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。     

不同动脉压水平对老年感染性休克患者血流动力学组织灌注和代谢的影响

目的 观察应用去甲肾上腺素联合多巴酚丁胺(NE+Dobu)在小同平均动脉压(MAP)水平对老年感染件休克患者血流动力学、组织灌注和代谢的影响. 方法 感染性休克患者18例,充分液体复苏后为基础状态,应用NE+Dobu并随机调节剂量,使MAP分别维持在65、75、85mm Hg 3种水平状态,持续4 h,观察不同MAP水平的血流动力学、氧合指标、血乳酸、肾功能和胃黏膜二氧化碳分压变化. 结果 当MAP达75 mm Hg和85 mm Hg时,心脏指数(4.7±0.6和5.1±0.7)、体循环阻力指数(1162±278和1276±319)、氧输送[(697±53)ml·min-1·m-2和(711±68)ml·min-1·m-2]、氧摄取率[(0.28±0.02)%和(0.27±0.02)%],均较MAP为65 mm Hg时[4.0±0.6、1011±225、(634±70)ml·min-1·m-2、(0.25±0.02)%]明显增加(P<0.05);尿量分别为(98±43)ml/h和(91±54)ml/h,较65 mm Hg时(74±49)ml/h有明显增加,但仅MAP75 mmHg时,差异有统计学意义(P<0.05). 结论 老年感染性休克患者充分液体复苏后,应用NE+Dobu提高MAP达75 mm Hg时,可改善全身血流动力学和肾功能,对于老年感染性休克患者可能需要适当提高MAP.     

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。     

西地那非治疗肺动脉高压疗效和安全性的初步观察

目的 探讨西地那非治疗对肺动脉高压患者的临床疗效及耐受性,为西地那非治疗肺动脉高压提供依据.方法 连续入选2007年5月至2009年4月阜外心血管病医院收治的肺动脉高压患者56例,其中男11例,女45例,年龄(31±11)岁.给予西地那非25 mg口服,3次/d,记录治疗前和治疗12周后患者心功能和肺动脉高压功能分级、6 min步行距离、Borg呼吸困难指数、血流动力学改变及临床症状,同时监测患者血液循环及实验室检测指标及不良反应.结果 治疗12周后,患者心功能和肺动脉高压功能分级有明显改善(P<0.01),其中2例Ⅳ级升高至Ⅲ级;8例Ⅲ级改善为Ⅱ级,2例升高至Ⅰ级;5例Ⅱ级升高至Ⅰ级.无纽约心功能分级及世界卫生组织肺动脉高压功能分级恶化病例;6 min步行距离由(352±80)m增加至(396±78)m;差值为(44±70)m(P<0.01);肺动脉平均压降低(6±14)mm Hg(1 mm Hg=0.133 kPa)、肺血管阻力降低(490±832) Dys·s·cm-5(均P<0.01)心输出量增加(1.1±2.0) L/min,P<0.01;心指数增加(0.7±1.1) L·min-1·m-2(均P<0.01).患者无临床恶化情况,耐受性良好,无严重不良事件.结论 西地那非治疗可显著改善肺动脉高压患者功能分级,活动耐力及血流动力学.

Abstract：

Objective To explore the safety and efficacy of oral sildenafil therapy for pulmonary arterial hypertension(PAH), and to provide evidence for sildenafil treatment for Chinese patients with PAH. Methods In this 12-week, prospective, open-label, uncontrolled study, 56 patients with PAH were given oral sildenafil (25 mg, tid). The primary end point was change from baseline to 12 weeks in exercise capacity assessed by 6 min walk (6MW) test. Secondary end points included changes in WHO class and cardiopulmonary hemodynamics. Clinical worsening was defined as death, transplantation, hospitalization for PAH, or initiation of additional therapies for PAH, such as intravenous epoprostenol or oral bosentan. Results After 12 weeks, the compliance was good in 56 patients. Significant improvement was seen in NYHA heart function class and WHO class as compared to baseline(P<0.01): from class Ⅳ to class Ⅲ in 2, from class Ⅲ to class Ⅱ in 8 and to class Ⅰ in 2 cases, and from class Ⅱ to class Ⅰ in 5 cases. No NYHA heart function class and WHO PAH function class deterioration were observed. Oral sidenafil increased 6MW distance, from (352±80) m to (396±78) m, with a change of (44±70) m(P<0.01). Significant improvement was seen in hemodynamics (mean pulmonary artery pressure, P<0.01; cardiac index, P<0.01; pulmonary vascular resistance, P<0.01) at week 12 as compared with baseline. Mean right atrial pressure decreased (3±11)mm Hg (1 mm Hg=0.133 kPa), mean pulmonary arterial pressure decreased (6±14) mm Hg, cardiac output increased (1.1±2.0)L/min, cardiac index increased (0.7±1.1)L·min-1·m-2, and total pulmonary resistance decreased (490±831) Dys·s·cm-5. Side effects were mild and consistent with those reported with sildenafil treatment. No statistically significant clinical worsening was observed with sildenafil therapy for PAH patients. Conclusions Sildenafil improves exercise capacity, WHO functional class, and hemodynamics in patients with pulmonary arterial hypertension.     

主动脉内球囊反搏在心脏外科围术期的临床应用

目的：探讨主动脉内球囊反搏术（IABP）在心脏外科围术期患者出现低心排血量综合征时应用的有效性及可行性。方法：回顾性分析40例使用IABP的原因及使用12h、24h、48h后平均动脉压（MAP）、心脏指数（CI）、肺动脉嵌压（PAWP）、氧输送指数（DO2I）、氧摄取率（O2ER）等血流动力学指标变化。结果：与IABP前比较,IABP12h、24h、48h后MAP[48h：（56．40±6．51）mmHg比（73．00±3．36）mmHg]、CI[48h：（1．74±0．21）L·min-1·m-2比（2．74±0．21）L·min-1·m-2]、DO2I[48h：（267．36±15．95）ml·min-1·m-2比（429．60±33．19）ml·min-1·m-2]均明显升高,尿量[48h：（25．44±3．88）ml／h比（99．48±9．48）ml／h]明显增加,PAWP[48h：（18．00±1．66）mmHg比（12．60±0．71）mmHg]、O2ER[48h：（44．45±4．00）％比（31．41±1．25）％]均明显下降（P均〈0．01）。40例中死亡7例（19．5％）。结论：主动脉内球囊反搏术可有效改善血流动力学,对于围术期低心排患者的治疗安全且有效。     

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。     

老年肺心病死亡患者的氧动力学变化

目的探讨老年肺心病死亡患者的氧动力学变化。方法选取1980～2000年间入住我院肺心病患者76例,随访5年,期间对死亡43例及存活33例的患者进行各项血流动力学及氧动力学监测对比。对并发消化道出血及感染性休克患者死亡前24～72h行血流动力学及氧动力学监测。结果死亡组及存活组平均肺动脉压(MPAP)分别为(41.3±10.6)mmHg,(28.9±6.5)mmHg(P<0.01);心脏指数(CI)分别为(1.92±0.41)L.min-1.m-2,(2.6±0.48)L.min-1.m-2(P<0.01);死亡组动脉血氧分压(PaO2)为(42.3±4.3)mmHg、氧输送(DO2)与氧耗(VO2)均较存活组明显下降、DO2与VO2呈正相关(r=0.71)。死亡年限愈短,MPAP愈高,CI、DO2、VO2、PaO2愈低。死亡的43例患者临终前39例(90.6%)有复合酸碱平衡紊乱,25例(58.13%)痰菌培养出致病菌57株。41例(95.3%)出现多器官功能障碍综合征(MODS)。12例肺心病感染性休克与消化道出血的临终前24～72h血流动力学及氧动力学变化明显的不同。结论导致肺心病的死亡除严重的低PaO2外,还存在组织氧的释放、摄取和利用障碍。     

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。     

吸入伊洛前列素在儿童先天性心脏病肺血管扩张试验中的应用

目的:该研究在心导管检查中通过对先天性心脏病(CHD)合并肺动脉高压(PAH)儿童,吸入伊洛前列素前后的血流动力学参数的测定,评价吸入伊洛前列素在CHD相关性PAH儿童急性肺血管扩张试验的安全性、有效性。方法:研究选择2007年6月至2010年5月,于北京安贞医院小儿心脏科住院的左向右分流型CHD合并重度PAH患儿,所有患儿行左、右心导管检查,并采用雾化吸入伊洛前列素,监测吸药前后血流动力学参数改变及不良反应。结果:该研究入选43例左向右分流型CHD合并重度PAH患儿,年龄(8.52±4.76)岁。吸入伊洛前列素后肺动脉平均压由(77.14±12.14)mmHg(1 mmHg=0.133 kPa)降至(69.35±12.14)mmHg(P<0.05);肺体循环血流量比值(Qp/Qs)由1.80±1.26升至3.06±2.90(P<0.05);肺血管阻力指数(PVRI)下降约5 Wood unit.m2〔吸入前(14.51±8.46)Wood unit.m2,吸入后(9.58±7.43)Wood unit.m2,P<0.05),体循环血压无明显变化〔吸入前(83.16±13.69)mmHg,吸入后(83.09±11.03)mmHg,P>0.05〕。以吸入伊洛前列素后PVRI较基础水平下降至少20%以上作为急性肺血管扩张试验阳性标准。肺血管反应阳性患儿31例,占72%,阴性反应患儿(PVRI下降<20%)12例,占28%。结论:对左向右分流型CHD合并重度PAH儿童在心导管检查中吸入伊洛前列素可明显降低肺血管阻力,体循环血压无明显变化,无明显不良反应发生,可作为安全有效的急性肺血管扩张试验的用药。     

老年原发性高血压患者红细胞分布宽度与早期肾功能损害的相关性分析

目的探讨老年原发性高血压患者红细胞分布宽度(RDW)与早期肾功能损害的关系。方法入选2014年7~12月首都医科大学宣武医院高血压门诊364例老年原发性高血压患者作为观察组,另连续选取100名同期年龄匹配的体检健康者作为对照组,记录两组年龄、性别、收缩压、舒张压、体质指数(BMI)、空腹血糖(FPG)、三酰甘油(TG)、总胆固醇(TC)、低密度脂蛋白胆固醇(LDL-C)、高密度脂蛋白胆固醇(HDL-C)、尿酸、肌酐、尿素氮、尿蛋白定性及血常规等。采用肾脏病饮食改良(MDRD)简化公式计算估算肾小球滤过率(eGFR),根据eGFR水平将高血压组患者分为两组:eGFR下降组(eGFR<90 ml·min-1·1.73 m-2)102例与e GFR正常组(eGFR≥90 ml·min-1·1.73 m-2)262例。并比较RDW、eGFR、BMI和生化指标等参数在各组间的变化差异,分析RDW与eGFR的相关性。结果 (1)与对照组比较,高血压组患者BMI[(25.84±2.95)kg/m2比(23.79±3.12)kg/m2]、TG[(1.96±1.34)mmol/L比(1.58±0.69)mmol/L]、血尿酸[(325.37±88.97)mmol/L比(296.63±80.11)mmol/L]和RDW(13.05%±0.87%比12.59%±0.61%)水平升高,HDL-C[(1.43±0.38)mmol/L比(1.61±0.33)mmol/L]和e GFR[(104.63±28.47)ml·min-1·1.73 m-2比(124.49±25.96)ml·min-1·1.73 m-2]水平下降,差异均有统计学意义(均为P<0.05);(2)在高血压患者中,与e GFR正常组比较,e GFR下降组的年龄[(62.6±9.3)岁比(59.9±8.8)岁]、BMI[(26.99±3.05)kg/m2比(25.39±2.78)kg/m2]、收缩压[(163.44±15.18)mm Hg比(154.42±12.27)mm Hg]、高血压病程[(12.4±3.7)年比(9.8±3.6)年]、血尿酸[(358.84±97.93)mmol/L比(312.34±81.79)mmol/L]、肌酐[(87.56±21.16)μmol/L比(58.60±11.01)μmol/L]、尿蛋白阳性率(46.10%比21.00%)及RDW(13.45%±0.94%比12.90%±0.79%)均升高,而eGFR[(73.85±12.32)ml·min-1·1.73 m-2比(116.61±23.54)ml·min-1·1.73 m-2]下降,差异均有统计学意义(均为P<0.05);经Pearson相关分析显示,高血压组患者RDW与eGFR水平呈负相关(r=-0.237,P=0.000);(3)Logistic多元回归分析显示,RDW是eGFR的危险因素(OR=1.485,P=0.015)。结论老年原发性高血压患者的RDW与eGFR呈负相关,RDW可作为评估老年原发性高血压患者早期肾功能损害的预测因素。     

Prospective cardiopulmonary screening program to detect chronic thromboembolic pulmonary hypertension in patients after acute pulmonary embolism

Background

Chronic thromboembolic pulmonary hypertension after pulmonary embolism is associated with high morbidity and mortality. Understanding the incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism is important for evaluating the need for screening but is also a subject of debate because of different inclusion criteria among previous studies. We determined the incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism and the utility of a screening program for this disease.

Design and Methods

We conducted a cohort screening study in an unselected series of consecutive patients (n=866) diagnosed with acute pulmonary embolism between January 2001 and July 2007. All patients who had not been previously diagnosed with pulmonary hypertension (PH) and had survived until study inclusion were invited for echocardiography. Patients with echocardiographic suspicion of PH underwent complete work-up for chronic thromboembolic pulmonary hypertension, including ventilation-perfusion scintigraphy and right heart catheterization.

Results

After an average follow-up of 34 months of all 866 patients, PH was diagnosed in 19 patients by routine clinical care and in 10 by our screening program; 4 patients had chronic thromboembolic pulmonary hypertension, all diagnosed by routine clinical care. The cumulative incidence of chronic thromboembolic pulmonary hypertension after all cause pulmonary embolism was 0.57% (95% confidence interval [CI] 0.02–1.2%) and after unprovoked pulmonary embolism 1.5% (95% CI 0.08–3.1%).

Conclusions

Because of the low incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism and the very low yield of the echocardiography based screening program, wide scale implementation of prolonged follow-up including echocardiography of all patients with pulmonary embolism to detect chronic thromboembolic pulmonary hypertension does not seem to be warranted.     

Epoprostenol in chronic thromboembolic pulmonary hypertension with distal lesions.

BACKGROUND: Although patients with primary pulmonary hypertension and patients with chronic thromboembolic pulmonary hypertension with distal lesions may share similar pathophysiological characteristics, scarce information is available on the usefulness of epoprostenol in this form of secondary pulmonary hypertension. The aim of this study was to evaluate the feasibility, safety and efficacy of epoprostenol therapy in surgically untreatable patients with chronic thromboembolic pulmonary hypertension. METHODS: Continuous infusive therapy with epoprostenol was undertaken in 16 patients with primary pulmonary hypertension and in 11 surgically untreatable thromboembolic pulmonary hypertension patients. The median follow-up was 12.4 months (range 6-23 months). Patients underwent clinical, echocardiographic and hemodynamic evaluation at baseline and a 6-min walk test every 3 months after beginning epoprostenol; ultrasound evaluations were repeated in a subgroup of patients. RESULTS: Epoprostenol therapy improved the clinical status, exercise tolerance and NYHA functional class. A greater left ventricular end-diastolic volume was recorded at echocardiography in both groups. CONCLUSIONS: Epoprostenol therapy may be feasible, safe and clinically effective in patients with surgically untreatable chronic thromboembolic pulmonary hypertension.     

Thrombotic risk factors in pulmonary hypertension.

Thrombotic lesions are consistently observed in chronic thromboembolic pulmonary hypertension (CTEPH) and frequently found in primary pulmonary hypertension (PPH). It remains unknown, however, whether thrombosis is related to defects of the antithrombotic pathway or to previous vascular injury. This study therefore analysed the frequency of both hereditary and acquired thrombotic risk factors in CTEPH and PPH. One hundred and forty-seven consecutive patients with CTEPH investigated in the author's institution were compared to 99 consecutive patients with PPH. In 116 CTEPH patients and 83 PPH patients, phospholipid-dependent antibodies (antiphospholipid antibodies and lupus anticoagulant) were analysed by both immunological and clotting assays. In patients enrolled since 1994 (46 CTEPH and 64 PPH), hereditary thrombotic risk factors were also determined. Antithrombin, protein C and protein S activities were measured by functional assays. Mutations of factor V and factor II were identified by polymerase chain reaction. The prevalence of hereditary thrombotic risk factors was not increased in patients with either PPH or CTEPH. In contrast, a high frequency of phospholipid-dependent antibodies was observed in PPH (10%) and more notably in CTEPH (20%). Moreover, in PPH, antibodies were present only in low titre whereas in CTEPH, half of the patients with antiphospholipid antibodies had high titres. In addition, in CTEPH all but one of the patients with lupus anticoagulant also had antiphospholipid antibodies. The most striking finding of this study was the high prevalence of phospholipid-dependent antibodies but their clinical relevance appears to be different in primary pulmonary hypertension and chronic thromboembolic pulmonary hypertension. In primary pulmonary hypertension, these antibodies in low titre probably reflect endothelial dysfunction. In contrast, in chronic thromboembolic pulmonary hypertension the presence of antibodies in high titre associated with lupus anticoagulant, underlines the role of thrombosis in the pathogenesis of this condition.     

Differentiation of patients with primary and thromboembolic pulmonary hypertension

Twenty-five consecutive patients with obliterative pulmonary hypertension were studied. Primary pulmonary hypertension (17 patients) or chronic thromboembolic pulmonary hypertension (eight patients) was diagnosed by pulmonary angiography or autopsy. Clinical symptoms, physical findings, chest roentgenograms, electrocardiograms, and pulmonary function studies did not differentiate the patients with primary pulmonary hypertension (PPH) from those with chronic thromboembolic pulmonary hypertension (TPH). All eight patients with TPH had a lung scan interpreted as high probability for pulmonary emboli while all 17 patients with PPH had a lung scan interpreted as normal or low probability for emboli. While there was close clinical similarity between patients with PPH and TPH, the presence of a normal or low probability lung scan excluded the diagnosis of TPH.     

Primary pulmonary hypertension and thromboembolic pulmonary hypertension--similarities and differences

In a retrospective study of 36 patients with primary pulmonary hypertension (PPH) and 16 patients with chronic large vessel thromboembolic pulmonary hypertension (TPH) the diagnostic value of clinical features, chest radiographs, electrocardiographs, radionuclide lung scanning and cardiac catheterization was assessed. PPH patients were younger, had higher prevalence of Raynaud's phenomenon, right axis deviation, right ventricular hypertrophy on electrocardiograph and higher pulmonary artery pressures than TPH patients, but these features were not diagnostic. Although pulmonary angiography is considered by some to be necessary in distinguishing these two conditions, radionuclide lung scanning proved a safe and effective noninvasive method for this purpose.     

Comparison of bronchopulmonary collaterals and collateral blood flow in patients with chronic thromboembolic and primary pulmonary hypertension.

OBJECTIVE: To compare the visualisation of bronchopulmonary collaterals and bronchopulmonary collateral blood flow in patients with chronic thromboembolic pulmonary hypertension 2nd primary pulmonary hypertension. SETTING: Referral centre for cardiology at an academic hospital. PATIENTS: Nine patients with chronic thromboembolic pulmonary hypertension and 17 with primary pulmonary hypertension. INTERVENTIONS: Bronchopulmonary collaterals were visualised by selective bronchial arteriography or thoracic aortography. Bronchopulmonary collateral blood flow was estimated by injecting indocyanine green into the ascending aorta and sampling below the mitral valve from the left ventricle. RESULTS: The degree of pulmonary hypertension was comparable in the two groups. Large bronchopulmonary collaterals were visualised in all the patients with thromboembolic pulmonary hypertension who had bronchial arteriography or aortography or both. None of the primary pulmonary hypertension group studied by aortography had bronchopulmonary collaterals (P < < 0.001). All the patients with chronic thromboembolic pulmonary hypertension had significant bronchopulmonary collateral blood flow, which was (mean (SD)) 29.8 (18.6)% of the systemic blood flow. There was no recordable collateral blood flow in 11 of 15 patients with primary pulmonary hypertension. In the remaining four patients the mean value was 1.1 (1.8)% of the systemic blood flow (P < < 0.001). CONCLUSIONS: Visualisation of bronchopulmonary collaterals by thoracic aortography or by bronchial arteriography, or the demonstration of an increased bronchopulmonary collateral flow, helps to distinguish patients with chronic thromboembolic pulmonary hypertension from those with primary pulmonary hypertension.     

Pulmonary hemodynamic and gas exchange effects of various oxygen concentrations in patients with severe pulmonary hypertension primarily affecting the pulmonary vasculature]

The aim of this study was to evaluate pulmonary hemodynamic and gas exchange response to oxygen inhalation in patients with severe pulmonary hypertension primarily affecting the pulmonary vasculature. This study included 7 patients with primary pulmonary hypertension (PPH), 11 with pulmonary hypertension related to collagen vascular diseases (CoPH), and 18 with chronic thromboembolic pulmonary hypertension (CTEPH). All patients had mean pulmonary arterial pressure (PPAm) of greater than 25 mm Hg. We divided the patients into two groups: a PPH + CoPH group comprising the 7 PPH and 11 CoPH patients, and the CTEPH group. We measured cardiopulmonary variables after 10 min inhalation of various oxygen concentrations (FiO2 0.24, 0.28, 0.4, 1.0). In the PPH + CoPH group, PPAm significantly decreased after the inhalation of oxygen concentrations of 40% or more. This was associated with a significant reduction in pulmonary arteriolar resistance (PAR), and suggested active pulmonary vasodilation was caused by oxygen inhalation. In the CTEPH group, on the other hand, PPAm significantly decreased after the inhalation of oxygen concentrations of 28% or more, apparently in association with a significant fall of cardiac output. However, PAR was unchanged regardless of the inspired oxygen concentration, indicating an absence of pulmonary vasodilation in the CTEPH group. When breathing room air, 7 patients in the PPH + CoPH group (38.9%) and 10 in the CTEPH group (55.6%) demonstrated mixed venous oxygen tension (PvO2) values of less than 35 Torr. Extra attention should be paid to PvO2 when administering oxygen therapy to patients with severe pulmonary hypertension.     

Pulmonary angiography in severe chronic pulmonary hypertension

We have analyzed the safety of doing pulmonary angiography in 67 consecutive patients with moderate-to-severe primary pulmonary hypertension or hypertension secondary to chronic thromboembolic occlusions of the pulmonary arteries. The average (+/- SD) pulmonary arterial systolic and diastolic pressures were 74 +/- 19 and 34 +/- 10 mm Hg, respectively. Fourteen patients had a right ventricular end-diastolic pressure of 20 mm Hg or more. Selective left and right main pulmonary artery injections were done using ionic contrast agents in 56 patients and nonionic contrast agents in 11. No major rhythm disturbances or systemic hypotension requiring therapy occurred, and there were no deaths. Thrombotic occlusions of the pulmonary arteries were identified in 52 patients and confirmed in all 42 of those who had a thromboendarterectomy. At autopsy, 3 of the 15 patients who had normal angiograms were found not to have had thrombotic occlusions. We conclude that pulmonary angiography can be done safely despite the presence of severe pulmonary hypertension and right ventricular failure, and that the procedure leads to the identification of chronic, major-vessel thromboembolic pulmonary hypertension that may be alleviated by thromboendarterectomy.     

Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension.

For chronic thromboembolic pulmonary hypertension not amenable to pulmonary endarterectomy, effective medical therapy is desired. In an open-label uncontrolled clinical trial, 104 patients (mean +/- sem age 62 +/- 11 yrs) with inoperable chronic thromboembolic pulmonary hypertension were treated with 50 mg sildenafil t.i.d. At baseline, patients had severe pulmonary hypertension (pulmonary vascular resistance 863 +/- 38 dyn.s.cm(-5)) and a 6-min walking distance of 310 +/- 11 m. Eight patients were in World Health Organization functional class II, 76 in class III and 20 in class IV. After 3 months' treatment, there was significant haemodynamic improvement, with reduction of pulmonary vascular resistance to 759 +/- 62 dyn.s.cm(-5). The 6-min walking distance increased significantly to 361 +/- 15 m after 3 months' treatment, and to 366 +/- 18 m after 12 months' treatment. A subset of 67 patients received a single dose of 50 mg sildenafil during initial right heart catheterisation. The acute haemodynamic effect of this was not predictive of long-term outcome. In this large series of patients with inoperable chronic thromboembolic pulmonary hypertension, open-label treatment with sildenafil led to significant long-term functional improvement. The acute effect of sildenafil may not predict the long-term outcome of therapy.