Rheumatic mitral valve stenosis is associated with impaired flow-mediated dilatation

Isolated pulmonary hypertension with clinical implication is rare in rheumatoid arthritis. We sought to study the prevalence of pulmonary arterial hypertension in an unselected population of 45 patients with rheumatoid arthritis (classified according to the ARA criteria) without cardiac disease and corresponding age and sex matched controls by Doppler echocardiography. The pulmonary artery systolic pressure was higher in patients with Rheumatoid Arthritis (27.49+/-12.66 mm Hg) than in controls (20.40+/-8.88) (p=0.003). Incidence of pulmonary artery systolic pressure>30 mm Hg suggesting pulmonary hypertension was significantly higher in patients with RA (26.7% versus 4.5% in controls; p=0.03) and 20% of patients had pulmonary hypertension without lung disease or cardiac disease evident on pulmonary function testing, and echocardiogram respectively. There was also a strong correlation between the pulmonary artery pressure and the disease duration (r=0.68, p<0.0001) suggesting a subclinical involvement of the pulmonary vasculature with disease progression and may be relevant to the high incidence of cardiovascular deaths observed in patients with Rheumatoid Arthritis.     

Plasma endothelin-1 levels in patients with systemic sclerosis: influence of pulmonary or systemic arterial hypertension.

OBJECTIVES--To investigate the behaviour of circulating endothelin-1 (ET-1) in patients affected by systemic sclerosis and to elucidate the relationship between systemic and pulmonary plasma peptide and arterial pressure levels. METHODS--Plasma ET-1 concentrations were determined in 48 patients affected by systemic sclerosis (41 women, seven men; mean age 47.2 (SD 5.5) years) with or without systemic or pulmonary hypertension (or both). A group of 18 normal volunteers served as controls (15 women, three men; mean age 45.0 (10.1) years). RESULTS--Plasma ET-1 levels were significantly greater in patients affected by systemic sclerosis (1.65 (0.29) pg/ml) than in controls (0.63 (0.19) pg/ml) (p < 0.0001). Pulmonary artery systolic hypertension alone was present in 14 patients with systemic sclerosis (50.5 (8.49) mm Hg, range 37-67 mm Hg), and systemic hypertension alone (160.7 (5.9)/100.6 (3.2) mm Hg) was present in 11 patients. Both conditions were present in 12 patients, while 11 patients had systemic hypertension. There were no significant differences in plasma ET-1 levels between patients with pulmonary hypertension alone (1.62 (0.21) pg/ml) and those with systemic hypertension alone (1.65 (0.43) pg/ml). In particular, patients with normal pulmonary artery and systemic pressures (n = 11) had plasma ET-1 concentrations identical to those found in patients (n = 12) with both pulmonary and systemic hypertension (1.70 (0.15) v 1.64 (0.35) pg/ml, respectively). No correlations were observed between plasma ET-1 and either pulmonary or systemic pressures. CONCLUSION--Systemic sclerosis is characterised by increased plasma ET-1 levels, but neither pulmonary nor systemic hypertension are accompanied by further increase in plasma peptide levels.     

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.     

Prevalence and clinical correlates of pulmonary arterial hypertension in progressive systemic sclerosis

Forty-nine patients with progressive systemic sclerosis who had undergone extensive studies including pulmonary artery catheterization as part of an ongoing prospective study of the natural course of progressive systemic sclerosis were evaluated. The overall prevalence of pulmonary arterial hypertension in this population of patients with progressive systemic sclerosis was 33 percent, and among 10 subjects with the CREST syndrome the prevalence of pulmonary hypertension was 50 percent. The relation between pulmonary arterial hypertension documented at catheterization and abnormal results of noninvasive studies suggesting pulmonary hypertension, including physical examination, chest x-ray, electrocardiography, echocardiography, single-breath diffusing capacity, and vital capacity, was studied. Diffusing capacity was significantly lower in those patients with definite pulmonary hypertension (mean pulmonary artery pressure of 22 mg Hg or more) compared with those with a normal mean pulmonary artery pressure, and a diffusing capacity below 43 percent of predicted showed the greatest sensitivity (67 percent) of any single diagnostic test in detecting definite pulmonary hypertension. Chest x-ray suggesting pulmonary hypertension was the least sensitive of the tests evaluated, but showed the greatest specificity (100 percent) in identifying patients with pulmonary hypertension. A classification matrix based on discriminant function analysis utilizing the combination of diffusing capacity below 43 percent of predicted and chest x-ray and electrocardiographic findings correctly identified 75 percent of patients with definite pulmonary hypertension and 97 percent of patients with a normal pulmonary artery pressure, but failed to identify correctly patients with mild pulmonary hypertension (mean pulmonary artery pressure of 20 mm Hg). These findings indicate that specific noninvasive studies are helpful in assessing the likelihood of normal or definitely elevated pulmonary artery pressures in patients with progressive systemic sclerosis, but patients with mild pulmonary hypertension are not likely to be identified by these noninvasive studies.     

Hemodynamic effects of nifedipine on secondary pulmonary hypertension in man

We evaluated the hemodynamic effects of the calcium antagonist nifedipine in 13 consecutive patients admitted to the intensive care unit with secondary pulmonary hypertension. Etiology of secondary pulmonary hypertension was: chronic obstructive pulmonary disease (n = 9), pulmonary emboli (n = 2), pulmonary fibrosis (n = 2). We obtained the resting hemodynamic parameters before, and 60, 120, 180 minutes after the sublingual administration of nifedipine 20 mg. All patients had normal pulmonary artery wedge pressure before nifedipine. After 60 minutes, systolic pulmonary artery pressure fell from 72.3 +/- 7 to 57.3 +/- 5.4 mm Hg (p less than 0.005) and mean pulmonary artery pressure from 44.6 +/- 4.0 to 33.6 +/- 3.2 mm Hg (p less than 0.001). Cardiac output rose from 6.36 +/- 0.56 to 7.65 +/- 0.64 l/min (p less than 0.005). The pulmonary vascular resistance fell from 431 +/- 58 to 238 +/- 36 dynes. sec. cm-5 (p less than 0.001). Heart rate, mean systemic arterial pressure, pulmonary artery wedge pressure, total systemic vascular resistance and arterial partial pressure of O2 (PaO2) remained unchanged. In this heterogenous population we were unable to reproduce the results of other authors, showing a correlation between PaO2 and fall of pulmonary vascular resistance. These findings confirm the pulmonary vasodilating effect of nifedipine in patients with secondary pulmonary hypertension.     

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.     

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.     

Doppler echocardiographic prediction of pulmonary arterial hypertension in congenital heart disease

This study determines the accuracy of Doppler echocardiography (echo) for predicting the presence of pulmonary artery (PA) hypertension from Doppler PA velocity traces. The patient group included 17 patients with congenital cardiac disease who had undergone catheterization. The control group was composed of 15 normal subjects. Doppler traces were analyzed qualitatively and quantitatively. Qualitative assessment included evaluation for a negative presystolic velocity that was the equivalent of the pulmonary a wave detected by M-mode echo. Quantitative assessment included measurement of the following time intervals and ratio of intervals: preejection period (PEP), time to peak velocity (TPV), right ventricular ejection time (RVET), PEP/RVET and TPV/RVET ratios. In the patient group, systolic PA pressure ranged from 22 to 90 mm Hg (mean 50 +/- 23), and mean PA pressure ranged from 12 to 60 mm Hg (mean 32 +/- 17). Five patients had systolic PA pressures of less than or equal to 30 mm Hg and 12 had systolic PA pressures greater than 30 mm Hg. Of 15 control subjects, 14 had a negative presystolic a wave. Of 5 patients with PA pressure less than or equal to 30 mm Hg, 4 had a presystolic negative velocity, and all with higher pressures had no presystolic negative velocity. One patient with pressure less than 30 mm Hg and 2 with PA pressure greater than 30 mm Hg had indeterminate status of presystolic velocity pattern because of turbulence or baseline blanking. The best quantitative indexes for separating patients with normal PA pressure from those with elevated PA pressure were TPV and TPV/RVET, which respectively correlated negatively with systolic PA pressure (r = -0.82, standard error of the estimate [SEE] = 0.02; and r = -0.70, SEE = 0.05). These measurements also correlated negatively with mean PA pressure (r = -0.75, SEE = 0.02; and r = -0.76, SEE = 0.05). Other intervals and ratios had enough individual variability to make them less useful as predictors of PA hypertension.     

Epoprostenol for treatment of pulmonary hypertension in patients with systemic lupus erythematosus

OBJECTIVE: Pulmonary hypertension with pathological changes similar to those observed in primary pulmonary hypertension occurs in patients with systemic lupus erythematosus (SLE). The efficacy of chronic epoprostenol therapy in SLE has not been well described. The objective of this paper is to describe our experience with long-term epoprostenol therapy in patients with pulmonary hypertension associated with SLE. DESIGN: Case series of six patients with SLE and associated pulmonary hypertension receiving chronic treatment with epoprostenol. RESULTS: All 6 patients had severe pulmonary hypertension. Mean pulmonary artery pressure (mPAP) was 57 +/- 9 mm Hg (mean +/- SD), and pulmonary vascular resistance was 14 +/- 7 units before beginning therapy with epoprostenol. In 4 patients who underwent repeat hemodynamic evaluation (9 to 16 months after starting epoprostenol), mean pulmonary artery pressure decreased by 38 +/- 21% and pulmonary vascular resistance by 58 +/- 12%. Clinically, all patients improved from New York Heart Association class III or IV to class I or II. Doses of epoprostenol ranged from 4 to 46 ng/kg/min, and the longest duration of therapy has been 2.5 years. Side effects from epoprostenol have not differed from those seen in patients with primary pulmonary hypertension, and except for one patient, there has been no exacerbation of SLE. CONCLUSION: Epoprostenol was effective for the treatment of pulmonary hypertension in this small group of patients with SLE. Further evaluation of epoprostenol therapy for patients with SLE and other diseases associated with pulmonary hypertension is warranted.     

Echocardiographic estimation of pulmonary artery pressure in transposition of the great arteries

To determine their usefulness in estimating pulmonary artery pressure, left ventricular systolic time intervals (STI) were determined by echocardiography in 65 patients with dextro-transposition of the great arteries (TGA). The STI were measured from recordings of pulmonary valve motion at 100 mm/sec paper speed. The pre-ejection period (PEP) and the ratio of PEP to left ventricular ejection time (PEP/LVET) were directly related to pulmonary artery pressure. The strongest correlations were that between PEP/LVET and pulmonary artery diastolic pressure (r = 0.70) and that between PEP/LVET and the ratio of mean pulmonary pressure to mean systemic pressure (r = 0.71). A value of PEP/LVET of less than 0.26 was consistently associated with pulmonary artery diastolic pressures of less than 20 mm Hg and, in 28 of 31 patients, pulmonary artery pressure less than one-third of mean systemic arterial pressure. Pulmonary hypertension was present in 18 of 22 patients with PEP/LVET of 0.30 or greater; elevated PEP/LVET was also present in four patients with abnormalities of cardiac rhythm or conduction, two of whom also had angiographic evidence of myocardial dysfunction.     

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.     

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.     

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.     

Hemodynamic effects of inhaled nitric oxide in women with mitral stenosis and pulmonary hypertension

Mitral stenosis (MS) is associated with elevated left atrial pressure, increased pulmonary vascular resistance (PVR), and pulmonary hypertension (PH). The hemodynamic effects of inhaled nitric oxide (NO) in adults with MS are unknown. We sought to determine the acute hemodynamic effects of inhaled NO in adults with MS and PH. Eighteen consecutive women (mean age 58 +/- 15 years) with MS and PH underwent heart catheterization. Hemodynamic measurements were recorded at baseline, after NO inhalation at 80 ppm, and after percutaneous balloon valvuloplasty (n = 10). NO reduced pulmonary artery systolic pressure (62 +/- 14 mm Hg [baseline] vs 54 +/- 15 mm Hg [NO]; p <0.001) and PVR (3.7 +/- 2.5 Wood U [baseline] vs 2.2 +/- 1.4 Wood U [NO]; p <0.001). NO had no effect on mean aortic pressure, left ventricular end-diastolic pressure, left atrial pressure, cardiac output, or systemic vascular resistance. Mitral valve area increased after valvuloplasty (0.9 +/- 0.2 cm2 [baseline] vs 1.6 +/- 0.3 cm2 [postvalvuloplasty]; p <0.001). A decrease in left atrial pressure (25 +/- 4 mm Hg [baseline] vs 17 +/- 4 mm Hg [after valvuloplasty]; p <0.001) and pulmonary artery systolic pressure (58 +/- 12 mm Hg [baseline] vs 45 +/- 8 mm Hg [after valvuloplasty]; p <0.001) was observed after valvuloplasty. No change in cardiac output or PVR was observed. Thus inhaled NO, but not balloon valvuloplasty, acutely reduced PVR in women with MS and PH. This suggests that a reversible, endothelium-dependent regulatory abnormality of vascular tone is an important mechanism of elevated PVR in MS.     

Doppler echocardiographic estimation of systolic pulmonary artery pressure in patients with aortic-pulmonary shunts

The objective of this study was to determine if the pressure drop across various types of aortic-pulmonary shunts could be accurately estimated by Doppler echocardiography, and if systolic pulmonary pressure could be estimated by referencing the pressure drop across the aortic-pulmonary shunt to systolic systemic arterial pressure measured by cuff sphygmomanometry. This was done in 22 patients and Doppler results were compared with pulmonary artery pressure measured directly by strain gauge manometry. Adequate Doppler waveforms were obtained in 21 of 22 patients; 3 had a Waterston shunt, 10 had a Blalock-Taussig shunt, 1 had a left pulmonary artery-aortic anastomosis, 6 had a patent ductus arteriosus and 1 had an aortic-pulmonary window. Systolic pulmonary artery pressure estimated by Doppler echocardiography ranged from 12 to 90 mm Hg (mean 41.3 +/- 21.4 [SD] ), and measured by strain gauge manometry ranged from 20 to 90 mm Hg (mean 44.7 +/- 20.7) (p = NS, r = 0.94, SEE = 7.4 mm Hg; slope = 0.90, y intercept = 7.4 mm Hg). Systolic pulmonary artery to aortic pressure ratios predicted by Doppler recording ranged from 0.1 to 1.0 (mean 0.4 +/- 0.2 [SD] ); when calculated from direct measurement it ranged from 0.2 to 1.0 (mean 0.4 +/- 0.2) (p = NS, r = 0.92; SEE = 0.08, slope = 0.80, y intercept = 0.09). This study demonstrates that Doppler echocardiography provides an estimation of pressure drop across aortic-pulmonary shunts, and that the data can be used to estimate systolic pulmonary artery pressure by subtracting the estimated pressure drop from the systolic systemic arterial pressure.     

Right ventricular contractile function in children with congenital heart disease

Indexes of right ventricular (RV) systolic function were evaluated in 41 patients undergoing cardiac catheterization. High-fidelity tracings were used to determine RV pressure, maximal RV dP/dt and the velocity of contractile element shortening at a developed pressure of 10 mm Hg (VCE10). In 14 children with an RV systolic pressure less than 35 mm Hg, normal RV volume, pulmonary vascular resistance (PVR) less than 3 units X m2 and no shunts (our normal group), mean (+/- standard deviation) RV dP/dt was 437 +/- 116 mm Hg X s-1 and VCE10 was 1.15 +/- 0.33 muscle length X s-1. In patients with RV systolic hypertension due to valvular pulmonary stenosis or isolated increases in PVR, mean values for RV dP/dt and VCE10 were significantly (p less than 0.05) greater than the normal values. In patients with a ventricular septal defect, RV hypertension and normal PVR, VCE10 was normal but RV dP/dt was significantly elevated. Children with chronic RV volume overload had normal RV contractile indexes. No patient in any group had values for RV dP/dt or VCE10 that were less than normal (mean normal - 2 standard deviations). This study establishes for the first time the indexes of RV isovolumic systole in children. It also shows that RV contractile function is preserved in young patients with chronic RV pressure or volume overload who do not have overt congestive heart failure.     

Hypoalbuminemia in elderly patients with acute diastolic heart failure

OBJECTIVES: This study evaluated the relative contribution of serum colloid osmotic pressure (COP) lowering and pulmonary artery wedge pressure (PAWP) elevation in the pathogenesis of pulmonary edema in patients with systolic or isolated diastolic heart failure (DHF). BACKGROUND: The role of hypoalbuminemia and the resulting low COP have been shown in some patients with acute systolic heart failure (SHF).Colloid osmotic pressure and PAWP were determined in 100 patients with acute heart failure (HF) (56 with DHF and 44 with SHF; mean age, 78 +/- 12 years), in 35 patients with acute dyspnea from pulmonary origin, and in 15 normal controls. Pulmonary artery wedge pressure was estimated using transthoracic Doppler echocardiography. RESULTS: Colloid osmotic pressure was significantly lower in the DHF group (20.5 +/- 5 mm Hg) than in the SHF group (24.2 +/- 3.7 mm Hg, p < 0.001), pulmonary disease group (25.1 +/- 4.2 mm Hg, p < 0.001), or normal control group (24.7 +/- 3 mm Hg). Low COP resulted from hypoalbuminemia due to age, malnutrition, and sepsis. Pulmonary artery wedge pressure was significantly higher in patients with SHF (26 +/- 6.3 mm Hg) than in the patients with DHF (20.3 +/- 7 mm Hg, p < 0.001) and was significantly higher in the patients with DHF than in the patients with pulmonary disease (13 +/- 4.2 mm Hg, p < 0.001). The COP-PAWP gradient was similar in patients with SHF (-1.6 +/- 7.1 mm Hg) and patients with DHF (0.7 +/- 6 mm Hg). CONCLUSIONS: Frequent hypoalbuminemia resulting in low COP facilitates the onset of pulmonary edema in patients with DHF who usually have lower PAWP than patients with SHF.     

Pulmonary hypertension in systemic lupus erythematosus: clinical association and survival in 18 patients.

OBJECTIVE: To examine the clinical and serologic features in patients with systemic lupus erythematosus (SLE) with pulmonary hypertension (PHT). METHODS: Case records were reviewed for 419 Chinese patients with SLE from a single center in Hong Kong between January 1985 and June 1997. Patients with PHT were compared with SLE controls. The diagnosis of PHT was made when the pulmonary artery systolic pressure (PASP), measured by 2 dimensional echocardiogram and Doppler studies at rest, was >30 mm Hg, or at postmortem. Clinical features, serologic profile, and outcome of those with PHT were compared with those without PHT. Thirteen patients had repeat echocardiogram to determine subsequent PASP. RESULTS: Eighteen of 419 patients, all female, were identified as having PHT. The clinical and serologic features of patients with SLE with PHT were similar to those without PHT except for serositis and Raynaud's phenomenon, which occurred more frequently in patients with PHT (p = 0.01, p = 0.03, respectively). The age at diagnosis of PHT was 30.5+/-10.2 (mean +/- SD) years. The duration of followup from time of diagnosis of PHT was 23.6+/-20.1 months. PASP was 52+/-23.4 mm Hg. There were 4 deaths. Survival of PHT patients as a group was 40.2+/-5.18 (95% CI 30.1-50.4) months. Repeat echocardiogram in 13 patients revealed a mean reduction in PASP of 21.3% over a median interval of 43 (range 4-52) months. CONCLUSION: Raynaud's phenomenon is associated in patients with SLE with PHT. The prognosis of these patients is variable, with the majority of patients having mild to moderate disease showing little change over 2 years. Nearly a quarter died and a few improved markedly.     

Influence of aging on pulmonary hemodynamics in a population free of coronary artery disease

Previous studies showing an increase in pulmonary artery (PA) pressures and vascular resistance with aging have not systematically excluded subjects with coronary artery disease or left ventricular systolic dysfunction. To better determine the influence of aging on PA hemodynamics in the absence of disease, we identified 47 normal subjects angiographically free of coronary artery disease (18 men, 29 women) with normal left ventricular systolic function (ejection fraction greater than or equal to 50% and left ventricular end-diastolic pressure less than 14 mm Hg) from 5,508 consecutive patients undergoing cardiac catheterization and coronary angiography between September 10, 1982 and March 9, 1987. All subjects met a set of clinical and laboratory criteria identifying them as normal. In group I (age greater than or equal to 60 years) mean PA pressure was 16 +/- 3 mm Hg, pulmonary vascular resistance was 124 +/- 32 dynes s cm-5 and the pulmonary/systemic vascular resistance ratio was 0.099 +/- 0.046. In contrast, in group II (age less than 60 years), these values were lower at 12 +/- 2 mm Hg, 70 +/- 25 dynes s cm-5 and 0.057 +/- 0.019, respectively (all p values less than 0.01 to 0.001). All these parameters increased linearly with age (r = 0.69, p less than 0.001 for pulmonary vascular resistance). The differences were not attributable to body surface area or gender. There was no difference in cardiac output, PA wedge pressure, aortic pressure or systemic vascular resistance between the 2 groups. Thus, PA pressure and vascular resistance increase with aging, a change not attributable to coronary disease or left ventricular systolic dysfunction.