伊洛前列素在小儿先天性心脏病肺动脉高压术后的应用研究

目的:探究伊洛前列素治疗小儿先天性心脏病(CHD)术后肺动脉高压(PAH)的安全性及有效性。方法:选择合并PAH的CHD体外循环下行双心室矫治术患儿50例,年龄6个月～18岁,中位数年龄4.5(2.78,8.0)岁,分为吸入伊洛前列素组(组Ⅰ)、静注前列腺素E1(PGE1)组(组Ⅱ)。所有患儿均于术前放置PICCO及Swan-Ganz导管。通过术后测定、FICK法计算2组患儿用药前后血流动力学指标,包括体循环阻力指数、肺血管阻力指数。在相同通气条件下,比较2组患儿用药前后血流动力学指标在用药后48 h内的变化趋势。结果:伊洛前列素组肺体血管压力比(Pp/Ps):用药后即刻、用药后30 min较用药前差异有统计学意义(P<0.001);肺体循环阻力比(Rp/Rs):用药后即刻较用药前差异有统计学意义(P<0.05),用药后30 min较用药前差异有统计学意义(P<0.001);心排指数(CI):用药后即刻及用药后30 min差异有统计学意义(P<0.05)。结论:吸入性伊洛前列素是高效的选择性肺血管扩张剂,可以显著降低合并PAH的CHD术后患儿的肺动脉压力(PAP)和肺血管阻力(PVR)。应用吸入性伊洛前列素可以提高CHD术后患儿的CI。伊洛前列素用于治疗CHD术后PAH是有效安全的。     

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。     

吸入伊洛前列素和纯氧在肺动脉高压急性肺血管扩张试验中的对比研究

目的:本研究通过对先天性心脏病(CHD)合并重度肺动脉高压(PAH)患者在心导管术中行急性肺血管扩张试验,评估吸入纯氧和吸入伊洛前列素对血流动力学的影响,为合理选择和应用血管扩张药物提供依据。方法:本研究选择CHD合并重度PAH患者为研究对象,在心导管术中测定基础状态、吸氧试验后、吸入伊洛前列素药物试验后血流动力学参数,并进行比较。结果:本研究入选71例简单CHD合并重度PAH患者,中位年龄15岁(1.5~44岁)。在吸氧试验和药物试验后,血流动力学参数均有明显改善。在吸氧后肺动脉平均压由(78.30±10.30)mm Hg(1mm Hg=0.133k Pa)降至(75.85±10.41)mm Hg,(P<0.01),肺血管阻力指数由(23.30±9.51)WU·m~2降至(16.52±8.47)WU·m~2。在吸入伊洛前列素后,肺动脉平均压降至(74.37±9.95)mm Hg(P<0.01),肺血管阻力指数降至(19.49±8.75)WU·m~2(P<0.01),主动脉平均压无明显变化(P>0.01),无严重不良反应发生。不同于药物试验,在吸氧试验中增加氧分压测定和校正可以有效减少误差。在判断肺血管反应性时,吸氧试验阳性率高于药物试验。结论:对于CHD合并重度PAH患者,吸入纯氧和伊洛前列素均可安全、有效的应用于急性肺血管扩张试验药物,但吸氧试验需要氧分压的校正。在本研究中吸氧试验阳性率要高于药物试验。     

先天性心脏病术后合并肺动脉高压心导管检查及急性肺血管扩张试验分析

目的:探讨先天性心脏病术后(CHD-PO)合并肺动脉高压(PAH)患者,行心导管检查及急性肺血管反应试验的临床价值。方法:研究选自2008年8月至2015年12月,收住北京安贞医院小儿心脏科,行左、右心导管检查及急性肺血管扩张试验的CHD-PO合并PAH的患者,监测血流动力学指标变化。结果:入选40例患者,男性11例(27.5%),女性29例(72.5%),年龄6.61~45.51岁,平均年龄(23.6±9.3)岁,中位手术年龄13.4岁;平均纽约心功能(NYHA)分级(1.88±0.68)[I~II/III~IV,33例(82.5%)/7例(17.5%)],术后最短行心导管检查的时间为0.5年,最长为25.4年,中位时间为3.0年。术中无肺动脉高压危象发生,无低血压现象,其中1例急性肺血管扩张试验阳性。心导管检查:基础状态下,平均肺动脉压力(MPAP)为(58.30±21.32)mm Hg(1mm Hg=0.133k Pa),肺小动脉阻力指数(PVRI)为(16.67±11.64)Wood U·m2,肺动脉收缩压/主动脉收缩压(PP/PS)为(0.75±0.25),肺循环阻力/体循环阻力(RP/RS)为(0.63±0.28),所有患者均被证实仍残存肺动脉高压。吸入伊洛前列素(Iloprost)后,肺动脉收缩压(SPAP)、MPAP、主动脉收缩压(SAOP)、PVRI、RP/RS降低,心指数(CI)增加,且均有显著临床意义(P0.05);平均主动脉压(MAOP)、体循环阻力指数(SVRI)降低,但无明显临床意义。结论:CHD合并重度PAH丧失最佳手术时机的患者,行心脏矫治术后,术后远期随访证实肺动脉压力仍未降至正常,需要长期随访及靶向药物治疗。CHD-PO合并PAH患者,与IPAH相比,其肺血管反应差,心导管检查过程中肺动脉高压危象发生率低。雾化吸入伊洛前列素(Iloprost),在增加或者维持CI不变的前提下,具有良好降低肺循环阻力及压力的作用。     

法舒地尔对先天性心脏病重度肺动脉高压的急性肺血管反应

目的:比较成人先天性心脏病(CHD)相关性重度肺动脉高压(PAH)患者,对雾化吸入伊洛前列素(万他维)与静脉泵入法舒地尔的急性肺血管扩张效应,以评价法舒地尔在CHD相关性重度PAH的急性肺血管反应。方法:回顾性分析161例进行急性肺血管扩张试验的成人CHD相关性重度PAH患者,其中万他维组126例,法舒地尔组35例,比较两组用药前后血流动力学参数变化。结果:万他维组与法舒地尔组用药后肺动脉平均压、肺小动脉阻力、肺总阻力、肺循环阻力与体循环阻力比值、平均肺动脉压力与体循环压力比值均显著降低,且肺循环血流量/体循环血流量比值(Qp/Qs)显著增加,差异均有统计学意义(P<0.05)。用药前后,万他维组右心房压(P=0.056)无明显变化,而法舒地尔组(P<0.01)明显降低。两组患者动脉氧饱和度(Sa O2)、体循环血压、心脏指数(CI)及体循环阻力等差异均无统计学意义。结论:静脉使用法舒地尔可明显降低肺动脉压及肺血管阻力,显著增加Qp/Qs,而CI、体循环血压及Sa O2无变化;法舒地尔可能成为CHD相关性PAH的另一种安全有效的治疗药物。     

先天性心脏病并重度肺动脉高压患者雾化吸入伊诺前列素的即刻效应

目的 评价雾化吸入伊洛前列素对重度成人先天性心脏病相关性肺动脉高压血液动力学的影响.方法 对165例经超声心动图诊断先天性心脏病合并重度肺动脉高压患者进行左右心导管检查获得基线血液动力学参数后雾化吸入伊洛前列素20 μg,10 min时再记录相关血液动力学资料,对比吸入伊诺前列素前后血液动力学的变化.结果 伊洛前列素吸入结束后即刻和吸入前比较,患者主动脉和肺动脉的压力略有下降(P＜0.05),但心率、主动脉平均压、肺动脉收缩压与主动脉收缩压的比值(Pp/Ps)差异均无统计学意义(P＞0.05).肺循环血量(Qp)明显增加[(7.2±4.8)L/min比(9.9±7.2)L/min,P＜0.01],肺血管阻力(PVR)明显下降[(13.4±8.7)Wood单位比(9.5±6.6)Wood单位,P＜0.01],左向右分流量明显增加[(3.2±4.4)L/min比(5.5±7.0)L/min,P＜0.01]及右向左分流量明显减少[(1.0±1. 0)L/min比(0.7±0.7)L/min,P＜0.01].亚组分析结果提示,不是所有先天性心脏病合并重度肺动脉高压患者血液动力学均呈相同的变换趋势,存在动脉导管未闭和(或)心室间隔缺损患者的肺动脉压力明显高于继发于心房间隔缺损患者,而且更易出现艾森曼格综合征(P＜0.05).结论 伊洛前列素雾化吸入可快速降低先天性心脏病相关性肺动脉高压的压力、肺血管阻力,明显增加右心排血量,对体循环压力及心率影响较小,可以作为评估急性肺血管反应的肺血管扩张剂.

Abstract：

Objective To investigate the immediately effects of inhaled aerosolized iloprost in adult patients with severe pulmonary arterial hypertension(PAH)secondary to congenital heart diseases(CHD).Methods Adult patients with severe PAH secondary to CHD(n = 165)were included in this study. Right heart catheterization was performed, Pulmonary and systemic blood flow, the oxygen consumption VO2(ml/min)were calculated using Fick's principle. Pulmonary vascular resistances(PVR)were calculated with standard formulas and indexed to body surface area. Hemodynamic parameters were measured before and after iloprost inhalation(20 μg). Results Post iloprost inhalation, heart rate, mean aortic pressure, pulmonary systolic pressure to aortic systolic pressure ratio all remained unchanged(P ＞ 0. 05), while pulmonary artery pressure(PAP)were significantly reduced and Qp significantly increased from(7. 2 ±4. 8)L/min to(9. 9 ±7.2)L/min(P＜0.01), PVR was also significantly reduced from(13.4 ±8.7)Wood units to(9.5 ±6. 6)Wood units(P ＜0. 01),and left-to-right shunt volume increased from(3.2 ±4. 4)L/min to(5. 5 ±7.0)L/min(P ＜ 0. 01)and right -to-left shunt volume decreased from(1.0 ± 1.0)L/min to(0. 7 ± 0. 7)L/min(P ＜ 0. 01). Subgroup analysis showed that adult patients with patent ductus arteriosus and/or ventricular septal defects are more likely to develop severe pulmonary arterial hypertension or Eisenmenger syndrome than patients with atrial septal defects. Conclusions Inhaled Aerosolised iloprost use is effective and safe for adult patients with severe pulmonary arterial hypertension secondary to congenital heart diseases.     

吸入伊洛前列素在GLENN术后的应用研究

目的:研究吸入伊洛前列素在先天性心脏病(先心病)患儿,上腔-右肺动脉吻合术(GLENN)术后的血流动力学作用。对象:2011年2月至2011年10月安贞医院小儿心脏中心,行GLENN手术后,肺动脉平均压力(mPAP)≥18 mmHg(1 mmHg=0.133 kPa)者30例。方法:所有患儿手术后置入肺动脉测压管。入ICU常规治疗基础上,测量mPAP≥18 mmHg者,1 h内开始吸入伊洛前列素500 ng/kg,1次/4 h,连续治疗至脱离呼吸机后24 h。观察指标:mPAP、氧合指数(OI)、心率(HR)、平均动脉血压(MAP)。观察时间点设为第1次、第4次、第7次及拔管后第1次吸药,每次治疗前及治疗停止后20 min。结果:29例患儿,男性16例,年龄(0.7～13.8)岁,平均6.26岁。其中单心室合并肺动脉狭窄11例,矫正性大动脉转位6例,肺动脉闭锁6例,右心室双出口合并肺动脉狭窄4例,右心室双出口合并完全性心内膜垫、肺动脉狭窄2例。合并中度房室瓣反流9例。应用体外循环11例。术毕测压mPAP(18.49±3.33)mmHg,ICU首次吸药前mPAP(20.38±3.01)mmHg。其余每次吸药前mPAP分别是[(19.28±2.07)、(17.21±2.37)、(16.07±2.66)mmHg],吸药后[(18.66±2.33)、(17.97±2.28)、(16.03±2.34)及(14.51±2.68)mmHg],均较治疗前显著下降(P<0.01)。带呼吸机时吸药前OI分别是(87.93±11.06)、(103.13±13.05)及(106.21±16.56),吸药后分别(90.52±12.27)、(110.69±13.41)及(117.76±12.99),均较吸药前明显升高(P<0.01)。但拔管后、吸药前后OI差异无统计学意义。每次吸药前后HR、MAP差异均无统计学意义。患儿术后气管切开1例,死亡1例。结论:GLENN术后mPAP>18 mmHg者,吸入伊洛前列素可以迅速降低mPAP,机械通气时升高OI。对HR、MAP无明显影响。     

心脏磁共振成像对左向右分流型先天性心脏病合并肺动脉高压的诊断价值及右心室功能评估

目的 探讨心脏磁共振成像(CMR)对左向右分流型先天性心脏病(CHD)合并肺动脉高压(PAH)的诊断效能及对患者右心室功能的评价作用.方法 选取2012年9月至2020年7月就诊于徐州医科大学附属医院确诊为CHD的42例患者,根据右心导管检查的平均肺动脉压力(mPAP)是否≥25 mmHg将患者分为CHD+PAH组(2...     

吸入伊洛前列素对不同类型肺动脉高压患者氧动力学的影响

目的 分析雾化吸入伊洛前列素对动脉型肺动脉高压(PAH)及慢性血栓栓寒性肺动脉高压(CTEPH)患者的氧动力学的急性影响.方法 顺序收集北京朝阳医院2006年6月至2009年1月连续收治的明确诊断为PAH的患者22例及CTEPH患者24例,均行右心导管检查,监测基线状态及吸人伊洛前列素20μg后的即刻血流动力学特征,同步抽取肺动脉及股动脉血行血气分析,计算氧动力学参数.结果 基线状态PAH及CTEPH患者的PaO2分别为(63±10)mm Hg(1 mm Hg=0.133 kPa)及(59 ±10)mm Hg,氧输送指数(DO2I)分别为(458±136)ml·min-1·m-2及(386 ±92)ml·min-1·m-2,氧消耗指数分别为(135±53)ml·min-1·m-2及(131±43)ml·min-1·m-2.吸入伊洛前列素后即刻,2组患者肺内分流率均显著升高(均P<0.01),P4O2显著升高(均P<0.01);动脉血氧含量均显著降低(均P<0.05);混合静脉血氧合指标及氧摄取率无明显变化;DO2I无明显增加,氧消耗指数出现不同程度降低;2组患者各项氧动力学参数变化幅度无差异.基线状态CTEPH患者混合静脉血氧饱和度(SvO2)、静脉血氧含量(CvO2)及DO2I均显著低于PAH患者(均P<0.05).吸药后,CTEPH患者PaO2、SvO2及CvO2均显著低于PAH患者(均P<0.05).结论 PAH及CTEPH患者存在低氧血症及氧动力学异常;雾化吸入伊洛前列素后患者肺内分流增加,氧动力学状态无改善.CTEPH患者的氧动力学状态较PAH患者更差,应用伊洛前列素时需加强氧合功能监测,必要时给予氧疗支持.     

急性肺血管药物扩张试验在评价先天性心脏病合并重度肺动脉高压中的应用进展

<正>一、先天性心脏病合并重度肺动脉高压继发于左向右分流型先天性心脏病(CHD)的肺动脉高压(PAH)是一种进行性病变,主要表现为肺动脉压力和肺血管阻力的进行性升高,最终导致右心衰竭甚至死亡。这类肺动脉高压按其性质可分为动力性肺动脉高压和梗阻性肺动脉高压,CHD合     

The short- and medium-term results of transcatheter closure of atrial septal defect with severe pulmonary arterial hypertension

We investigated the short-term and medium-term results in patients with pulmonary arterial hypertension (PAH) associated with atrial septal defect (ASD) undergoing transcatheter closure. Fifteen patients with severe PAH associated with ASD who underwent successful occluder implantation from 2007 to 2010 were included. Clinical, echocardiographic, and hemodynamic data were reviewed. Severe PAH was defined as pulmonary arterial systolic pressure measured by catheterization was ≥60 mmHg and pulmonary vascular resistance (PVR) ≥6 Wood Units (WU). Compared with baseline, the 6-minwalking distance significantly increased by 29.7 ± 26.3 m (P < 0.001) at 3 months (short-term) and 65.4 ± 63.6 m (P < 0.001) at 23.4 ± 9.7 months (medium-term), World Health Organization function class considerably improved after postclosure short-term and medium-term. Repeat cardiac catheterization (n = 7) showed that mean pulmonary arterial pressure decreased from 51.6 ± 9.4 mmHg at baseline to 21.0 ± 3.8 mmHg (P < 0.001) at follow-up of 12 months. The PVR decreased by 5.6 ± 1.1 WU (P < 0.001). Through carefully selected patients with severe PAH associated with ASD, transcatheter closure can be safely performed with a promising short-term and medium-term outcome. Trial occlusion is an effective way for deciding the reversibility of severe PAH in ASD patients. The role of aerosolized iloprost for pulmonary vasoreactivity testing in patients with severe PAH secondary to ASD requires further investigation.     

先天性心脏病重度肺动脉高压对腺苷的急性反应

目的: 探讨先天性心脏病（CHD）并发重度肺动脉高压（PAH）患者对腺苷的急性血流动力学反应。方法: 对25例CHD并发严重PAH患者采用腺苷进行急性肺血管扩张试验,检测其血流动力学指标变化。结果: 25例患者中,仅7例患者达到最大剂量无不良反应。给予腺苷后,肺动脉压力和主动脉压力均显著降低（P<0.05）,股动脉血氧饱和度,肺血管阻力,肺循环/体循环血流量比值,肺动脉/主动脉平均压比值,肺血管阻力/体循环阻力比值（Rp/Rs）均无明显变化。没有患者肺动脉平均压降至40 mmHg以下。12例患者肺血管阻力和Rp/Rs降低10%以上,与另外13例患者比较,二者在年龄,肺动脉压力,肺动脉/主动脉平均压比值,肺循环/体循环血流量比值,肺血管阻力和Rp/Rs等方面均无明显差异。结论: 在CHD并发重度PAH患者中,采用腺苷进行急性肺血管扩张试验可引起肺动脉和体循环压力同步降低,而对肺血管阻力无明显影响。     

Assessment of pulmonary hypertension in the pediatric catheterization laboratory: Current insights from the magic registry

Objectives: To assess protocols, demographics, and hemodynamics in pediatric patients undergoing catheterization for pulmonary hypertension (PH). Background: Pediatric specific data is limited on PH. Methods: Review of the Mid‐Atlantic Group of Interventional Cardiology (MAGIC) collaboration PH registry dataset. Results: Between November 2003 and October 2008, seven institutions submitted data from 177 initial catheterizations in pediatric patients with suspected PH. Pulmonary arterial hypertension associated with congenital heart disease (APAH‐CHD) (n = 61, 34%) was more common than idiopathic PAH (IPAH) (n = 36, 20%). IPAH patients were older with higher mean pulmonary arterial pressures (mPAP) (P < 0.01). Oxygen lowered mPAP in patients with IPAH (P < 0.01) and associated PAH not related to congenital heart disease (APAH‐non CHD) (P < 0.01). A synergistic effect was seen with inhaled Nitric Oxide (iNO) (P < 0.01). Overall 9/30 (29%) patients with IPAH and 8/48 (16%) patients with APAH‐non CHD were reactive to vasodilator testing. Oxygen lowered pulmonary vascular resistance index (PVRI) in patients with APAH‐CHD (P < 0.01). There was no additive effect with iNO but a subset of patients required iNO to lower PVRI below 5 WU·m². General anesthesia (GA) lowered systemic arterial pressure (P < 0.01) with no difference between GA and procedural sedation on mPAP or PVRI. Adverse events were rare (n = 7) with no procedural deaths. Conclusions: Pediatric patients with PH demonstrate a higher incidence of APAH‐CHD and neonatal specific disorders compared to adults. Pediatric PH patients may demonstrate baseline mPAP < 40 mm Hg but > 50% systemic illustrating the difficulty in applying adult criteria to children with PH. Catheterization in children with PH is relatively safe. © 2010 Wiley‐Liss, Inc.     

Long-term treatment of pulmonary hypertension with aerosolized iloprost.

Pulmonary arterial hypertension (PAH), defined as elevated pulmonary arterial pressure and pulmonary vascular resistance, is an end-point of a variety of conditions. The only therapy that has been shown to improve both quality of life and survival is intravenous prostacyclin (prostaglandin I2 (PGI2), epoprostenol). The effect of long-term aerosolized iloprost (Ilomedin, Schering, Berlin, Germany and Vienna, Austria), a stable prostacyclin analogue and potent vasodilator, on haemodynamics and functional status was investigated in 12 patients with severe pulmonary hypertension. Haemodynamic measurements and vasodilator testing by right heart catheterization were performed prior to and after long-term iloprost inhalation therapy. Haemodynamic improvement or increased exercise tolerance was not observed in any of the patients. After a mean+/-SD treatment period of 10+/-5 months, mean+/-SD pulmonary vascular resistance had increased from 11+/-3 Wood Units (mmHg.L(-1).min) to 13+/-4 Wood Units, with unchanged arterial oxygen saturation (92+/-4%, versus 91+/-4%). Within the study period, three patients went into right heart failure and had to be placed on intravenous epoprostenol. The authors conclude that inhaled iloprost in addition to conventional therapy in the presently recommended dose of 100 microg.day(-1) delivered in 8-10 2 h portions, is not an efficient vasodilator therapy in severe pulmonary hypertension. It remains to be shown whether dose increases and/or combination protocols will be effective, or whether inhalation of iloprost may be safe for selected cases of pulmonary hypertension.     

Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis

OBJECTIVE: A prospective study to evaluate echocardiography and gas transfer (DLCO) by comparison with cardiac catheterization in discriminating between patients with and without systemic sclerosis-associated pulmonary arterial hypertension (SScPAH). METHOD: A total of 137 (52 with and 85 without pulmonary fibrosis) had echocardiography and lung function tests within 3 months of their definitive invasive study. RESULTS: At cardiac catheterization 99 of these patients were found to have PAH, while PAH was excluded in 38. Echocardiographically estimated tricuspid gradient (TG) showed a moderate positive correlation (r(2) = 0.44, P<0.005) with both mean pulmonary pressure and invasively determined tricuspid gradient. DLCO showed a weak correlation (r(2 )= 0.09, P = 0.006), when compared with mean pulmonary arterial pressure. In total, 97% of patients with an echocardiographically determined TG of > 45 mmHg were found to have pulmonary hypertension at catheterization. However, no threshold could be defined with either screening test that safely excluded PAH. CONCLUSIONS: The positive predictive accuracy of currently used non-invasive tests are adequate for the diagnosis of advanced PAH provided sufficiently high thresholds (TG > 45 mmHg or DLCO < 55% predicted) are used. These tests cannot be relied upon to exclude pulmonary hypertension where pre-test probability is high.     

成人先天性心脏病并发肺动脉高压外科治疗术后不良事件和右房改善的危险因素

目的 分析先天性心脏病（CHD）并发肺动脉高压（PAH）（CHD-PAH）患者手术后不良事件、右房大小改善的危险因素。方法 收治79例CHD-PAH患者。初诊,右心导管检查和吸氧实验,确诊PAH、肺血管反应性,其中13例患者肺血管阻力（PVR）<10 Wood单位、体循环血流量（Qp）与肺循环血流量（Qs）的比值(Qp/Qs)≥1.5、血氧饱和度(SaO2)≥95%,且心功能较好者直接行手术治疗,其余66例患者接受靶向药物治疗3个月后,复查右心导管进行评估手术。结果 术后不良事件发生17例,其中肺高压危象11例,撤机困难需气管切开4例以及低心排综合征2例。53例患者术后右房恢复正常大小。有无不良事件组间比较结果显示:术前PVR≥10 Wood单位、Qp/Qs、SaO2、肺动脉收缩压/主动脉收缩压（SPAP/SBP）>0.75、SPAP、肺动脉舒张压（DPAP）是术后不良事件的影响因素（均P<0.01）,多因素Logistic回归分析结果显示SaO2（OR=0.584,P=0.015）越高,不良事件发生率越低;术后右房大小改善是否满意组间单因素分析显示:PVR≥10 Wood单位、右房压（RAP）>8 mmHg(1 mmHg=0.133 kPa)、血流分流方向是其影响因素（均P<0.05）,多因素Logistic回归分析结果显示其危险因素是RAP>8 mmHg（OR=3.398,P=0.029）。结论 CHD-PAH患者术后不良事件的危险因素是SaO2的降低,影响右房改善的危险因素是RAP>8 mmHg。     

Successful treatment with sildenafil in systemic sclerosis patients with isolated pulmonary arterial hypertension: two case reports

We describe two systemic sclerosis (SSc) patients with isolated pulmonary arterial hypertension (PAH) who were given treatment with 50 mg oral sildenafil per day. We evaluated the efficacy of oral sildenafil for isolated PAH in SSc patients by direct assessment with cardiac catheterization before and 6 months after the initiation of sildenafil. Right-heart catheterization demonstrated decreased mean pulmonary artery pressure, decreased pulmonary vascular resistance, and increased cardiac output after treatment with sildenafil. Brain natriuretic peptide levels were gradually decreased. The 6-min walking distance was greatly extended. Moreover, the physical conditions of both patients were much improved. We recognized no adverse events. We propose that oral sildenafil may be beneficial as a selective pulmonary vasodilator and as long-term treatment in SSc patients with isolated PAH.