[Show abstract][Hide abstract] ABSTRACT: Objectives: Increased arterial stiffness is a risk factor of atherosclerosis and cardio-vascular complications. The aim of the study was to determine whether peripheral vascular function might be an early marker of impaired health status in patients with a single ventricle after Fontan procedure.Methods and results: Twenty five consecutive adults (11 women and 14 men) aged 24.7 +/- 6.2 years after the Fontan procedure and 25 sex, age and BMI match healthy volunteers underwent physical examination, blood analysis, transthoracic echocardiography and noninvasive assessment of aortic stiffness. Augmented pressure and Augmentation Index (AI) were both significantly elevated in Fontan when compared to the controls (6,08 +/- 0,7 vs. 2,0 +/- 3,7; p = 0.002 and 17,01 +/- 3,3 vs. 6,05 +/- 11; p < 0.001, respectively). There were no differences in pulse wave velocity (PWV), Mean blood pressure (BP), brachial pulse pressure (PP), central: systolic BP, diastolic BP and PP. In Fontan group we find negative correlation between PWV and SatO2 (r = -0.68; p = 0.04) and positive correlation with WBC (0.72; p = 0.72; p = 0.013), INR (0.81; p = 0.008), TNFalpha (r = 0.45; p = 0.04), and postoperative time (r = 0.77; p = 0.02). AI correlates positively only with age at surgery (r = 0.45; p = 0.04). Bilirubin level correlates positively with brachial PP (r = 0.71; p = 0.02) and central PP (r = 0.68; p = 0.03).The multivariate model showed that SatO2 (beta = -0.44, p = 0.04) was the only independent predictor of PWV (R2 = 0.32, p = 0.03).
Adult Fontan patients have an increased arterial stiffness assessed by a noninvasive technique. Low arterial oxygen saturation postoperative time, age at surgery, white blood cells, TNFalpha and bilirubin level are associated with arterial stiffening in these patients. The combination of blood parameters of the hepatic function and noninvasive measurements of arterial stiffness could be helpful in comprehensive care of patients with Fontan circulation.
[Show abstract][Hide abstract] ABSTRACT: Patients who have undergone a Fontan operation (FO) may suffer from both systolic and diastolic single ventricle (SV) dysfunction.
The aim of the study was to quantify non-invasively the systolic and diastolic single ventricle function in adult FO patients and to assess its effect on exercise tolerance.
We investigated 21 patients (12F; 9M) after FO with morphological left ventricle with a mean age of 26 +/- 6 years, and 17 age-matched, healthy people (control group). Pulse-wave Doppler signals from the mitral inflow, aortic flow and tissue Doppler imaging of the mitral annulus were obtained. A cardiopulmonary exercise test (CPET) was performed.
The Fontan patients, as compared to the controls, had a lower value of SV ejection fraction (SVEF), E velocity, E/A ratio, aortic valve velocity time integral, exercise time, SatO2, VO(2peak) and significantly higher E/E' ratio, VE/VCO(2peak) and VE/VO(2peak'). We found negative correlations between E/E' and exercise time and VO(2peak). Positive correlations were found between E/E' and VE/VCO(2peak) and age at surgery. S' velocity correlated positively with SVEF, VO(2peak) and negatively with E/E' ratio. Patients after FO with E/E' ratio > or = 12 had a significantly lower oxygen uptake when compared to those with E/E' ratio < 12.
Adult patients after Fontan operations are characterized by both systolic and diastolic dysfunction of the single ventricle, associated with severe exercise intolerance reflected by shorter duration of exercise with decreased oxygen uptake and increased ventilatory response. E/E' ratio assessed by Tissue Doppler echocardiography can be a powerful predictor of oxygen uptake and ventilatory response.
[Show abstract][Hide abstract] ABSTRACT: Background
Growing evidence indicates that iron-deficiency anemia is common in patients with congenital heart diseases.
The aim of this study was to characterize hematologic changes and iron metabolism in adult Fontan patients. We also searched for the associations between these parameters and physical performance in the study group.
Methods and results
Thirty-two white Fontan patients with a mean age of 25 ± 4.5 years and 30 healthy control subjects matched for age and sex were studied. Complete blood count together with iron-related parameters was determined in plasma of peripheral venous blood. The cardiopulmonary exercise test was performed.
The Fontan patients had higher red blood cell counts (6.0 ± 2.1 × 109/μl vs. 4.8 ± 0.4 × 109/μl, p < 0.001), hemoglobin (16.7 ± 1.4 g/dl vs. 14.2 ± 1.3 g/dl, p < 0.001), hematocrit (49 ± 3.4% vs. 42.1 ± 3.1%, p < 0.001), red cell distribution width (RDW) (14.3 ± 2.4% vs. 12.8 ± 0.5%, p < 0.001), while mean corpuscular volume, mean corpuscular hemoglobin, and mean corpuscular hemoglobin concentration were similar in both the groups. Compared to the controls, the Fontan patients had higher unsaturated iron binding capacity (46.1 ± 12.6 μmol/l vs. 38.4 ± 11.9 μmol/l, p = 0.02), total iron-binding capacity (62.8 ± 9.8 μmol/l vs. 57.8 ± 8.5 μmol/l, p = 0.04), lower transferrin saturation (27.4 ± 11.4% vs. 34.6 ± 13.4%, p = 0.03), and oxygen uptake, while iron and ferritin levels were comparable in both the groups. The multivariate model showed that SatO2 and cystatin C were independent predictors of RDW, and alanine aminotransferase was an independent predictor of ferritin level. Interestingly RDW was an independent predictor of oxygen uptake.
Adult patients after Fontan operation despite having increased hemoglobin, hematocrit, and red blood cells have insufficient iron stores. Red cell distribution width is an indicator of iron deficiency in adult Fontan patients and it correlates with lower exercise capacity. Elevated ferritin levels in adult patients after Fontan surgery are associated with liver failure.
Journal of Cardiology 01/2014; · 2.30 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Thrombosis occurs in up to 30% of patients with various forms of congenital single ventricle after the Fontan procedure. We investigated hemostatic abnormalities in adult Fontan patients.
Forty-eight Fontan patients between ages 18 and 40 years, including 10 (21%) patients with previous thromboembolism 5 to 15 years after surgery, and 35 control subjects matched for age and sex were studied. Coagulation factors and inhibitors, together with markers of fibrinolysis, platelets, and endothelial activation, were determined in peripheral venous blood plasma.
Compared with control subjects, Fontan patients showed lower, although mostly within normal ranges, values of all coagulation factors, as well as reduced free protein S, in association with higher antithrombin and free tissue factor pathway inhibitor levels. Thrombin generation, reflected by prothrombin fragment 1.2, and platelet activation markers were increased in Fontan patients. The plasma clot lysis time was prolonged in Fontan patients, which was associated with an increased activity of thrombin-activatable fibrinolysis inhibitor. Fontan patients with previous thromboembolism had lower oxygen saturation, coagulation factors V and VIII, and free protein S, and increased von Willebrand factor, soluble CD40 ligand, and P-selectin. Other laboratory or clinical parameters were not associated with prior thrombotic episodes.
Adult Fontan patients are characterized by enhanced platelet activation and endothelial injury, heightened thrombin formation, and impaired fibrinolysis. Patients showed reduced free protein S levels, increased platelet activation, and endothelial damage after thromboembolic events observed late after Fontan surgery. Our findings indicate novel prothrombotic mechanisms in adult Fontan patients, which might help to optimize thromboprophylaxis.
The Journal of thoracic and cardiovascular surgery 07/2013; · 3.41 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Mortality in systemic lupus erythematosus (SLE) patients is influenced by an increased occurrence of severe cardiovascular complications. Statins have been proven to protect a wide spectrum of SLE patients from these complications. This study was conducted to determine the possible efficacy of atorvastatin in SLE patients as assessed by multi-detector computed tomography (MDCT)-based coronary calcium scoring and single photon emission computed tomography (SPECT) of the myocardium.
Sixty SLE patients in stable clinical conditions were randomized to receive either atorvastatin (40 mg daily; n = 28) or placebo (n = 32). Clinical and biochemical evaluation together with MDCT-based coronary calcium scoring and SPECT studies (Tc-99 m sestamibi) were performed at the time of randomization and after 1 year of treatment.
At randomization, SPECT revealed perfusion defects at rest in 22 (36.7%) patients and exercise-induced defects in 8 (13.3%), whereas MDCT revealed coronary calcifications in 15 subjects (25%). Coronary calcium deposits increased after 1 year in the placebo group (plaque volume change from 35.2 ± 44.9 to 62.9 ± 72.4, P < 0.05; calcium score from 32.1 ± 39.1 to 59.5 ± 64.4; P < 0.05), but not in the atorvastatin group (plaque volume 54.5 ± 62.4 vs. 51.0 ± 47.6, P not significant; calcium score 44.8 ± 50.6 vs. 54.9 ± 62.5, P not significant). The atorvastatin group showed a decrease in total serum cholesterol (from 5.1 ± 1.2 to 4.4 ± 0.7 mmol/L, P < 0.05), LDL cholesterol (2.9 ± 1.0 to 2.3 ± 0.6 mmol/L, P < 0.05), triglycerides (1.6 ± 0.6 to 1.2 ± 0.5 mmol/L, P < 0.05), and C-reactive protein (CRP) (4.4 ± 4.1 to 2.7 ± 1.7 mg/L, P < 0.05). There was no change in the mean Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score in patients from both groups. Perfusion defects observed at randomization showed no change after one year treatment with atorvastatin.
In SLE patients 40 mg of atorvastatin daily for 1 year led to a decrease in serum lipids and CRP levels. Additionally the progression of atherosclerosis, as assessed by MDCT-based coronary calcium scoring, is restrained by atorvastatin treatment. The value of statin treatment in patients with SLE free from cardiovascular disease clinical symptoms should be addressed in large, prospective clinical trials.
Arthritis research & therapy 07/2011; 13(4):R117. · 4.27 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Conventional risk factors for coronary artery disease fail to explain the increased frequency or cardiovascular morbidity in systemic lupus erythematosus (SLE) patients. This study was conducted to determine the possible influence of autoimmune and inflammatory phenomena markers on coronary artery calcifications and myocardial perfusion abnormalities in SLE patients.
Multi-detector computed tomography (MDCT)-based coronary calcium scoring and single photon emission computerized tomography (SPECT) studies (Tc-99m sestamibi) were performed in 60 SLE patients in stable clinical condition, without a prior history of coronary artery disease. Laboratory evaluation included serum C-reactive protein (CRP), complement C3c and C4 components and antiphospholipid antibodies (aPL). The latter included anticardiolipin (aCL) and anti-β2-glycoprotein I (aβ2GPI) antibodies, of both IgG and IgM classes, and lupus anticoagulant (LA) in plasma.
SPECT revealed persistent perfusion defects in 22 (36.7%) patients and exercise-induced defects in eight (13.3%), while MDCT revealed coronary calcifications in 15 (25%). Calcium scores ranged from 1 to 843.2 (mean 113.5 ± 259.7). No association was found between conventional coronary artery disease risk factors (obesity, hypertension, tobacco use, hyperlipidaemia, diabetes) nor CRP, C3c or C4 levels and coronary calcifications or myocardial perfusion defects. On the contrary, in patients with these pathologies, augmented autoimmunization was found, reflected by increased aCL IgG and antiβ2GPI IgG levels. In patients with aCL IgG >20 RU/ml or antiβ2GPI IgG >3 RU/ml, the relative risk of coronary calcification formation was 4.1 compared to patients with normal values. Accordingly, in LA-positive patients the relative risk of coronary calcification formation was 4.4 compared to LA-negative patients.
Conventional risk factors for coronary artery disease as well as markers of an ongoing inflammation did not show any association with perfusion defects and/or coronary artery calcifications in SLE patients. On the contrary, calcified atherosclerotic plaques and myocardial perfusion defects were observed mainly in patients with elevated levels of anticardiolipin and aβ2GPI antibodies of the IgG class. It might be speculated that coronary artery calcifications and perfusion defects are a result of antiphospholipid antibodies-induced coronary artery microthrombosis.
Agents and Actions 07/2011; 60(10):973-80. · 1.59 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Heart pathology strongly influences the course and prognosis of patients with generalized autoimmune diseases. In spite of autoimmunity being a common denominator of these diseases, systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and dermato/polymyositis (DPM) differ significantly in the pathogenesis of organ damage. The aim of the study was to compare pathologic changes in heart structure and function in these diseases by means of standard echocardiography and tissue Doppler (TDE).
Four groups were examined: 60 SSc, 60 SLE and 15 DPM patients in stable clinical conditions and 30 healthy control subjects. Echocardiography with TDE was performed with the assessment of systolic (S) and diastolic (E) velocities of mitral and tricuspid annuli.
Heart in SSc was characterized by significant diastolic left ventricular dysfunction (mitral E 8.61 +/- 2.3 cm/s vs. 12.4 +/- 3.5 cm/s in the control group; P < 0.01) with preserved systolic function (mitral S 7.85 +/- 1.5 cm/s vs. 7.95 +/- 0.9 cm/s in control group; ns). SLE and DPM resulted mainly in pathologic thickening of valvular leaflets and/or pericardium [mitral or aortic leaflets thickened in 38 (63.3%) of SLE patients, 7 (46.7%) of DPM patients; pericardium thickened in 36 (60%) of SLE patients]. Pulmonary capillary wedge pressure was elevated in SSc (13.8 +/- 3.5 mmHg) and DPM (13.2 +/- 2.5 mmHg) patients as compared to the control group (9.2 +/- 3.7 mmHg, P < 0.01). Right ventricular systolic and diastolic dysfunction was frequent irrespective of the presence or absence of pulmonary hypertension.
Echocardiography with TDE reveals characteristic pathology in different forms of generalized autoimmune diseases reflecting their different pathogenetic mechanisms. Overproduction of collagen in SSc results in diastolic left ventricular dysfunction, while generalized inflammation in SLE and DPM leads mainly to pathologic changes on valvular leaflets and/or pericardium. Interestingly, right ventricular dysfunction is common in all diseases analyzed, regardless of the presence of pulmonary hypertension. Echocardiography, preferably with TDE, could add valuable information about usually asymptomatic heart pathology in an individual patient with generalized autoimmune disease.
[Show abstract][Hide abstract] ABSTRACT: We focused on neurohumoral activity and its clinical correlates early and late after fenestrated, lateral intra-atrial total cavopulmonary connection (TCPC). Between 2007 and 2010, we prospectively studied 28 early and 48 late postoperative TCPC patients. Plasma concentrations of vasopressin, endothelin-1, proBNP, proANP were determined. We reviewed clinical data to determine relationship between neurohumoral activation and clinical status after TCPC. There was a significant influence of preoperative ventricular end-diastolic pressure (VEDP) (P=0.008) and vasopressin concentration (P=0.02) on the appearance of prolonged pleural effusions. A significant correlation between a combined predictor (a product of preoperative vasopressin concentration and VEDP) and time of effusions (r=0.59, P=0.006) was found. The mean respiratory equivalent of carbon dioxide at peak exercise (VE/VCO(2peak)) was significantly lower in patients operated before the second year of life compared to patients operated after two years of age (27.5±1.39 vs. 48.6±3.86; P=0.039). There was a significant correlation of endothelin-1 (r=0.84; P=0.008) and proBNP (r=0.88; P=0.02) concentrations with VE/VCO(2peak). The prolonged postoperative pleural effusions can be predicted based on the product of preoperative vasopressin concentration and VEDP. Exercise performance is related to the age at TCPC. Endothelin-1 and proBNP can be useful for identification of high-risk Fontan patients.
Interactive Cardiovascular and Thoracic Surgery 03/2011; 13(1):40-5. · 1.11 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Cardiac resynchronization therapy (CRT) is an acknowledged treatment for advanced heart failure in acquired dilated cardiomyopathy, resistant to pharmacotherapy. Although there are no therapeutic standards regarding heart failure originating from congenital heart defects with systemic right ventricle, a number of CRT implantations by transvenous approach in congenitally corrected transposition of the great arteries (CCTGA) have been reported since 2001, even though none of them expressly referred to a case concomitant with dextrocardia and situs inversus anomaly. We present a 57 year-old patient with dextrocardia and CCTGA, who underwent surgical closure of interatrial and interventricular septal defects at the age of 19, and in whom a VVI pacemaker was subsequently implanted at age 36. A three-lead CRT system was implanted by transvenous approach. Imaging techniques, including multi-slice computed tomography, targeted to pacing system and unusual anatomical relationships were applied. Within a 20-month follow-up, a significant improvement of functional NYHA class, systemic right ventricle ejection fraction and exercise capability were observed. Entirely transvenous CRT system implantation is feasible in patients with dextrocardia and CCTGA, and has substantial potential for long-term benefits.
[Show abstract][Hide abstract] ABSTRACT: To assess changes of systolic function using tissue Doppler imaging (TDI) during stress echocardiography and its impact on exercise capacity in heart failure (HF) patients (pts).
80 pts (65 male), mean age of 59.3 +/- 10.9 years, NYHA class 1.95 +/- 0.8, left ventricle ejection fraction (LVEF) 27.2 +/- 9.5 (10-45%). The etiology of HF was ischemic (ICM) in 50 pts and dilated cardiomyopathy (DCM) in 30 pts. Peak myocardial velocity (IVV) and acceleration (IVA) during isovolumic contraction and peak myocardial velocity during ejection phase (S') were measured at baseline and peak exercise during semi-supine stress-echo (20 Watts, 2-min increments). Concurrently peak oxygen uptake (VO(2) peak) was measured.
Rest values of analyzed parameters were comparable in groups according to etiology of HF and physical capacity. However, peak stress parameters mainly S' were significantly higher in the DCM group and the group with better VO(2) peak. The best correlation with exercise capacity was S' at peak stress (r = 0.66; p < 0.0001). The most useful parameter for identifying severe exercise intolerance, VO(2) peak < 14 ml/kg/min, was S' with an area under ROC curve of 0.82 +/- 0.05 (95% CI 0.71-0.89). The cutoff of 5.75 cm/s for S' at peak stress showed a sensitivity of 61% with a specificity of 96%.
The evaluation of systolic function by means of TDI instead of LVEF shows more clearly that systolic function is at least partly responsible for exercise tolerance in HF. Assessment of echocardiographic systolic parameters at peak stress provides more accurate information about exercise capacity in HF pts.
[Show abstract][Hide abstract] ABSTRACT: Diastolic dysfunction and elevated left ventricular (LV) filling pressure at rest are key factors of exercise intolerance in patients with heart failure (HF). There are few studies, however, that have addressed the issue of changes of LV diastolic function and filling pressure during exercise in patients with HF with severe systolic dysfunction. The ratio of early diastolic velocity of mitral inflow (E) and early myocardial diastolic velocity (E') strongly correlates with invasively obtained LV filling pressure.
We sought to assess dynamic changes of diastolic function, including LV filling pressure using Doppler tissue imaging, during stress echocardiography and its impact on exercise capacity in patients with ischemic HF.
We studied 50 adult patients (44 male and 6 female) with a mean age of 62.9 +/- 8.8 (46-79) years, mean New York Heart Association class of 1.97 +/- 0.86, and mean ejection fraction of 28.4 +/- 9.5 (10%-45%). The following conventional and tissue Doppler parameters were measured at baseline and peak exercise during semisupine stress echocardiography (20 W, 2-minute increments): peak early (E) and late (A) diastolic velocity of the mitral inflow, E/A ratio, peak early myocardial diastolic velocity (E'), and E/E' ratio. Diastolic Doppler tissue imaging indices were derived from septal, lateral, anterior, and inferior border of the mitral annulus in the apical 4- and 2-chamber views. Simultaneously during stress echocardiography peak oxygen uptake was measured. Patients were divided into two groups according to peak oxygen uptake value: group 1 with 23 patients (< 14 mL/kg/min) and group 2 with 27 patients (> or = 14 mL/kg/min).
There were significant differences in terms of E' and E/E' ratios both at rest and peak exercise between the two groups. The best correlation with exercise capacity was E/E' at peak stress (r = -0.75, P < .0001). The most useful parameter for identifying severe exercise intolerance, as indicated by peak oxygen uptake less than 14 mL/kg/min, was E/E' at peak stress with an area under receiver operating characteristic curve of 0.92. The cut-off of 18.2 for E/E' at peak stress showed a sensitivity of 85.2% with a specificity of 95.6%.
The evaluation of hemodynamic response of diastolic function, including LV filling pressure, during exercise is feasible during stress echocardiography and provides valuable information in predicting exercise capacity in patients with ischemic HF.
Journal of the American Society of Echocardiography: official publication of the American Society of Echocardiography 08/2008; 21(7):834-40. · 2.98 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Little is known about the relation between echocardiographic parameters and cardiopulmonary capacity in patients with atrial septal defect (ASD). The study aimed to assess the value of right atrial, right ventricle and left ventricle dimensions and function in predicting cardiopulmonary capacity in adults with ASD. 52 consecutive adult patients with hemodynamic significant ASD (mean age 38.6+/-15, range: 18-68 years; mean Qp/Qs: 2.7+/-0.7, range: 1.5-4.3) were enrolled into the study. The right ventricle indices were defined as follows: end-diastolic and end-systolic area, ejection fraction (area-length method) and right ventricle systolic pressure (RVSP). The following right atrial parameters were taken into consideration: end-diastolic and end-systolic right atrial area. Left ventricular systolic and diastolic diameters and LV ejection fraction were measured as well. All patients underwent the cardiopulmonary exercise test (CPX) with estimation of peak oxygen uptake -VO2, anaerobic threshold -AT and VE/VCO2. The mean VO2 was 23.4+/-18.4 ml/kg/min (63+/-18.4% of the predicted value). The dimensions and ejection fraction of left ventricle did not correlate significantly with any CPX parameter. Echocardiographic parameters of both right atrial and right ventricle function were related to peak oxygen uptake in adults with ASD. The strongest correlation between VO2peak and RVSP was found (r=-0.7; p<0.001). Echocardiography seems to be efficient in predicting cardiopulmonary capacity in ASD patients.
[Show abstract][Hide abstract] ABSTRACT: Percutaneous transluminal septal myocardial ablation (PTSMA) is becoming an alternative to surgical myectomy in the treatment of severe, drug refractory, hypertrophic obstructive cardiomyopathy (HOCM). The aim of our study was to analyze early results, complications and long-term follow-up in patients after PTSMA.
Out of eighteen patients [11 M] initially accepted for PTSMA, the procedure was performed in 12 patients [6 M] age from 22 to 70 y. All the patients underwent clinical evaluation, echocardiography and cardiopulmonary exercise testing (CPX) before the procedure, and after a median of 38 months of observation. In echo-cardiography left ventricle outflow tract gradient (LVOTG) and intraventricular septum diastolic diameter were assessed. The following parameters of CPX were analyzed: exercise duration, anaerobic threshold, peak oxygen consumption, peak exercise heart rate and carbon dioxide ventilating equivalent.
The procedure was successful in 11 patient. There were 2 acute complications: 3rd degree AV block requiring peacemaker implantation and LVOTG increase with SAM exacerbation requiring urgent cardiosurgical intervention. During long-term follow-up 1 cerebral stroke and 1 death occurred. PTSMA resulted in significant reduction of left ventricle outflow tract gradient (89 +/- 44 vs. 17 +/- 17 mmHg) and intraventricular septum diastolic diameter (24 +/- 4 vs 18 +/- 5 mm) (p < or = 0.01 for both). We also observed improvement of CPX parameters: exercise duration (487 +/- 268 vs. 730 +/- 292 sec), anaerobic threshold (34.3 +/- 8.9 vs. 53.2 +/- 13.4% VO2max predicted), peak oxygen consumption (18.5 +/- 6 vs. 26.8 +/- 10.1 ml/kg/min), peak exercise heart rate (70.3 +/- 8.3 vs. 83.6 +/- 11.7%) and carbon dioxide ventilating equivalent (31 +/- 6.1 vs. 28 +/- 4.8); (p < or = 0.01 for all).
PTSMA is an effective method of treatment in patients with severe, drug refractory HOCM. PTSMA is safe and is associated with a low percentage of severe complications.