Pulmonary hypertension in systemic lupus erythematosus

A prospective study was performed in our center on 60% (n = 36) of patients with systemic lupus erythematosus (SLE) to determine the prevalence and severity of pulmonary hypertension. Twenty-six healthy subjects of similar age and sex served as controls. Pulmonary artery systolic pressure was calculated from the sum of the peak tricuspid insufficiency Doppler pressure gradient and an estimate of right atrial pressure based on inferior vena cava size and its degree of inspiratory collapse. Five patients with SLE (14%) had pulmonary hypertension, defined as pulmonary artery systolic pressure greater than 30 mm Hg. Cardiac indices determined by planimetry of biplane apical 2-dimensional echocardiographic images were low or normal in the patients with pulmonary hypertension implying increased pulmonary vascular resistance as the etiology for elevated pulmonary artery pressure. The mean pulmonary artery systolic pressure in patients with SLE was 25 +/- 10 mm Hg vs 20 +/- 2 in controls (p = 0.002). No control had a pulmonary artery systolic pressure greater than 23 mm Hg. Patients with pulmonary hypertension had a shorter duration of SLE and steroid therapy and a higher prevalence of cytotoxic treatment and Raynaud's phenomenon in comparison to those with normal pulmonary artery pressures. The prevalence of systemic hypertension, interstitial lung disease, pleurisy, pericarditis, cutaneous manifestations, arthritis, renal disease, central nervous system involvement, and hematologic abnormalities was similar in patients with SLE with normal and elevated pulmonary artery pressure. Our study suggests that pulmonary hypertension in SLE is common but usually mild.     

Echocardiographic and Doppler findings in long-term treated rheumatoid arthritis patients without clinically evident cardiovascular disease

OBJECTIVE: To assess the frequency of echocardiographic and Doppler abnormalities in long-term treated rheumatoid arthritis (RA) patients without clinically evident cardiovascular manifestations. METHODS: Forty-seven patients with RA were recruited from Hospital Xeral-Calde, Lugo, Spain. Patients were required to have been treated for at least 5 years and to be on treatment with 1 or more disease-modifying antirheumatic drugs. Patients seen during the period of recruitment who had cardiovascular risk factors or had suffered cardiovascular or cerebrovascular events were excluded. Forty-seven healthy matched controls were also studied. Echocardiographic and Doppler studies were performed in all cases and controls. Patients were HLA-DRB1 genotyped by using molecular-based methods. RESULTS: In patients with RA, the prevalence of aortic regurgitation (17%) and tricuspid regurgitation (17%) was not higher than that seen in controls (15% and 6%). The pulmonary artery systolic pressure was higher in patients with RA (30.3 +/- 8.0 mm Hg) than in controls (26.2 +/- 4.8) (P =.004). Incidence of pulmonary artery systolic pressure >35 mm Hg was significantly higher in patients with RA (21% versus 4% in controls; P =.03). Diastolic dysfunction caused by impaired relaxation was also more common in patients with RA (66%) than in controls (43%) (P =.02). It was more frequent in the older patients. Extra-articular manifestations were more common in patients with RA with diastolic dysfunction (P =.05). The HLA-DRB1 genotype was not implicated in the risk of developing diastolic dysfunction. CONCLUSIONS: The present study confirms a high frequency of left ventricular diastolic dysfunction and pulmonary hypertension in patients with RA without evident cardiovascular disease.     

Pulmonary hypertension secondary to serum hyperviscosity in a patient with rheumatoid arthritis

A patient with rheumatoid arthritis who was evaluated for dyspnea of six months' duration is described. Although no primary cardiac or parenchymal lung disease was identified, right heart catheterization revealed marked pulmonary hypertension. The patient was presumed to have pulmonary arteritis. Evaluation of her hyperproteinemia, however, led to the discovery of a polyclonal gammopathy with a marked increase in plasma viscosity. Although the classic clinical findings of the hyperviscosity syndrome were minimal, the patient underwent plasmapheresis, resulting in a marked reduction of pulmonary artery pressures (from 53 +/- 4 mm Hg, mean +/- SD, to 30 +/- 3 mm Hg, p less than 0.05) and pulmonary vascular resistance (from 707 +/- 63 dynes/second/cm5 to 421 +/- 72 dynes/second/cm5, p less than 0.05) concomitant with a return to normal plasma viscosity. Her dyspnea completely resolved. This represents the first successful treatment of pulmonary hypertension by plasmapheresis. Protein evaluation revealed the presence of intermediate complexes of IgG rheumatoid factor. The hyperviscosity syndrome should be considered in the differential diagnosis of pulmonary hypertension in patients with rheumatoid arthritis and other disorders associated with a polyclonal or monoclonal gammopathy. Pulmonary hypertension secondary to the hyperviscosity syndrome is reversible by plasmapheresis. Immunosuppressive therapy that reduces immunoglobulin production may provide a means of long-term treatment.     

The effect of chronic pulmonary hypertension on left ventricular size, function, and interventricular septal motion

The effect of right ventricular pressure overload secondary to chronic pulmonary arterial hypertension on left ventricular size and function and on interventricular septal motion was studied in 13 patients in whom coronary artery disease, hypertension, and hypoxemia were excluded. Regional and global left ventricular function were assessed by computer-assisted analysis of two-dimensional directed M-mode echocardiograms obtained within 24 hours of a hemodynamic study. Septal position and motion were further analyzed by delineating seven points along the right and left sides of the septum during a single cardiac cycle. All echocardiographic data were compared to those of 10 normal subjects. Mean values for right ventricular systolic, mean pulmonary artery and pulmonary capillary wedge pressures were: 71 +/- 26 mm Hg, 46 +/- 16 mm Hg, and 7 +/- 1 mm Hg, respectively. Septal motion was interpreted from the M-mode echocardiograms as normal in seven patients (group I) and abnormal in the remaining six patients (group II). The only hemodynamic parameter which distinguished these two patterns was delta P, the transseptal systolic pressure gradient across the interventricular septum, which was significantly different (p less than 0.02) in group I (delta P = 65 +/- 16 mm Hg) from that of group II (delta P = 21 +/- 24 mm Hg). As a result of abnormal septal position, the septal-free wall dimensions of the left ventricle were reduced, but there was no evidence of depressed left ventricular performance in these patients. We conclude that resting left ventricular function is well preserved in patients with pulmonary hypertension, despite significant alterations in septal position and left ventricular size.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pulmonary hypertension and cardiac function in adult cystic fibrosis: role of hypoxemia.

STUDY OBJECTIVES: To determine (1) the prevalence of pulmonary hypertension and cardiac dysfunction in adult cystic fibrosis (CF) patients with severe lung disease, (2) the relationship between these cardiovascular abnormalities and hypoxemia, and (3) the impact of subclinical pulmonary hypertension on survival. DESIGN: Single-blind, cross-sectional study. SETTING: Ambulatory clinic of the Adult CF program at a tertiary-level hospital. PATIENTS: Clinically stable patients with severe lung disease (FEV1 < 40% of predicted normal value) who were not receiving supplemental oxygen. A second cohort of patients in stable condition with less severe lung disease (FEV1 40 to 65% predicted) was also recruited to enable multivariate analysis for the determinants of pulmonary hypertension. MEASUREMENTS AND RESULTS: Eighteen patients with severe lung disease (FEV1 28 +/- 7% of predicted normal value) were initially studied. Each patient had overnight polysomnography, pulmonary function tests, and Doppler echocardiography. Arterial oxygen saturation (SaO2) was reduced during wakefulness (87.1 +/- 6.1%) and fell during sleep (84.0 +/- 6.6%) while transcutaneous PCO2 was normal during wakefulness (41.1 +/- 6.9 mm Hg) and increased during sleep (46.6 +/- 4.7 mm Hg). Left ventricular size, systolic function, and diastolic function were normal except in one patient who had had a previous silent myocardial infarction due to coronary artery disease. Qualitative assessment of right ventricular function was normal in all patients. Pulmonary artery systolic pressure (PASP) was increased (> 35 mm Hg) in seven patients without clinical evidence of cor pulmonale. Regression analysis was performed by combining these data with data from an additional 15 CF patients with moderately severe lung disease (FEV1 56.3 +/- 8.9% predicted normal) who were recruited to a modified study protocol that included overnight oximetry, pulmonary function tests, and Doppler echocardiography. None of these patients had evidence of hypoxemia and only three had mild elevation of PASP (36, 37, and 39 mm Hg). Linear regression analysis revealed that PASP was significantly correlated with FEV1 (r = -0.44; p = 0.013), and SaO2 during wakefulness (r =-0.60; p = 0.0003), during sleep (r = -0.56; p = 0.0008), and after 6 min of exercise (r = -0.75; p < 0.0001). Multivariate analysis revealed that awake SaO2 was a significantly better predictor of PASP than FEV1 (p = 0.0104). Clinical follow-up of the original cohort for up to 5 years revealed that mortality was significantly higher in those with pulmonary hypertension than those without pulmonary hypertension (p = 0.0129). CONCLUSIONS: In adult CF patients with severe stable lung disease, left and right ventricular function is well maintained in the absence of significant coronary artery disease; pulmonary hypertension develops in a significant proportion of patients and is strongly correlated with oxygen status, independent of lung function; and subclinical pulmonary hypertension is associated with an increased mortality.     

The clinical significance of reversed flow in the main pulmonary artery detected by doppler color flow imaging

BACKGROUND: Using Doppler color flow imaging, abnormal flow patterns were reported to occur with pulmonary artery (PA) dilation. We have frequently observed red signals in the main PA, suggesting reversed flow (RF) in patients without overt pulmonary hypertension. The clinical implication of these signals has not been extensively studied. PATIENTS AND METHODS: We studied 191 of 412 patients referred for echocardiography (99 men and 92 women; mean +/- SD age, 62 +/- 13 years), in whom the main PA diameter had been adequately measured. If a red signal was observed by color flow imaging, a pulsed Doppler echocardiogram of the red signal was recorded simultaneously. The presence of the red signal was correlated with the PA diameter and the PA systolic pressure determined using the modified Bernoulli equation. In 54 patients who also underwent cardiac catheterization studies, the red signal was correlated with PA and pulmonary capillary wedge (PCW) pressures, and with pulmonary vascular resistance. RESULTS: Red signals adjacent to the medial PA border were detected in parallel with systolic blue signals in 127 patients (66%). Pulsed Doppler recordings revealed that they were caused by RF occurring immediately after the forward systolic signal and persisted in diastole. The PA diameter (28 +/- 4.8 mm) and the estimated PA systolic pressure (34 +/- 16 mm Hg) of patients with the RF signal were significantly greater (p < 0.001 and p < 0.05, respectively) than those of patients without the signal (22 +/- 2.5 mm and 28 +/- 6.0 mm Hg, respectively). Among patients who had hemodynamic studies, PA and PCW pressures were significantly higher (p < 0.05) in the 41 patients with the RF signal (22 +/- 12 mm Hg vs 15 +/- 2.6 mm Hg and 11 +/- 5.5 mm Hg vs 8 +/- 3.1 mm Hg, respectively). CONCLUSION:: RF signals in the main PA occur mostly as a result of PA dilation, which may be caused by primary pulmonary hypertension or chronic elevation of left atrial pressure in left-sided cardiac abnormalities.     

Cardiac involvement in systemic sclerosis assessed by tissue-doppler echocardiography during routine care: A controlled study of 100 consecutive patients

OBJECTIVE: To assess the prevalence of primary cardiac complications in a large population of patients with systemic sclerosis (SSc), using recently developed echocardiographic techniques. METHODS: We prospectively studied 100 consecutive patients (mean +/- SD age 54 +/- 14 years; 86 women) presenting with SSc without pulmonary arterial hypertension or clinical manifestations of heart failure. All patients underwent standard echocardiography, along with measurements of longitudinal velocities by tissue Doppler imaging (TDI) to assess left ventricular (LV) and right ventricular (RV) contractility and LV diastolic function. Results were compared with those in 26 age- and sex-matched healthy controls. RESULTS: Patients with SSc had a wider mean left atrial diameter and impaired relaxation compared with the controls. A trend was observed toward a smaller LV ejection fraction (EF) in the patients (mean +/- SD 64.9 +/- 0.6%) than in the controls (67.2 +/- 0.7%), as well as higher pulmonary artery pressure (mean +/- SD 33.3 +/- 0.6 mm Hg versus 30.8 +/- 1.0 mm Hg). LVEF was <55% in 7 patients versus none of the controls. Peak systolic mitral annular velocity as measured by TDI was <7.5 cm/second in 14 patients versus none of the controls (P = 0.040). Mitral annulus early diastolic velocity was <10 cm/second in 30 patients versus 2 of the controls (P = 0.022). Fifteen patients and none of the controls had reduced peak systolic tricuspid annular velocity (P = 0.039). The TDI results correlated with each other, but not with lung abnormalities or other disease characteristics. CONCLUSION: Depression of LV and RV systolic and LV diastolic function is common in patients with SSc and is due to primary myocardial involvement. Considering the major contributions of TDI, the addition of this simple technique to standard measurements may improve the detection of heart involvement in patients with SSc.     

Clinical risk factors for portopulmonary hypertension

Portopulmonary hypertension affects up to 6% of patients with advanced liver disease, but the predictors and biologic mechanism for the development of this complication are unknown. We sought to determine the clinical risk factors for portopulmonary hypertension in patients with advanced liver disease. We performed a multicenter case-control study nested within a prospective cohort of patients with portal hypertension recruited from tertiary care centers. Cases had a mean pulmonary artery pressure > 25 mm Hg, pulmonary vascular resistance > 240 dynes x second x cm(-5), and pulmonary capillary wedge pressure < or = 15 mm Hg. Controls had a right ventricular systolic pressure < 40 mm Hg (if estimable) and normal right-sided cardiac morphology by transthoracic echocardiography. The study sample included 34 cases and 141 controls. Female sex was associated with a higher risk of portopulmonary hypertension than male sex (adjusted odds ratio = 2.90, 95% confidence interval 1.20-7.01, P = 0.018). Autoimmune hepatitis was associated with an increased risk (adjusted odds ratio = 4.02, 95% confidence interval 1.14-14.23, P = 0.031), and hepatitis C infection was associated with a decreased risk (adjusted odds ratio = 0.24, 95% confidence interval 0.09-0.65, P = 0.005) of portopulmonary hypertension. The severity of liver disease was not related to the risk of portopulmonary hypertension. CONCLUSION: Female sex and autoimmune hepatitis were associated with an increased risk of portopulmonary hypertension, whereas hepatitis C infection was associated with a decreased risk in patients with advanced liver disease. Hormonal and immunologic factors may therefore be integral to the development of portopulmonary hypertension.     

Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease

Doppler echocardiography is commonly used to estimate systolic pulmonary artery pressure and to diagnose pulmonary hypertension, but data relating to its utility in patients with advanced lung disease are limited. In a cohort study of 374 lung transplant candidates, the performance characteristics of echocardiography compared with right heart catheterization in the determination of systolic pulmonary artery pressure and diagnosis of pulmonary hypertension were investigated. The prevalence of pulmonary hypertension was 25% in the study population. Estimation of systolic pulmonary artery pressure by echocardiography was possible in 166 patients (44%). The correlation between systolic pulmonary artery pressure estimated by echocardiography and measured by cardiac catheterization was good (r = 0.69, p < 0.0001). However, 52% of pressure estimations were found to be inaccurate (more than 10 mm Hg difference compared with measured pressure), and 48% of patients were misclassified as having pulmonary hypertension by echocardiography. Sensitivity, specificity, and positive and negative predictive values of systolic pulmonary artery pressure estimation for diagnosis of pulmonary hypertension were 85%, 55%, 52%, and 87%, respectively. In conclusion, despite a statistically significant correlation with directly measured values, estimation of systolic pulmonary artery pressure by echocardiography is frequently inaccurate in patients with advanced lung disease and leads to considerable overdiagnosis of pulmonary hypertension.     

肺动脉血栓内膜剥脱术的远期疗效

目的　观察肺动脉血栓内膜剥脱术 (PTE)对慢性栓塞性肺动脉高压 (CTEPH )患者的远期疗效。方法　 1997年 3月～ 2 0 0 2年 6月 ,共对 15例CTEPH患者行PTE ,14例存活出院。其中 13例随访时间平均 4 0 (17～ 77)个月 ,1例失访 ;随访项目包括心功能状态、血气分析和通过超声心动图测量肺动脉收缩压。结果　随访期内 13例患者均存活 ,其术前心功能NYHA分级均为Ⅲ、Ⅳ级 ;术后 12例患者心功能NYHA分级Ⅰ级、1例Ⅲ级 ;动脉血氧分压 (PaO2 )由术前的 (5 8 1± 8 6 )mmHg上升至(90 8± 6 0 )mmHg (P <0 0 1) ;动脉血氧饱和度由术前的 (90± 5 ) %上升至 (96± 1) % (P <0 0 5 ) ;肺动脉收缩压由术前的 (97 1± 2 2 4 )mmHg下降至 (4 2 6± 10 7)mmHg (P <0 0 1)。结论　肺动脉血栓内膜剥脱术对慢性栓塞性肺动脉高压患者具有很好的远期疗效 ,它能显著改善患者的心肺功能 ,提高其生活质量     

Adenosine plasma concentration in pulmonary hypertension.

OBJECTIVE: In this study, we sought to appreciate the role of adenosine in the regulation of pulmonary vascular tone, especially in the case of clinical pulmonary hypertension, by investigating the relationship between endogenous plasma adenosine levels and pulmonary artery vasoconstriction. METHODS: Adenosine plasma concentrations, were measured simultaneously in the distal right pulmonary artery and in the femoral artery, both at steady state (room air) and during pure oxygen inhalation. Three clinical situations were considered: (1) normal hemodynamics [7 control subjects, mean pulmonary artery pressure (MPAP) = 18.5 +/- 1 mm Hg], (2) moderate pulmonary hypertension secondary to chronic obstructive pulmonary disease (COPD), (8 patients, MPAP = 31 +/- 3 mm Hg), (3) severe primary pulmonary hypertension (PPH), (8 patients, MPAP = 70 +/- 5 mm Hg). RESULTS: In every instance, adenosine evaluated by HPLC was higher in the pulmonary than in the systemic circulation. For room air, adenosine plasma concentrations were significantly lower in COPD (0.49 +/- 0.16 mumol l-1) and PPH patients (0.45 +/- 0.14 mumol l-1) than in controls (1.26 +/- 0.12 mumol l-1). During O2 administration, adenosine plasma concentrations all decreased but more so in COPD and PPH patients. The significant correlations between adenosine plasma concentrations and both pulmonary vascular resistance and PvO2, in controls, were not found in COPD or PPH patients. CONCLUSION: The adenosine plasma concentrations in the pulmonary circulation of PPH and COPD patients are low, and may contribute to pulmonary artery hypertension.     

Cardiovascular abnormalities in hyperthyroidism: a prospective Doppler echocardiographic study

PURPOSE: We investigated the prevalence and clinical importance of cardiovascular abnormalities in patients with hyperthyroidism. METHODS: All consecutive patients diagnosed with hyperthyroidism during a period of 24 months were included in the study. Medical history, complete physical examination results, electrocardiographic findings, laboratory determinations, and Doppler echocardiographic findings were obtained for all patients within 24 hours of diagnosis, and after euthyroidism had been achieved. Age- and sex-matched controls also were studied. RESULTS: Thirty-nine patients (mean [+/-SD] age, 52 +/- 20 years; range, 25 to 86 years; 72% women), and 39 age- and sex-matched controls, were included. Atrial fibrillation was present in 7 patients (18%). Moderate or severe mitral or tricuspid regurgitation, or both, were present in 9 patients (23%) and in only 1 control (3%; P= 0.01). Mean pulmonary arterial systolic pressure was 38 +/- 12 mm Hg (range, 17 to 64 mm Hg) in patients and 27 +/- 4 mm Hg (range, 19 to 37 mm Hg) in controls (P= 0.001). Sixteen patients (41%) and 1 control (3%) had pulmonary arterial systolic pressure >or=35 mm Hg. Left ventricular systolic dysfunction was detected in 1 patient. After correction of hyperthyroidism, a significant decrease in pulmonary arterial systolic pressure was observed, and the levels became similar to those of controls. CONCLUSION: In patients with hyperthyroidism, there is a high prevalence of pulmonary hypertension and atrioventricular valve regurgitation. These abnormalities usually correct after treatment for hyperthyroidism.     

Pulmonary artery pulse pressure and wave reflection in chronic pulmonary thromboembolism and primary pulmonary hypertension

OBJECTIVES: The purpose of this time-domain study was to compare pulmonary artery (PA) pulse pressure and wave reflection in chronic pulmonary thromboembolism (CPTE) and primary pulmonary hypertension (PPH). BACKGROUND: Pulmonary artery pressure waveform analysis provides a simple and accurate estimation of right ventricular afterload in the time-domain. Chronic pulmonary thromboembolism and PPH are both responsible for severe pulmonary hypertension. Chronic pulmonary thromboembolism and PPH predominantly involve proximal and distal arteries, respectively, and may lead to differences in PA pressure waveform. METHODS: High-fidelity PA pressure was recorded in 14 patients (7 men/7 women, 46 +/- 14 years) with CPTE (n = 7) and PPH (n = 7). We measured thermodilution cardiac output, mean PA pressure (MPAP), PA pulse pressure (PAPP = systolic - diastolic PAP) and normalized PAPP (nPAPP = PPAP/MPAP). Wave reflection was quantified by measuring Ti, that is, the time between pressure upstroke and the systolic inflection point (Pi), deltaP, that is, the systolic PAP minus Pi difference, and the augmentation index (deltaP/PPAP). RESULTS: At baseline, CPTE and PPH had similar cardiac index (2.4 +/- 0.4 vs. 2.5 +/- 0.5 l/min/m2), mean PAP (59 +/- 9 vs. 59 +/- 10 mm Hg), PPAP (57 +/- 13 vs. 53 +/- 13 mm Hg) and nPPAP (0.97 +/- 0.16 vs. 0.89 +/- 0.13). Chronic pulmonary thromboembolism had shorter Ti (90 +/- 17 vs. 126 +/- 16 ms, p < 0.01) and higher deltaP/PPAP (0.26 +/- 0.01 vs. 0.09 +/- 0.07, p < 0.01). CONCLUSIONS: Our study indicated that: 1) CPTE and PPH with severe pulmonary hypertension had similar PA pulse pressure, and 2) wave reflection is elevated in both groups, and CPTE had increased and anticipated wave reflection as compared with PPH, thus suggesting differences in the pulsatile component of right ventricular afterload.     

Noninvasive evaluation of pulmonary hypertension by quantitative contrast M-mode echocardiography

Although it has been shown that pulmonary flow velocity can be calculated from contrast M-mode echocardiographic tracings, the clinical value of this noninvasive method has not been established. We used contrast M-mode echocardiography to examine the flow velocity pattern at the pulmonary valve in 30 adults referred for diagnostic cardiac catheterization. In the 15 patients with normal pulmonary artery pressure (PAP) (mean pressure less than or equal to 20 mm Hg), midsystolic pulmonary flow velocity was significantly (p less than 0.001) higher (654 +/- 140 mm/sec) compared to the 15 patients with pulmonary hypertension (342 +/- 85 mm/sec, mean pressure greater than 20 mm Hg). Fourteen of the 15 patients with pulmonary hypertension exhibited an early systolic flow velocity peak, whereas all patients with normal PAP showed a dome-shaped systolic flow velocity profile with maximal flow velocity occurring in midsystole. A significant close correlation was found between the relative early to midsystolic flow velocity change and mean PAP (r = 0.96; p less than 0.001). Thus quantitative contrast M-mode echocardiography reliably differentiates patients with pulmonary hypertension from patients with normal mean PAP. In addition, this technique allows a noninvasive estimation of PAP.     

Isolated chronic mitral regurgitation with preserved systolic left ventricular function and severe pulmonary hypertension

Pulmonary hypertension in chronic mitral valve disease has been related most commonly to left ventricular dysfunction or mitral stenosis; its association with chronic, isolated mitral regurgitation and preserved left ventricular systolic function is unclear. In 41 catheterized patients with chronic mitral regurgitation (known history of mitral regurgitation for greater than 18 months) and preserved left ventricular systolic function (ejection fraction greater than 0.55), historic, electrocardiographic, echocardiographic and hemodynamic variables were analyzed. Ten patients (Group I) had normal pulmonary artery systolic pressure (less than 30 mm Hg), whereas 31 patients had pulmonary hypertension. Pulmonary artery systolic pressure was mildly increased (30 to 49 mm Hg) in 13 patients (Group II) and was greater than or equal to 50 mm Hg in 18 patients (Group III). Univariate analysis showed the more frequent occurrence of male gender and ruptured chordae tendineae in the groups with pulmonary hypertension. Mean pulmonary capillary wedge pressure, size of the V wave in pulmonary capillary wedge pressure and pulmonary arteriole resistance were higher, whereas cardiac index was lower in the hypertension groups. Multivariate stepwise analysis revealed higher mean pulmonary capillary wedge pressure and pulmonary arteriole resistance as the only variables independently differing among groups. In conclusion, pulmonary hypertension occurs frequently (76% of cases) in patients with chronic, isolated mitral regurgitation with preserved left ventricular systolic function. In these patients, a severe increase in pulmonary capillary wedge pressure is associated with elevation in pulmonary artery resistance, a finding similar to that in mitral stenosis.     

Unexplained pulmonary hypertension in elderly patients

BACKGROUND: Idiopathic pulmonary arterial hypertension (IPAH) preferentially affects young women. However, a subset of patients with IPAH is elderly. Our objective was to compare elderly (age >/= 65 years) vs younger persons with unexplained pulmonary hypertension (PH) and a presumptive diagnosis of IPAH. METHODS: Clinical, echocardiographic, hemodynamic, and survival data were collected on consecutive patients with suspected IPAH after evaluation in a large tertiary center PH clinic. RESULTS: Of 197 patients (mean age +/- SD, 52 +/- 16 years; 80% female), 48 patients (24%) were elderly. Elderly and younger patients had similar symptom severity, systolic pulmonary artery (PA) pressure (82.7 +/- 20.3 mm Hg vs 86.9 +/- 18.8 mm Hg, respectively; p = 0.21), and severity of right ventricular enlargement and dysfunction. Elderly patients had higher pulmonary capillary wedge pressure (PCWP) [15.3 +/- 7.3 mm Hg vs 11.1 +/- 5.3 mm Hg; p < 0.0001] and more frequently failed (56%) to meet hemodynamic criteria for IPAH (PH with PCWP < 15 mm Hg) than did younger patients (19%). Elderly patients also had higher systemic systolic (p < 0.0001) and pulse (p < 0.0001) pressures and more cardiovascular disease. Among those patients with normal PCWP, elderly patients had worse survival than young patients (p = 0.007). Among those patients with elevated PCWP, elderly patients had lower PA pressures (p = 0.04) and better survival (p = 0.02). CONCLUSIONS: Elderly patients with clinically suspected IPAH often fail to meet hemodynamic criteria for IPAH due to elevated PCWP. Studies to define the proper diagnostic strategy and the safety and efficacy of pulmonary vasodilators in elderly patients with unexplained PH are needed.     

Plasma beta-endorphin and adenosine concentration in pulmonary hypertension

To determine whether beta-endorphin plays a role in the regulation of pulmonary vascular tone in patients with pulmonary hypertension, we investigated the relations between hemodynamics and beta-endorphin and adenosine concentrations in 3 clinical situations: (1) normal hemodynamics (7 subjects, mean pulmonary artery [PA] pressure 18.5 +/- 1 mm Hg); (2) moderate pulmonary hypertension secondary to chronic obstructive pulmonary disease (COPD) (8 patients, mean PA pressure 31 +/- 3 mm Hg); and (3) severe primary pulmonary hypertension (PPH) (8 patients, mean PA pressure 70 +/-5 mm Hg). Plasma beta-endorphin and adenosine were measured in a distal PA and in the femoral artery in room air and during oxygen inhalation. Beta-endorphin levels were similar in the pulmonary and systemic circulations. No difference was observed between patients with COPD and PPH, but relative to controls, both had significantly higher beta-endorphin levels. Pulmonary adenosine was significantly lower in patients with pulmonary hypertension than in controls (-60% in COPD [p <0.005] and -70% in PPH [p <0.001]). Pure oxygen administration significantly decreased adenosine and beta-endorphin levels, much more so in patients with COPD and PPH. We found a negative correlation between beta-endorphin and adenosine concentrations (r = -0.751, p <0.001): the higher the adenosine, the lower the beta-endorphin level. These observations suggest that because adenosine release by pulmonary vascular endothelium is reduced in pulmonary hypertension, the resulting worsened hypoperfusion and tissue oxygenation may cause increased beta-endorphin release.     

Doppler echocardiographic measurement of pulmonary artery pressure from ductal Doppler velocities in the newborn

The ductal flow velocities in 37 newborns (group 1: persistent pulmonary hypertension [n = 16], transient tachypnea [n = 3], other [n = 2]; group 2: respiratory distress syndrome [n = 16]) were prospectively evaluated by Doppler ultrasound for the purpose of deriving systolic pulmonary artery pressures. Maximal tricuspid regurgitant Doppler velocity in 21 of these patients was used to validate the pulmonary artery pressures derived from ductal flow velocities. There was a significant linear correlation between tricuspid regurgitant Doppler velocity and pulmonary artery systolic pressure derived from ductal Doppler velocities in patients with unidirectional (pure left to right or pure right to left) ductal shunting (p less than 0.001, r = 0.95, SEE 8) and in those with bidirectional shunting (p less than 0.001, r = 0.95, SEE 4.5). Systolic pulmonary artery pressure in group 1 (67 +/- 13 mm Hg) was significantly higher than that in group 2 (39 +/- 10 mm Hg) (p less than 0.001). In those with bidirectional shunting, duration of right to left shunting less than 60% of systole was found when pulmonary artery pressure was systemic or less, whereas duration greater than or equal to 60% was associated with suprasystemic pulmonary artery pressures. Serial changes in pulmonary artery systolic pressure, reflected by changes in ductal Doppler velocities, correlated with clinical status in persistent pulmonary hypertension of the newborn. Persistently suprasystemic pulmonary artery pressure was associated with death in five group 1 patients. It is concluded that ductal Doppler velocities can be reliably utilized to monitor the course of pulmonary artery systolic pressures in newborns.     

Usefulness of the pulmonary arterial systolic pressure to predict pulmonary arterial wedge pressure in patients with normal left ventricular systolic function

Tissue Doppler imaging combined with transmitral Doppler permits estimation of pulmonary artery wedge pressure (PAWP) in many, but not all, patients, whereas pulmonary artery systolic pressure (PASP) and cardiac output (time-velocity integral method) are routinely measured. It was hypothesized that simple Doppler echocardiographic measurements could be used to estimate PAWP in many patients by rearranging the equation for pulmonary vascular resistance ([mean pulmonary artery pressure - (left atrial pressure/cardiac output)] x 80). Data from 69 patients (mean age 59 +/- 15 years) were reviewed, including cardiac output, transmitral mitral E wave velocity, and lateral tissue Doppler imaging mitral annular early diastolic velocity. PAWP was determined in the 2 ways of (1) measured (PAWPm) using the regression equation PAWPm = 1.91 + (1.24 * transmitral mitral E wave velocity/mitral annular early diastolic velocity) developed and validated by Nagueh, and (2) using a nomogram that we developed to predict PAWP when cardiac output and PASP were known. Moderately strong correlation was found between PASP and PAWPm (r = 0.73), and this correlation improved when excluding patients with pulmonary or liver disease and restricting cardiac output to 3.5 to 6.0 L/min (physiologic range; r = 0.81). Furthermore, the relation between PAWPm and PASP allowed for discrimination of high versus low PAWP: 36 of 37 patients with PASP < or =30 mm Hg had PAWPm < or =15 mm Hg (sensitivity 97%, specificity 47%). Conversely, 9 of 9 patients with PASP > or =40 mm Hg had PAWPm > or =12 mm Hg (sensitivity 100%, specificity 70%). Predicted PAWP correlated well with PAWPm (r = 0.63) and improved when patients with liver or pulmonary disease were excluded (r = 0.83). In conclusion, PASP strongly correlated with PAWP, and this principle can be exploited to rapidly detect patients with low or high PAWP.     

Hemodynamic characterization of patients with severe emphysema

In 120 patients with severe emphysema evaluated for participation in the National Emphysema Treatment Trial, pulmonary hemodynamics and ventricular function were assessed. Pulmonary function tests were (%predicted): FEV(1) = 27%; residual volume = 224.6%; diffusion capacity = 26.7%. In 90.8% of patients, end-expiratory pulmonary artery mean pressure was > 20 mm Hg; in 61.4%, end-expiratory wedge pressure was > 12 mm Hg. Cardiac index was normal. Mean pulmonary artery pressure correlated inversely with arterial PO(2), and severity of emphysema, and directly with wedge pressure. Multiple stepwise regression revealed that arterial PO(2) was not an independent predictor of mean pulmonary artery pressure. No correlation was found between indices of emphysema severity and PA pressures. Diastolic ventricular pressures were increased without evidence of systolic dysfunction. We conclude that (1) elevations of pulmonary vascular pressures are common, (2) pulmonary hypertension may be related to factors other than hypoxia, (3) pulmonary hypertension does not impair resting systemic O(2) delivery, and (4) elevated cardiac diastolic pressures do not represent systolic dysfunction.