超声心动图测量肺动脉高压对急性肺栓塞患者的危险分层及预后的应用价值

目的研究超声心动图测量肺动脉高压(PAH)对急性肺栓塞(APE)患者的危险分层及预后的应用价值。方法选取2012年1月-2015年4月我院收治的APE患者64例,均接受右心导管检查和彩色多普勒超声心动图检查,比较不同危险分层APE患者肺动脉收缩压(PASP)及PAH的发生率,两种检查方法的血流动力学参数的相关性;将患者分为PAH组(30 mmHg)、正常组(≤30 mmHg),比较两组患者的临床症状及预后等。结果随着APE危险程度的增加,PASP、PAH发生率依次升高(P0.05);Pearson相关分析显示,超声心动图所得血流动力学参数mRAP、mPAP、PASP与右心导管呈显著正相关(P0.05);PAH组临床症状(咯血、下肢浮肿)、D-D水平、心电图异常的发生率显著高于正常组(P0.05)。结论超声心动图测量PAH可作为APE患者病情危险分层及预后评估的重要指标,尤其适用于疑似或待确诊APE患者的确诊、疗效评估及随访。     

经胸超声心动图和右心导管检测评估肺动脉高压患者血流动力学参数的比较

目的 探讨经胸超声心动图(TTE)和右心导管检查(RHC)评估肺动脉高压(PAH)患者血流动力学参数的价值.方法 选取2017年10月至2019年10月于河北医科大学第二医院心内科收治的PAH患者78例.根据肺动脉收缩压(sPAP)分为轻度28例,中度31例和重度19例.采用TTE和RHC测量PAH患者血流动力学参数,...     

西地那非治疗肺动脉高压的临床疗效观察

目的:评价口服西地那非治疗肺动脉高压(Pulmonary hypertension PH)的有效性及安全性。方法:对我院呼吸科2007年1月1日至2008年12月31日门诊及病房就诊的PH患者进行筛选,符合入组标准的23例患者,给予口服西地那非,共12 w;对患者的随访采取电话及预约的方法。主要终点为治疗12 w后6 min步行测试中行走距离的改变,次要终点为治疗12 w后Brog呼吸困难评分及WHO PH患者功能分级、心脏多普勒超声心动图测定的肺动脉收缩压(SPAP)、血气分析动脉血氧分压(PaO2)的改变,并随访患者血压、心率及生化指标。结果:1.经西地那非治疗后,主要终点:6 min步行距离从(296.78±118.77)m增加至(400.57±96.58)m(P<0.01),增加了(103.78±60.21)m。经西地那非治疗后,次要终点的Brog呼吸困难评分及WHO PH患者功能分级明显改善(P<0.01);多普勒超声心动图测定的肺动脉收缩压(SPAP)由基线时的(101.68±20.89)mmHg(1 mmHg=0.133 kPa)降低到(82.74±20.04)mmHg(P<0.01);降低了(18.94±15.48)mmHg。血气分析血氧分压(PaO2)由(62.68±7.92)mmHg增加至(71.48±6.30)mmHg(P<0.01)。2.不良反应:经随访观察,在服药期间,无患者出现血压下降,治疗前后心率、血压及肝、肾功能比较差异均无统计学意义(P>0.05)。结论:1.口服西地那非可以显著改善患者的活动耐量、临床症状及WHO PH患者功能分级和血流动力学参数,且在治疗过程中,无患者出现临床病情恶化,证实了其治疗PH有效性。2.口服西地那非治疗后,未发现血压下降及严重的药物副作用,证实了其治疗PH的安全性。     

COPD伴肺动脉高压患者吸入不同浓度氧时血管和心脏的反应性

慢性缺氧性肺血管收缩是COPD患者发生肺动脉高压(PAH)的主要原因。研究旨在通过测定不同氧吸入分数(FiO_2)时的血流动力学,探讨COPD伴PAH患者的急性肺血管反应性。患者和方法 COPD伴PAH男患者26例,平均年龄63±2岁,平均肺动脉压(MPAP)3.63±0.13kPa。均处于临床稳定状态,无左心室功能不全证据。行右心导管检查,分别于基础状态以及通过面罩吸入100％、15％、21％和30％浓度氧各20min后测定动脉血气和血流动力学参数。     

超声检查在肺动脉高压诊疗中可靠性的Meta-分析

目的:综合评价超声检查在肺动脉高压(PAH)诊疗过程中的可靠性。方法:在数据库PubMed(1966年1月至2006年5月),Embase(1980年至2006年),中国期刊网(1978年1月至2006年5月)中,检索以PAH为研究对象,应用超声检查和右心导管检查,评价患者的血流动力学状态,对二种方法所测得的肺动脉收缩压进行比较的临床研究,对超声检查在PAH诊疗过程中的可靠性进行Meta-分析。结果:1.共有8项研究进行了超声检查结果与右心导管检查结果相关性分析,入选PAH患者256例。二者具有良好的相关性,合并后的R值为0.679,95%可信区间为0.623～0.735。2.共有6项研究对超声检查和右心导管检查进行了肺动脉收缩压测量值的比较,超声检查的结果明显低估了右心导管的结果;对其中48h内完成二种检查的2项研究进行综合比较,发现超声检查的结果仍低于右心导管检查结果,但二者差异的显著性降低。结论:在PAH的诊疗过程中超声检查的结果低估了肺动脉收缩压但可以反映其变化趋势。     

儿童特发性肺动脉高压预后相关因素的分析

目的:通过临床资料分析及随访,评价与儿童特发性肺动脉高压(IPAH)预后相关的影响因素。方法:入选2010年1月至2014年12月,入住北京安贞医院小儿心脏中心确诊为IPAH的患儿(年龄<18岁),收集常规实验室检查、心脏超声检查及心导管检查等指标,并进行随访。对临床资料进行生存分析,按患儿生存情况分组,对相关指标进行单因素及多因素分析,评价各种因素与儿童IPAH预后的相关性。结果:确诊IPAH患儿38例,其中男性25例(65.8%),年龄0.8~18.3岁,平均年龄(7.3±4.2)岁,平均确诊时间16.8个月。所有患儿均有呼吸困难和乏力症状,另外晕厥、咯血及水肿的发生率分别为42.1%、10.5%及15.8%。47.4%患儿在确诊时NYHA心功能为Ⅲ~IV级。78.9%患儿接受PAH靶向药物治疗,其余6例(21.1%)接受钙通道阻滞剂治疗。平均随访时间34.2个月,2例患儿失访,平均存活时间为确诊后20.5个月,1、2及3年生存率分别为91%、82%及68%。随访过程中11例患儿死亡[平均死亡年龄(7.3±3.9)岁],右心衰竭、肺动脉高压危象及咯血导致窒息是主要死因。与预后可能相关的指标单因素Cox分析显示:NYHA心功能分级(RR=4.981,P=0.025)、超声心动图估测肺动脉收缩压(P=0.007,RR=1.039)、红细胞分布宽度(red cell distribution width,RDW)(RR=1.287,P=0.014)、直接胆红素(RR=P=0.021,1.404)、全肺阻力(RR=1.048,P=0.032)和肺小动脉阻力指数(RR=1.072,P=0.037);进一步多因素Cox回归分析显示:NYHA心功能分级(RR=10.94,P=0.019)、超声心动图估测肺动脉收缩压(RR=1.033,P=0.036)、肺小动脉阻力指数(RR=1.202,P=0.022)及RDW(RR=1.439,P=0.007)是与预后相关的影响因素。结论:儿童IPAH临床表现多样、预后不良,进展性右心功能衰竭是儿童IPAH最重要的死亡原因。心导管检查是评价病情和预后、指导诊治的重要检查手段。NYHA心功能分级、肺小动脉阻力指数、超声心动图估测肺动脉收缩压、红细胞分布宽度是儿童IPAH预后相关的影响因素。     

结缔组织病相关肺动脉高压的临床分析

目的探讨结缔组织病(CTD)相关肺动脉高压(PAH)的临床、诊治和预后。方法回顾性分析北京协和医院1997年1月-2004年9月2189例CTD[包括混合性结缔组织病(MCTD)、系统性硬化(SSc)、原发性干燥综合征(pSS)、系统性红斑狼疮(SLE)、未分化结缔组织病(UCTD)、皮肌炎(DM)和白塞病(BD)]住院患者中并发PAH的临床和随访资料。结果(1)2189例CTD共发生PAH82例(3.7%),女75例,男7例,年龄12~71岁,平均41岁。PAH在SLE和MCTD患者中出现的时间(3年和2年)早于pSS患者(6年)。(2)主要临床表现是呼吸困难(84.1%)和肢端雷诺征(56.1%)。肺动脉收缩压(PASP)为(65.71±20.44)mmHg(1mmHg=0.133kPa),肺CO弥散量(DLCO)占预计值的百分比为(51±14)%,PaO2为(70.37±15.02)mmHg,PaCO2为(27.88±6.46)mmHg,PAH功能分级Ⅰ、Ⅱ、Ⅲ、Ⅳ级分别占13%、32%、29%、8%。(3)治疗后仅SLE患者PASP由(76.47±18.20)mmHg降至(69.08±20.77)mmHg。平均随访4.33年。82例并发PAH者13例(15.85%)死亡,明显高于未并发PAH的CTD患者的病死率(2.75%);死亡者与存活者相比,其PaO2更低,PAH功能分级Ⅲ、Ⅳ级的比例更多。结论CTD患者并发PAH不少见,一般在CTD发病后4年,SLE和MCTD并发PAH较早,pSS并发PAH较晚。CTD相关PAH的主要表现是呼吸困难和肢端雷诺征。严重PAH将影响CTD患者的预后,PAH功能分级或PaO2水平对预后评估有意义。定期对CTD患者行超声心动图和肺功能检查对筛查PAH非常必要。     

西地那非在儿童先天性心脏病相关性肺动脉高压中的应用

目的:观察西地那非治疗先天性心脏病(CHD)相关性肺动脉高压(PAH)儿童患者的安全性和有效性。方法:选择13例年龄<18岁的CHD合并PAH的患者,口服西地那非每次0.25～1mg/kg,3次/d进行治疗。对比患者用药前后的6 min步行试验距离(6 MWT)、平均肺动脉压力(mPAP)、肺血管阻力指数(PVRI)、肺循环与体循环平均压比率(Pp/Ps)及肺循环与体循环阻力比率(Rp/Rs)的变化。结果:13例服药患者平均随访(9.5±6.2)个月,6 MWT平均增加(47.36±15.7)m,P<0.05。其中11例分别行用药前后的心导管检查,检查结果示mPAP从(87.1±8.4)mmHg(1 mmHg=0.133 kPa)降至(82.2±3.7)mmHg,P=0.1;PVRI从(24.5±7.4)Wood units m2降至(20.3±5.4)Wood units m2,P<0.05;Pp/Ps从(0.99±0.09)降至(0.89±0.05),P<0.05;Rp/Rs从(0.91±0.25)降至(0.86±0.17),P=0.5。所有患者服药期间无明显不良反应及肝肾功能异常。结论:西地那非在CHD相关性PAH儿童患者中应用是安全的,能显著改善患者的活动耐量,降低肺血管阻力。     

原发性干燥综合征患者肺动脉压力变化的临床研究

目的探讨合并间质性肺病(ILD)的原发性干燥综合征(pSS)患者肺动脉压力变化及其与肺功能、预后的关系。方法分析南京市鼓楼医院及南京鼓楼医院集团宿迁市人民医院2009年1月至2013年1月确诊的pSS-ILD患者108例,按超声心动图检测的肺动脉收缩压(SPAP)结果,依据肺动脉高压(PAH)诊断标准将其分为PAH组(26例)和非PAH组(82例),收集两组的临床资料、超声心动图、肺功能的检测结果,电话随访患者的用药、治疗及死亡情况,并行PAH组的肺动脉收缩压与肺功能指标相关性研究。结果(1)108例pSS-ILD患者中有PAH者26例(24.0%),PAH组中SPAP为35~82 mmHg,平均(56.8±9.2)mmHg,PAH的发生与患者年龄、性别、病程无关。(2)PAH组FVC%、TLC%、DLCO%较非PAH组降低(P0.05),肺动脉收缩压与FVC%、TLC%、DLCO%呈负相关,与FEV1/FVC无相关性。随访12月后PAH组再入院率、死亡率较非PAH组升高。结论 pSS-ILD患者合并肺动脉高压后,肺功能减退明显,预后较差。定期对患者行超声心动图和肺功能检查对诊断PAH以进行早期治疗非常必要。     

波生坦对房间隔缺损合并重度肺动脉高压女性患者血管内皮舒张功能的影响

目的:观察波生坦对继发孔型房间隔缺损(ASD)合并重度肺动脉高压(PAH)女性患者血管内皮舒张功能的短期影响。方法:给予11例经右心导管检查确诊为先天性心脏病,ASD合并PAH的女性患者,给予波生坦62.5mg,每日2次,口服4周治疗。记录并比较患者治疗前、后6分钟步行距离(6MWD)、WHO心功能分级、内皮依赖性(FMD)和非内皮依赖性血管舒张功能(EID)及超声心动图检测指标。结果:波生坦治疗4周后,患者6MWD由(238±173.0)m增至(307±141.4)m(P=0.005);WHO心功能分级3例患者(27%)降低1级,8例患者维持原有分级不变;治疗前、后患者的肺动脉收缩压和平均压呈下降趋势,但差异无统计学意义;FMD由治疗前的2.7(2.0,3.3)%升至4.4(3.3,5.6)%(P=0.033),EID无显著变化。患者随访期间未观察到严重不良反应。结论:半剂量波生坦短期治疗,可以显著改善ASD合并PAH女性患者肱动脉内皮依赖性血管舒张功能。     

波生坦治疗特发性肺动脉高压患者的疗效及安全性

目的 观察内皮素受体拮抗剂波生坦治疗中国特发性肺动脉高压患者的有效性、安全性及耐受性.方法 全国多中心Ⅳ期临床试验,共入选79例右心导管等技术确诊的新发特发性肺动脉高压患者,口服波生坦每日2次,每次62.5 mg,治疗4周后增至每日2次,每次125 mg至16周试验结束.分别在基线及治疗16周时记录6分钟步行距离,WHO肺高压功能分级、Borg呼吸困难评分及超声心动图评价资料.结果 波生坦治疗16周后,患者6分钟步行距离从(343.7±93.7)m增至(397.5±104.4)m(P＜0.01),WHO肺高压功能分级显著改善(P＜0.01),Borg呼吸困难评分由3.0±1.5降低至2.5±1.5(P＜0.01),超声心动图估测肺动脉收缩压从(97.8±25.2)mm Hg(1 mm Hg=0.133 kPa)下降至(92.8±29.5)mmHg(P＜0.05).无患者因无法耐受不良反应退出.结论 波生坦能显著改善中国特发性肺动脉高压患者的运动耐量、心功能,且具有良好的安全性和耐受性.

Abstract：

Objective To investigate the efficacy, safety and tolerance of bosentan, a dual endothelin receptor antagonist, in Chinese patients with idiopathic pulmonary arterial hypertension (IPAH).Methods Totally 79 IPAH patients (hemodynamic criteria confirmed by right heart catheterization) were included in this open-label, prospective multicenter study. Patients received 62. 5 mg of bosentan twice daily for the first 4 weeks, and then up-titrated to 125 mg twice daily for another 12 weeks. The primary end point was the change in exercise capacity showed by six-minute walk distance (6MWD) from baseline to 16 weeks. Secondary end points included the change in World Health Organization (WHO) functional class,Borg dyspnoea scale and systolic pulmonary artery pressure measured by echocardiography. Results The 6MWD increased from (343. 7 ± 93.7) meters at baseline to (397.5 ± 104. 4) meters after 16 weeks ( P ＜0. 01 ), WHO functional class and Borg dyspnoea scale were also significantly improved after 16 weeks therapy compared to baseline levels (all P ＜0. 01 ). Furthermore, the systolic pulmonary artery pressure was significantly decreased from (97.8±25.2) mm Hg (1 mm Hg=0. 133 kPa) to (92.8 ±29.5) mm Hg (P ＜0. 05) after 16 weeks bosentan treatment. There was no patient withdrawal from this study for safety consideration. Conclusion Bosentan therapy is well tolerated and can improve the exercise capacity and WHO functional class in Chinese IPAH patients.     

Investigation of pulmonary hypertension

Pulmonary hypertension (PH) is defined by a mean pulmonary artery pressure (PAPm) superior than 25mmHg at rest or superior than 30mmHg with exercise. The classification of PH differentiates between "secondary" PH which results from a well-known disease, such as PH due to thromboembolic disease (obstructive PH), left cardiac failure (passive PH), or chronic respiratory diseases (hypoxic PH), and pulmonary arterial hypertension (PAH). PAH is a rare disease characterized by a progressive increase of pulmonary vascular resistance leading to right ventricular failure. PAH is classified as idiopathic, familial, or associated with various conditions (connective tissue diseases, congenital heart diseases with systemic-to-pulmonary shunts, portal hypertension, infection with the human immunodeficiency virus, or appetite-suppressant drugs). Transthoracic Doppler echocardiography is the investigation of choice for non invasive detection of PAH but right-heart catheterization is necessary to confirm the diagnosis of PAH and determine its mechanism. Pulmonary function tests and chest CT scan may detect an underlying chronic pulmonary disease (hypoxic PH). Lung perfusion scan and contrast-enhanced chest spiral CT scan can lead to the diagnosis of thromboembolic PH, which is to be confirmed by pulmonary angiography. Assessment of the severity of PH is based on clinical parameters (NYHA, right heart failure), functional tests (six-minute walk test), echocardiography and hemodynamics. Characterization of PH is essential in the management of PH because it determines the appropriate treatment: an etiological treatment in passive, obstructive or hypoxemic PH, or vasodilatator and antiproliferative therapies in PAH.     

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     

Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension.

AIMS: This study investigated the relationship between right ventricular (RV) structure and function and survival in idiopathic pulmonary arterial hypertension (IPAH). METHODS AND RESULTS: In 64 patients, cardiac magnetic resonance, right heart catheterization, and the six-minute walk test (6MWT) were performed at baseline and after 1-year follow-up. RV structure and function were analysed as predictors of mortality. During a mean follow-up of 32 months, 19 patients died. A low stroke volume (SV), RV dilatation, and impaired left ventricular (LV) filling independently predicted mortality. In addition, a further decrease in SV, progressive RV dilatation, and further decrease in LV end-diastolic volume (LVEDV) at 1-year follow-up were the strongest predictors of mortality. According to Kaplan-Meier survival curves, survival was lower in patients with an inframedian SV index or= 84 mL/m(2), and an inframedian LVEDV相似文献   

Serum N-terminal brain natriuretic peptide as a prognostic parameter in patients with pulmonary hypertension

STUDY OBJECTIVES: Baseline prognostic assessment in patients with pulmonary hypertension (PH) may help in the selection of treatment. High plasma levels of natriuretic peptide type B have been reported in patients with right ventricular (RV) dysfunction and suggest poor prognosis in patients with idiopathic pulmonary arterial hypertension (IPAH). We prospectively assessed the correlation of N-terminal brain natriuretic peptide (NT-proBNP) with echocardiographic and hemodynamic indexes of RV function as well as with baseline functional status and long-term survival of PH patients.Patients and design: Fifty-five consecutive patients with a mean (+/- SD) age of 41 +/- 15 years and severe PH (including 36 patients with IPAH) were followed up for up to 36 months. Serum samples for NT-proBNP were secured, and 6-min walk test (6 MWT), RV catheterization, and echocardiography were all performed on the same day, before the introduction of targeted treatment. RESULTS: The median baseline serum NT-proBNP concentration was 1,674 pg/mL (range, 51 to 10,951 pg/mL). NT-proBNP concentration correlated with 6MWT distance (r = 0.6; p < 0.001), cardiac index, pulmonary vascular resistance, and right atrial pressure (RAP), but not with pulmonary arterial pressure. NT-proBNP levels were also related to the ratio of the diastolic area of the RV and the LV, and to pericardial effusion during echocardiography. Receiver operating characteristic analysis identified > or = 1,400 pg/mL as the best NT-proBNP threshold predicting fatal outcome for the entire study group as well as for IPAH patients (sensitivity, 88% and 100%, respectively; specificity, 53% and 56%, respectively). In multivariate analysis, NT-proBNP, troponin T, and RAP were identified as independent factors for poor prognosis for the entire study group, while only NT-proBNP and RAP were identified as markers for poor prognosis in the IPAH subgroup. CONCLUSIONS: NT-proBNP level is related to the right heart morphology and dysfunction in PH patients. A serum NT-proBNP level of > or = 1,400 pg/mL was found to be useful in identifying patients with poor long-term prognosis both in the whole studied group and in the IPAH subgroup.     

NT-proBNP as a tool to stratify disease severity in pulmonary arterial hypertension

The recent development of treatment modalities for patients with idiopathic pulmonary arterial hypertension has been based on the evaluation of many different markers such as functional capacity, addressed by NYHA classification, six-minute walk test (6MWT) and hemodynamic parameters. The aim of this study was to evaluate the correlation of N-terminal fragment (NT-proBNP) with other markers in IPAH and its potential for patient stratification. We studied 42 IPAH patients consecutively evaluated through right heart catheterization in the absence of any specific treatment for pulmonary hypertension. Blood samples, clinical evaluation and 6MWF distance were collected at baseline. The levels of NT-proBNP showed a high correlation with hemodynamic parameters, such as pulmonary vascular resistance (r=0.80, P<0.001). A significant difference was found among patients with different functional classes, addressed by NYHA classification (P< 0.02 for all groups comparison). The discriminant analysis reinforced the ability of NT-proBNP to stratify patients according to NYHA functional class. Compared to the other variables studied (hemodynamics and 6MWT), NT-proBNP had the lowest level of overlap in the stratification of IPAH patients. We conclude that NT-proBNP differs among the different functional classes and correlates with other measures of disease severity, although its role in predicting survival still needs to be addressed.     

Pulmonary artery diastolic pressure: a simultaneous Doppler echocardiography and catheterization study

Pulmonary hypertension is an important determinant of the clinical presentation of and surgical approach to patients with heart disease. To confirm the utility of continuous wave Doppler echocardiography in assessing the pulmonary artery diastolic pressure in patients with pulmonary regurgitation, 51 patients representing the wide hemodynamic spectrum of pulmonary artery pressure underwent simultaneous determination of pulmonary artery diastolic pressure by continuous wave Doppler echocardiography and cardiac catheterization. Pulmonary artery diastolic pressure was estimated from the Doppler recordings by the end-diastolic pressure gradient obtained by the modified Bernoulli equation plus the estimated right atrial pressure. A correlation was observed (r = 0.935, SEE = 7.4 mmHg) between Doppler and catheterization pulmonary artery diastolic pressure. In addition, comparison between the mean diastolic pressure gradient across the pulmonary valve by Doppler and pulmonary artery diastolic pressure at catheterization yielded a high correlation (r = 0.947, SEE = 5.1 mmHg). These data demonstrate that continuous wave Doppler echocardiography is a useful noninvasive technique for evaluating the pulmonary artery diastolic pressure in patients with pulmonary regurgitation.     

Echocardiographic Screening for Pulmonary Hypertension in Stable COPD Out-Patients and NT-proBNP as a Rule-Out Test

Abstract

Pulmonary hypertension (PH) worsens the prognosis in chronic obstructive pulmonary disease (COPD). The diagnosis of PH is established by right heart catheterisation (RHC), while echocardiography can be used for screening. We aimed to asses the outcome of echocardiographic screening for PH in a group of stable COPD out-patients, and to evaluate NT-proBNP as a first line screening tool. Criteria for PH on echocardiography were a tricuspid regurgitation pressure gradient > 40 mmHg, a tricuspid annular plane systolic excursion < 1.8 cm or right ventricular dilatation. Positively screened patients were asked to undergo RHC. Results (Mean ± SEM): 16 of 117 patients (14%) had PH on echocardiography. They had a higher mortality (hazard ratio for death: 2.7 ± 1.3, p = 0.037) and lower six minute walk test (224 ± 33 vs. 339 ± 15, p = 0.006). NT-proBNP below 95 ng/l excluded PH on echocardiography with a negative predictive value of 100 (95% CI: 89–100%). RHC was obtained in six patients screened positive. In three of these, PH was not confirmed. Conclusions: Signs of PH on echocardiography as defined here was found in 14% and had prognostic significance in COPD. A value of NT-proBNP less than 95 ng/l may be used to exclude signs of PH.     

Doppler velocimetry in superior vena cava provides useful information on the right circulatory function in patients with congestive heart failure

BACKGROUND: Although flow velocities curves recorded with pulsed-wave Doppler in systemic vein are known to provide functional data on the right circulatory function, little information is available on the relationship between right heart filling dynamics and right ventricular function. METHODS: Consecutive patients with chronic heart failure due to severe systolic left ventricular dysfunction and in sinus rhythm underwent echocardiography and right heart catheterization. In the initial part of the study, the hemodynamic correlates of different flow velocity patterns recorded into the superior vena cava were evaluated in 120 patients. The accuracy of the prediction of different right heart hemodynamic profiles by means of the different venous flow patterns was then prospectively tested in a subsequent series of 86 patients. RESULTS: The venous flow pattern was closely related to right heart hemodynamics. A normal Doppler pattern identified patients with normal right heart hemodynamics (sensitivity 86%, specificity 78%); a "predominant systolic wave" pattern identified patients with a reduced thermodilution-derived right ventricular ejection fraction (< 30%) and normal or slightly elevated right atrial pressure (< or = 8 mmHg) (sensitivity 69%, specificity 81%); a "predominant diastolic wave" pattern identified patients with a reduced right ventricular ejection fraction (< 3 0%) and elevated right atrial pressure (> 8 mmHg) (sensitivity 52%, specificity 95%). The observed and the predicted hemodynamic profiles turned out to be concordant in 80% of patients. CONCLUSIONS: The analysis of the flow velocity pattern into the superior vena cava is a useful tool to estimate the extent of the right circulatory impairment in patients with congestive heart failure.     

Pulmonary arterial hypertension in connective tissue diseases

Pulmonary hypertension (PH) was found to be the primary cause of death in mixed connective tissue disease (MCTD). This led to investigation of the prevalence of PH in other connective tissue diseases (CTD). In 1998, the Ministry of Health and Welfare's MCTD Research Committee revealed complication of PH diagnosed by physicians in 5.02% MCTD patients, 0.90% systemic lupus erythematosus patients, 2.64% systemic sclerosis patients, and 0.56% polymyositis/dermatomyositis patients. These results have been supported by a similar survey performed in North America. As quite a few rheumatologists find right heart catheterization difficult to perform, doppler echocardiography is frequently used for screening and diagnosing PH. The MCTD Research Committee set the revised criteria for MCTD-PH, in which the threshold of estimated pulmonary arterial systolic pressure value for diagnosis of pulmonary arterial hypertension (PAH) is set at 36 mmHg, as proposed by the European Society of Cardiology. Right heart catheterization is strongly recommended for commencing the treatment. Since PH due to thromboembolism can potentially be cured surgically, lung perfusion scintigraphy should be performed for all patients diagnosed with PH. Most CTD-PH are PAH, and since idiopathic PAH (IPAH) patients sometimes have immune disorders, treatment for IPAH may be applicable to CTD-PH. The greatest difference between the treatment strategy for CTD-PH and IPAH is the usage of corticosteroids and other immunosuppressants. The MCTD Research Committee updated its therapeutic guidelines for MCTD-PH in 2011. Validation of these guidelines is also needed.