Rheumatoid hyperviscosity: analysis of a patient with intermediate complexes that block other autoantibodies and a review of the literature

Objectives:

To report a patient whose rheumatoid arthritis presented withhyperviscosity syndrome, analyze this patient's rheumatoid factor, and review the previously reported patients.

Methods:

Immunofluorescence for antinuclear antibodies, double immunodiffusion,enzyme-linked immunosorbent assay, and size exclusion chromatography were used before and after plasmapheresis to study the patient's rheumatoid hyperviscosity. A systematic Medline search of rheumatic illnesses, hyperviscosity, and polyclonal gammopathy and references in identified papers was used to identify previously reported patients.

Results:

Similar to several previous patients, this patient's sera contained bothIgG and IgM rheumatoid factor and abundant intermediate complexes. Other autoantibodies, either from the patient or from other patients, were masked by rheumatoid factor or intermediate complexes from the reported patient's sera. Rheumatic hyperviscosity is seen uncommonly, being reported in only 18 patients with rheumatoid arthritis and nine with other rheumatic illnesses.

Conclusions:

There are two mechanisms by which rheumatoid factor can leadto hyperviscosity, both of which require large amounts of rheumatoid factor. Rheumatoid hyperviscosity must be recognized because this life-threatening syndrome usually can be successfully treated with plasmapheresis acutely and immunosuppressives for long-term control.     

An analysis of the pulsatile hemodynamic responses of the pulmonary circulation to acute and chronic pulmonary venous hypertension in the awake dog

In this study we measured high fidelity pulsatile pressure and flow waveforms at the inlet to the pulmonary vascular bed to assess the differences in adaptation to acute and chronic pulmonary venous hypertension in awake dogs. Acute elevations in left atrial pressure (P1a) were effected by inflation of left atrial balloons, while chronic elevations were accomplished by placement of aorta to left atrial shunts. Pulmonary artery hydraulic impedance was calculated and analysis of these data revealed marked differences between the responses to acute and chronic elevations of left atrial pressure. The acutely stressed dogs (n = 12) had significantly decreased pulmonary vascular resistance (when P1a = 16.9 +/- 1.0 mm Hg, PVR = 212 +/- 57 dynes sec/cm5; when P1a = 28.6 +/- 1.4 mm Hg PVR = 18 +/- 115 dynes sec/cm5; control P1a = 6.1 +/- 1.5 mm Hg, and PVR = 355 +/- 69 dynes sec/cm5) and normal characteristic impedances (ZO) (210 +/- 36, 227 +/- 39, 178 +/- 14 dynes sec/cm5, respectively), indicating recruitment of arteriolar-capillary perfusion density and no change in proximal pulmonary arterial physical properties. The chronic pulmonary venous hypertension group (n = 11) retained normal PVR (496 +/- 30 dynes sec/cm5) but demonstrated a markedly higher characteristic impedance, ZO = 361 +/- 11 dynes sec/cm5 (P < 0.001). This indicated a measurably different and extremely potent effect of chronic venous hypertension on the physical properties of the pulmonary vessels with an apparently increased arterial stiffness correlating with a 4-fold increase in Young's elastic modulus. These changes were not reversed by alpha-adrenergic blockade or acute lowering of left atrial pressures.     

Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: a case series.

BACKGROUND: Treatment of patients with secondary pulmonary hypertension has been unsatisfactory. OBJECTIVE: To describe exercise capacity, functional class, and hemodynamic variables after long-term intravenous infusion of prostacyclin in patients with secondary pulmonary hypertension. DESIGN: Case series. SETTING: Academic referral center. PATIENTS: 33 patients with secondary, precapillary pulmonary hypertension (New York Heart Association class III or IV). INTERVENTION: Continuous intravenous prostacyclin administered by portable infusion pump on a compassionate-use basis. MEASUREMENTS: Functional class, treadmill time, and hemodynamic variables. RESULTS: Patients were followed for an average of 12.7 +/- 5.6 months. Exercise tolerance and New York Heart Association class improved in each patient. The duration of treadmill exercise increased from 186 seconds to 491 seconds, an increase of 305 seconds (95% CI, 194 to 417 seconds; P < 0.001). Mean pulmonary artery pressure decreased from 60 mm Hg to 46 mm Hg, a decrease of 14 mm Hg (CI, 9 to 19 mm Hg; P < 0.001). Cardiac output increased from 3.90 L/min to 6.30 L/min, an increase of 2.40 L/min (CI, 1.56 to 3.25 L/min; P < 0.001). The pulmonary vascular resistance decreased from 1143 dynes x s/cm5 to 575 dynes x s/cm5, a decrease of 567 dynes x s/cm5 (CI, 407 to 727 dynes x s/cm5; P < 0.001). Patients with collagen vascular disease, congenital heart disease, and portopulmonary hypertension were analyzed with other patients and separately. All groups had a statistically significant reduction in mean pulmonary artery pressure and a statistically significant increase in cardiac output. CONCLUSION: Intravenous prostacyclin may be effective in the treatment of patients with certain types of secondary pulmonary hypertension.     

Progressive improvement in pulmonary vascular resistance after percutaneous mitral valvuloplasty

Percutaneous mitral valvuloplasty has been proposed as a nonsurgical technique for treating high-risk patients with mitral stenosis who are deferred from mitral valve replacement. The effect of this technique on patients with pulmonary hypertension, however, has not been fully evaluated. Accordingly, serial assessment of pulmonary vascular resistance was made in 14 patients with critical mitral stenosis and pulmonary hypertension (pulmonary vascular resistance greater than 250 dynes.sec/cm5 or mean pulmonary artery pressure greater than 40 mm Hg or both) who underwent percutaneous balloon dilatation of the mitral valve. Balloon valvuloplasty was performed with either one (n = 10) or two (n = 4) balloons through the transseptal approach, and it resulted in significant improvement in mean mitral gradient (from 18 +/- 4 to 9 +/- 4 mm Hg, p less than 0.001), systemic blood flow (from 3.7 +/- 1.2 to 5.0 +/- 2.2 l/min, p less than 0.001), and calculated mitral valve area (from 0.7 +/- 0.2 to 1.6 +/- 0.7 cm2, p less than 0.001). Immediately after balloon mitral valvuloplasty, pulmonary vascular resistance fell from 630 +/- 570 to 447 +/- 324 dynes.sec/cm5. Repeat catheterization 7 +/- 4 months after valvuloplasty showed further improvement of pulmonary hypertension in 12 of the 14 patients, with a mean pulmonary vascular resistance for the group as a whole of 280 +/- 183 dynes.sec/cm5, p less than 0.005. In two patients, mitral valve restenosis to a mitral valve area less than 1.0 cm2 was associated with a return of pulmonary hypertension to predilatation values.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pulmonary vascular response during Raynaud's phenomenon in progressive systemic sclerosis

Progressive systemic sclerosis and Raynaud's phenomenon are associated with a distinctly vascular form of pulmonary involvement and pulmonary hypertension. To investigate a possible underlying vasospastic predisposition in these patients, the pulmonary vascular response to Raynaud's phenomenon induced by cold-water hand immersion was examined in nine patients. Four patients had pulmonary fibrosis and four patients had the CREST syndrome; no patient had pre-existing pulmonary hypertension. During Raynaud's phenomenon, there was no significant rise in mean pulmonary artery pressure (15 +/- 3 versus 15 +/- 2 mm Hg, p = NS) or pulmonary vascular resistance (112 +/- 38 versus 118 +/- 50 dynes X second X cm-5, p = NS) over baseline, despite a significant rise in mean aortic pressure (104 +/- 14 versus 92 +/- 11 mm Hg, p less than 0.01) and systemic vascular resistance (1,700 +/- 450 versus 1,500 +/- 470 dynes X second X cm-5, p less than 0.01). It is concluded that pulmonary vasospasm with transient pulmonary hypertension does not occur in patients with progressive systemic sclerosis and Raynaud's phenomenon during episodes of Raynaud's phenomenon. Abnormal pulmonary vasospasm in these patients in response to other stimuli, however, is not excluded.     

Selective bone marrow involvement of lymphoplasmacytic cells secreting monoclonal IgA rheumatoid factor in a patient with Sj?gren's syndrome and serum hyperviscosity.

The clinical features and results of serological studies of a patient with Sjögren's syndrome, IgA kappa monoclonal gammopathy, and hyperviscosity syndrome are reported. The novel aspect of this case is the selective localisation to the bone marrow of lymphoplasmacytoid cells secreting IgA kappa morphologically identical to the cells infiltrating the salivary glands. The serum of the patient contained large amounts of immunoglobulin-anti-immunoglobulin immune complexes. By gel filtration chromatography it was shown that the immune complexes formed a peak of molecular weight 680 kilodaltons. The immune complexes were dissociable under acidic conditions. The immunoglobulin with rheumatoid activity was characterised as monoclonal IgA kappa protein. Treatment with plasmapheresis combined with immunosuppressive treatment with cyclophosphamide reduced the serum viscosity with concomitant clinical improvement.     

Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study

OBJECTIVE: Screening allows for early management of pulmonary arterial hypertension (PAH), a severe complication of systemic sclerosis (SSc). Since no consensus has been reached on the method and criteria for optimal screening, we sought to develop an algorithm based on symptoms, Doppler echocardiography, and right heart catheterization (RHC) for application to a nationwide multicenter SSc population in France. METHODS: This prospective study was conducted from September 2002 to July 2003 by experts at 21 SSc centers. At each center, SSc patients without severe pulmonary function abnormalities underwent Doppler echocardiography by an experienced cardiologist. Patients with a peak velocity of tricuspid regurgitation (VTR) of >3 meters/second or 2.5-3 meters/second with unexplained dyspnea were asked to undergo RHC to confirm PAH according to international guidelines. RESULTS: Of the 599 patients analyzed, 29 had known PAH and 33 had suspected PAH, based on Doppler echocardiography, and underwent RHC. Of these 33, 18 were found to have PAH, 3 had left ventricular dysfunction, and 12 had no PAH. Newly diagnosed cases of PAH were of mild severity (mean +/- SD pulmonary artery pressure [mPAP] 30 +/- 9 mm Hg, mean +/- SD total pulmonary resistance [TPR] 524 +/- 382 dynes x second/cm(5)). Hemodynamic findings in patients with known PAH were mPAP 49 +/- 17 mm Hg and TPR 1,007 +/- 615 dynes x second/cm(5). The estimate of PAH prevalence was 7.85% (95% confidence interval 5.70-10.00). CONCLUSION: This screening algorithm, based on dyspnea, Doppler echocardiographic evaluation of VTR, and RHC, enabled early detection of PAH at a mild stage. Whether mild PAH will evolve to severe PAH in reported cases and whether this early diagnosis translates into improved prognosis for patients with mild PAH will be evaluated in the ongoing 3-year followup of this cohort.     

Polymerized Monoclonal IgA in Two Patients with Myelomatosis and Hyperviscosity Syndrome

Two cases of multiple myeloma with IgA gammopathy and the syndrome of serum hyperviscosity are reported. A common feature of both patients was the production of large amounts of polymeric IgA (more than 50% of the monoclonal proteins existed as dimers or larger forms). However, similar large amounts of polymeric IgA were present in sera with only slightly raised viscosity, suggesting that factors such as the molecular configuration of the polymers or their sub-units may play a role in determining serum viscosity. The association of plasmapheresis and chemotherapy produced remarkable improvements of the symptoms attributable to increased serum viscosity, and 16 months after diagnosis one of the patients has survived relatively free of symptoms. The hyperviscosity syndrome is being diagnosed with increasing frequency in association with IgA multiple myeloma, and should be investigated in all cases with monoclonal proteins exceeding a concentration of 3-0 g/dl.     

Nifedipine in pulmonary arterial hypertension. Importance of Raynaud's phenomenon

We studied (via acute vasodilator testing with nifedipine) 27 patients with pulmonary arterial hypertension (PAH) (11 primary, 16 secondary PAH, [including six patients with Raynaud's phenomenon]) in order to identify predictors of hemodynamic response and specifically to assess whether patients with Raynaud's phenomenon and pulmonary hypertension were more likely to respond to nifedipine. Nifedipine decreased resting mean pulmonary artery (PA) pressure and pulmonary vascular resistance (PVR) in patients with Raynaud's phenomenon (delta PA - 6.8 +/- 10.5 mm Hg; delta PAD - PCW gradient - 9.3 +/- 4.7 mm Hg; delta PVR - 255 +/- 201 dynes.s.cm-5, all p less than .05) versus (delta PA 0.3 +/- 4.0 mm Hg; delta PAD - PCW gradient 0.4 +/- 5.0 mm Hg; delta PVR - 58 +/- 132 dynes.sec.cm-5, all NS), in the patients without Raynaud syndrome. These data suggest that patients with both primary and secondary PAH may benefit from nifedipine therapy, but that patients with Raynaud's phenomenon may respond particularly well, perhaps because of vasodilator-reversible pulmonary vasoconstriction. An alternative hypothesis is that prior chronic vasodilator therapy in the majority of our patients with Raynaud's phenomenon preserved pulmonary vasoreactivity.     

Tocilizumab is effective for pulmonary hypertension associated with multicentric Castleman’s disease

A 38-year-old man, diagnosed as having multicentric Castleman’s disease (plasma cell type) in 1995, had been treated with melphalan and prednisolone or prednisolone alone, but there was no remarkable response. In 2002, he was admitted to our hospital with a chief complaint of increasing dyspnea on effort. Laboratory data showed high serum IgG (10050 mg/dl), interleukin-6 (37.9 ng/ml), and vascular endothelial growth factor (VEGF 1920 pg/ml) levels. In addition, serum viscosity was very high (6.0 cp). Electrocardiogram, echocardiogram, and cardiac catheterization demonstrated pulmonary hypertension (PH). There were no other demonstrable causes of PH suggesting that PH was due to hyperviscosity syndrome and high VEGF level. He was treated with plasmapheresis, resulting in a transient improvement of dyspnea. Then, he was given humanized anti-interleukin-6 receptor antibody (tocilizumab), which resulted in the dramatic improvement of dyspnea and PH a few weeks later. PH is a rare complication of MCD, and could be successfully treated with tocilizumab.     

Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis.

Pulmonary hypertension is a life-threatening complication of lung fibrosis. Vasodilator therapy is difficult owing to systemic side effects and pulmonary ventilation-perfusion mismatch. We compared the effects of intravenous prostacyclin and inhaled NO and aerosolized prostacyclin in randomized order and, in addition, tested for effects of oxygen and systemic calcium antagonists (CAAs) in eight patients with lung fibrosis and pulmonary hypertension. Aerosolized prostaglandin (PG)I(2) caused preferential pulmonary vasodilatation with a decrease in mean pulmonary arterial pressure from 44.1 +/- 4.2 to 31.6 +/- 3.1 mm Hg, and pulmonary vascular resistance (RL) from 810 +/- 226 to 386 +/- 69 dyn. s. cm(-)(5) (p < 0.05, respectively). Systemic arterial pressure, arterial oxygen saturation, and pulmonary right-to-left shunt flow, measured by multiple inert gas analysis, were not significantly changed. Inhaled NO similarly resulted in selective pulmonary vasodilatation, with RL decreasing from 726 +/- 217 to 458 +/- 81 dyn. s. cm(-)(5). In contrast, both intravenous PGI(2) and CAAs were not pulmonary selective, resulting in a significant drop in arterial pressure. In addition, PGI(2) infusion caused a marked increase in shunt flow. Long-term therapy with aerosolized iloprost (long-acting PGI(2) analog) resulted in unequivocal clinical improvement from a state of immobilization and severe resting dyspnea in a patient with decompensated right heart failure. We concluded that, in pulmonary hypertension secondary to lung fibrosis, aerosolization of PGI(2) or iloprost causes marked pulmonary vasodilatation with maintenance of gas exchange and systemic arterial pressure. Long-term therapy with inhaled iloprost may be life saving in decompensated right heart failure from pulmonary hypertension secondary to lung fibrosis.     

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.     

Pre- and postoperative systemic hemodynamic evaluation in patients subjected to esophagogastric devascularization plus splenectomy and distal splenorenal shunt： A comparative study in schistomomal portal hypertension

AIM： To investigate the systemic hemodynamic effects of two surgical procedures largely employed for treatment of schistosomal portal hypertension.
METHODS： Thirty-six patients undergoing elective surgical treatment of portal hypertension due to hepatosplenic mansonic schistosomiasis were prospectively evaluated. All patients were subjected to preoperative pulmonary artery catheterization; 17 were submitted to esophagogastric devascularization and splenectomy （EGDS） and 19 to distal splenorenal shunt （DSRS）. The systemic hemodynamic assessment was repeated 4 d after the surgical procedure.
RESULTS： Preoperative evaluation revealed （mean ± SD） an increased cardiac index （4.78 ± 1.13 L/min per m^2）, associated with a reduction in systemic vascular resistance index （1457 ± 380.7 dynes.s/cm^5.m^2）. The mean pulmonary artery pressure （18 ± 5.1 mmHg） as well as the right atrial pressure （7.9 ± 2.5 mmHg） were increased, while the pulmonary vascular resistance index （133 ± 62 dynes.s/cm^5.m^2） was decreased. Four days after EGDS, a significant reduction in cardiac index （3.80 ± 0.4 L/min per m^2, P 〈 0.001） and increase in systemic vascular resistance index （1901.4 ± 330.2 dynes.s/cm^5. m^2, P 〈 0.001） toward normal levels were observed. There was also a significant reduction in pulmonary artery pressure （12.65 ± 4.7 mmHg, P 〈 0.001） and no significant changes in the pulmonary vascular resistance index （141.6 ±102.9 dynes.s/cm^5.m^2）. Four days after DSRS, a non-significant increase in cardiac index （5.2 ± 0.76 L/min per m^2） and systemic vascular resistance index （1389 ± 311 dynes.s/cm^5.m^2） was observed. There was also a non-significant increase in pulmonary artery pressure （19.84 ± 5.2 mmHg）, right cardiac work index （1.38 ± 0.4 kg.m/m^2） and right ventricular systolic work index （16.3 ± 6.3 g.m/m^2）, without significant changes in the pulmonary vascular resistance index （139.7 ± 67.8 dynes.s/cm^5.m^2）.
CONCLUSIO     

Cardiac function in alcohol-associated systemic hypertension

The pathogenesis of alcohol cardiomyopathy is obscure. Because systemic hypertension is observed in one-third of alcoholics, the relation of this finding to left ventricular (LV) function was analyzed in 66 alcoholics (26 with a blood pressure of 160/95 mm Hg or higher) 4 to 5 days after alcohol withdrawal. Hypertensive alcoholics had a more abnormal ratio of preejection period/LV ejection time (PEP/ET) (0.398 +/- 0.01 vs 0.35 +/- 0.01, p less than 0.02) than normotensive alcoholics (matched normal 0.290 +/- 0.01). Hypertensive alcoholics (transitory hypertension) with blood pressures of 120/80 mm Hg or less at time of study also had more abnormal PEP/LVET than matched normotensive alcoholics (0.415 +/- 0.03 vs 0.331 +/- 0.01, p less than 0.05). In both hypertensive (77 +/- 6 dynes/cm2 X 10(3)) and normotensive alcoholics (67 +/- 4 dynes/cm2 X 10(3) LV stress was elevated (normal 46 +/- 3 dynes/cm2 X 10(3), both p less than 0.02). However, LV mass was not increased (hypertensive 96 +/- 4 g/m2; vs normotensive 100 +/- 4 g/m2; (normal 92 +/- 5 g/m2), resulting in a markedly increased stress to mass ratio (hypertensive 0.8 +/- 0.06; Normal 0.05 +/- 0.05, p less than 0.02). Hypertensive alcoholics also had LV "hyperfunction," with an increased stress/LV end-systolic volume ratio (1.7 +/- 0.1 vs 1.3 +/- 0.1 dynes/cm2 X 10(3)/ml, p less than 0.02). Thus, hypertensive alcoholics, even those with transitory hypertension, have more abnormal cardiac function than normotensive alcoholics. Presence of hypertension with hyperdynamic LV features may be a prelude to heart failure.     

Early improvement in left ventricular diastolic function after relief of chronic right ventricular pressure overload

Chronic right ventricular pressure overload is associated with left ventricular diastolic dysfunction. Whether or not an abrupt reduction in pulmonary artery pressure in patients with chronic pulmonary hypertension results in early improvement of left ventricular diastolic function is unknown. To assess this, the Doppler indexes of left ventricular diastolic function and echocardiographic measures of left ventricular volume were analyzed in 22 patients (age, 41 +/- 14 years, mean +/- SD) before and within 1 week after pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension. Mean duration of cardiopulmonary symptoms was 37 months (range, 4 months to 9 years). After operation, mean pulmonary artery pressure and pulmonary vascular resistance decreased (50 +/- 13 to 29 +/- 9 mm Hg and 904 +/- 654 to 283 +/- 243 dynes.sec/cm5, respectively, both p less than 0.001), pulmonary artery wedge pressure was unchanged (11 +/- 5 to 12 +/- 5 mm Hg), and cardiac index increased (2.0 +/- 0.5 to 2.8 +/- 0.7 l/min/m2 p less than 0.001). Left ventricular end-diastolic volume and stroke volume increased significantly (58.5 +/- 18.0 to 76.6 +/- 25.0 ml and 30.3 +/- 12.3 to 41.8 +/- 12.5 ml, respectively, both p less than 0.001) after surgery.(ABSTRACT TRUNCATED AT 250 WORDS)     

Portopulmonary hypertension: Results from a 10-year screening algorithm

Portopulmonary hypertension (POPH) is the elevation of pulmonary artery pressure due to increased resistance to pulmonary blood flow in the setting of portal hypertension. Increased mortality has occurred with attempted liver transplantation in such patients and thus, screening for POPH is advised. We examined the relationship between screening echocardiography and right heart catheterization determinations of pressure, flow, volume, and resistance. A prospective, echocardiography-catheterization algorithm was followed from 1996 to 2005. Consecutive transplantation candidates underwent Doppler echocardiography to determine right ventricular systolic pressure (RVSP). Of 1,235 patients, 101 with RVSP >50 mm Hg underwent catheterization to measure mean pulmonary artery pressure (MPAP), flow via cardiac output (CO), central volume via pulmonary artery occlusion pressure (PAOP), and resistance via calculated pulmonary vascular resistance (PVR). Bland-Altman analysis suggested marked discordance between echocardiography-derived RVSP and catheterization results. All-cause pulmonary hypertension (MPAP >25 mm Hg) was documented in 90/101 (90%) patients. Using current pressure and resistance diagnostic guidelines (MPAP >25 mm Hg, PVR > or =240 dynes/s/cm(-5)), POPH was documented in 66/101 (65%) patients. Elevated MPAP was due to increased CO and/or PAOP in 35/101 (35%) patients with normal resistance (PVR <240 dynes/s/cm(-5)). The transpulmonary gradient (MPAP-PAOP) further characterized POPH in the presence of increased volume. Model for end stage liver disease (MELD) scores correlated poorly with MPAP and PVR. In conclusion, right heart catheterization is necessary to confirm POPH and frequently identifies other reasons for pulmonary hypertension (e.g., high flow and increased central volume) in liver transplantation candidates. Severity of POPH correlates poorly with MELD scores.     

Prevalence of pulmonary hypertension in patients with hepatosplenic Mansonic schistosomiasis--prospective study

BACKGROUND/AIMS: Thirty-four patients with portal hypertension and previous history of esophageal varices hemorrhage due to hepatosplenic Mansonic schistosomiasis were prospectively studied. METHODOLOGY: All patients underwent invasive hemodynamic monitoring with introduction of a pulmonary artery catheter. Hemodynamic evaluation was characterized by an increased cardiac index (4.90 +/- 1.27 L/min/m2) associated to a decrease in systemic vascular resistance index (1461 +/- 443.04 dynes.sec/cm5.m2). RESULTS: Mean pulmonary artery pressure (17.97 +/- 6.97 mmHg) and right atrial pressure (7.65 +/- 3.67 mmHg) were increased while pulmonary vascular resistance index was decreased (147.95 +/- 126.21 dynes.sec/cm5.m2). Twenty-four patients (70.5%) presented pulmonary hypertension (mean pulmonary artery pressure > 15 mmHg); in fifteen (44.1%) pulmonary pressure was between 15 and 20 mmHg, in three between 20 and 25 mmHg and, in four patients, pulmonary pressure was higher than 25 mmHg. CONCLUSIONS: In conclusion, pulmonary hypertension is a frequent complication in patients with portal hypertension due to hepatosplenic Mansonic schistosomiasis and, in 20.6% of the cases, it can be considered as moderate or severe. Our results suggest that shunt surgeries, which can aggravate pulmonary hypertension, should be employed very cautiously in the treatment of schistosomal portal hypertension.     

Cardiac energetics after intravenous enoximone in idiopathic dilated cardiomyopathy

The acute effects of enoximone on left ventricular (LV) function, myocardial oxygen metabolism and central and systemic hemodynamics were investigated in 12 patients with idiopathic dilated cardiomyopathy. Enoximone was administered intravenously at a rate of 12.5 mg/min; the average dose was 1.42 mg/kg. LV systolic pressure decreased significantly (p less than 0.01) from 128 +/- 18 to 96 +/- 16 mm Hg (mean +/- standard deviation), LV end-diastolic pressure from 16 +/- 3 to 5 +/- 3 mm Hg, LV end-diastolic volume from 288 +/- 43 to 210 +/- 58 ml, LV end-diastolic wall stress from 33 +/- 15 to 11 +/- 5 10(3) dynes/cm2 and LV peak systolic wall stress from 243 +/- 73 to 159 +/- 42 10(3) dynes/cm2. Heart rate increased from 86 +/- 18 to 100 +/- 20 beats/min, ejection fraction from 43 +/- 7 to 52 +/- 14% (p less than 0.05). Cardiac index, stroke volume index and dP/dtmax did not change significantly. Systemic vascular resistance decreased significantly (p less than 0.01) from 1,311 +/- 444 to 1,027 +/- 356 dynes s cm-5, mean pulmonary artery pressure from 13 +/- 6 to 8 +/- 2 mm Hg, mean right atrial pressure from 4 +/- 2 to 2.6 +/- 2 mm Hg and mean arterial pressure from 95 +/- 13 to 74 +/- 13 mm Hg.(ABSTRACT TRUNCATED AT 250 WORDS)     

Early changes of right heart geometry after pulmonary thromboendarterectomy

To determine the changes in right heart hemodynamics and geometry early after surgery for chronic pulmonary hypertension due to large vessel thromboembolic occlusion, 30 patients were evaluated 8 +/- 8 days (mean +/- SD) before and 6 +/- 4 days after pulmonary thromboendarterectomy by two-dimensional echocardiography and right heart catheterization. Surgery resulted in an early significant improvement in hemodynamic variables including mean pulmonary artery pressure (48 +/- 12 to 28 +/- 8 mm Hg, p less than 0.001), right ventricular systolic pressure (76 +/- 20 to 47 +/- 15 mm Hg, p less than 0.001), pulmonary vascular resistance (935 +/- 620 to 278 +/- 252 dynes.s.cm-5, p less than 0.001) and cardiac index (2.0 +/- 0.5 to 2.9 +/- 0.6 liters/min per m2, p less than 0.001). Similarly, echocardiographic variables of right heart structures, which were well outside the normal range preoperatively, improved significantly early after thromboendarterectomy. These included diameters of the pulmonary artery (2.8 +/- 0.3 to 2.4 +/- 0.4 cm, p less than 0.001), inferior vena cava (2.9 +/- 0.6 to 2.2 +/- 0.4 cm, p less than 0.001) and right atrium (6.8 +/- 1.5 to 5.9 +/- 1.5 cm, p less than 0.001) as well as right ventricular short axis (4.5 +/- 0.8 to 3.7 +/- 0.8 cm, p less than 0.001) and long axis (8.7 +/- 0.9 to 8.1 +/- 0.9 cm, p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)     

Relation between hemodynamic and ventilatory responses in determining exercise capacity in severe congestive heart failure

The cause of exercise intolerance in congestive heart failure is unclear. Hemodynamic and ventilatory responses were measured during symptomatic maximal upright bicycle exercise in 28 patients with chronic severe left ventricular failure who achieved a maximal oxygen uptake of only 12 +/- 4 ml/min/kg (+/- standard deviation). All patients reached anaerobic metabolism as the respiratory exchange ratio rose and arterial pH fell significantly. Pulmonary capillary wedge pressure increased from 20 +/- 10 mm Hg at rest to 38 +/- 9 mm Hg at peak exercise and cardiac index increased from 2.51 +/- 0.73 to 4.54 +/- 1.65 liters/min/m2 (both p less than 0.001). Systemic vascular resistance decreased, but pulmonary vascular resistance did not change during exercise. Despite the marked pulmonary venous hypertension at peak exercise, blood gases were unchanged (PaO2, 96 +/- 15 mm Hg; PaCO2, 35 +/- 7 mm Hg). Systemic arterial oxygen content increased from 16 +/- 2 to 17 +/- 2 vol% (p less than 0.01). Changes in pulmonary capillary wedge pressure did not correlate with changes in arterial oxygen content. Results were similar whether patients were limited by dyspnea or fatigue. Thus, exercise intolerance in patients with severe left ventricular failure is associated with marked elevation of pulmonary capillary wedge pressure and anaerobic metabolism without hypoxemia or altered carbon dioxide tension. These findings suggest that exercise ability in congestive heart failure is more dependent on cardiac output than on ventilatory consequences of pulmonary congestion.