertension: an age-based approach

Secondary hypertension is a type of hypertension with an underlying, potentially correctable cause. A secondary etiology may be suggested by symptoms (e.g., flushing and sweating suggestive of pheochromocytoma), examina- tion findings (e.g., a renal bruit suggestive of renal artery stenosis), or laboratory abnormalities (e.g., hypokalemia suggestive of aldosteronism). Secondary hypertension also should be considered in patients with resistant hyper- tension, and early or late onset of hypertension. The prevalence of secondary hypertension and the most common etiologies vary by age group. Approximately 5 to 10 percent of adults with hypertension have a secondary cause. In young adults, particu- larly women, renal artery stenosis caused by fibromuscular dyspla- sia is one of the most common secondary etiologies. Fibromuscular dysplasia can be detected by abdominal magnetic resonance imag- ing or computed tomography. These same imaging modalities can be used to detect atherosclerotic renal artery stenosis, a major cause of secondary hypertension in older adults. In middle-aged adults, aldosteronism is the most common secondary cause of hyperten- sion, and the recommended initial diagnostic test is an aldosterone/ renin ratio. Up to 85 percent of children with hypertension have an identifiable cause, most often renal parenchymal disease. Therefore, all children with confirmed hypertension should have an evaluation for an underlying etiology that includes renal ultrasonography.     

Transfusion-associated noncardiogenic pulmonary edema. Report of a case and a warning regarding treatment

Although noncardiogenic pulmonary edema (NCPE) is a recognized complication of blood transfusion, the precise etiology is not well understood. NCPE may be secondary to complement-mediated pulmonary capillary injury initiated by either donor or recipient anti-leukocyte antibodies. It is not caused by simple volume overload. Recent blood banking texts and published case reports continue to suggest diuretics as part of the initial therapy for this complication. We report a case of transfusion-associated NCPE in which empirical diuretic therapy clearly was detrimental and suggest that the use of diuretics for treatment of this condition is not warranted. Reversal of progressive hypoxemia is the mainstay of therapy. Hemodynamic monitoring is important in differentiating NCPE from pulmonary edema secondary to cardiac failure or volume overload and should be used as a guide for further therapy.     

成功救治肺源性心脏病并发胃肠道穿孔、脓毒症休克一例

脓毒症是患者入住ICU的常见病因,也是ICU患者死亡的主要原因之一。急性肾功能损伤是脓毒症常见脏器损伤,且与患者病死率相关。慢性阻塞性肺疾病患者由于长期肺动脉高压会导致肺源性心脏病,并进一步引起右心肥大甚至心功能不全,严重影响患者心肺功能。嘉兴市第二医院收治1例老年肺源性心脏病并发胃肠道穿孔、脓毒症休克患者,术后出现低心排、急性肾功能损伤及体循环淤滞,经相关积极救治后顺利出院,现将本例的临床资料及诊疗体会报道如下。     

Noninvasive imaging for the diagnosis and prognosis of pulmonary hypertension

Pulmonary hypertension (PH) is a major cause of morbidity and mortality, but it often presents with nonspecific symptoms, thereby delaying diagnosis and treatment. While invasive cardiac catheterization is essential to confirm the diagnosis, patients with breathlessness are commonly encountered in clinical practice creating a demand for noninvasive screening methods. Preliminary investigations such as the electrocardiogram and chest radiograph lack sensitivity even in advanced cases. Echocardiography is used to screen patients; however, over-reliance on a single estimation of pulmonary artery systolic pressure is unwise, instead multiple parameters should be assessed. Once a diagnosis of PH is made, radionuclide imaging should be performed to exclude chronic thromboembolic disease, and computed tomography is vital for eliminating parenchymal lung disease as a potential etiology. Currently, the primary contribution of cardiac MRI is the accurate assessment of right ventricular size and function. In this respect, cardiac MRI may be supportive during diagnosis of PH, but the main importance of this is in defining prognosis although new outcome variables are anticipated.     

Pulmonary Hypertension: Evaluation and Management

Pulmonary hypertension (PH) is a hemodynamic state characterized by elevation in the mean pulmonary arterial pressure and pulmonary vascular resistance leading to right ventricular failure and premature death. PH can be the result of a variety of diseases of different etiologies. Pulmonary arterial hypertension (PAH) should be distinctly differentiated from pulmonary venous hypertension (PVH) as a result of left heart disease. PAH is commonly caused by or associated with an underlying pulmonary, cardiac, or systemic disease (APAH). In the absence of an identifiable etiology or associated underlying disease, PAH is referred to as idiopathic (IPAH). IPAH, formerly known as primary pulmonary hypertension (PPH), is a rare disease most commonly seen in women of childbearing age. Presenting symptoms and signs are nonspecific and include dyspnea on exertion, fatigue, and a loud pulmonary component of the second heart sound. Transthoracic Doppler echocardiography is an excellent noninvasive test to detect the presence of pulmonary hypertension, although every patient should receive a right heart catheterization to confirm the diagnosis. A detailed work up, including laboratory tests and imaging studies, is also indicated to rule out known causes of pulmonary hypertension. Several targeted treatment options have become available in recent years and include parenteral and inhaled prostanoids, oral endothelin receptor antagonists, and oral phosphodiesterase type-5 inhibitors. As a result of their complex care, patients should be referred to centers with expertise in pulmonary hypertension.     

Rapid change in pulmonary vascular hemodynamics with pulmonary edema during cardiopulmonary resuscitation

Previous studies have shown that pulmonary edema occurs in half of all pre-hospital cardiac arrest victims who cannot be successfully resuscitated and is a major cause of hypoxemia and poor lung compliance during resuscitation. Pulmonary vascular hypertension and elevation of pulmonary capillary wedge pressure have been observed during cardiac resuscitation in humans. To further define the time course of the pulmonary hemodynamic changes, pulmonary artery diastolic pressure (PAd) was measured on a computerized trend recorder prior to, during, and immediately after arrest in three adult patients. Prior to arrest, PADP was 20.9 +/- 3.1 mm Hg. The PADP rose in all three patients by an average of 30.6% after 5-10 minutes and 71.3% after 10-15 minutes of CPR. Peak PADP reached 35.8 +/- 5.1 mm Hg (difference from pre-arrest level significant, P less than 0.001). In both patients who were resuscitated successfully, the PADP returned to baseline within 5 minutes of effective spontaneous circulation. The finding that such hemodynamic changes occur rapidly during resuscitation and can reverse quickly with resumption of effective spontaneous circulation is consistent with the time course for the early development of pulmonary edema. Development of pulmonary edema many hours following successful resuscitation likely involves other mechanisms.     

Percutaneous angioplasty for renovascular hypertension due to fibromuscular dysplasia.

Because the cause of hypertension is reversible in only 5 percent of patients, extensive initial work-up should only be considered in selected cases. Secondary causes should be suspected in patients whose hypertension begins before age 30 or after age 50 and in patients whose hypertension suddenly worsens after a long period of good control, becomes severe or malignant, or remains refractory to maximal medical therapy. A sudden reduction in renal function in a hypertensive patient and the discovery of a unilateral small kidney may also raise suspicion of a secondary cause. Renovascular disease, one of the most common secondary causes of hypertension, is usually the result of atherosclerosis in older patients and the result of fibromuscular dysplasia in younger patients. Physical examination seldom contributes to the diagnosis. The classic upper abdominal or flank bruit occurs in only 30 to 50 percent of patients with renovascular disease, and is not uncommon in patients with essential hypertension. The gold standard for diagnosis of renovascular disease remains the arteriogram. Transluminal renal angioplasty may be performed during arteriography if a high-grade stenosis is identified. Other management options include medical therapy and surgical revascularization with grafts.     

Diagnosis and evaluation of secondary hypertension

Although patients with secondary hypertension comprise only a small percentage of those with elevated blood pressure, this subgroup should not be ignored. In many cases, correcting the cause of secondary hypertension can lead to a cure, thus avoiding the need for long-term medical therapy, with its attendant risks and economic toll. Moreover, effective treatment of secondary hypertension can prevent chronic complications, such as left ventricular hypertrophy and coronary artery disease, which markedly increase morbidity and mortality. Nearly all forms of secondary hypertension are related to decreased renal function and/or derangement of hormonal balance or secretion. If hypertension is secondary to chronic renal failure of any etiology, it can be recognized from biochemical assays for blood urea nitrogen and creatinine.     

Exacerbation of underlying pulmonary disease in pregnancy

This article examines the management and outcomes of pregnant women with cystic fibrosis, primary pulmonary hypertension, and sarcoidosis. Pregnancy and the puerperium are associated with important cardiopulmonary changes that can adversely affect the clinical condition. Management of pregnant women with CF should be done with careful attention to complications of altered body weight, diabetes, and liver disease. Primary pulmonary hypertension is characterized by a progressive increase in pulmonary pressure and resistance in the absence of an identified cardiac or pulmonary cause. A multidisciplinary approach to the management of patients with primary pulmonary hypertension is of great importance for a successful maternal and fetal outcome. Good maternal and fetal outcomes are possible in women with restrictive lung disease in general and sarcoidosis in particular. The management of pregnancy, labor, and delivery are not altered by the presence of sarcoidosis.