Pawel Rubis

Heart structure and function in patients with generalized autoimmune diseases: echocardiography with tissue Doppler study.

Objective Heart pathology strongly infl uences 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) diff er signifi cantly 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). Material and methods 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. Results Heart in SSc was characterized by signifi cant 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 leafl ets and/or pericardium [mitral or aortic leafl ets 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. Conclusions Echocardiography with TDE reveals characteristic pathology in diff erent forms of generalized autoimmune diseases refl ecting their diff erent pathogenetic mechanisms. Overproduction of collagen in SSc results in diastolic left ventricular dysfunction, while generalized infl ammation in SLE and DPM leads mainly to pathologic changes on valvular leafl ets 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.

Usefulness of the Evaluation of Isovolumic and Ejection Phase Myocardial Signals during Stress Echocardiography in Predicting Exercise Capacity in Heart Failure Patients

Aim: 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). Material and Methods: 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 (VO2 peak) was measured. Results: 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 VO2 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, VO2 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%. Conclusions: 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.

Myocardial Viability Detected by Myocardial Contrast Echocardiography—Prognostic Value in Patients after Myocardial Infarction

Objective: This study aimed to assess the role of myocardial contrast echocardiography (MCE) as a predictor of cardiac events and death in patients with acute myocardial infarction (AMI). Methods: Eighty‐six patients underwent primary percutaneous coronary angioplasty for AMI. Segmental perfusion was estimated by MCE in real time at mean 5 days after PCI using low MI (0.3) after 0.3–0.5 ml bolus injection of intravenous Optison. MCE was scored semiquantitatively as: (1) normal perfusion (homogenous contrast effect), (2) partial perfusion (patchy myocardial contrast enhancement), (3) lack of perfusion (no visible contrast effect). A contrast score index (CSI) was calculated as the sum of MCE scores in each segment divided by the total number of segments. The patients were followed up for cardiac events and death. Results: A CSI of >1.68 was taken to be a predictor of cardiac events and death. Death occurred only in patients with CSI >1.68. Patients with CSI >1.68 had a significantly (P = 0.03) higher incidence of cardiac death or cardiac events (75%) compared to those with CSI <1.68 (27%). The absence of residual perfusion within the infarct zone was an independent predictor of death and cardiac events (P = 0.02). Conclusions: The absence of residual myocardial viability in the infarct zone supplied by an infarct‐related artery is a powerful predictor of cardiac events in patients after AMI. (Echocardiography 2010;27:430‐434)

RELATIONS BETWEEN FIBROSIS-LINKED MICRORNAS (MIR-21, MIR-26, MIR-29, MIR-30 AND MIR-133A) AND RIGHT VENTRICULAR MORPHOLOGY AND FUNCTION IN DILATED CARDIOMYOPATHY

Background: Relations between right ventricle (RV) and microRNAs in dilated cardiomyopathy (DCM) are poorly understood. Methods: We studied 70 DCM patients (pts) (48 ± 12.1 years, EF 24.4 ± 7.4%). Basal RV (RVd1) > 41 mm and/or mid-cavity RV dimension (RVd2) > 35 mm was diagnostic for RV

Comparison of two European models estimating risk of ­sudden cardiac death in hypertrophic cardiomyopathy

Objective Hypertrophic cardiomyopathy (HCM) is associated with a risk of sudden cardiac death (SCD). Several models have been developed to estimate SCD risk and guide preventive therapy. The comparison of the previous 2003 with novel 2014 SCD risk models have never been studied. Methods Over a year we included 103 consecutive HCM patients without previous cardiac arrest and/or ICD implanted (65% males; aged 53.3 ± 13.9 years; mean EF 62.3 ± 18%). The SCD risk was calculated for each patient. Results Based on the 2003 model, patients had following scores: 0 points -15 (15%) patients, 1-28 (27%), 2-34 (33%), 3-18 (17%), ≥ 4-8 (8%). According to the 2014 model 83 (80%) had low risk, 12 (12%) intermediate risk, and only 8 (8%) high risk. Patients who had an intermediate or high risk in the 2003 model but a low risk in 2014 model were the oldest, experienced less frequently syncope and nsVT, but had more often abnormal blood pressure response to exercise and an intermediate LVOT gradient in comparison to the patients who had consistently low or high risk in both models. Conclusions Calculation of SCD risk using two models provides completely different risk estimates and consequently different guidance on SCD primary prevention. According to the 2003 model up to 85% of patients have indications for ICD, whereas based on the 2014 risk model only 20% of the patients had non-negligible SCD risk and are candidates for ICD therapy. SCD risk markers, used in the 2003 and 2014 models, have various discriminating power.

EXTRACELLULAR MATRIX TURNOVER IS NOT RELATED TO THE DURATION OF THE DISEASE IN DILATED CARDIOMYOPATHY

Fibrosis of extracellular matrix (ECM) is a hallmark of dilated cardiomyopathy (DCM). The relation between ECM turnover and duration of DCM is unknown. Since July 2014 till April 2015 we included 53 consecutive DCM patients (pts) (47.4 ± 12.4 years, EF 24.2 ± 7.6%). Pts were divided into early (

4th Symposium on Rare Cardiovascular Diseases – ESC Barcelona 2014

Cardiovascular Diseases organized the 4th Satellite Symposium during the ESC Congress in sunny Barcelona, Spain. This consec‐ utive endeavor was only possible because of the EU Project MRPO 08.02.00‐12‐424/10, which is under realization by the Krakow Center for Rare Cardiovascular Diseases. Our session on rare car‐ diovascular diseases has gradually maturated over the years and as in the case of last two ESC Congresses in Munich and Am‐ sterdam, was officially included in the Program of the Congress. The Symposium took place on Sunday, 31st of August in the pre‐ miere lunch time between 1 p.m. till 1:45 p.m. in the Cairo room. It was planned that our long‐term Partner Professor Sabine Pan‐ kuweit from Marburg, Germany, the international Expert and au‐ thor of the guidelines on myocarditis, co‐chair the session. How‐ ever, just before the Congress an acute flu‐like infection stopped her from going to the ESC. Fortunately, the inventor of the famous “Alfieri stich” – Professor Ottavio Alfieri from Milan, Italy kindly agreed to substitute the missing Chairmen. The second chairmen was Doctor Pawel Rubis from the Krakow Center for Rare Cardio‐ vascular Diseases, Poland. The session program was organized according to the same prin‐ ciples that worked out during recent Congresses. In short, after welcoming and introductory lecture, was a main topic lecture, and finally we had two presentations of rare clinical cases that were pre‐ sented by the managing physician and commented by two experts. Naturally, the session was opened by the Director of the Krakow Center for Rare Cardiovascular Diseases, Professor Piotr Podolec. This year Professor Podolec presented data on the registries that are currently underway in the Krakow Centre. Moreover, he stressed the usefulness of the newly developed Classification of Rare Car‐ diovascular Diseases, which was presented last year, and proved to be the “tool” we needed to conduct those registries. On his final slides Professor Podolec presented the impressive number of pa‐ tients with rare diseases that were included in the registries, e. g. in the registry of rare diseases of the pulmonary circulation (class II according to CRCD Classification) there are 223 patients, in the reg‐ istry of congenital diseases (class IV) are 421 patients, whereas in the pregnancy registry (class VII) are 25 women with newly discov‐ ered significant heart problems. Although the Centre has already registered the substantial number of rare cases, Professor Podolec stressed that this is just a beginning and that we will carry on with recruiting more patients. As in previous years, he warmly encour‐ aged all interested in the rare cardiovascular diseases to join our network for the sake of all those “neglected” patients. The Keynote Lecture, entitled “Coronary anomalies and the risk of sudden death” was delivered by the Professor Gary Webb, uniquely an Expert in both pediatric and adult rare cardiovascular diseases, from Cincinnati, US. In the beginning Professor Webb defined cor‐ 4th Symposium on Rare Cardiovascular Diseases – ESC Barcelona 2014. Chairperson’s Perspective