Pulmonary hypertension in patients with pulmonary fibrosis awaiting lung transplant.

Pulmonary hypertension (PH) may complicate idiopathic pulmonary fibrosis (IPF) but the prevalence of PH in IPF remains undefined. The present authors sought to describe the prevalence of PH in IPF. The lung transplant registry for the USA (January 1995 to June 2004) was analysed and IPF patients who had undergone right heart catheterisation (RHC) were identified. PH was defined as a mean pulmonary arterial pressure ((Ppa)) > or =25 mmHg and severe PH as a (Ppa) >40 mmHg. Independent factors associated with PH were determined. Of the 3,457 persons listed, 2,525 (73.0%) had undergone RHC. PH affected 46.1% of subjects; approximately 9% had severe PH. Variables independently associated with mild-to-moderate PH were as follows: need for oxygen, pulmonary capillary wedge pressure (P(pcw)) and forced expiratory volume in one second (FEV(1)). Independent factors related to severe PH included the following: carbon dioxide tension, age, FEV(1), P(pcw), need for oxygen and ethnicity. A sensitivity analysis in subjects with P(pcw) <15 mmHg did not appreciably alter the present findings. Pulmonary hypertension is common in idiopathic pulmonary fibrosis patients awaiting lung transplant, but the elevations in mean pulmonary arterial pressure are moderate. Lung volumes alone do not explain the pulmonary hypertension. Given the prevalence of pulmonary hypertension and its relationship with surrogate markers for quality of life (e.g. activities of daily living), future trials of therapies for this may be warranted.     

Pulmonary hypertension and pulmonary function testing in idiopathic pulmonary fibrosis

BACKGROUND: Pulmonary hypertension (PH) is commonly seen in patients with idiopathic pulmonary fibrosis (IPF). We sought to examine the relationship between pulmonary function tests (PFTs), including the percentage of predicted FVC (FVC%), percentage of predicted total lung capacity, percentage of predicted diffusing capacity of the lung for carbon monoxide (Dlco%), the composite physiologic index (CPI), and PH. The ability of FVC%, Dlco%, and FVC%/Dlco% ratio to predict underlying PH was assessed. METHODS: Retrospective review of IPF patients seen at a tertiary referral center over an 8-year interval in whom both PFT and right-heart catheterization data were available. RESULTS: The study cohort consisted of 118 patients, of whom 48 patients (40.7%) had PH. There was no correlation between measures of lung volumes or the CPI with underlying PH. There was a modest association between Dlco% and PH, with Dlco% < 30 having a twofold-higher prevalence of PH (56.4%) compared to Dlco% >/= 30 (28.6%). Cardiac dysfunction might have played a small role, since 16.1% of the patients had an associated elevated pulmonary capillary wedge pressure. There was a trend to a higher prevalence and greater severity of PH in those patients with FVC% > 70 compared to the group with FVC% < 40. CONCLUSION: PH is common in patients with IPF. There is a poor correlation between lung function measures and PH, suggesting that factors other than fibrosis may play a role in the etiology. The unexpected high prevalence and severity of PH in patients with well-maintained lung function have implications for the prognosis and management of the disease.     

Extremely thickened media of small pulmonary arteries in fatal pulmonary hypertension with congenital heart disease--a morphometric and clinicopathological study

There are patients with congenital heart disease and fatal pulmonary hypertension in whom the medial hypertrophy of the small pulmonary arteries is quite beyond the extent of ordinary cases of hypertension, a condition described as pulmonary hypertension with extremely thickened media of small pulmonary arteries (PH/ETM). Lungs from 6 infants, all younger than 2 years of age, who had congenital heart disease and fatal pulmonary hypertension, were analyzed by accurately measuring the media using Suwa's method. In PH/ETM, the media of the small pulmonary arteries was shown to be not only unusually thick, but extending toward the periphery, whereas the intimal changes were unexpectedly mild. In the PH/ETM group, the % wall thickness at a diameter of 50 microm (%Tw(50)), determined from regression analysis, was 23.2+/-1.3%, which was significantly higher than in either the control (10.3+/-1.2%) or ventricular septal defect group (18.9+/-1.6%). In persistent pulmonary hypertension of the newborn (PPHN), it was 22.3+/-1.8%, not significantly different from PH/ETM. The striking medial hypertrophy in PH/ETM and PPHN was apparently confined to small pulmonary arteries and in both conditions is likely to be the result of maldevelopment of these arteries. Surgical intervention may trigger a critical elevation of the pulmonary arterial resistance.     

Prevalence of pulmonary hypertension and its association with respiratory disturbances in obese patients living at moderately high altitude

OBJECTIVE: To determine the point prevalence of pulmonary hypertension (PH) and its relationship with respiratory disturbances in obese patients living at moderate altitude. SUBJECTS: A total of 57 obese patients comprised the final sample and consisted of 34 women and 23 men, with a mean age of 42.7+/-12.1 ys and a mean body mass index (BMI) 47.1+/-10.6 kg/m(2) (range from 30.1 to 76.1). The mean living altitude was 2248.7 m, range 2100-2400 m above sea level. MEASUREMENTS: Doppler echocardiography, pulmonary function tests, arterial blood gas analysis, and polysomnography were performed. RESULTS: Data showed that 96.5% of the studied sample had daytime PH defined as calculated systolic pulmonary artery pressure (PSAP) >30 mmHg (mean PSAP=50, s.d.=13 mmHg). The severity of diurnal PH was found to be related to the presence of alveolar hypoventilation and BMI. The main risk factor for severity of diurnal PH was hypoventilation with a significant odds ratio (OR) 7.96, 95% CI 1.35-46.84, BMI was (OR 1.12, 95% CI 1.02-1.25) and apnea/hypopnea index was not a predictor of pulmonary hypertension severity (OR 0.99, 95% CI 0.97-1.02). CONCLUSION: We concluded that prevalence of diurnal PH is high in obese patients living at moderate altitude, and that hypoventilation is the main risk factor associated with the severity of pulmonary hypertension.     

Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension

RATIONALE: Although pulmonary hypertension (PH) is a common complication of sickle cell disease (SCD) associated with high mortality, there exist few data characterizing hemodynamics and cardiopulmonary function in this population. OBJECTIVES: To characterize hemodynamics and cardiopulmonary function in patients with SCD with and without PH. METHODS: Patients with SCD with PH (n = 26) were compared with control subjects with SCD but without PH (n = 17), matched for age, hemoglobin levels, and fetal hemoglobin levels. MEASUREMENTS AND MAIN RESULTS: Upon catheterization, 54% of the patients with PH had pulmonary arterial hypertension, and 46% had pulmonary venous hypertension. When compared with control subjects, patients with PH exhibited lower six-minute-walk distance (435 +/- 31 vs. 320 +/- 20 m, p = 0.002) and oxygen consumption (50 +/- 3% vs. 41 +/- 2% of predicted, p = 0.02), and also had mild restrictive lung disease and more perfusion abnormalities on radionuclide lung scans. The six-minute-walk distance in this population inversely correlated with tricuspid regurgitant jet velocity (r = -0.55, p < 0.001), and mean pulmonary artery pressure (r = -0.57, p < 0.001), and directly correlated with maximal oxygen consumption (r = 0.49, p = 0.004), even after adjustment for hemoglobin, supporting an independent contribution of increasing pulmonary artery pressures to loss of exercise capacity. CONCLUSIONS: Patients with SCD-associated PH have both pulmonary arterial and venous PH associated with severe limitations in exercise capacity, likely compounded by interstitial lung fibrosis and severe anemia. These data support the use of the six-minute-walk distance as an index of PH and cardiopulmonary function in patients with SCD.     

High prevalence of hypothyroidism in patients with primary pulmonary hypertension.

PURPOSE: To characterize the prevalence of hypothyroidism in a population with primary pulmonary hypertension (PPH). METHODS: Retrospective record review of 41 patients with PPH seen between 1991 and 1997 at a tertiary care center. Data abstracted included: history of previous thyroid disease, intake of thyroid supplement, and thyroid function tests. Hypothyroidism was defined as a serum thyroid stimulating hormone (TSH) level higher than 5.5 U/L, intake of thyroid supplement, or low serum thyroxine level. RESULTS: Of the 40 patients with PPH included in the study (11 men and 29 women), ages ranged from 11 to 76 years (mean 43.5 years). The mean pulmonary artery pressure was 58.7 mm Hg. Thirty-three patients had normal serum TSH levels (3 of whom were on levothyroxine supplement); 1 had low TSH; 5 had high TSH (range, 6.8-9.9 U/L, mean 8.4 U/L), and 1 had low thyroxine (T4 < 1.0 microg/dL). Nine of 40 patients (22.5%) had evidence of hypothyroidism, which is much more than expected in the general population of similar age range (2.8% in men, 7.5% in women) (p = 0.002). CONCLUSIONS: The prevalence of hypothyroidism in patients with PPH is high (22.5%). Patients with PPH should be investigated for the possibility of coexisting hypothyroidism.     

Plasma markers of endothelial dysfunction in pulmonary hypertension

STUDY OBJECTIVES: To evaluate and to correlate endothelial cell dysfunction, using recently available plasma markers, with the magnitude of pulmonary artery pressure in patients with severe pulmonary hypertension (PH). DESIGN: Selected plasma markers of endothelial cell dysfunction were studied: nitric oxide (NO), thrombomodulin, tissue factor pathway inhibitor, and soluble endothelium, leukocyte, and platelet selectins (sE-, sL-, sP-selectins, respectively). SETTING: Padova University Hospital and Department of Pathology and Pharmacology, Loyola University of Chicago, Chicago, IL. PATIENTS: Fifteen patients had severe PH (four men and 11 women; mean age, 49.7 +/- 2.9 years: seven patients had primary pulmonary hypertension [PPH] and eight patients had secondary pulmonary hypertension [SPH]), and 20 patients were healthy control subjects. Measurement and results: In patients with PH, sP- and sE-selectins were elevated, whereas sL-selectin was lower in comparison with the selectin levels in control subjects. However, the differences between patients with PH and control subjects were significant only for sL-selectin (p < 0.0001) and sE-selectin (p < 0.03). The NO level was significantly lower in patients with PH compared with the NO level in control subjects (p < 0.01). No difference in tissue factor pathway inhibitor level was noted between control subjects and patients with PH. Only a weak correlation was found between thrombomodulin plasma levels and magnitude of systolic pulmonary artery pressure (r = -0.528, p < 0.05). CONCLUSIONS: Our data are in keeping with the evidence for significant endothelial cell dysfunction in patients with PH and the need for chronic anticoagulation believed to increase survival in these patients. In addition, these data seem to suggest a need for newer agents that are able to increase the antithrombotic endothelial function.     

Pulmonary hypertension in patients with chronic renal failure: role of parathyroid hormone and pulmonary artery calcifications

RATIONALE: The aim of this work was to study the association of pulmonary hypertension (PH) with pulmonary artery calcifications (PACs) and hyperparathyroidism in patients with chronic renal failure (CRF) receiving regular hemodialysis. BACKGROUND: Scarce data are available regarding the prevalence and the predictors of PH in patients with CRF. Abnormal (99m)Tc diphosphonate lung uptake was reported in these patients, suggesting a role of PACs. METHODS: We studied 51 patients (28 men and 23 women) with end-stage renal disease, who were receiving regular hemodialysis. Patients underwent two-dimensional, Doppler echocardiographic imaging. Laboratory investigations included BUN, serum creatinine, calcium, phosphorus, alkaline phosphatase, and intact molecule parathormone. PH was defined as pulmonary artery systolic pressure > 35 mm Hg as determined by Doppler echocardiographic evaluation. RESULTS: PH was detected in 15 patients (29%). Women had a higher prevalence of PH (48% vs 14%, p = 0.01). There was no significant differences between patients with PH and those without PH with regards to age, duration of dialysis, serum calcium (9.6 +/- 2 mg/dL vs 10 +/- 2 mg/dL), phosphorus (6 +/- 1.4 mg/L vs 6.2 +/- 1.9 mg/L), alkaline phosphatase (609 +/- 768 U/L vs 473 +/- 574 U/L), parathyroid hormone (PTH) [420 +/- 512 pg/mL vs 354 +/- 519 pg/mL] or the prevalence of an abnormal (99m)Tc diphosphate lung scan result (60% vs 73%, respectively [+/- SD]). CONCLUSIONS: This study demonstrated that 29% of patients with CRF receiving regular hemodialysis have PH. The presence of PH was not related to the level of PTH or the severity of other metabolic abnormalities. There was no relation between PH and the presence or the severity of PAC. PH is detected more frequently in women. This study does not support a role of secondary hyperparathyroidism and subsequent PAC as the etiology of PH in patients with CRF.     

选择性肺动脉造影评价先天性心脏病肺动脉高压的初步分析

通过选择性肺动脉造影检查，了解肺血管病变程度。对４３例先天性左向右分流型心脏病病人行选择性肺动脉造影检查，通过分析肺循环时间、肺动脉终末单支血管计数、肺动脉分级、毛细血管像等指标，并与右心导管（４３例）和肺病理（７例）结果进行比较分析。结果显示：随着肺血管病变加重，肺循环时间延长；肺动脉终末单支血管计数、肺动脉分级减少；肺毛细血管象分布不均。结论：选择性肺动脉造影检查可以比较准确地判定肺血管病变程度。     

N-Terminal Natriuretic Peptide and Ventilation-Perfusion Lung Scan in Sickle Cell Disease and Thalassemia Patients with Pulmonary Hypertension

The aim of this study was to determine the prevalence of pulmonary hypertension (PH) in sickle cell disease and thalassemia patients in relation to clinical and laboratory parameters of hemolysis and hemosidersosis, as well as plasma N-terminal pro-brain natriuretic peptide (NT-pro-BNP). The study also aimed to define the role of thromboembolic pulmonary artery (PA) obstruction in its etiology. Forty sickle cell disease and 30 thalassemia patients [15 β-thalassemia major (β-TM) and 15 β-thalassemia intermedia (β-TI)] were screened for PH defined as tricuspid regurgitant velocity (TRV) >2.5 m/sec and evaluated for PA obstruction using ventilation-perfusion lung scan (V/Q), together with measurement of their plasma levels of NT-pro-BNP. Patients were prospectively followed up for a mean of 18 ± 6.1 months. The prevalence of PH was 37.5, 40.0 and 26.7% in sickle cell disease, β-TI and β-TM patients, respectively. Pulmonary hypertension patients were older, had longer disease duration, higher serum ferritin, serum lactate dehydrogenase (LDH) and NT-pro-BNP with lower hemoglobin (Hb) levels compared to patients without PH. N-terminal pro-BNP was positively correlated with duration of illness, TRV, LDH, serum ferritin, and negatively correlated with Hb levels. The strongest predictor for TRV was serum ferritin followed by the NT-pro-BNP level. Forty-six-point-seven percent of sickle cell disease patients with PH had either high or intermediate probability V/Q scan results compared to 10% of thalassemic patients with PH who had high probability V/Q scan results. Pulmonary hypertension is highly prevalent in young sickle cell disease and thalassemia patients, where elevated serum ferritin and NT-pro-BNP are the main indicators.     

重叠综合征患者血浆内皮肽1水平与睡眠呼吸参数的相关性

目的:研究重叠综合征(OS)患者血浆内皮肽1(ET-1)水平,探讨其与OS合并肺动脉高压(PH)的相关性以及OS合并PH的危险因素。方法:选取吸烟男性患者155例,其中正常对照组30例,阻塞性睡眠呼吸暂停低通气综合征(OSAHS)组45例,慢性阻塞性肺疾病(COPD)组40例,OS组40例。记录年龄、体质量指数(BMI)等临床参数,测定第1秒用力呼气容积(FEV1)占预计值百分比(FEV1%)及呼吸暂停低通气指数(AHI)、脉搏血氧饱和度(SpO2)120 ng/L者合并PH的相对危险度是120 ng/L)可能是OS合并PH的危险因素。     

肺动脉高压合并甲状腺功能异常临床特点分析

目的:分析肺动脉高压合并甲状腺功能异常患者的临床特点。方法:回顾首都医科大学附属北京安贞医院2018年1月至2018年12月,就诊住院的肺动脉高压患者52例,分为肺动脉高压不合并甲状腺功能异常组(n=22),肺动脉高压合并甲状腺功能异常组(n=30),比较两组患者在临床资料、超声心动图参数、实验室检查等方面的差异。结果:两组患者在年龄、性别、BMI、病因、NYHA分级等方面,差异均无统计学意义(P>0.05)。与不合并甲状腺功能异常相比,肺动脉高压合并甲状腺功能异常患者的吸烟指数高,BNP水平、超声估测的肺动脉收缩压力较高(P<0.05),而LVEF、血气分析氧分压低于不合并甲状腺功能异常组。结论:肺动脉高压合并甲状腺功能异常患者较不合并患者的临床指标偏重,常规检测甲状腺功能可能对肺动脉高压患者病情进行早期预警。     

Natural history of mild-moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma

OBJECTIVE: To determine risk factors for developing pulmonary hypertension (PH) in patients with scleroderma (SSc, systemic sclerosis). METHODS: We used a cohort of 1136 SSc patients using severe PH as the primary outcome in a natural history study. RESULTS: Among 361 individuals with no initial echocardiographic PH, 92 (26.0%) developed mild-moderate PH and 48 (13.6%) severe PH. Patients developing severe PH had lower initial DLCO (48.8% of predicted) than those who did not develop PH (56.8% of predicted). Patients with mild-moderate PH had a 17% probability of progressing to severe PH, and 15.6% probability of regressing to no PH. Individuals with limited disease, mild-moderate PH, and age > or= 47 years at diagnosis had a 27.3% probability of developing severe PH, compared to 8.5% in individuals with diffuse disease, no evidence of PH, and age < 47 years at diagnosis. Longitudinal regression models estimated that individuals with limited disease, mild-moderate PH, and DLCO < 50% predicted had an age-adjusted odds ratio of 8.6 of developing severe PH within 2 years compared to individuals without these risk factors. CONCLUSION: Development of severe PH is uncommon in certain subgroups of SSc patients. Risk factors for progression of PH include older age, limited skin disease, and elevated pulmonary artery pressures at the time of initial evaluation.     

Pulmonary hypertension in sickle cell disease

BACKGROUND: Recurrent vaso-occlusive episodes lead to progressive end-organ damage in patients with sickle cell disease. We sought to determine the prevalence of pulmonary hypertension in adult patients with sickle cell disease and to identify factors associated with this life-threatening complication. METHODS: Sixty patients (> or =18 years of age; mean [+/- SD] age, 37 +/- 13 years) followed at a University Medical Center were evaluated. They were selected by a systematic sampling of patients presenting to the clinic for routine follow-up visits. All enrolled subjects underwent a clinical examination, Doppler echocardiography, pulmonary function tests, and hematologic tests during a single visit. Pulmonary hypertension was defined using an age- and body mass index-adjusted nomogram. RESULTS: The prevalence of pulmonary hypertension was 30% (18/60). Ten patients had mild pulmonary hypertension (up to 44 mm Hg), 5 had moderate pulmonary hypertension (45 to 74 mm Hg), and 2 had severe pulmonary hypertension (> or =75 mm Hg). In a logistic regression model, both lower fetal hemoglobin level and lower systolic blood pressure were associated with the presence of pulmonary hypertension. CONCLUSION: We found that the prevalence of pulmonary hypertension in adult patients with sickle cell disease was substantial, particularly in those with lower levels of fetal hemoglobin and lower systolic blood pressure.     

Prevalence of thyroid disease in an older Australian population

Aim: To determine the prevalence of thyroid disease in an older Australian population in a population‐based cross‐sectional study. Background: Community‐living subjects, aged 49 years or older, in two Blue Mountains postcodes were invited to participate in an eye, nutrition and health study between 1997 and 2000. Methods: Three thousand five hundred and nine of the 4489 identified persons participated. Fifty‐seven per cent of 3504 who completed questionnaires were women; their mean age was 66.8 years. Thyroid‐stimulating hormone (TSH) was measured in 2665 subjects (76% of those completing the questionnaire). The main outcome measures were serum TSH and free thyroxine levels, serum lipids, urate and sugar levels and questionnaire responses. Results: The prevalence of recognized thyroid disease (either self‐reported history of thyroid disease or current thyroxine treatment) was 10% (95% confidence interval (CI) 8.9–11.1%). An additional 3.6% (95%CI 2.9–4.3%) of participants had unrecognized thyroid disease (abnormal TSH). The TSH was abnormal in 7.1% (95%CI 5.8–8.4%) of women and 3.7% (95%CI 2.6–4.8%) of men. Sixty‐five per cent of those with an abnormal TSH did not report a history of thyroid disease, whereas 25% of those taking thyroxine replacement therapy had an abnormal TSH level. The prevalence of hypothyroidism increased with increasing age in women. The mean fasting cholesterol was 0.36 mmol/L (95%CI 0.15–0.57) higher in hypothyroid subjects than in euthyroid subjects. Conclusion: Thyroid disease in older Australian women is relatively common and may be undiagnosed. Ongoing monitoring of patients on thyroxine replacement therapy is important, given that 25% of treated patients had an abnormal TSH.     

Pulmonary hypertension in an adult sickle cell population in Guadeloupe

Pulmonary hypertension (PH) is a severe complication of sickle cell disease (SCD). The main purpose of this study was to evaluate the prevalence of PH in an adult SCD population in Guadeloupe. Echocardiography findings of 427 patients with SCD were analyzed. Subjects with a pulmonary artery systolic pressure (PASP) >30 mm Hg, at rest, were studied. PH was found in 49 (11.5%) SCD patients. Mean age was 37 years and mean PASP was 40 mm Hg. The most frequent symptom was exertional dyspnea. Thirteen subjects (26%) had left heart diseases. In the overall study population, 22 (5%) patients died. Seven (14%) patients had PH and 15 (4%) were PH free, p = 0.006. An early PH screening is recommended in SCD patients at steady state owing to the absence of specific symptoms and to the significant mortality rate related to this complication.     

Epidemiology of pulmonary hypertension: new data from the Swiss registry

BACKGROUND: since 1999 data from pulmonary hypertension (PH) patients from all PH centres in Switzerland were prospectively collected. We analyse the epidemiological aspects of these data. METHODS: PH was defined as a mean pulmonary artery pressure of >25 mm Hg at rest or >30 mm Hg during exercise. Patients with pulmonary arterial hypertension (PAH), PH associated with lung diseases, PH due to chronic thrombotic and/or embolic disease (CTEPH), or PH due to miscellaneous disorders were registered. Data from adult patients included between January 1999 and December 2004 were analysed. RESULTS: 250 patients were registered (age 58 +/- 16 years, 104 (41%) males). 152 patients (61%) had PAH, 73 (29%) had CTEPH and 18 (7%) had PH associated with lung disease. Patients <50 years (32%) were more likely to have PAH than patients >50 years (76% vs. 53%, p <0.005). Twenty-four patients (10%) were lost to followup, 58 patients (26%) died and 150 (66%) survived without transplantation or thrombendarterectomy. Survivors differed from patients who died in the baseline six-minute walking distance (400 m [300-459] vs. 273 m [174-415]), the functional impairment (NYHA class III/IV 86% vs. 98%), mixed venous saturation (63% [57-68] vs. 56% [50-61]) and right atrial pressure (7 mm Hg [4-11] vs. 11 mm Hg [4-18]). DISCUSSION: PH is a disease affecting adults of all ages. The management of these patients in specialised centres guarantees a high quality of care. Analysis of the registry data could be an instrument for quality control and might help identify weak points in assessment and treatment of these patients.     

60岁以上老年人肺动脉高压病因学研究

目的 回顾性分析老年人肺动脉高压（pulmonary hypertension,PH）的病因,以改善PH的综合管理.方法 回顾性分析2012年1月-2014年1月在上海长海医院就诊住院的年龄大于60岁的患者,超声心动图检查提示肺动脉高压者,按病因分组进行统计,对不同疾病导致的经超声心动图三尖瓣返流速度估测的肺动脉收缩压进行比较分析.结果 资料完整的60岁以上PH病例数共540例,男278例,女262例,平均年龄（74.2±8.4）岁,中位年龄70岁.其中无特发性PAH=PH,均为疾病相关性,其中心源性共287例（扩心病19例,肥厚性心肌病8例）;先心病共27例.肺脏疾病相关性PAH为31例.瓣膜疾病相关性PAH、先心病相关性PAH及结缔组织相关性PAH=PH肺动脉收缩压明显高于心肌病及高血压引起的PAH=PH.按欧洲心脏协会2009年分类标准中属于第一组动脉型肺动脉高压为30例,占5％,左心疾病相关性PH为351例,占65％;第三组为肺疾病或/和缺氧相关性PH,为31例,占6％.其中第一组患者的肺动脉收缩压明显高于其他组.按照肺动脉收缩压升高程度来分,主要为中度升高.结论老年人PAH=PH主要是疾病相关性,最主要导致PH的疾病是左心疾病相关性.     

先天性心脏病伴肺动脉高压患者双源CT肺血管影面积百分比与肺小动脉形态变化的关系

目的:探讨先天性心脏病伴肺动脉高压(PH)患者双源CT肺血管影面积百分比［EA(%)=右肺层面中血管影面积和/层面中右肺总面积］与肺小动脉形态变化的关系。方法: 随机选取2009年1月~12月收治92例左向右分流先天性心脏病患者,按肺动脉压力情况分为3组,先心病不并发PH组（对照组）29例,肺动脉平均压(MPAP)<20 mmHg;先心病并发PH组(PH组)55例, 80 mmHg>MPAP>20 mmHg;先心病并发紫绀组8例(紫绀组),MPAP>90 mmHg。术前行肺血管双源CT,测定EA(%)值。取肺组织进行活检,观察肺血管病理变化及肺小动脉形态学变化指标。结果: 肺活检切片按Heath和Edwards标准进行病理分级,无肺高压对照组29例,Ⅰ级11例,Ⅱ级16例,Ⅲ级19例,Ⅳ级9例;紫绀组(≥V级)8例。对照组EA(%)为9.37±0.13,;肺小动脉血管壁厚度占血管外径的百分比WT(%)为15.27±1.98,血管壁面积占血管总面积百分比WA(%)为21.13±4.30。参照对照组各项检查指标,随病理分级的增加,双源CT肺血管影EA(%)呈下降趋势,显影的肺血管面积占肺总面积比例减少; WT(%)及WA(%)均随病理分级增加呈上升趋势,各组之间有显著性差异（P<0.01）,一定程度上反映了病理变化程度越重,肺血管壁增生、管腔萎缩越重。结论: 肺高压患者双源CT肺血管影EA(%)的变化在一定程度上能够反映肺动脉高压患者肺血管病理的变化。     

Prevalence of thyroid abnormalities in patients with dermatitis herpetiformis and in control subjects with HLA-B8/-DR3

PURPOSE: The prevalence of thyroid dysfunction as measured by the presence of overt thyroid disease, abnormal results of thyroid function tests, or antithyroid antibodies was compared in patients with dermatitis herpetiformis (DH) and a normal control group who had the HLA-B8/-DR3 haplotype. PATIENTS AND METHODS: The study population consisted of 56 patients with DH and 26 control subjects with the HLA-B8/-DR3 haplotype. All were examined for thyroid function abnormalities and thyroid autoantibodies. RESULTS: Patients with DH had a statistically significant increase in the prevalence of abnormal thyroid function test results and autoantibodies: 32% versus 4% for controls (Z = 2.01, p less than 0.02). In patients with DH, hypothyroidism was the most common thyroid abnormality (12 of 56) followed by hyperthyroidism (four of 56). Two patients had normal thyroid function test results with thyroid autoantibodies. Risk factors for thyroid abnormalities in patients with DH were increasing age (chi 2 = 6.55, p less than 0.02, significant) and the presence of thyroid microsomal antibodies. The HLA-B8/-DR3 haplotype was not a risk factor for thyroid abnormalities. CONCLUSION: The findings suggest that thyroid disease is independently associated with DH. Examination of patients with DH should include thyroid function tests along with assays for antithyroid antibodies.