5型磷酸二酯酶抑制剂改善特发性肺动脉高压患者预后

目的:探讨5型磷酸二酯酶(PDE5)抑制剂对特发性肺动脉高压(IPAH)患者预后的影响.方法:纳入2005-05至2009-08期间在我院本部肺血管病诊治中心住院并新诊断为IPAH的患者89例.根据患者是否接受PDE5抑制剂分为PDE5抑制剂组(52例)及对照组(37例).通过回顾性查阅病历收集患者的基线资料,申话随访和(或)查阅患者门诊病历获取患者生存状态及治疗情况.结果:PDE5抑制剂组及对照组在年龄、性别、身高、体重、血压、心率、世界卫生组织(WHO)肺动脉高压功能分级、6分钟步行距离、血流动力学及接受基础治疗比例,差异均无统计学意义(P均>0.05).对89例患者平均随访(20.12±14.69)月,随访期间26例患者死亡.PDFA抑制剂组1、2、3年生存率明显高于对照组,差异有统计学意义(P<0.05).WHO肺动脉高压功能分级为III/IV级的患者,PDE5抑制剂组1、2、3年生存率明显高于对照组,差异有统计学意义(P<0.05);WHO肺动脉高压功能分级为I/Ⅱ级的患者,PDE5抑制剂组1、2、3年生存率较对照组有改善的趋势,但差异无统计学意义(P>0.05).当未包括6分钟步行距离时,84例患者多因素COX分析结果显示:体重(风险比=0.944,P=0.047),混合静脉血氧饱和度(风险比=0.934,P=0.006),接受PDE5抑制剂治疗(风险比=0.314,P=0.006)三参数将有助于评估IPAH患者的预后.当纳入6分钟步行距离时(n=41),多因素COX分析显示年龄(风险比=0.860,P=0.004)、6分钟步行距离(风险比=0.984,P=0.001)及接受PDE5抑制剂治疗(风险比=0.072,P=0.001)三参数将有助于评估IPAH患者的预后.结论:PDE5抑制剂对改善IPAH患者的预后有益.     

Idiopathic Pulmonary Arterial Hypertension Misdiagnosed as Asthma

Idiopathic pulmonary arterial hypertension (IPAH) is a rare disorder that is progressive and often leads to right heart failure if left untreated. Because of the vague nature of symptoms at presentation, IPAH may take several months to diagnose. The most common presenting complaint in patients with IPAH is dyspnea with exertion, which is also commonly seen with asthma. This report describes an adult female with refractory exertional dyspnea who was diagnosed with asthma 2 years earlier but was ultimately diagnosed with IPAH. Exclusion of other etiologies is a necessity for refractory dyspnea in the setting of asthma.     

特发性肺动脉高压的诊断与治疗

特发性肺动脉高压(Idiopathic Pulmonary ArterialHypertension,IPAH)是指没有明确原因呈单克隆细胞增生和致丛性病变的肺动脉高压.早期诊断和及时治疗有助于改善IPAH患者的预后,因此综合分析患者的个人史、家族史、临床表现、体格检查和实验室检查,进行规范的IPAH诊断评估,进而明确诊断和规范治疗,对于提高IPAH患者的生存期和改善生活质量具有十分重要的意义.     

Pulmonary Artery Pulse Wave Velocity in Idiopathic Pulmonary Arterial Hypertension

Background

Idiopathic pulmonary artery (PA) hypertension (IPAH) is associated with severe PA remodelling. Although the resulting increase in pulse wave velocity (PWV) might be of major pathophysiological relevance, little is known about PA-PWV in IPAH. The aim of this study was to characterize PA-PWV and its predictors in patients with IPAH.

Methods

We studied 26 consecutive patients with incident IPAH aged 55.0 (45.0-66.0) years (62% female) and 10 control subjects without pulmonary hypertension. PA-PWV was measured invasively; PA wall thickness and diameter were assessed using intravascular and transthoracic ultrasonography.

Results

PA-PWV was higher in IPAH than in control subjects (10.0 [7.5-14.0] m/s vs 3.5 [1.9-4.0] m/s; P < 0.001) as was also PA diameter and PA wall thickness. In IPAH patients, in univariate analysis PA-PWV was greater in men than in women and in patients with body mass index (BMI) < 25 kg/m² than with BMI ≥ 25 kg/m² and correlated positively with symptomatic disease duration, mean PA pressure, pulmonary vascular resistance, creatinine level, and negatively with low-density lipoprotein (LDL) cholesterol and triglyceride level but not with PA diameter or PA wall thickness. In multiple regression analysis mean PA pressure, LDL cholesterol level and BMI < 25 kg/m² were the main predictors of PA-PWV in IPAH patients (R² = 77%; P < 0.001).

Conclusions

PA-PWV is increased in IPAH patients. High PA pressure, low LDL cholesterol level, and BMI < 25 kg/m² explain most of its variability in this group.     

特发性肺动脉高压患者血尿酸水平变化及其临床意义

<正>大量研究表明,慢性心功能衰竭[1],右向左分流先天性心脏病[2],和阻塞性肺病[3]等机体存在低氧状况时血尿酸水平增高,血尿酸水平已被证明是慢性心力衰竭病人远期死亡的独立危险因素[4]。特发性肺     

Left Ventricular Mass is Preserved in Patients with Idiopathic Pulmonary Arterial Hypertension and Eisenmenger's Syndrome

BackgroundLeft ventricular (LV) atrophic remodelling was described for chronic thromboembolic pulmonary hypertension (PH) but not in other forms of PH. We aimed to assess LV morphometric changes in idiopathic pulmonary arterial hypertension (IPAH) and Eisenmenger's syndrome(ES).MethodsFifteen patients with IPAH, 15 patients with ES and 15 healthy volunteers were included. Magnetic resonance was used to measure masses of LV, interventricular septum (IVS), LV free wall (LVFW), and LV end diastolic volume (LVEDV) indexed for body surface area.ResultsBetween patients with IPAH, ES and controls no differences in LVmass_index (54.4[45.2-63.3] vs 58.7[41.5-106.1] vs 52.8[46.5-59.3], p = 0.50), IVSmass_index (21.6[18.2-21.9)] vs 27.4[18.0-32.9] vs 20.7[18.2-23.2], p = 0.18), and LVFWmass_index ([32.4[27.1-40.0] vs 36.7[30.9-62.1] vs 32.5[26.9-36.1], p = 0.29) were found. LVEDV_index was lower in IPAH patients than in controls and in ES patients (54.9[46.9-58.5] vs 75.2[62.4-88.9] vs 73.5[62.1-77.5], p < 0.001). In IPAH LVEDV but not LV mass correlated with pulmonary vascular resistance (r = -0.56, p = 0.03) and cardiac output (r = 0.59, p = 0.02).ConclusionsLV mass is not reduced in patients with IPAH and with ES and is not affected by haemodynamic severity of PH. LVEDV is reduced in IPAH patients in proportion to reduced pulmonary flow but preserved in patients with ES, where reduced pulmonary flow to LV is compensated by right-to left shunt.     

The Use of Antidepressants and the Risk of Idiopathic Pulmonary Arterial Hypertension

BackgroundSerotonin has been implicated in the development of idiopathic pulmonary arterial hypertension (IPAH). Drugs modulating serotonin pathways, including antidepressants, have been associated with the incidence of IPAH, with conflicting reports as to the direction of the effect. We aimed to determine whether antidepressant exposure is associated with the incidence of IPAH.MethodsA nested case-control study was conducted using the United Kingdom Clinical Practice Research Datalink and the Hospital Episodes Statistics repository between January 1, 1988 and September 30, 2011. Incident cases of IPAH were identified and matched to all controls in the case's risk set on age, sex, general practice, and date of registration with the practice. Rate ratios (RRs) and 95% confidence intervals (CIs) were estimated for the use of antidepressants on the risk of IPAH, with an 18-month lag period before the diagnosis.ResultsOne hundred ninety-five IPAH cases were identified (incidence 3.84/million per year). Use of any antidepressant was associated with a 67% increased risk of IPAH (RR, 1.67; 95% CI, 1.17-2.37). The rate of IPAH was similar across antidepressant classes, whether with selective serotonin reuptake inhibitors (SSRIs) (RR, 1.67; 95% CI, 1.09-2.57) or non-SSRI antidepressants (RR, 1.66; 95% CI, 1.07-2.59). In sensitivity and exploratory analyses, no change in risk was observed with different lag times, serotonin transporter affinities, or durations of exposure.ConclusionsThe use of antidepressants was associated with a significantly increased risk of IPAH. However, the consistency of this risk across all antidepressants and absence of a dose-response relationship suggests a noncausal association.     

Pulmonary Hypertension or Pulmonary Arterial Hypertension in Idiopathic Pleuroparenchymal Fibroelastosis: An Updated Comprehensive Review

Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a little-known entity with unique clinical, radiological, and pathological features. iPPFE is chronic interstitial pneumonia characterized by the thickening of elastic fibers in the pleura and subpleural parenchyma involving the upper lobes. Computed tomography pulmonary angiography (CTPA) usually depicts bilateral pleural thickening, with a left scalloped appearance that conditions retraction of the structures of the superior mediastinum and both pulmonary hila, associated with pulmonary consolidations with bronchogram air and thickening of the peribronchovascular interstitium, in addition to areas of left apical air trapping. When severe enough, the disease leads to progressive loss of volume of the upper lobes, decreased body mass, and platythorax. Some patients with iPPFE follow an inexorably progressive course culminating in irreversible respiratory failure and premature death. Up to 20% of patients might develop pulmonary hypertension (PH); transthoracic echocardiography is used as a screening test for PH; right heart catheterization performed in a tertiary-care hospital will confirm the diagnosis. Because iPPFE can be easily confused and misdiagnosed with infectious pathologies, such as pulmonary tuberculosis, and easily confuse physicians with little expertise in diffuse interstitial lung diseases, knowing the differential diagnoses, clinical presentation, imaging, and complications of the iPPFE allows for an early diagnosis and gives patients who suffer from it a better quality of life. This report presents a comprehensive review of PPFEi, discussing severe precapillary pulmonary hypertension and the associated findings demonstrated by right heart catheterization (RHC), which be of interest for cardiopulmonologists.     

Association of HLA Class II Genes with Idiopathic Pulmonary Arterial Hypertension in Koreans

The aim of this study was to compare the frequency of the HLA-DRB1 and HLA-DQB1 alleles in Korean patients with idiopathic pulmonary arterial hypertension (IPAH) and in normal controls and to determine any association that may exist between clinical characteristics of IPAH and specific HLA alleles. IPAH patients seen between October 1998 and September 2001 were retrospectively assessed, and 19 patients and 193 controls were HLA typed at the HLA-DRB1 and DQB1 loci. Clinical characteristics and hemodynamic parameters were reviewed. The patients with IPAH had a significantly higher frequency of the HLA-DRB1*0406 allele (18% vs. 6%, p = 0.004) and the HLA-DQB1*0302 allele (24% vs. 12%, p = 0.034), as well as a significantly higher frequency of haplotype DRB1*0406-DQB1*0302 (p = 0.0006). All 6 patients with haplotype DRB1*0406-DQB1*0302 (H+ group) were women, compared with 8 of the 13 patients lacking the DRB1*0406-DQB1*0302 haplotype (H- group), but without statistical significance. Three of 19 patients showed a positive short-term hemodynamic response to NO inhalation, all 3 of whom had the DRB1*0406-DQB1*0302 haplotype. There were no other significant differences in clinical characteristics and hemodynamic parameters between the H+ and H- groups. We conclude from this study that the HLA-DRB1*0406-DQB1*0302 haplotype is associated with IPAH in Korean patients. These results suggest that certain clinical characteristics of IPAH may be controlled in part by patients' HLA alleles.