Pulmonary dysfunction in systemic lupus erythematosus and anti-phospholipid syndrome patients.

OBJECTIVE: To assess and compare parameters of pulmonary function in systemic lupus erythematosus (SLE) and anti-phospholipid syndrome (APS) patients. METHODS: Consecutive patients (n = 74) who were free of respiratory symptoms were divided into four groups: 1) SLE (n = 23); 2) SLE with anti-phospholipid antibodies (aPL) (n = 18); 3) SLE with APS (n = 20); and 4) primary APS (PAPS) (n = 13). Pulmonary function testing, single breath diffusion capacity of carbon monoxide (DLCO/SB) and echocardiography studies were performed. Induced sputum cytology was analysed. RESULTS: Forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), and DLCO were significantly reduced in SLE compared to PAPS patients (p = 0.039; p = 0.017; p = 0.029, respectively). Elevated pulmonary arterial pressure was observed in two patients with SLE and aPL and in two with SLE and APS. Lymphocyte and eosinophil counts in induced sputum showed no significant differences; however, a trend towards lower CD4 counts in SLE vs. PAPS was noted (p = 0.086), while in patients with both SLE and APS, a low CD4/CD8 ratio was seen. Patients with APS were older than patients without APS (47.12+/-14.86 vs. 34.29+/-12.6, p = 0.0001), while SLE patients were younger than PAPS patients (38.19+/-14.68 vs. 48.53+/-13.97, p = 0.023). CONCLUSION: Abnormal pulmonary functions tests were detected frequently in asymptomatic patients with SLE or PAPS. Although SLE patients were younger, pulmonary function was significantly more impaired in SLE as compared to PAPS patients.

Prevalence of asymptomatic coronary arterial abnormalities detected by angiography in grown-up patients with congenital heart disease.

AIMS: Coronary arterial abnormalities, congenital and acquired, may co-exist with structural congenital malformations of the heart. This study analyses information obtained from coronary angiography in grown-up patients with congenital cardiac disease undergoing cardiac catheterisation. METHODS AND RESULTS: We studied 448 patients, of whom 181 (40%) had selective coronary angiography. This was undertaken as a routine measure in 165 (37%), and because of clinical indications in 16 (4%). Aortic root angiography was carried out in 107 (24%). Coronary arterial abnormalities were demonstrated in one-third of those undergoing selective angiography, and in 69% of those with a clinical indication for angiography, compared with 30% who had a routine procedure. In those patients undergoing aortography alone, abnormalities were recognized in 17%, while the angiographic images were inconclusive in 24%. In the remaining 59%, no abnormality was apparent, but only the proximal coronary arteries could be seen clearly. In total, congenital abnormalities of the coronary arteries were found in 11% of the patients, acquired lesions were demonstrated in 5%, and surgically acquired lesions in 2%. CONCLUSION: Routine coronary angiography during catheterization of grown-up patients with congenital heart disease revealed a high incidence of abnormalities of the coronary arteries. Selective injection into the arteries had a higher sensitivity than aortography. Consideration should be given to performing routine coronary arteriography during invasive investigation of patients with congenital cardiac disease, as it provides useful and often unexpected information, which may be valuable for the interpretation of symptoms and the planning of subsequent surgery.

Distal arterial flow in patients undergoing upper extremity dialysis shunting: a prospective study using doppler sonography.

OBJECTIVE: The objective of this study was to document changes in the distal circulation after creation of a proximal upper extremity dialysis shunt and to correlate these findings with the patient's clinical condition. SUBJECTS AND METHODS: We prospectively examined 18 patients scheduled for upper extremity shunt creation. We used color and spectral Doppler sonography to examine flow in the radial and ulnar arteries, noting flow direction and peak systolic velocity. After the shunt procedure, we repeated the measurements and correlated them statistically with hand symptomatology. RESULTS: Six (33%) of 18 patients were symptomatic. The mean peak systolic velocities in the radial and ulnar arteries were 52 and 61 cm/sec, respectively, before surgery, and decreased to 12 cm/sec after surgery in the radial artery and 44 cm/sec in the ulnar artery. The mean percentage of decrease in peak systolic velocity was 77% in the radial artery and 28% in the ulnar artery. Eight patients showed reversed flow. No statistical correlation was found between change in peak systolic velocity values before and after surgery and the presence of hand symptoms. Similarly, no correlation was found between flow reversal and symptoms. The most consistent factor associated with symptoms was diabetes; all symptomatic patients were diabetic, but only 54% of the diabetic patients were symptomatic. CONCLUSION: The difference in the peak systolic velocities in the radial and ulnar arteries after shunt construction does not correlate with symptoms. The hand can tolerate a significant decrease in the peak systolic velocity and even flow reversal without symptomatology.

The use of subcutaneous erythropoietin and intravenous iron for the treatment of the anemia of severe, resistant congestive heart failure improves cardiac and renal function and functional cardiac class, and markedly reduces hospitalizations.

OBJECTIVES: This study evaluated the prevalence and severity of anemia in patients with congestive heart failure (CHF) and the effect of its correction on cardiac and renal function and hospitalization. BACKGROUND: The prevalence and significance of mild anemia in patients with CHF is uncertain, and the role of erythropoietin with intravenous iron supplementation in treating this anemia is unknown. METHODS: In a retrospective study, the records of the 142 patients in our CHF clinic were reviewed to find the prevalence and severity of anemia (hemoglobin [Hb] <12 g). In an intervention study, 26 of these patients, despite maximally tolerated therapy of CHF for at least six months, still had had severe CHF and were also anemic. They were treated with subcutaneous erythropoietin and intravenous iron sufficient to increase the Hb to 12 g%. The doses of the CHF medications, except for diuretics, were not changed during the intervention period. RESULTS: The prevalence of anemia in the 142 patients increased with the severity of CHF, reaching 79.1% in those with New York Heart Association class IV. In the intervention study, the anemia of the 26 patients was treated for a mean of 7.2 +/- 5.5 months. The mean Hb level and mean left ventricular ejection fraction increased significantly. The mean number of hospitalizations fell by 91.9% compared with a similar period before the study. The New York Heart Association class fell significantly, as did the doses of oral and intravenous furosemide. The rate of fall of the glomerular filtration rate slowed with the treatment. CONCLUSIONS: Anemia is very common in CHF and its successful treatment is associated with a significant improvement in cardiac function, functional class, renal function and in a marked fall in the need for diuretics and hospitalization.

Reduction of reinfarction and angina with use of low-molecular-weight heparin therapy after streptokinase (and heparin) in acute myocardial infarction.

This study examined whether extending the anticoagulation effect of heparin by low-molecular-weight heparin (clexane) can prevent recurrent myocardial infarction (AMI) treated by streptokinase. On the fifth day after AMI and after heparin therapy cessation, 103 patients were randomly assigned to either treatment with low-molecular-weight heparin (40 mg subcutaneously per day for 25 days, n=43) or control (no treatment, n=60). All patients were followed carefully for 6 months after the infarction date. A total of 32 patients (31%) sustained a cardiac event during the 6-month observation period. There were 12 patients (20%) with reinfarction in the control group versus 2 patients (4.6%) in the low-molecular-weight heparin group during the first 30 days of the study (p=0.02). One additional patient sustained reinfarction at 3 months of followup in the control group, which yielded a total of 13 patients (21.6%) sustaining reinfarction in the control group versus 2 patients (4.6%) in the low-molecular-weight heparin group during 6 months of followup (p=0.01). Angina pectoris after AMI was diagnosed in 13 control patients (21.6%) versus 4 low-molecular-weight heparin-treated patients (9.3%) (p=0.078) during the study period. No major bleeding events were reported in either low-molecular-weight heparin-treated or control patients. Among patients with recently diagnosed AMI treated by streptokinase, extending the anticoagulant effect of heparin for 25 days may prevent recurrent coronary events for at least one month.

Improvement of cardiac performance by intravenous infusion of L-arginine in patients with moderate congestive heart failure.

OBJECTIVES: The aim of this study was to evaluate the hemodynamic effect of L-arginine infusion in patients with congestive heart failure. BACKGROUND: Endothelium-dependent vasodilation is impaired in patients with congestive heart failure. Nitric oxide, which was identified as endothelium-derived relaxing factor, is generated by nitric oxide synthase from L-arginine. Our hypothesis was that administration of L-arginine in patients with congestive heart failure may increase nitric oxide production and have a beneficial hemodynamic effect. METHODS: Twelve patients with congestive heart failure (New York Heart Association class II or III) due to coronary artery disease (left ventricular ejection fraction < 35%) were given 20 g of L-arginine by intravenous infusion over 1 h at a constant rate. Stroke volume, cardiac output and left ventricular ejection fraction were determined with Doppler echocardiography at baseline and at 30 and 60 min and 1 h after the end of infusion. Blood and urinary levels of nitrite/nitrate (NO2/NO3), stable metabolites of nitric oxide, were measured and clearance was calculated. RESULTS: One hour of infusion of L-arginine resulted in a significant increase in stroke volume (from 68 +/- 18 ml to 76 +/- 23 ml [mean +/- SD], p = 0.014) and cardiac output (from 4.07 +/- 1.22 liters/min to 4.7 +/- 1.42 liters/min, p = 0.006) without a change in heart rate. Mean arterial blood pressure decreased (from 102 +/- 11 mm Hg to 89 +/- 9.5 mm Hg, p < 0.002), and systemic vascular resistance decreased significantly. Within 1 h after cessation of L-arginine infusion, blood pressure, stroke volume, cardiac output and systemic vascular resistance were statistically not different from baseline values. Clearance of NO2/NO3 increased significantly during L-arginine administration (from 13.28 +/- 0.42 ml/min to 29.97 +/- 1.09 ml/min, p < 0.001). CONCLUSIONS: Infusion of L-arginine in patients with congestive heart failure results in increased production of nitric oxide, peripheral vasodilation and increased cardiac output, suggesting a beneficial hemodynamic and possibly therapeutic profile.

One-year clinical and echocardiographic follow-up of patients with congestive cardiomyopathy treated with captopril compared to placebo.

The beneficial hemodynamic and clinical effects of angiotensin-converting enzyme (ACE) inhibition in patients with severe congestive heart failure has recently been documented in large-scale studies. This mode of therapy when added to digitalis and diuretics improves survival. To evaluate the clinical effect and the changes in cardiac dimensions of captopril compared to placebo we followed 50 patients with severe congestive heart failure over 1 year using echo-Doppler cardiography. After randomization, 25 patients were started on captopril and 25 patients on placebo. At baseline and at 6 and 12 months, each patient underwent exercise tolerance test, radionuclide angiography to estimate left and right ventricular ejection fraction, M mode and two-dimensional echocardiography and Doppler cardiography to calculate cardiac dimensions and stroke volumes. During follow-up two patients in the captopril group and four in the placebo group died. Due to clinical deterioration nine patients in the placebo group had to be started on open-label captopril. Treatment with captopril was associated with a more significant improvement in functional class and exercise duration compared to placebo. Forward stroke volume estimated from Doppler echocardiography increased significantly by captopril from 47 +/- 3 to 55 +/- 3 ml and decreased in the placebo-treated patients from 49 +/- 5 to 44 +/- 4 ml. This improvement was associated with a trend towards reduced heart rate by captopril. Left ventricular end diastolic volume tended to increase in the placebo group and did not change in the captopril group. Calculated mitral regurgitant volume at 6 and 12 months tended to be lower in the captopril-treated patients. Thus captopril therapy proved efficacious in patients with severe congestive heart failure and resulted in increased forward stroke volume; it may have a beneficial effect on cardiac dimensions and on mitral regurgitation.

Lupus anticoagulant, thrombosis and thrombocytopenia in systemic lupus erythematosus.

Thirty-six patients were studied for the presence of the lupus anticoagulant and its possible clinical and laboratory associations. Seven patients were found to have the anticoagulant. These patients had a significantly increased incidence of both thromboembolic complications (5/7 vs. 2/29) and thrombocytopenia (4/7 vs. 4/29). None of them had a bleeding tendency. Except for these associations, patients with the anticoagulant did not differ from those without the anticoagulant by any demographic, clinical, or laboratory parameter. The present study confirms observations that SLE patients with the lupus anticoagulant have an increased risk of thrombosis.