The pulmonary venous wedge pressure in pulmonary arterial hypertension

The comparability of the main pulmonary artery pressure (PAP) and the pulmonary venous wedge pressure (PVWP) was assessed during cardiac catheterization in 89 patients with pulmonary artery hypertension (PAH) and increased pulmonary blood flow. Preliminary evaluation revealed a wide disparity between the 2 determinations. Fifty-five pull-back pressure recordings from branch-to-main pulmonary artery were analyzed. Twenty-four percent (13/55) had systolic pressure gradients >20 mm Hg. between branch and main pulmonary artery. When PVWP and only ipsilateral branch PAP were compared (n=48), diastolic and mean (m), but not systolic PVWP, correlated closely with branch PAP (r=0.77, r=0.73 and r=0.59, respectively). In 46 of 48 patients the PVWP_m was not significantly greater than the ipsilateral PAP. Twenty-nine of 30 patients with PVWP_m <30 mm Hg. had an ipsilateral PAP_m <40 mm Hg. In 15 patients with PVWP_m between 30 and 39 mm Hg, there was a wide range (30–59 mm Hg) of PAP_m. Three patients with PVWP_m >40 mm Hg. had severe PAH. It is concluded that: 1) hemodynamically significant branch-to-main PAP gradients are present in some patients with PAH and may result in erroneously high pulmonary arteriolar vascular resistance when calculated from main PAP; 2) properly performed PVWP determination can define the lower limit of mean pressure in the ipsilateral branch pulmonary artery; 3) a PVWP_m <30 mm Hg. usually indicates an ipsilateral PAP_m <40 mm Hg; 4) a PVWP_m >30 mm Hg. is compatible with either moderate or severe PAH; 5) correlation of PVWP with PAP is not related to pulmonary blood flow.     

Preventive treatment with atorvastatin ameliorates endothelial dysfunction of small pulmonary arteries in monocrotaline-induced pulmonary hypertensive rats

This study examined the effects of preventive atorvastatin (Ator) treatment on vasodilatation of small pulmonary arteries (SPAs) in monocrotaline (MCT)-induced pulmonary hypertensive rats. SD rats were randomly assigned to: normal control (Ctr), pulmonary arterial hypertension (PAH), PAH treated with 5 mg/kg/d Ator (LAtor), or 10 mg/kg/d Ator (HAtor). PAH was induced by MCT injection (40 mg/kg, i.p.). Mean pulmonary artery pressure (mPAP), right ventricular hypertrophy index (RVHI%), endothelium-dependent relaxations (EDdRs), and endothelium-independent relaxations (EDiRs) were determined. Four weeks after MCT injection, mPAP was higher in PAH group compared to that in Ctr group, and this effect was suppressed by Ator treatment (PAH: 32.19 ± 0.91 mm Hg vs. LAtor: 19.13 ± 1.01 mm Hg, HAtor: 17.55 ± 0.20 mm Hg, p < 0.05). Similar trend of changes in RVHI% was found in the same way. EDdRs of SPA rings in PAH group were markedly decreased 2 and 4 weeks after MCT injection, while in Ator treated groups, the impairment can only be detected 4 weeks after MCT injection. There were no differences in EDiRs among all groups 1 week after MCT injection. However, 2 weeks and 4 weeks after MCT injection, EDiRs were significantly impaired, while in HAtor and LAtor groups, EDiR was only impaired 4 weeks but not 2 weeks after MCT injection. Preventive treatment with atorvastatin for 2 weeks ameliorated endothelium-dependent and endothelium-independent vasodilative dysfunction in small pulmonary artery rings of MCT-induced PAH rats. It suggests that MCT-induced damage of endothelial function was progressing, and Ator was only beneficial in the early stage of MCT-induced PAH.     

Evolution and prognosis of pulmonary hypertension in chronic obstructive pulmonary diseases

The prognostic value of the presence and degree of pulmonary arterial hypertension (PAH) in chronic obstructive pulmonary diseases (COPD) has been well established by recent studies including the European WHO multicentric study where 591 COPD patients have been included. The level of pulmonary artery mean pressure (PAP) is one of the best predictors of mortality in these patients along with FEV1 and arterial blood gases. The natural history of the course of PAP in COPD was relatively obscure until recent years. The first studies in that field were performed in 1968 but included small numbers of patients. In consideration of the data of three studies published in 1977 to 1979 which concern a relatively homogeneous group of 163 patients with severe COPD, it appears that changes in PAP after an average follow-up period of four years are rather small from 21.4 to 23.6 mm Hg (0.5 to 0.6 mm Hg/year). These results have been confirmed by a very recent study of our group including 93 COPD patients followed-up for five to twelve years (mean = 90 months): the yearly increase in PAP was of only 0.5 mm Hg. Long-term oxygen therapy (LTO) for greater than or equal to 15 h/day rarely normalizes PAP, but can reverse the progression of PAH, particularly in those patients who exhibit a marked worsening of PAH due to a progressive deterioration of arterial blood gases. Very recent studies have clearly indicated that LTO had beneficial hemodynamic effects in a high percentage of COPD patients with PAH.     

Balloon pulmonary valvuloplasty in the management of cyanotic congenital heart defects

Twenty-three children with cyanotic congenital heart defects, aged 3 days to 11.5 years, weighing 2.9 to 30 kg, underwent percutaneous balloon pulmonary valvuloplasty to improve pulmonary oligemia. The patients were divided into two groups: group I with intact ventricular septum and group II with ventricular septal defect. In 12 group I patients, there was an increase of systemic arterial oxygen saturation [83 ± 8% (mean ± SD) vs. 94 ± 5%, P < 0.001] and pulmonary-to-systemic flow ratio (0.7 ± 0.1 vs. 1.0 ± 0.2, P < 0.001). Peak systolic pressure gradient across the pulmonary valve decreased (P > 0.001) from 105 ± 48 to 25 ± 18 mm Hg. In 11 group II patients, arterial oxygen saturation (67 ± 13 vs. 83 ± 13%, P < 0.01) and pulmonary-to-systemic flow ratio (0.7 ± 0.4 vs. 1.2 ± 0.5, P < 0.02) increased following valvuloplasty. Peak systolic pressure gradient across the pul-monic valve (52 ± 16 vs. 32 ± 22 mm Hg, P < 0.05) decreased while infundibular and total pulmonary outflow tract gradients were unchanged (P > 0.1). Immediate surgical intervention was avoided in all cases in both groups. On follow-up, 1 to 36 months after valvuloplasty, arterial oxygen saturation, pulmonary-to-systemic flow ratio, and pulmonary valve gradients remain improved in both groups. However, in group I, repeat balloon valvuloplasty was required in two children. In group II, six children with tetralogy of Fallot (TOF) underwent successful total surgical correction 4 months to 2 years after valvuloplasty. Two children with transposition of the great arteries required Blalock-Taussig shunts 6 and 18 months after valvuloplasty for further improvement of hypoxemia. Demonstrable increase in pulmonary artery diameter occurred in several children. These data suggest that pulmonary valvuloplasty offers an excellent relief of pulmonary valve obstruction in the intact ventricular septum group and palliation of pulmonary oligemia in the ventricular septal defect group. In the latter group, avoiding immediate surgical shunts in all patients and achieving growth in pulmonary arterial size in many patients, thus making them suitable for further surgical palliation or correction, are additional advantages.     

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.     

Total anomalous pulmonary venous connection. Results of repair in 50 infants

Fifty infants with isolated total anomalous pulmonary venous connection (TAPVC) were operated between 1/01/73 and 31/12/87. The average weight at surgery, which was performed under hypothermia with circulatory arrest in 92 per cent of cases, was 4.5 Kg. The preoperative pulmonary to systemic pressure ratios (PAP/PS) enabled identification of two groups of patients: Group I: TAPVC without severe pulmonary hypertension (PAP/PS less than 0.85) (n = 35), and Group II: TAPVC with severe pulmonary hypertension (PAP/PS greater than 0.55) (n = 15). The hospital mortality was 22 per cent (8 cases) in Group I compared with 73 per cent (11 cases) in Group II (p less than 0.05). Patients in Group II were younger (64 days compared with 137 days, p less than 0.02), lighter (p less than 0.05) and had preoperative mean pulmonary artery systolic pressures of 83 mmHg (p greater than 0.001). Three patients in Group I required early reoperation for stenosis of the pulmonary veins at the site of repair resulting in pulmonary hypertension, and all died. The global survival was 28 patients with an average follow-up of 7 years (range 1 to 15 years). Six of these patients were reoperated (2 phrenoplications, 4 atrial shunts). All survivors are asymptomatic and have no conduction defects. Control echocardiography in 15 of the 28 survivors was judged to be normal. These results show that obstructive forms of TAPVC (Group II) carry a very poor prognosis: immediate results in this group could only be improved by earlier surgery. The clinical long-term results in those who survive surgery are very satisfactory.     

Characterization of brain natriuretic peptide in long-term follow-up of pulmonary arterial hypertension

STUDY OBJECTIVES: Pulmonary arterial hypertension (PAH) leads to substantial morbidity and mortality. Noninvasive parameters in the follow-up assessment of PAH could be helpful in clinical decision making. The brain natriuretic peptide (BNP) has been shown to correlate with the functional status and prognosis of these patients and could be a valuable parameter in this respect. The aim of our study was to investigate whether BNP levels could reflect clinical and hemodynamic changes, including the response to therapy during long-term follow-up in patients with PAH. STUDY DESIGN: We measured pulmonary hemodynamics, functional parameters including the 6-min walk distance (6MWD), and plasma BNP levels at baseline and after a mean (+/- SEM) follow-up period of 12.6 +/- 1.5 months in patients with PAH. RESULTS: In group A (n = 18), with decreasing BNP levels mean pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR) decreased (PAP, 60.89 +/- 3.44 to 53.47 +/- 3.24 mm Hg; PVR, 1,207.47 +/- 111.75 to 942.35 +/- 103.15 dyne.s.cm(-5); p < 0.01) and 6MWD increased (408.24 +/- 29.57 to 470 +/- 25.54 m; p < 0.01). In group B (n = 12), with increasing BNP levels mean PAP and PVR increased (PAP, 52 +/- 3.31 to 60.17 +/- 5.03 mm Hg; PVR, 946.13 +/- 115.35 to 1,236.6 +/- 180.23 dyne . s . cm(-5); p < 0.01) and mean 6MWD decreased from 463.64 +/- 27.77 to 367.27 +/- 38.87 m (p < 0.05). Comparing groups revealed statistically significant differences regarding changes in PAP (group A, -11.58 +/- 3.57%; group B, +13.29 +/- 5.44%; p = 0.001) and PVR (group A, -19.21 +/- 5.87%, group B, +30.35 +/- 7.72%; p < 0.001). Correlations existed between the changes in BNP levels and pulmonary hemodynamics. CONCLUSION: We concluded that BNP levels parallel changes in pulmonary hemodynamics and functional parameters, including the 6MWD, in PAH patients. Consequently, we suggest BNP as a parameter for the follow-up assessment of PAH patients.     

系统性红斑狼疮肺动脉高压63例临床分析

目的 探讨系统性红斑狼疮(SLE)并肺动脉高压(PAH)患者的临床特点、发病机制以及诊治方法.提高对该病的认识.方法 选取本院近5年住院的SLE合并PAH的患者63例,对其临床症状、实验室指标、超声心动图特点、SLE病情活动评分等进行回顾性分析.结果 该组患者抗RNP抗体阳性率较高,肺动脉压力与SLE病情活动评分无关,PAH的轻重与血液中自身抗体的出现无明显关系,PAH重者易有雷诺现象发生,而且与其他系统受累无关联,比较有无肾脏受累的患者在临床以及实验室指标的不同,没有明显差别.结论 SLE患者PAH发生与其他脏器受累无关,发病隐匿,临床上应该重视SLE肺动脉压的筛查,早期发现和治疗以利于病情的控制和改善预后.     

Hemodynamic effects of nifedipine versus hydralazine in primary pulmonary hypertension

The acute hemodynamic effects of both sublingual nifedipine (N) and intravenous hydralazine (Hy) were studied in 5 patients with primary pulmonary hypertension to ascertain whether the capacity for pulmonary vasodilatation was generalized or drug-specific, and to determine which of the 2 agents had preferential pulmonary vasodilatory effects. For the group as a whole, neither N nor Hy produced changes in heart rate, mean pulmonary capillary wedge or right atrial pressures. Both N and Hy reduced mean systemic arterial pressure (before N 90 ± 8 mm Hg, after N 76 ± 7 mm Hg, p < 0.01; before Hy 92 ± 11 mm Hg, after Hy 68 ± 8 mm Hg, p < 0.05), and decreased systemic vascular resistance (before N 1,558 ± 645 dynes s cm^?5, after N 1,192 ± 430 dynes s cm^?5, p < 0.05; before Hy 1,700 ± 415 dynes s cm^?5, after Hy 957 ± 285 dynes s cm^?5, p < 0.05). In addition, N administration resulted in an increased cardiac output (before N 4.5 ± 2.0 liters/min, after N 4.8 ± 2.0 liters/min, p < 0.01); Hy administration was associated with a more varied effect on cardiac output (before Hy 4.0 ± 1.0 liters/min, after Hy 5.3 ± 1.8 liters/min, p < 0.10, difference not significant [NS]). Although for the group neither agent decreased mean pulmonary artery pressure (PAP) (before N 51 ± 13 mm Hg, after N 44 ± 13 mm Hg, NS; before Hy 50 ± 15 mm Hg, after Hy 51 ± 15 mm Hg, NS) or pulmonary vascular resistance (before N 873 ± 458 dynes s cm^?5, after N 680 ± 450 dynes s cm^?5, NS; before Hy 945 ± 454 dynes s cm^?5, after Hy 715 ± 309 dynes s cm^?5, NS), 4 of 5 patients had a decrease in PAP after N and 1 had no change, and only 1 of 5 patients had a decreased PAP after Hy administration. Moreover, compared with the changes after Hy administration, PAP declined after N administration (δPAP after Hy 0.2 ± 9 mm Hg, after N ?7 ± 9 mm Hg, p < 0.05). The PA diastolic to mean PA wedge pressure gradient tended to decrease after N administration (after N ?5.0 ± 10 mm Hg, after Hy +4.7 ± 7 mm Hg, NS), suggesting more pulmonary vasodilatation after N administration. Moreover, the ratio of pulmonary to systemic vascular resistances was unchanged after N but increased after Hy administration (before N 0.55 ± 0.2, after N 0.53 ± 0.2, NS; before Hy 0.55 ± 0.2, after Hy 0.74 ± 0.3, p < 0.02), indicating the more balanced vasodilatory effect of N. Two patients were treated chronically with Hy but had intolerable adverse effects; 1 was subsequently treated successfully with N. A third patient had pulmonary edema (presumably neurogenic) 30 minutes after Hy administration; this patient later died. Another patient has symptomatically improved with chronic N therapy. Thus, N appears to be a more specific pulmonary arterial vasodilator than Hy in acute drug testing; in this small group of patients with primary pulmonary hypertension, N appears to be more efficacious when administered chronically.     

The role of isoproterenol in the preoperative evaluation of high-pressure, high-resistance ventricular septal defect

The experience we describe derives from the short-term administration of isoproterenol in 15 patients with ventricular septal defect (VSD) and severe pulmonary artery hypertension (PAH). For the whole study group, mean pulmonary artery pressure (PAP) was 68.5 +/- 2.6 mm Hg, pulmonary vascular resistance (Rp) was 11.6 +/- 0.9 U/m2, pulmonary vascular resistance/systemic vascular resistance ratio (Rp/Rs) was 0.9 +/- 0.03, and the pulmonary vascular gradient (PAd-PWP) was 45 +/- 3.5 mm Hg. Infusions of isoproterenol decreased PAP, Rp, Rp/Rs ratio, and PAd-PWP an average of 10.2 mm Hg, 2.88 U/m2, 0.13 and 6.6 mm Hg, respectively, for the whole group (P less than 0.001). On the basis of isoproterenol response, the patients could be divided into two groups: A (n = 4) and B (n = 11). In group A, the PAP decreased from 61.7 +/- 1 to 45 +/- 4 mm Hg, the Rp from 8.9 +/- 0.3 to 4.62 +/- 0.5 U/m2, the Rp/Rs from 0.84 +/- 0.02 to 0.55 +/- .05, and the PAd-PWP from 34.5 +/- 0.9 to 24 +/- 2 mm Hg (mean +/- 1 SE). In group B a less significant change in these measurements was observed. Group A patients underwent VSD repair, and the mean average postoperative decrease in PAP was 31 mm Hg (P less than 0.001). Our findings suggest that in patients with VSD and severe PAH, in whom surgical treatment is controversial, a trial with isoproterenol should be routinely attempted. If the preceding hemodynamic parameters improve significantly, the VSD repair should be performed.     

Two‐ and four‐dimensional echocardiography with high‐definition flow imaging and spatiotemporal image correlation in the diagnosis of fetal isolated partial anomalous pulmonary venous connection

Partial anomalous pulmonary venous connection (PAPVC) is a rare malformation. We describe a case of PAPVC, in which the left pulmonary veins coursed to the left innominate vein through a vertical vein and finally drained into the right superior vena cava; the right pulmonary veins were connected to the left atrium. Tracing the origin and destination of abnormal vessels presented at the three‐vessel and trachea view is useful for the diagnosis. Four‐dimensional echocardiography with high‐definition flow imaging and spatiotemporal image correlation facilitates the identification of the drainage of fetal pulmonary veins, which should be considered as a complementary modality in obstetric ultrasonic examination when cardiac abnormalities are suspected.     

Usefulness of latent left ventricular dysfunction assessed by Bowditch Treppe to predict stress-induced pulmonary hypertension in minimally symptomatic severe mitral regurgitation secondary to mitral valve prolapse

We assessed whether the presence of latent myocardial dysfunction, evaluated by echocardiographic derived force-frequency relationship (FFR) during exercise, predicts the appearance of stress-induced pulmonary hypertension in minimally symptomatic patients with severe mitral regurgitation (MR). Two groups of patients were identified: group I with normal (40 mm Hg) peak stress systemic pulmonary artery pressure. Group I had normal and upsloping FFR and group II had abnormal flat or biphasic FFR. Therefore, in patients with severe MR and apparently normal left ventricular function, the stress-induced pulmonary hypertension seems to be related to the presence of latent left ventricular dysfunction.     

Prevalence of elevated pulmonary arterial pressures measured by echocardiography in a multicenter study of patients with systemic sclerosis

OBJECTIVE: To estimate the prevalence of elevated pulmonary arterial pressures (PAP) as a correlate for pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc) in rheumatology centers in Canada. METHODS: During the one-year study period (June 2002-May 2003), charts of patients with SSc were reviewed to determine demographics, SSc characteristics, percentage of patients with a PAH diagnosis, and the site criteria for such diagnosis. Subjects with no PAH that had symptoms of dyspnea and/or fatigue were invited to undergo Doppler echocardiography to estimate their systolic PAP (sPAP). RESULTS: A total of 539 patients with SSc (age 56 yrs +/- 13 SD, 84% female, 41% with diffuse SSc, 58% limited SSc, SSc disease duration 9 yrs +/- 7 SD) at 8 rheumatology centers were reviewed. Twenty-three percent of patients were diagnosed with elevated sPAP based on the site diagnosis criteria (i.e., > 30 mm Hg or > 35 mm Hg). From the non-PAH, not recently screened patients that had symptoms of dyspnea or fatigue, a total of 89 patients underwent a Doppler echocardiograph; 40% had sPAP > 35 mm Hg. CONCLUSION: Elevated PAP are common in both limited and diffuse SSc disease, occurring in 21% of limited and 26% of diffuse SSc patients. During the screening most patients had mild PAP elevations that would require further assessments such as right heart catheterization to diagnose PAH where appropriate. A high index of suspicion is important and routine echocardiography in symptomatic patients may allow earlier diagnosis of PAH and intervention. The proportion of SSc patients with mild to moderate elevations of PAP who will develop significant PAH is unknown and longterm studies are needed to address the natural progression.     

The efficacy of using pulmonary vein wedge pressure for the estimation of pulmonary artery pressure in children with congenital heart disease.

The objectives of this study were to assess the accuracy of pulmonary vein wedge pressure (PVWP) in estimating pulmonary artery pressure (PAP) in various types of congenital heart disease, including single-ventricle physiology. The systolic, diastolic, and mean values of both PAPs and PVWPs were measured in 30 patients (a total of 46 points). Pulmonary artery pressure ranged from 13 to 74 (34 +/- 15) mm Hg in systole, 5 to 25 (13 +/- 6) mm Hg in diastole, and 6 to 48 (18 +/- 10) mm Hg in mean. As a whole, good correlations between PAPs and PVWPs were observed (systole, r = 0.70; diastole, r = 0.85; mean, r = 0.82; P < 0.0001). However, with an increase in PAP, the discrepancy between PAPs and PVWPs increased. When the mean PVWP was more than 18 mm Hg, the mean PVWP in 14 of 24 (58%) underestimated the mean PAP by up to 22 mm Hg (mean difference, -1.7 +/- 5.8 mm Hg). On the other hand, all of the patients with mean PVWPs less than 18 mm Hg (n = 22) had mean PAPs less than 18 mm Hg (r = 0.86; PAP = 1.11 x PVWP - 1.41; P < 0.0001), and the mean difference was -0.2 +/- 1.8 mm Hg. The mean PVWP can accurately estimate the mean PAP in children with congenital heart disease who have a mean PVWP less than 18 mm Hg.     

Efficacy of balloon valvuloplasty in treating mild pulmonary stenosis.

Balloon pulmonary valvuloplasty (BPV) is a well accepted treatment for moderate and severe pulmonary stenosis. However, the efficacy of BPV in treating mild pulmonary stenosis is still unanswered. Therefore, the efficacy of BPV in treating patients with mild pulmonary stenosis was compared to that in treating patients with moderate or severe pulmonary stenosis. A total of 46 patients with pulmonary stenosis were arbitrarily divided into 3 groups; Group I consisted of 9 patients with a pressure gradient less than 40 mm Hg, Group II consisted of 5 patients with a pressure gradient ranged from 40 to 50 mm Hg, and Group III consisted of 32 patients with a gradient greater than 50 mm Hg. Following BPV, the gradient reduced significantly in all 3 groups (p less than 0.05 in each group). The efficacy of BPV in the 3 groups was evaluated and compared by two parameters; one is the percentage of gradient reduction, the other is the right ventricular pressure ratio (RV post-bpv/RV pre-bpv). The percentage of gradient reduction in Group I, II, and III were 28 +/- 20, 55 +/- 17 and 70 +/- 9% respectively. The right ventricular pressure remained 84 +/- 11, 59 +/- 16, and 46 +/- 12% of pre-valvuloplasty level in Group I, II and III respectively. If the efficacy of BPV was compared among the 3 groups. Groups III had the best efficacy and Group I had the worst.(ABSTRACT TRUNCATED AT 250 WORDS)     

慢性阻塞性肺疾病与肺动脉高压自然病程的探讨

目的 探讨慢性阻塞性肺疾病(chronic obstructive pulmonary disease,COPD)并发肺动脉高压(pulmonary arterial hypertension,PAH)的发生和发展.方法 对88例[COPD肺动脉平均压(meanpulmonary arterial pressure,MPAP)≤20 mm Hg(1 mm Hg=0.133 kPa)]进行肺功能、心电图、血气及右心漂浮导管检测血流动力学,每5年一次共随访20年.结果 ①88例COPD随访至终点(20年或死亡),36例并发PAH(A组)死亡24例(66.7%);其余52例未并发PAH(B组)死亡18例(34.6%).MPAP平均每年上升0.51 mm Hg.②COPD急性加重次数>3次/年A组为22例(66.1%),B组为12例(23.1%),两组差异有统计学意义(P<0.05).③COPD急性加重时MPAP、肺血管阻力、内皮素1、血栓素B2均高于稳定期(P<0.05).动脉血氧分压、氧输送量、一氧化氮、6-前列素Fia、均低于稳定期(P<0.05).结论 PAH是COPD自然病程中一种常见的并发症,它的出现可以增加病死率;COPD急性加重频繁发作是诱发COPD并发PAH的重要因素之一.     

Exercise capacity in idiopathic pulmonary fibrosis: the effect of pulmonary hypertension

Background and objective: Increased pulmonary arterial pressure (PAP) usually coexists with impaired lung function in IPF. Data on the effect of pulmonary hypertension (PH) on cardiopulmonary responses during exercise in IPF patients is very limited. We sought to investigate the impact of PH on exercise capacity and the correlation between systolic PAP (sPAP) and pulmonary function testing, as well as cardiopulmonary exercise parameters, in patients with IPF and PH. Methods: Eighty‐one consecutive patients with IPF, who were evaluated over a 6‐year period, were retrospectively studied. Patients underwent pulmonary function testing, Doppler echocardiography and maximal cardiopulmonary exercise testing. PH was defined as sPAP > 35 mm Hg. Results: PH was diagnosed in 57% of the patients. Categorization of patients according to severity of PH indicated a significant reduction in maximum work rate, peak O₂ uptake, anaerobic threshold and peak O₂ pulse in those with sPAP > 50 mm Hg. In IPF patients with PH, estimated sPAP correlated with peak O₂ uptake, anaerobic threshold, peak O₂ pulse and end‐tidal CO₂ at anaerobic threshold, while the strongest correlation was between sPAP and ventilatory equivalent for CO₂ at anaerobic threshold (r = 0.611, P < 0.001). There were no differences in pulmonary function or exercise parameters indicative of lung volume reduction, across the patient categories, and none of these parameters correlated with sPAP. Conclusions: PH has a negative impact on exercise capacity in IPF patients. In IPF patients with PH, resting sPAP correlated with exercise parameters indicative of gas exchange and circulatory impairment, but not with defective lung mechanics.     

The acute effects of 30 mg vs 60 mg of intravenous Fasudil on patients with congenital heart defects and severe pulmonary arterial hypertension

Objective: The optimal dose of Fasudil is still controversial in congenital heart disease accompanied with severe pulmonary hypertension (CHD‐PAH). This study aimed to compare acute hemodynamic changes after different doses of Fasudil in 60 consecu‐ tive adult patients with CHD‐PAH.
Design: Prospective randomized controlled trial.
Setting: Tertiary cardiology center.
Patients: Adult patients with CHD‐PAH.
Interventions: Patients were randomized to Fasudil 30 or 60 mg.
Outcome Measures: The hemodynamic parameters were measured at baseline and after 30 minutes of Fasudil through right cardiac catheterization. Blood gas results were obtained from the pulmonary artery, right ventricle, right atrium, superior and inferior vena cava, and femoral artery. Pulmonary vascular resistance (PVR) and sys‐ temic arterial resistance (SVR) were calculated.
Results: The changes in systolic pulmonary artery pressure (sPAP) (−13.1% vs −9.3%, P < .05), diastolic PAP (dPAP) (−17.6% vs −14.5%, P < .05), mean PAP (mPAP) (−12.4% vs −8.5%, P < .05), and PVR (−35.8% vs −22.2%, P < .05) were more pronounced in the 60‐mg group than in the 30‐mg group. All patients had no obvious adverse reac‐ tions related to peripheral blood pressure.
Conclusions: Fasudil could improve the hemodynamics of patients with CHD‐PAH, especially with the 60‐mg dose. There were no serious adverse reactions.     

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。