COPD患者肺动脉高压与急性加重的相关研究

目的 探讨COPD患者肺动脉高压对急性加重的预测作用.方法 收集2013至2015年因COPD急性加重入院的76例患者资料,随访8个月,记录心脏彩超所测收缩期肺动脉压(systolic pulmonary artery pressure,sPAP)、急性加重次数.结果 肺动脉压正常组和轻度肺动脉高压组间急性加重次数差异无统计学意义;中度、重度肺动脉高压组的急性加重次数均多于肺动脉压正常组、轻度肺动脉高压组,中度肺动脉高压组与重度肺动脉高压组的急性加重次数差异无统计学意义.肺动脉压力和有无急性加重发生的 ROC曲线下面积 AUC=0.786 (95% CI :0.684~0.887), P=0.000,最佳界值点为sPAP=50 mmHg,敏感度64%,特异度88.5%,阳性预测值91.2%,阴性预测值56.97%.结论 因COPD急性加重而住院的患者 sPAP>50 mmHg 可作为再次急性加重的预测因子,中重度肺动脉高压的COPD患者可发生更频繁的急性加重.     

慢性阻塞性肺疾病稳定期夜间低氧的治疗进展

慢性阻塞性肺疾病(chronic obstructive pulmonary disease,COPD)稳定期患者常发生夜间低氧,特别是易发生在快动眼睡眠时相,持续夜间低氧会进一步发展为肺动脉高压、肺心病.为改善COPD稳定期夜间低氧,其治疗主要集中在长期氧疗、夜间无创正压通气、药物治疗,并取得了一定的疗效.本文对COPD稳定期夜间低氧的治疗进展作一综述.     

Pulmonary hypertension in chronic obstructive pulmonary disease.

Pulmonary hypertension is a common complication of chronic obstructive pulmonary disease (COPD). Its presence is associated with shorter survival and worse clinical evolution. In COPD, pulmonary hypertension tends to be of moderate severity and progresses slowly. However, transitory increases of pulmonary artery pressure may occur during exacerbations, exercise and sleep. Right ventricular function is only mildly impaired with preservation of the cardiac output. Structural and functional changes of pulmonary circulation are apparent at the initial stages of COPD. Recent investigations have shown endothelial dysfunction and changes in the expression of endothelium-derived mediators that regulate vascular tone and cell growth in the pulmonary arteries of patients with mild disease. Some of these changes are also present in smokers with normal lung function. Accordingly, it has been postulated that the initial event in the natural history of pulmonary hypertension in COPD could be the lesion of pulmonary endothelium by cigarette-smoke products. Long-term oxygen administration is the only treatment that slows down the progression of pulmonary hypertension in chronic obstructive pulmonary disease. Nevertheless, with this treatment pulmonary artery pressure rarely returns to normal values and the structural abnormalities of pulmonary vessels remain unaltered. Vasodilators are not recommended on the basis of their minimal clinical efficacy and because they impair pulmonary gas exchange. Recognition of the role of endothelial dysfunction in the physiopathology of pulmonary hypertension in chronic obstructive pulmonary disease opens new perspectives for the treatment of this complication.     

Significance of pulmonary artery pressure in emphysema patients with mild-to-moderate hypoxemia

The guidelines of the Global Initiative for Chronic Obstructive Lung Disease (GOLD) do not recommend the measurement of pulmonary artery pressure in patients with chronic obstructive pulmonary disease (COPD). This is on the basis that the mean pulmonary artery pressure (mPAP) does not provide more clinical information than measurement of the oxygen tension in arterial blood (PaO2). The mPAP correlates well with PaO2 in emphysema patients with severe hypoxemia (PaO2 < or = 7.3 kPa (55 mmHg)). However, the occurrence and significance of mPAP is unclear in patients without severe hypoxemia (PaO2 > 7.3 kPa (55 mmHg)). In order to evaluate the usefulness of measurement of mPAP in emphysema patients without severe hypoxemia, we performed right heart catheterization and investigated the pulmonary hemodynamics of 53 patients without severe hypoxemia. In addition, we identified long-term prognostic factors with a mean follow-up term of 77 months after right heart catheterization. Seventeen of 27 patients with mild-to-moderate hypoxemia exhibited pulmonary hypertension (mPAP > or = 2.7 kPa (20 mmHg)) and the classification according to severity in GOLD exhibited a greater correlation to mPAP than PaO2. Moreover, only mPAP was found to be a significant prognostic factor according to multivariate proportional hazards analysis (P = 0.01). We conclude that mPAP is more informative about the severity of emphysema than PaO2 in patients with mild-to-moderate hypoxemia.     

Unusual presentation: pulmonary hemosiderosis with celiac disease and retinitis pigmentosa in a child

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease characterized by anemia, hemoptysis and recurrent alveolar hemorrhage. The combination of IPH and celiac disease (CD) is extremely rare. We report a 9-year-old boy with Lane-Hamilton syndrome, co-occurrence of pulmonary hemosiderosis with CD. This presentation is unique presentation because he has also retinal pigmentation.     

Therapeutic application of calcium-channel antagonists for pulmonary hypertension

Calcium-channel antagonists may provide an effective approach to the treatment of pulmonary hypertensive disorders. Biochemical evidence suggests that pulmonary vasoconstriction results from the transmembrane flux of calcium into vascular smooth muscle; accordingly, the pulmonary pressor responses in experimental hypoxic pulmonary hypertension can be attenuated by verapamil and nifedipine. In patients with chronic obstructive lung disease, nifedipine decreases pulmonary artery pressures and pulmonary vascular resistance in proportion to the severity of hypoxemia before treatment. However, little pulmonary vasodilator effect is seen when hypoxemia is corrected by inhalation of oxygen, and systemic arterial oxygen desaturation can occur after nifedipine in patients breathing room air; most importantly, long-term studies in patients with chronic lung disease are lacking. In selected patients with primary pulmonary hypertension and other obliterative diseases of the pulmonary vasculature, nifedipine produces short- and long-term hemodynamic improvement at rest and during exercise, and these benefits are frequently paralleled by amelioration of dyspnea and fatigue. However, in patients in whom right ventricular function has been severely compromised by chronic pressure overload, both verapamil and nifedipine may exert notable depressant effects on right ventricular performance, despite the decrease in right ventricular afterload that would be expected to accompany a decrease in pulmonary vascular resistance. These negative inotropic actions may result in serious deleterious clinical reactions. Although calcium-channel antagonists represent a promising approach to the management of patients with pulmonary hypertension, the long-term efficacy and safety of these drugs in this disorder remain to be established.     

Long-term outcome of idiopathic pulmonary hemosiderosis in children

We retrospectively analyzed the long-term outcome of idiopathic pulmonary hemosiderosis (IPH) in 15 children. IPH started at a mean age of 5 years, and the mean duration of follow-up was 17.2 years (range, 10-36 yr). Four patients developed immune disorders, 3 cases of rheumatoid polyarthritis or rheumatoid polyarthritis-like diseases and 1 case of celiac disease. Respiratory outcome showed that 3 patients had severe symptoms: 2 patients developed severe pulmonary fibrosis resulting in major chronic respiratory insufficiency, and 1 patient had severe asthma. Twelve patients (80%) had mild or no respiratory problems and were able to lead a normal life. According to chest X-ray and pulmonary function test data, 4 patients had normal chest X-ray and no evidence of restrictive syndrome, 6 patients had an interstitial pattern on chest X-ray and evidence of restrictive pattern, 1 patient had an interstitial pattern but normal lung function, and 1 patient had a normal chest X-ray but evidence of mixed obstructive and restrictive pattern. Our results show that long-term survival is possible in patients with IPH. Factors of poor prognosis seem to be the presence of antineutrophil cytoplasm antibodies (ANCA) or other autoantibodies. No other clinical or biological predictive factors for prolonged survival were found.     

Atrial natriuretic peptide concentrations and pulmonary hemodynamics in patients with pulmonary artery hypertension

To define the relationship between plasma levels of immunoreactive atrial natriuretic peptide (IR-ANP) and hemodynamic parameters in patients with chronic pulmonary artery hypertension, we measured plasma concentrations of the peptide in 15 patients during right heart catheterization. Eleven patients had chronic obstructive pulmonary disease and 4 had pulmonary vascular disease of diverse etiology. At rest, plasma concentrations of IR-ANP positively correlated with mean pulmonary artery pressure (r = 0.70, p less than 0.01) and pulmonary vascular resistance (r = 0.88, p less than 0.001), but not with right atrial pressure. Nine of these patients, all with chronic obstructive pulmonary disease, were also evaluated during exercise. Plasma concentrations of IR-ANP increased from 131 +/- 22 to 191 +/- 30 pg/ml (p less than 0.003) at maximal exercise, whereas pulmonary artery pressure increased from 29 +/- 1.5 to 56 +/- 2.5 mm Hg and right atrial pressure from 5 +/- 1 to 13 +/- 2 mm Hg. Increases of plasma IR-ANP concentrations correlated with changes in pulmonary artery pressure and right atrial pressure but not with changes in pulmonary capillary wedge pressure. These findings suggest that ANP is released in response to an increase in pulmonary artery pressure and are consistent with the hypothesis that ANP could modulate the pulmonary vascular tone in patients with pulmonary artery hypertension.     

肺心病合并肺血栓栓塞40例临床分析

目的 探讨COPD所致慢性肺原性心脏病并肺血栓栓塞（PTE）临床表现特点。方法 回顾性分析40例COPD所致肺心病合并PTE与单纯的COPD所致慢性肺心病40例进行对比。结果 肺心病合并肺栓塞组顽固性右心衰的发生率高、肺动脉压明显升高；氧分压明显下降，与单纯肺心病组比较，差异有显著性（P〈0．05）。结论 在诊断COPD所致慢性肺心病时，遇有难于解释的顽固性右心衰、高肺动脉压及低氧血症时应警惕合并PTE，尽快作胸部CT肺动脉造影或磁共振肺动脉造影（MRIPA）等检查以进一步明确诊断。     

Effects of captopril on hemodynamics and blood gases in chronic obstructive lung disease with pulmonary hypertension

The effects of Captopril, an angiotensin-converting enzyme inhibitor, on pulmonary hemodynamics and blood gases were studied in 9 patients with chronic obstructive lung disease (COLD) and pulmonary hypertension (PA-P greater than 20 mm Hg). Hemodynamic data were recorded prior to Captopril administration (50 mg per os) and for the next 60 min. Following Captopril administration, significant reductions in mean pulmonary artery pressure (PA-P) (p less than 0.05), in mean pulmonary wedge pressure (PW-P) (p less than 0.05), and in total pulmonary resistance (TPR) were noted; significant reductions in mean brachial artery pressure (BA-P) and systemic vascular resistance (SVR) were also recorded, while cardiac output, heart rate and blood gas tensions showed no significant changes. Furthermore, the higher the hypoxemia, the greater was the reduction in BA-P (p less than 0.05). We therefore feel that Captopril, when administered to COLD patients with pulmonary hypertension, may protect the pulmonary circulation from hypoxic pulmonary vasoconstriction.     

Prevalence and diagnosis of severe pulmonary hypertension in patients with chronic obstructive pulmonary disease

PURPOSE OF REVIEW: The possible development of pulmonary hypertension is a well-known complication in the course of chronic obstructive pulmonary disease. When present, pulmonary hypertension is in general of mild severity at rest. It is therefore generally considered as a second-rank marker of the disease, mainly because the patients are limited in their exercise capacity for respiratory rather than for circulatory reasons. Apart from the common hemodynamic profile, however, some patients have a moderate-to-severe level of mean pulmonary artery pressure. RECENT FINDINGS: These patients with a predominant vascular disease have been individualized recently. They have in common some particularities, the most characteristic profile being a mild-to-moderate airway obstruction, contrasting with the severity of dyspnea and hypoxemia. Another typical feature in these patients is the apparently poor prognosis. SUMMARY: Such patients have to be recognized because they are potential candidates for specific therapies aiming at reducing pulmonary vascular resistance.     

Idiopathic pulmonary hemosiderosis in a patient of advanced age

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown etiology characterized by hemoptysis, diffuse pulmonary infiltrates and iron-deficiency anemia. It is typically found in children and young adults. Finding that the concentration of siderophages exceeds 20% in bronchoalveolar lavage indicates a diagnosis of IPH. We report the case of a 72-year-old man with repeated diagnoses of respiratory infection, bronchiectasis and melena. Because such a clinical picture is rare for IPH, the final diagnosis was obtained by open lung biopsy.     

Diffuse alveolar hemorrhage with chronic thyroiditis in an advanced-age adult

Idiopathic pulmonary hemosiderosis (IPH) is one of the rare causes of diffuse alveolar hemorrhage (DAH), and usually occurs in children. The mechanism underlying this disease development has not been defined. During the acute phase, death due to massive alveolar hemorrhage and subsequent severe respiratory failure with multiple organ failure often occurs. We report a case of IPH which occurred in an advanced-aged adult during following thyroidectomy for chronic thyroiditis. Following surgery this 83-year-old male developed acute onset dyspnea and pulmonary hemorrhage. In a search for underlying causes, no disorders were found and the only finding was the presence of anti-thyroid antibody. Systemic corticosteroid therapy was effective and he fully recovered. To our knowledge, this is the second documentation of IPH in association with chronic thyroiditis.     

Renin-angiotensin-aldosterone system and pulmonary hemodynamics in patients with pulmonary artery hypertension

To investigate the relationship between renin-angiotensin-aldosterone system and pulmonary hemodynamic parameters in patients with chronic pulmonary artery hypertension, we measured plasma levels of renin activity, angiotensin II and aldosterone in 11 patients during right heart catheterization. All patients had chronic obstructive pulmonary disease. At rest, plasma concentration of angiotensin II positively correlated with mean pulmonary artery pressure (r = 0.76, P less than 0.01) and pulmonary vascular resistance (r = 0.64, P less than 0.05). During exercise, plasma level of angiotensin II increased from 70 +/- 21 to 81 +/- 24 pg/ml (P less than 0.01) and plasma renin activity from 0.66 +/- 0.54 to 1.28 +/- 1.2 ng/ml/h (P less than 0.05), whereas mean pulmonary artery pressure increased from 3.73 +/- 0.85 to 6.27 +/- 1.81 kPa (28 +/- 6.4 to 47 +/- 13.6 mmHg). Increase of angiotensin II correlated with changes in mean pulmonary artery pressure (r = 0.69, P less than 0.05) but not with systemic artery pressure. The results of present study suggest that angiotensin II might play a role in the development of pulmonary artery hypertension in patients with chronic obstructive pulmonary disease.     

Idiopathic pulmonary hemosiderosis in an adult patient responded well to corticosteroid therapy

Idiopathic pulmonary hemosiderosis (IPH) is a very rare disorder of unknown etiology characterized by recurrent or chronic hemorrhage and accumulation of hemosiderin in the lung parenchyma. It is most common in children but can occur in adults. Clinical manifestations of the disease include iron deficiency anemia without any known cause, pulmonary symptoms such as hemoptysis, dyspnea and cough, and parenchymal lesions on chest X-ray. The clinical course of the disease may vary from patient to patient however, in general, the prognosis of the disease is worse. Treatment is symptomatic and supportive. Corticosteroids and other immune suppressive agents were used for the therapy of IPH. Since it is seen rarely in adults and the clinical course of the disease vary from patient to patient we presented an adult male patient with IPH responded well to steroid therapy clinically and radiologically.     

Approach to patients with heart failure and pulmonary hypertension

Opinion statement Pulmonary hypertension (PH), defined as a mean pulmonary artery pressure greater than 25 mm Hg, is not a diagnosis, but rather the physiologic consequence of the interaction between pulmonary blood flow, pulmonary vascular impedance, and downstream pulmonary venous pressure. The diagnosis and appropriate treatment of PH in patients with or without heart failure (HF) requires an understanding of the underlying pathogenesis, whether it be due to increased pulmonary venous pressure, increased pulmonary vascular resistance (PVR), increased pulmonary blood flow, or a combination thereof. Furthermore, an explanation for the underlying cause must also be sought. For example, a rise in pulmonary venous pressure may relate primarily to an increase in left ventricular end-diastolic pressure in a patient with a known cardiomyopathy; however, it may be complicated by severe mitral regurgitation. Similarly, an increased PVR may reflect reactive changes in the pulmonary vasculature due to long-standing pulmonary venous hypertension, concomitant hypoxemia/hypercapnia, or it may be the harbinger of chronic thromboembolic disease. It is imperative that reversible causes of PH be considered. Although most often diagnosed by Doppler echocardiography, full hemodynamic characterization of PH requires right heart catheterization to measure biventricular filling pressures and PVR. Integration of invasive pulmonary hemodynamics with an assessment of right ventricular function is essential to appreciate the clinical and prognostic significance of PH of an individual patient. Right heart catheterization is not practically feasible in all patients with HF and PH; however, at a minimum it should be performed in patients with a Doppler-estimated pulmonary artery pressure greater than 60 mm Hg, those who present clinically with predominant right HF, significant mitral valve disease, and in particular, patients with impaired right ventricular function.     

Effects of hydralazine on hemodynamics, ventilation, and gas exchange in patients with chronic obstructive pulmonary disease and pulmonary hypertension

Reports on hemodynamic effects of hydralazine on pulmonary hypertension (primary or secondary) usually include cases with severe disease or with mixed varieties of pulmonary vascular disease. Serious side effects and death have been reported. Effects of this drug on ventilation and gas exchange are unknown. We investigated the short-term effects of hydralazine treatment on hemodynamics, ventilation, and gas exchange in a relatively homogeneous group of patients with severe chronic obstructive pulmonary disease and moderate exertional pulmonary hypertension (mean pulmonary artery pressure, 43 +/- 3 mmHg). Hydralazine produced significant improvement in cardiac index, total pulmonary resistance, and oxygen transport. We also observed significant improvement in alveolar ventilation (mean PaCO2, decreased from 47 +/- 2 to 40 +/- 3 mmHg at rest and from 51 +/- 3 to 43 +/- 3 mmHg during exercise). The severe exertional hypoxemia of the group (mean PaO2, 48 +/- 2 mmHg) improved significantly (mean PaO2, 57 +/- 3 mmHg). Four of 11 patients showed increased exercise tolerance after hydralazine. This change is probably related to a combined improvement in hemodynamics plus a newly observed improvement in gas exchange and ventilation. Three of 14 patients could not tolerate hydralazine because of marked tachycardia. Serious side effects were not observed in the remaining group.     

Segmental wall motion abnormalities in an individual with idiopathic pulmonary hemosiderosis

Idiopathic pulmonary hemosiderosis (IPH) is a rare condition characterized by diffuse pulmonary hemorrhage of unknown etiology. Cardiac involvement in the form of myocarditis and right ventricular hypertrophy have been reported to occur in association with IPH, although findings on echocardiography have not been described. Herein is presented a case of an adult with IPH and echocardiographic abnormalities.     

Normal pulmonary capillary blood volume in patients with chronic infiltrative lung disease and high pulmonary artery pressure

STUDY OBJECTIVES: Pulmonary capillary blood volume (Qc), a component of diffusing capacity of the lung for carbon monoxide (Dlco), is increased in postcapillary pulmonary hypertension due to valve disease, but is decreased in primitive and thromboembolic pulmonary hypertension. This study was performed to evaluate which way pulmonary Qc is affected in patients with chronic infiltrative lung disease according to the value of systolic pulmonary artery pressure (SPAP). PATIENTS AND METHODS: Twenty-four patients who were nonsmokers and had chronic infiltrative lung disease secondary to connective tissue disease (12 patients), asbestosis (1 patient), sarcoidosis (5 patients), or of unknown origin (6 patients), and 8 control subjects underwent pulmonary function tests and Doppler echocardiography. MEASUREMENTS AND RESULTS: Total lung capacity, alveolar-arterial oxygen pressure difference, Dlco, and conductance of the alveolar-capillary membrane (Dm) did not differ between patients with low SPAP (LPAP) [ie, < 30 mm Hg] or high SPAP (HPAP). Patients with LPAP, but not HPAP, experienced significant decreases in pulmonary Qc, whatever the cause of the disease. There was a strong positive correlation between SPAP and Qc scaled by Dm to account for infiltrative disease severity (r = 0.68; p < 0.001). CONCLUSIONS: We thus conclude that pulmonary Qc is not decreased as expected in patients with chronic infiltrative lung disease and high pulmonary artery pressure. A high Qc/Dm ratio should encourage the physician to look for HPAP compatible with pulmonary hypertension, whatever the etiology of lung infiltrative disease.