Abnormal spatial QRS-T angle, a marker of ventricular repolarisation, predicts serious ventricular arrhythmia in systemic sclerosis.

OBJECTIVES: Cardiac involvement may be under-diagnosed in asymptomatic patients with systemic sclerosis (SSc). Standard electrocardiography-derived spatial QRS-T angle (spQRS-Ta) is an established marker of ventricular repolarisation heterogeneity, and a strong independent predictor of cardiac morbidity and mortality, including sudden death, in the general population. We examined whether spQRS-Ta is abnormal in asymptomatic SSc patients and assessed its predictive value for possibly concurrent, serious ventricular arrhythmia. METHODS: SpQRS-Ta and 24-hour Holter recordings were obtained from 69 SSc patients (aged 51±13 years, 63 women) without clinically evident cardiac involvement and having left ventricular ejection fraction at least 50% by echocardiography. 'Healthy' subjects matched 1:1 with patients for age, gender and body mass index served as controls. RESULTS: SpQRS-Ta was wider in SSc (median value 15.6°, interquartile range 10.6-24.3°) than controls (10.5°, 7.3-13.5°, p=0.0001) and not associated with skin fibrosis extent or specific clinical manifestations and autoantibodies. Twenty-four-hour Holter recordings revealed couplets of ventricular beats in six (Lown class IVa) and non-sustained ventricular tachycardia in five patients (Lown class IVb); spQRS-Ta was wider in those eleven patients with serious ventricular arrhythmia than the remaining patients (24.9°, 14.9-31.3° vs. 14.4°, 9.6-22.3°; p=0.02). A spQRS-Ta>19.3° demonstrated 80% sensitivity and 68% specificity (area under the curve 0.81, p=0.02) to predict the presence of non-sustained ventricular tachycardia in Holter monitoring. CONCLUSIONS: Ventricular repolarisation heterogeneity, as reflected by wider spQRS-Ta, is common in SSc. Increased spQRS-Ta could serve as a simple screening test for further investigation to identify patients at risk or prone to develop life-threatening ventricular arrhythmia.

The association between the spatial QRS-T angle with cardiac autonomic neuropathy in subjects with Type 2 diabetes mellitus.

AIMS: To examine differences in the spatial QRS-T angle in patients with Type 2 diabetes mellitus with and without cardiac autonomic neuropathy. METHODS: Two hundred and thirty-two patients with diabetes mellitus (105 with cardiac autonomic neuropathy and 127 without cardiac autonomic neuropathy) and 232 control subjects, matched by gender and age, were studied. Diagnosis of cardiac autonomic neuropathy was based on the classic autonomic function tests. All subjects underwent a digital electrocardiographic recording. Electrocardiographic parameters were measured using the Modular Electrocardiographic Analysis (MEANS) program. Left ventricular mass index (LVMi) and global myocardial performance index (Tei index) of the left ventricle were assessed by ultrasonography. RESULTS: The spatial QRS-T angle was higher in the patients with diabetes in comparison with the control subjects (24.5 ± 10.7 vs. 9.7 ± 4.5°, P < 0.001) and in the patients with diabetes and cardiac autonomic neuropathy than in those without cardiac autonomic neuropathy (30.1 ± 11.3 vs. 19.5 ± 7.1, P < 0.001). No differences were found in the QT interval between the studied groups. Multivariate linear regression analysis in subjects with diabetes after controlling for age, gender, BMI, blood pressure, diabetes duration, HbA(1c) , lipids, microalbuminuria and insulin resistance, demonstrated significant and independent associations between the spatial QRS-T angle with presence and severity of cardiac autonomic neuropathy, all parameters of heart rate variability, LVMi and Tei index. CONCLUSIONS: The spatial QRS-T angle is increased in patients with Type 2 diabetes who have cardiac autonomic neuropathy, suggesting increased ventricular arrhythmogenicity, and is associated with the structural and functional properties of the myocardium. Further research is warranted to evaluate its role in cardiovascular risk stratification of patients with diabetes.

Hypertrophic obstructive cardiomyopathy in rheumatoid arthtritis - coincidence or association? A case report.

A 60-year-old woman with a history of rheumatoid arthritis was admitted to the hospital for investigation of dyspnea on exertion (New York Heart Association class II), polyarthralgias and mild fever. An echocardiogram revealed asymmetric hypertrophy of the interventricular septum with signs of subaortic obstruction.The coexistence of rheumatoid arthritis and hypertrophic cardiomyopathy could be connected with the human lymphocyte antigen DR4, which is common in both conditions. Further studies are necessary to assess whether a true association of the above diseases exists.

Myocardial performance and aortic elasticity are impaired in patients with ankylosing spondylitis.

OBJECTIVE: To measure aortic stiffness and global left ventricular (LV) function in patients with ankylosing spondylitis (AS) and no clinical evidence of heart disease. METHODS: Fifty-seven consecutive patients with AS (54 males, three females, mean age 41.78+/-10.02 years) without clinical evidence of cardiac involvement and 78 healthy subjects (73 males, five females, mean age 39.92+/-9.11 years) underwent complete echocardiographic study. Aortic stiffness was determined non-invasively by aortic distensibility (AoD) and the global LV function was evaluated by the myocardial performance index (the Tei index). RESULTS: AoD in patients with AS [(2.21+/-0.24)x10(-6) cm(2) dyn(-1)] was decreased compared to controls [(2.58+/-0.19) )x10(-6) cm(2) dyn(-1), p<0.01], confirming that aortic stiffness is increased in AS. The LV Tei index was significantly increased in the patient group compared to the control group (0.392+/-0.031 vs. 0.370+/-0.034, p<0.01). The ejection fraction (EF) did not differ between the two groups (p>0.05). In multivariate linear regression analysis, AoD was significantly associated with the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and LV isovolumic relaxation time (IVRT) whereas the LV Tei index was associated with BASDAI and the LV mass index. CONCLUSIONS: Patients with AS and no clinical evidence of cardiac disease have increased stiffness of the aorta and decreased global myocardial performance and both of these abnormal measurements correlate with disease activity. The abnormal Tei index may reflect an early manifestation of cardiac dysfunction in these patients.

The presence of anti-centromere antibodies may predict progression of estimated pulmonary arterial systolic pressure in systemic sclerosis.

OBJECTIVE: To define the risk factors associated with a relatively rapid increase in estimated pulmonary arterial systolic pressure (PASP) in patients with systemic sclerosis (SSc). METHODS: SSc patients undergoing screening for pulmonary arterial hypertension (PAH) by echocardiography were identified and their charts were retrospectively reviewed. In all patients, we recorded PASP, pulmonary function, and clinical and laboratory data. PAH was defined as an estimated PASP> or =40 mmHg. In each patient, the PASP values with their corresponding time intervals were fitted to a linear function and the slope of the line was calculated. RESULTS: Seventy-one patients with at least two echocardiographic studies each were analysed. In 16 (23%) patients, the rate of PASP progression was > or =2.5 mmHg/year whereas in the remaining 55 (77%) patients the rate of progression was <2.5 mmHg/year. In multiple logistic regression analysis, anti-centromere antibodies (ACA) (OR 8.75, CI 1.12-68.38, p = 0.039) and age > or =50 years at diagnosis (OR 8.76, CI 1.28-60.14, p = 0.027) were independently associated with a rise of PASP by > or =2.5 mmHg/year. Baseline forced vital capacity (FVC) <70% (predicted), Raynaud's duration preceding skin manifestations by > or =5 years, and fibrosis on lung computed tomography (CT) were not associated with a rapid rise of PASP (p>0.05). CONCLUSIONS: Old age at diagnosis and ACA are associated with a relatively rapid rise of PASP estimated by echocardiography in SSc. Screening for PAH in these patients may, if followed by right heart catheterization, detect PAH at an earlier stage and guide therapeutic decisions.

Is the heart affected in primary Sjögren's syndrome? An echocardiographic study.

OBJECTIVE: To evaluate whether patients with primary Sjögren's syndrome without overt cardiac disease have echocardiographic abnormalities and their relation with clinical and laboratory data. METHODS: One hundred and seven consecutive patients with primary Sjögren's syndrome and 112 healthy controls, matched for age and gender, underwent complete echocardiographic study. RESULTS: Thirty-two patients had mitral valve regurgitation (p<0.001) whereas tricuspid and aortic valve regurgitation were, also, more frequent in the patient group (p=0.022 and p=0.007 respectively). In multivariate analyses, low C4 levels of complement and age were strong predictors of mitral valve regurgitation whereas age was predictor of aortic valve regurgitation. Tricuspid valve regurgitation was associated with pulmonary hypertension. Clinically silent pericardial effusion, found in 9 patients (p=0.008), was associated with cryoglobulinemia and primary biliary cirrhosis. Twenty-four patients had pulmonary hypertension (p<0.001) whereas hypocomplementemia and cryoglobulinemia were strong predictors of pulmonary artery systolic pressure. The analyses reveal that easy fatigability was associated with pulmonary hypertension and low C4 levels. The patients' left ventricular mass index differed significantly from the controls (108.9+/-17.21 gm(-2) vs. 85.8+/-6.73 gm(-2), p<0.001) and was associated with palpaple purpura and anti-Ro/SSA. From the diastolic function indices only the left ventricular isovolumic relaxation time differed significantly among patients and controls. CONCLUSION: Valvular regurgitation, pericardial effusion, pulmonary hypertension and increased left ventricular mass index occur with disproportionately high frequency in patients with primary Sjögren's syndrome and no clinically apparent heart disease. Thus echocardiographic studies may need to be performed in these patients especially when palpable purpura, antibody reactivity and low C4 levels are present.

Impaired aortic elastic properties in patients with systemic sarcoidosis.

BACKGROUND: Systemic sarcoidosis (Sar) is a granulomatous disorder involving multiple organs. Widespread vascular involvement and microangiopathy are common in patients with Sar. In addition, subclinical cardiac involvement is increasingly recognized in patients with Sar. However, data on the effect of Sar on the elastic properties of the arteries and myocardial performance are limited. In this study we looked for differences in aortic distensibility (AoD) which is an index of aortic elasticity, and myocardial performance of the ventricles, between patients with Sar and healthy subjects. In addition, we examined potential associations between AoD and clinical, respiratory and echocardiographic findings in patients with Sar. MATERIALS AND METHODS: A total of 83 consecutive patients (26 male/57 female, mean age 51.1 +/- 13.3 years) with Sar, without cardiac symptoms, were included. All patients underwent echocardiographic and respiratory evaluation including lung function tests. Additionally, 83 age- and sex-matched healthy subjects served as controls. AoD was determined non-invasively by ultrasonography. RESULTS: AoD was lower in the Sar compared to the control group (2.29 +/- 0.26 vs. 2.45 +/- 0.20 .10(-) (6) cm2 x dyn(-1), P < 0.01), while left ventricular mass (LVM) was higher in the Sar group (221.3 +/- 50.2 vs. 195.6 +/- 31.3 g, P = 0.007). Furthermore, myocardial performance of both ventricles was impaired in the Sar group. Multivariate linear regression analysis in the total sample population demonstrated a significant and independent inverse relationship between AoD and the presence of Sar (P < 0.001). The same analysis in the Sar patients showed that AoD was associated significantly and independently with the stage of Sar, age, systolic blood pressure, LVM and myocardial performance of both ventricles. No significant relationship was found between AoD and disease duration, pulmonary artery pressure or lung function tests. CONCLUSIONS: Presence and severity of Sar are associated with reduced aortic distensibility, irrespective of the disease duration, pulmonary artery pressure and lung function. In addition, patients with Sar have increased LVM and impaired myocardial performance.

Spatial QRS-T angle: association with diabetes and left ventricular performance.

The spatial QRS-T angle obtained by vectorcardiography is a combined measurement of the electrical activity of the heart and predicts cardiovascular morbidity and mortality. Disturbances in repolarization and depolarization are common in diabetes. No data, however, exist on the effect of diabetes on QRS-T angle. In this study we examined differences in QRS-T angle between type 2 diabetic and non-diabetic subjects; in addition, the potential relationship between QRS-T angle and left ventricular performance as well as glycaemic control were also examined. A total of 74 subjects with type 2 diabetes and 74 non-diabetic individuals, matched for age and sex with the diabetic subjects were examined. All subjects were free of clinically apparent macrovascular complications. Spatial vectorcardiogaphic descriptors of ventricular depolarization and repolarization were reconstructed from the 12-electrocardiographic leads using a computer-based electrocardiogram. Left ventricular mass and performance were measured using M-mode and Doppler echocardiography. QRS-T angle values were higher (by almost 2-fold) in the diabetic in comparison with the non-diabetic subjects (P < 0.001). After multivariate adjustment, QRS-T angle was independently associated with age (P = 0.01), HbA(1c) (P = 0.003), and low-density lipoprotein cholesterol levels (P = 0.04) in the non-diabetic, and with HbA(1c) (P = 0.03) as well as Tei index (P = 0.003) in the diabetic subjects. The spatial QRS-T angle is high in subjects with type 2 diabetes and is associated with glycaemic control and left ventricular performance. The prognostic importance of the higher spQRS-T angle values in subjects with diabetes remains to be evaluated in prospective studies.

Echocardiographic findings in patients with temporal arteritis: apropos of one case of temporal arteritis-associated verrucous (Libman-Sachs) endocarditis.

We report the case of a 70-year-old patient who presented with fever of unknown origin. The initial diagnosis was infective endocarditis as a mitral valve vegetation was found in a transesophageal echocardiogram. Lack of response to empiric antibiotic treatment and further diagnostic work-up established the correct diagnosis of marantic endocarditis due to temporal arteritis. Treatment with steroids and aspirin led to rapid clinical improvement and disappearance of the vegetation. Apropos of this case, we reviewed the records of 25 patients with a new diagnosis of temporal arteritis and analyzed the echocardiographic findings in comparison to those of 40 age- and sex-matched controls. Abnormal echocardiographic findings were present in 13 (52%) out of 25 patients with temporal arteritis and in 5 (12.5 %) out of 40 controls (p < 0.001, chi-square test).

Complete heart block in a patient with systemic sclerosis.

We describe a patient with diffuse systemic sclerosis and presyncopal episodes where the electrocardiogram revealed complete atrioventricular heart block associated with left posterior and right heart bundle block. The patient underwent implantation of a permanent pacemaker.

Hypertrophic cardiomyopathy in systemic sclerosis. A report of two cases.

We describe two patients with diffuse systemic sclerosis (SSc) where the echocardiogram revealed asymmetric hypertrophy of the interventricular septum with signs of subaortic obstruction consistent with hypertrophic obstructive cardiomyopathy. Hypertrophic cardiomyopathy is associated with the human lymphocyte antigen (HLA DR3), and this may provide a possible link with SSc, as this HLA phenotype is common in the latter condition. However, further studies should examine whether a true association exists.

Systolic and diastolic function in middle aged patients with sickle beta thalassaemia. An echocardiographic study.

OBJECTIVE: To evaluate the right and left ventricular systolic and diastolic function in middle aged patients with sickle beta thalassaemia. METHODS: Forty three patients with sickle beta thalassaemia were recruited for echocardiographic study while 55 controls, matched for age and sex, served as the control group. Parameters measured included: dimensions and wall thickness of left (LV) and right (RV) ventricle and left atrium, LV mass, and cardiac index. LV and RV contractility variables--ejection fraction, circumferential fibre shortening velocity, end systolic stress, end systolic stress/volume index ratio, mitral and tricuspid annulus systolic excursion, and Tei index--were also calculated. The study also evaluated parameters of RV and LV diastolic function including early and late atrioventricular flow velocities (E and A wave respectively), E/A ratio, deceleration time (DT), isovolumic relaxation time (IVRT) as well as pulmonary and hepatic veins systolic to diastolic (S/D) ratio. RESULTS: Chamber enlargement, greater LV mass index, cardiac index, and RV wall thickness were found in the anaemic group compared with controls. The LV and RV contractility variables of the patients were similar to controls. Conversely the LV and RV Tei index was significantly greater in the patient group. Diastolic dysfunction was present in the anaemic patients resulting from the increased LV and RV A-wave, the longer LVIVRT, RVIVRT, and RVDT, as well as the higher hepatic and pulmonary veins S/D ratio. CONCLUSIONS: The results show that in middle aged patients with sickle beta thalassaemia the diastolic function is abnormal in both ventricles but still more in RV, whereas the systolic function remains unchanged.

Increased plasma ghrelin levels in chronic renal failure are not associated with hemodynamic parameters.

BACKGROUND/AIMS: Hardly anything is known about the effect of renal function on plasma ghrelin levels. Ghrelin is an orexigenic hormone with important hemodynamic effects. We examined differences in plasma ghrelin levels between chronic renal failure (CRF) patients and healthy subjects, and ghrelin's relationship with indices of left ventricular (LV) function. METHODS: Fasting total plasma ghrelin levels were measured in 122 CRF patients (57 on, 65 not on hemodialysis) and 57 control subjects. Indices of LV function were evaluated using echocardiography. RESULTS: Total plasma ghrelin levels were higher in patients with CRF compared to controls, but were not different between patients on and those not on hemodialysis. In a multivariate linear regression model, presence of kidney dysfunction explained 41 % of the variability of ghrelin values. The etiology of renal failure (diabetic nephropathy or not) had no influence on ghrelin levels in the renal patients. Ghrelin levels were not associated with indices of LV systolic function or blood pressure in these patients. CONCLUSION: Fasting plasma ghrelin concentrations are higher in CRF patients regardless of their need for hemodialysis compared to controls. The etiology of renal failure does not have any effect on plasma ghrelin levels. In addition, ghrelin levels are not associated with hemodynamic parameters in patients with CRF.

Transfusional hemosiderosis and combined chelation therapy in sickle thalassemia.

Although the indications for transfusions in sickle cell syndromes are well listed, and chronic transfusion has become practicable since the recent advances in chelation therapy have essentially eliminated the risk of secondary iron overload, multi-transfused, non-compliant to long-term chelation therapy patients confront the complication of iron overload and secondary hemosiderosis. In thalassemia major patients, combined therapy with desferrioxamine and deferiprone has maximized tissue iron removal and may reduce the overall occurrence of hemosiderotic heart failure. Despite this, safety and contradictions of chelating agents are still controversial. The aim of this report is to present the results of this combination in a long-term transfused sickle beta-thalassemic patient suffering from severe heart failure and liver dysfunction.

Left atrial systolic reserve in idiopathic vs. ischaemic-dilated cardiomyopathy.

PURPOSE: There are studies indicating more pronounced left atrial (LA) systolic dysfunction at rest in idiopathic (IDDC) than in ischaemic-dilated cardiomyopathy (ISDC). It was hypothesized that the findings would be similar with regards LA systolic reserve. METHODS: Twenty-six patients with IDDC, 28 with ISDC and 25 normal controls underwent low-dose dobutamine stress echocardiography (5-10 microg kg(-1) min(-1) IV). Left atrial volumes were echocardiographically determined at rest and during stress at the mitral valve opening (maximal, Vmax), electrocardiographic P wave (onset of atrial systole, Vp) and mitral valve closure (minimal, Vmin) from the apical 4- and 2-chamber views (biplane area-length method). Left atrial systolic function was assessed with the LA-active emptying volume (ACTEV) = Vp-Vmin and fraction (ACTEF) = ACTEV/Vp. RESULTS: Vmax at rest was similar in IDDC and ISDC and greater than in the controls (54.2 +/- 12 vs. 48.5 +/- 18 vs. 27.1 +/- 6.3 cm(3) m(-2), respectively, P < 0.001) and did not change with stress (53.9 +/- 13.8 vs. 46.9 +/- 16.2 vs. 25.8 +/- 5.9 cm(3) m(-2), P < 0.001). The ACTEV at rest was similar in IDDC and ISDC and greater than in the controls (8.6 +/- 3.5 vs. 9.7 +/- 2.9 vs. 6.1 +/- 2.2 cm(3) m(-2) P < 0.01), whereas during the dobutamine infusion it remained unaltered in IDDC (10.8 +/- 4.6 cm(3) m(-2), P = NS vs. rest) and increased in ISDC (11.8 +/- 3.3 cm(3) m(-2), P < 0.05) and the controls (13.1 +/- 3.2 cm(3) m(-2), P < 0.01). The ACTEF was lower in IDDC than ISDC and the controls at rest (20 +/- 10% vs. 33 +/- 8% vs. 36 +/- 10%, P < 0.01). Dobutamine infusion was associated with no significant increase in ACTEF in IDDC (25 +/- 12%, P = NS vs. rest), and with an increase in this variable in ISDC (39 +/- 10%, P < 0.05) and the controls (49 +/- 12%, P < 0.01). CONCLUSIONS: Dobutamine infusion is associated with an increase in LA ACTEV and fraction in ISDC and no significant change in these indices in IDDC. These findings indicate a reduced LA systolic reserve in IDDC.

Clinical significance of matrix metalloproteinases activity in acute myocardial infarction.

Matrix metalloproteinases (MMP) degrade myocardial fibrillar collagen in acute myocardial infarction (MI) patients. Their activity is tightly controlled in normal myocardium by a family of closely related tissue inhibitors known as TIMP. An imbalance in their activity might contribute to post-MI remodeling. Plasma levels of MMP-1, TIMP-1 and MMP-1/TIMP-1 complex were measured, using relevant ELISA kits, in 24 (22 males-2 females), acute MI patients with a mean age 59 +/- 14 years. Blood samples were taken on admission (0 h), and 3 h, 6 h, 9 h, 18 h, 24 h, 36 h, 48 h, 3rd, 4th, 5th, 7th, 15th, 30th days after MI. All patients underwent coronary arteriography with ventriculography for estimation of left ventricular ejection fraction (LVEF) and extent of coronary artery diseases, and echocardiographic study for measuring end-diastolic diameter (EDD). Ten patients with an LVEF < 45%, an EDD > 47.5 mm, and heart failure symptoms were included in group A and compared against 12 patients with an LVEF > 45% an EDD < 47.5 mm in group B. Mean plasma concentrations of MMP-1 were higher by 21% in group A (1.3 +/- 0.2 ng/mL) compared to group B (1 +/- 0.1 ng/mL) over the total study period. TIMP-1 plasma concentrations showed very little difference between the 2 groups, (704 +/- 213 ng/mL versus 691 +/- 165 ng/mL, (6%)). Finally, plasma concentrations of MMP-1/TIMP-1 complex were lower by -36% in group A with a mean value of 2.7 +/- 0.6 ng/mL versus 3.7 +/- 0.5 ng/mL in group B. Mean values for the differences were significant at time points 0, 6, 18, 24 and 48 hours for MMP-1 (p < 0.036), and on 48 h and the 4th day for MMP-1/TIMP-1 complex (p < 0.031). Moreover, a good correlation was found between plasma concentrations of creatine kinase (CK) and MMP-1 at 18 h (r = 0.422, p = 0.041) and on the 4th day (r = 0.67, p = 0.046), and TIMP-1 on the 4th day (r = 0.67, p = 0.047). Additionally, mean values for LVEF were 35.8 +/- 8.8% in group A versus 51.2 +/- 1.8% (p = 0.00014) in group B. Also, the EDD in-group A was 52.1 +/- 6.9 mm versus 42.9 +/- 3.2 mm in group B (p = 0.00013). In acute MI patients, increased MMP-1, with no change in TIMP-1, is associated with left ventricular dysfunction and dilatation, suggesting that increased collagenolytic activity contributes to loss of LV function.

Severe aortic regurgitation in von Recklinghausen's disease--a case report.

A 71-year-old man with von Recklinghausen's disease presented with NYHA class II exertional dyspnea and easy fatigability since about 20 days. Echocardiography and angiography demonstrated dilatation of aortic root, ascending aorta, and left ventricle, together with severe aortic regurgitation. Because of very few cases of cardiovascular abnormalities having been reported with von Recklinghausen's disease, it is logical to concur that this is simply co-existence, whereas other possible mechanisms are endothelial dysfunction or sympathetic denervation.

Impaired elastic properties of ascending aorta in patients with giant cell arteritis.

OBJECTIVE: To investigate the elastic properties of the ascending aorta in untreated patients with giant cell arteritis compared with age and sex matched normal controls. METHODS: Distensibility of the ascending aorta and aortic strain were measured in 22 patients with a recent diagnosis of giant cell arteritis (documented by a positive temporal artery biopsy) before initiation corticosteroid treatment, and in 44 age and sex matched healthy subjects. Aortic distensibility was calculated as 2x[pulsatile change in aortic diameter]/[(diastolic aortic diameter)x(aortic pulse pressure)], and aortic strain as [pulsatile change in aortic diameter]/[diastolic aortic diameter]. Aortic diameters were measured by echocardiography. Aortic pressures were obtained by external sphygmomanometry. RESULTS: Distensibility of the ascending aorta and aortic strain were both lower in patients with giant cell arteritis than in the controls (p<0.01). In the patients with giant cell arteritis, aortic distensibility was inversely correlated with white blood cell count (p<0.05), but not with erythrocyte sedimentation rate or C reactive protein. CONCLUSIONS: Compared with healthy subjects, aortic distensibility and aortic strain are decreased in patients with giant cell arteritis before initiation of corticosteroid treatment. There was an association between the degree of reduction of aortic distensibility and the white blood cell count in the patient group.

Aortic stiffness in systemic sclerosis is increased independently of the extent of skin involvement.

OBJECTIVE: To study the stiffness of large arteries in relation to the extent of skin and lung fibrosis, aortic distensibility was examined in patients with diffuse and limited systemic sclerosis (SSc). METHODS: Consecutive patients (55 with diffuse and 51 with limited SSc) without signs and symptoms of heart failure or a previous history of arterial hypertension underwent echocardiography and lung function tests. Aortic stiffness was determined non-invasively by aortic distensibility and aortic strain measurements in all patients and in 50 healthy subjects, matched for age and gender. RESULTS: Aortic distensibility in patients with either diffuse (2.03 +/- 0.26 x 10(-6) cm(2) dyn(-1)) or limited SSc (2.12 +/- 0.33) was similarly decreased compared with controls (2.49 +/- 0.36, P<0.001). Moreover, aortic strain was significantly reduced in both patient groups compared with controls, confirming that aortic stiffness is increased in SSc independently of the extent of skin involvement. Left ventricular performance was similar between patients and controls, while left ventricular mass and tricuspid systolic gradient were significantly increased in both SSc groups, the latter being associated with aortic stiffness in multivariate analysis. No association with serum levels of C-reactive protein or lung function abnormalities indicative of pulmonary fibrosis were found. CONCLUSIONS: Stiffness of the aorta is increased in patients with established SSc regardless of the extent of the inflammatory fibrotic process in the skin and lungs, suggesting that additional pathogenetic mechanisms contribute to the compromise of large arteries.