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The objectives of this study were to determine the relationship between carotid-femoral (cfPWV) and aortic pulse wave velocity (aPWV) and to compare their modulators and association with coronary artery disease (CAD). We studied 107 consecutive patients (68 men) with a mean age of 60.49+/-8.31 years who had stable angina and had been referred for coronary angiography. cfPWV and aPWV were measured simultaneously during cardiac catheterization using the Complior device and aortic pressure waveform recordings, respectively. Based on the presence or absence of significant coronary artery stenosis (CAS) patients were subdivided into a CAS+ or CAS- group. The mean values of cfPWV and aPWV were 10.65+/-2.29 m/s and 8.78+/-2.24 m/s, respectively. They were significantly higher in the CAS+ (n=71) compared with the CAS- (n=36) group and predicted significant CAS independently of cardiovascular risk factors and mean or systolic aortic blood pressure. aPWV and cfPWV were significantly correlated (r=0.70; p

Peripartum cardiomyopathy (PPCM) is defined as an idiopathic cardiomyopathy, presenting with heart failure (HF) secondary to left ventricular systolic dysfunction (LVSD) at the end of pregnancy or in the early months after delivery. Importantly, there should be no other identifiable cause of HF. The pathophysiology of PPCM is not fully understood and is probably multifactional. The data on the PPCM prognosis are sparse. Though initial reports suggested that mortality in PPCM vary geographically. We present a case of a a nulliparous 25-year-old Caucasian woman, who gradually developed dyspnea on exertion and fatigue 2 months after successful vaginal twin deliv- ery. She did not have any typical risk factors for cardiovascular diseases, her family history was unremarkable, and the whole pregnancy period was uneventful. After initiation of anti-HF therapy her clinical status gradually improved and she was discharged home with only mild symptoms and improved LV systolic function. JRCD 2015; 2 (3): 85–88

The 2nd International Conference on Rare Cardiovascular Diseases was organized by the Krakow CRCD between 16th and 17th October 2014 at the John Paul II Hospital premises. The two-days Conference’s program was diverse, educational and touch most of the important topics in orphan diseases.

Hypertrophic cardiomyopathy (HCM) is the most common inheritable cardiac disorder with an estimated prevalence of 1:500 in the general population. In minority of patients, with the prevalence approximately of 3–5%, HCM progresses into dilated, end-stage or burn-out phase, characterized by LV wall thinning, systolic dysfunction and LV cavity dilatation. We present a case of a 45 year-old man with endstage HCM with postcapillary pulmonary hypertension, who was experimentally treated with sildenafil. It resulted in noticeable clinical and haemodynamical improvement. JRCD 2014; 2 (1): 23–26

The changes in hemodynamics, as well as changes in the aortic media during pregnancy, put the women with bicuspid aortic valve (BAV) and significant aortic stenosis (AS) and/or dilated aortic roots at risk for complications during this period. From 1988–2014,89 pregnant patients with BAV, mean 26.8 ±4.6 years, were observed. There were 52 patients with AS: 22 with mild AS, 30 with moderate to severe AS and 37 patients with aortic insufficiency (AI). Medical history, physical examination, NYHA class assessment, ECG and echocardiography were performed during each trimester (TR) of pregnancy and after delivery. During pregnancy all patients with mild AS remained in NYHA class I. All of them delivered vaginally healthy babies on term. Patients with moderate to severe AS in I TR remained in NYHA class I-II, in 6/30 patients clinical deterioration was observed within III TR. Seventeen women with severe AS delivered by cesarean section, the remaining vaginally. In the group of IA clinical deterioration was observed in III TR in 5/37 patients with severe AI, enlarged left ventricle (LV) and depressed systolic function. Six of them delivered by cesarean section, the remaining vaginally. Conclusions: Patients with mild AS tolerate well the increased cardiovascular demand of pregnancy. In patients with severe AS pregnancy intensifies the hemodynamic disorders. Volume overload of pregnancy is well tolerated in patients with mild and moderate IA. Cardiac complications can be expected in patients with severe IA, LV enlargement and impaired systolic function. In the observed group of BAV patients there was no pregnancy-associated dissection. JRCD 2014; 2 (1): 9–14

Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy due to abnormal morphogenesis of the endocardium and myocardium that occurs in early stages of foetal life. Given the increased risk of sudden cardiac death associated with LVNC, athletes with this diagnosis should be excluded from most competitive sports with the possible exception of those of low intensity (class IA) in selected cases. We report de novo diagnosed LVNC in an asymptomatic young footballer. JRCD 2014; 1 (7): 21–23

Cardiac tumors are quite rare, and differential diagnosis of them is challenging. We present the case report of a young man complaining on non specific chest pain who was reported to Cardiology Ward due to suspicion of thrombus in left ventricle. The patient's echocardiography showed an additional mobile structure located in apex of left ventricle. For further investigation he had a cardiac Magnetic Resonance Imaging (MRI) performed which drew out the suspicion of rather unmalignant tumor. Ultimately patient underwent noncomplicated cardiac surgery with total excision of the tumor. Histopatology examination reveald capillary hemangioma.

Desmin – related myopathy is a chronic neuromuscular disorder caused by a mutation of desmin, an intermediate filament of the myocardial, skeletal, and smooth muscles. Clinical manifestations include skeletal myopathy, cardiac abnormalities, conduction disorders, or various types of arrhythmias. We present a case of a 33‑year‑old male with an end – stage restrictive cardiomyopathy and peripheral muscles myopathy caused by desmin mutation. JRCD 2013; 1 (5): 20–24

A 45-year-old man with a 2-month history of breathlessness, fatigue and irregular heart rate was referred to our centre after first-ever cardiopulmonary decompensation. At presentation he was stable with heart rate of 120/min, blood pressure of 140/90 mmHg, in NYHA class II. 12-lead ECG showed atrial fi brillation, low R-waves voltage and deep S-waves with non specific ST changes in V1-V6. A posterior-anterior chest X-ray showed an enlarged cardiac silhouette, whereas on lateral picture an unusual structure was identified in the thorax cavity. An echocardiogram showed an enlarged, hypertrophied left ventricle (LV) with severe global systolic impairment. A computed tomography revealed an intestinal loop, characterized as the anterior Morgagni type congenital diaphragm hernia, in the anterior mediastinum resulting in the heart dislocation to the right side. Cardiac magnetic resonance revealed a thinned apex coupled with increased trabeculation at the apex, posterior, lateral and anterior wall. The measurements of double-layered LV wall, confi rmed the diagnosis of LV non-compaction (LVNC). This case is an illustrate of a constellation of rare anomalies of LVNC, persistent left superior vena cava with a congenital anterior Morgagni-type diaphragm hernia, causing heart dextroposition. On multidisciplinary team meeting the general decision of conservative treatment was made with typical systolic heart failure pharmacotherapy, restoration of sinus rhythm, considering referral to ICD and/or CRT-D, and regular clinical and echocardiographic follow-up of both patient and patient’s pedigree. JRCD 2012; 1: 18–23 Key words: Cardiac malposition; Dextroposition; Diaphragmatic hernia; Left ventricular non-compaction

Gradual impairment of exercise tolerance is the commonest sign of heart failure (HF). Little is known as to which cardiac contributors of poor exercise capacity carry an independent prognostic information in HF.We investigated the prognostic role of exercise echocardiography (ex-echo) in HF patients.We studied 85 consecutive, symptomatic HF patients (66 males, mean age 62.5 ± 11.8 [range 21-83] years, mean left ventricular ejection fraction [LVEF] 27.2 ± 9.5%). The end-point was all-cause mortality. During the follow-up period (mean 43 ± 21 months) 21 patients died. Resting echocardiography and ex-echo, with the simultaneous measurement of peak oxygen uptake (VO(2peak)), was performed in each patient using a semi-supine ergometer (20 W, 2-min increments). Apart from conventional assessment of systolic and diastolic function (EF, E/A, DT, IVRT) or right ventricular systolic pressure (RVSP), tissue Doppler imaging was used for the assessment of LV and RV peak velocity (IVV) as well as acceleration during isovolumic contraction (IVA), peak velocity during ejection phase (S'), peak early diastolic velocity (E'), peak late diastolic velocity (A'), and ratio of early diastolic mitral/tricuspid velocity to peak early diastolic velocity (E/E').Patients who died were significantly older, had lower exercise capacity, more advanced HF, greater impairment of baseline systolic function, higher baseline pulmonary artery systolic pressure, and most importantly a lack of improvement in EF, diastolic function, and further increase of RVSP during exercise. Out of all echocardiographic parameters, only peak stress EF (x(2) 6.1; p = 0.01), baseline and peak exercise RVSP (x(2) 12.5 and c(2) 18.7; p 〈 0.001; respectively), and mitral E/E' ratio (x(2) 8.9; p 〈 0.01) were univariate predictors of prognosis and remained independently prognostic when adjusted for age and sex but were eliminated from the model by NT-proBNP.During exercise, more severe systolic and diastolic dysfunction with the elevation of pulmonary arterial pressure is more prevalent in HF patients who have a poorer outcome. The estimation of common parameters such as EF, RVSP and E/E' using ex-echo, provides prognostic information in HF.

Contrast-enhanced magnetic resonance imaging (CE-MRI) can identify myocardial scarring following acute myocardial infarction (AMI).To compare myocardial contrast echocardiography (MCE) and CE-MRI in detection of resting perfusion defect in patients with acute myocardial infarction.Twenty four patients (21 men, 3 women, mean age 58.7 +/- 11.4 years) underwent primary percutaneous coronary angioplasty (PCI) for anterior AMI. All patients underwent MCE: segmental perfusion was estimated in real time before and immediately after PCI and on third day after PCI, using low mechanical index (0.3) after 0.3-0.5 ml bolus injections of intravenous OptisonTM. The MCE was scored semiquantitatively as: 1--homogenous contrast enhancement, 2--patchy contrast enhancement, 3--no contrast (non-viable myocardium). All patients underwent CE-MRI on a 1.5 T scanner (SONATA, Siemens) on the third day after PCI. Acquisition of short axis slices was performed before and 20 min after injection of Gd-DPTA (0.15 mmol/kg) with an inversion recovery TurboFLASH sequence (TE 1.1 ms, TR 700 ms, flip angle 300) in multiple breath-holds. The pattern of hyperenhancement representing MI (which intensity was more than 150% intensity of myocardium) was quantified by planimetry. The CE-MRI was scored according to the severity of myocardial scar as: 1--without scar, 2--50% of myocardial thickness, 3 -50% of myocardial thickness.Myocardial perfusion was analysed using MCE and contrast-enhanced MRI in 362 segments. Agreement between MCE and CE-MRI for identification of viable versus necrotic myocardium on third day after PCI was 86% (kappa = 0.73). Thirteen (54%) patients showed transmural necrosis at CE-MRI while 11 (46%) showed non-transmural necrosis. Patients from the transmural necrosis group showed a higher creatine kinase peak (p = 0.0001), higher CK-MB (p = 0.00002) and higher troponine level (p = 0.008), and more impaired baseline regional contractile function (p = 0.045). All angiographic parameters were less favourable in this group before as well as after PCI than in patients with non-transmural necrosis.Myocardial contrast echocardiography correlates very well with CE-MRI in the assessment of myocardial perfusion after PCI in AMI. Contrast-enhanced MRI is accurate technique for assessing the infarct zone. Identification by CE-MRI of transmural necrosis was associated with more impaired left ventricular function, non-reperfused MI, and presence of Q waves in ECG.

Renal dysfunction is an important factor of cardiovascular risk. Renal artery stenosis (RAS) is a potential cause of secondary hypertension and by renal ischemia may lead to progressive renal insuficiency. In RAS patients a significant increase in prevalence of coronary artery disease (CAD) as well as revascularisation rate and mortality rate was observed.To determine the prevalence of RAS in patients with suspected CAD.The study group consisted of 1036 consecutive patients (700 men; 67.6% ) in the mean age of 62.1+/-9.7 (25-85) years admitted to coronary angiography. Simultaneously renal angiography was performed in all patients.Stenosisor = 50% in at least one main branch of coronary artery was found in 633 (66.1%) patients. The proportion of patients with one, two or three vessel CAD was respectively 291 (46%), 169 (26%) and 173 (27.3%). Non-significant coronary lesions50% were found in 108 (10.4%) patients, whereas in 295 (28.5%) patients no angiographic evidence of CAD was documented. In the whole group of patients RAS was found in 339 (32.7%) of patients - 124 (12%) had bilateral lesions. RAS prevalence in patients with CAD was 38.3% (284/741) and its frequency increased with severity of CAD: from 25% (27/108) in patients with insignificant coronary lesions up to 36.4% (106/291), 40.2% (68/169) and 48% (83/173) in 1, 2, and 3-vessel disease, respectively (p0.001). RAS prevalence in patients with normal coronary arteries was 18.6% (55/295). RAS30% was detected in 194 (18,7%) patients; RAS between 30-49% in 81 patients (8.7%); RAS 50-69% in 38 patients (3.7%) and RASor = 70% in the remaining 26 patients (2.5%). RASor = 50% was noted in 8 (2.7%) patients without coronary lesions; in 5 (4.6%) with insignificant coronary artery atherosclerosis and 51 (8%) with coronary artery stenosis50% (p=0.0008). Stepwise regression analysis identified 4 independent predictors of RASor = 50%: CAD severity (p=0.014), serum creatinine concentration (p0.001), cigarette smoking (p=0.02) and stenosis of aortic arch branches (p0.001).RAS is a frequent finding in patients with suspected CAD. CAD severity, number of involved aortic arch arteries, cigarette smoking and serum creatinine are independent RAS predictors.

Aortic stiffening is the most important determinant of elevated systolic blood pressure which in turn is the main contributor to the burden of disease attributable to hypertension. Endothelial function may affect arterial stiffening as has been shown for carotid-aorto-femoral segments in healthy humans or subjects with cardiovascular risk factors. We investigated whether this association is present selectively for aorta and whether it extends to patients with advanced atherosclerosis. Direct measurements of aortic pulse wave velocity (aPWV) to assess aortic stiffness and brachial artery flow-mediated dilatation (bFMD) tests to assess endothelial function were performed in 111 consecutive patients suspected of coronary artery disease. Progression of atherosclerosis was determined on the basis of the presence or absence of significant coronary artery stenosis, CAS (>or=50%) in angiography. bFMD was lower (P

Short QT (SQTS) syndrome is a rare inherited autosomal dominant cardiac channelopathy associated with malignant ventricular and atrial arrhythmias. It is the severest form of the major channelopathies, with cardiac arrest or sudden cardiac death (SCD) as the most common presentation. We report a case of a young patient in whom ventricular fibrillation was the first manifestation of the disease.

Eosinophilic myocarditis is a rare form of myocardial inflammation that may lead to heart failure and death, if left untreated. A previously healthy 26-year-old man was admitted to the department with chest pain and dyspnoea, that started two days before. Few days prior to the onset of symptoms he started to take weight-loss supplement with Garcinia cambogia. On laboratory tests myocardial necrosis markers were positive and blood hypereosinophilia was noted. Left ventricular ejection fraction was slightly decreased to 45% with regional wall motion abnormalities and significantly thickened left ventricle walls. Coronary angiography did not show any abnormalities. Endomyocardial biopsy revealed eosinophil infiltration of the myocardium. Stool cultures were positive for Giardia lamblia. The patient initially received standard heart failure therapy and then due to eosinophil infiltration of the myocardium, systemic steroids and antiparasitic treatment were added. His general condition and ejection fraction improved after a week and he was discharged home on standard heart failure treatment and prednisone. In the setting of acute coronary syndrome symptoms in patients without atherosclerotic lesions and decreased ejection fraction myocarditis should be suspected. Blood hypereosinophilia may raise suspicion of the eosinophilic myocarditis. The proper diagnosis should be supported by endomyocardial biopsy results as it plays key role in the initiation of steroid therapy which leads to heart failure symptoms relieve and improvement of left ventricle function.

Heart failure (HF) is characterised by poor prognosis and increasing prevalence regardless of the progression in diagnosis and therapy. A hallmark of HF is a reduced ability to perform physical exercise. Pathophysiology of impaired exercise capacity is complex; the basis is pump failure central mechanism but peripheral responses are also affected. For an exercise tolerance assessment self-administered activity questionnaires, like functional NYHA classification, are used. Exercise testing has been widely used in assessment of chronic, stable HF patients (pts). Cardiopulmonary exercise test (CPX) and direct measurement of maximal oxygen uptake (VO2max) is established as a mainstay of the objective evaluation of functional activity in HF. Rest echocardiography is of limited value in predicting exercise tolerance. Both systolic and diastolic function can be assessed by stress echocardiography. Non-invasive estimation of left and right ventricular filling pressure is well correlated with exercise duration and VO2max. HF is not a contraindication for exercise training and cardiac rehabilitation. However, careful pts selection, supervision during exercise and follow-up are mandatory. The benefits of exercise training in HF pts include improvement in exercise duration, quality of life and mortality reduction.

Currently, degenerative changes are the most important cause of valvular aortic stenosis. Epidemiological as well as experimental studies suggest that inflammatory process induced and intensified by common atherosclerosis risk factors as age, hyperlipidemia, diabetes, hypertension, male sex and smoking, is responsible for these changes. Microscopic examination of the affected valves reveals: endothelium derangement, thickening of the subendothelial layer, intra- and extracellular lipid, lipoprotein and protein deposits, inflammatory infiltrate composed of macrophages, T lymphocytes, foam cells and intensive calcification in the zones of lipid accumulation. The method of choice in the assessment of aortic valve stenosis is echocardiography. New, more sensitive, quantitative radiological methods like Electron Beam Computed Tomography and Multislice Computed Tomography may be useful in a detection of early lesions and monitoring of their progress. As the process of aortic valve degeneration is long and progressive in nature, early introduction of effective prevention would decrease the number of patients operated on for aortic stenosis. A number of studies indicate the role of statins in slowing the progression of aortic valve degeneration. Nevertheless, no large randomized prospective study has emerged in this field and there is no strong enough evidence for the efficacy of statins in degenerative aortic valve disease.

To evaluate quality of life (QoL) in patients after aortic homograft implantation for aortic valve disease and in those who had undergone reoperation due to homograft dysfunction.QoL was assessed in 354 patients (72 women and 282 men, mean age 55.1 +/- 11.5 years, range 13-69) after aortic homograft implantation. Patients were divided into two groups: I--patients after aortic homograft implantation without reoperation (291 patients) and II--patients after reoperation (68 patients). We used two questionnaires: SF 36 (Short Formulation 36) and a self-developed questionnaire for patients after cardiac operation (SDQ). SF 36 has three levels: a) 36 items; b) eight scales: physical activity, social activity, limitations in every day activity, body pain, mental health, emotional problems, vitality and health perception; c) two summary measures that aggregate scales; general physical health which constitutes of physical activity, limitations in every day activities, body pain, health perception and general mental health which constitutes the rest of four scales: social activity, mental health, emotional problems, vitality. Each scale is standardized from 1 to 100 with50 indicating better than general population average. SDQ is focused on social and demographic factors, clinical symptoms before and after operation, risk factors and physical and occupational activity.By SF 36, only health perception was below general population average. We found a statistically positive relationship between QoL and high physical and occupational activity in both groups (p0.05). The presence of dyspnea, chest pain, palpitations, edema, faints, fear/anxiety and hypertension were related to decreased general physical and mental health in both groups (p0.05).Patients after aortic homograft implantation have high QoL. This holds also for those after reoperation for homograft dysfunction. Symptomatic patients have lower QoL. Higher QoL is associated with physical and occupational activity. There is no difference in general physical health in both groups. General mental health is decreased in reoperated patients. Since reoperated patients more often suffer from emotional problems, they may particularly benefit from the psychological support.

Systemic autoimmune diseases form a diverse group which includes: systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), scleroderma, dermato-polymyositis, Wegener's granulomatosis, Sjögren syndrome. Although multisystem involvement is the hallmark of these diseases, the heart seems to be less affected than other organ systems. The aim of the study was to study possible cardiac abnormalities in patients with documented systemic autoimmune diseases and to assess whether there was any relation between antiphospholipid, anti-dsDNA antibodies and myocardial dysfunction findings. 76 patients (53 with SLE, 9 with MCTD, 8 with scleroderma, 6 with Wegener's granulomatosis) were subjected to our study, 69% of these patients manifested cardiac involvement, based on two-dimentional echocardiografic examination (36%--post-inflammatory valvular thickening, 20%--pericardial effusions, 15%--valvular regurgitation, 7%--left atrial enlargement, 5%--left ventricular hypertrophy, 4%--left ventricular dysfunction). None of the patients showed characteristic, acute Libman-Sacks endocarditis, which probably can be explained by chronic corticosteroid-treatment. Clinical evidence of cardiac abnormalities has been observed, in as many as 58% of cases with positive echocardiographic findings. The frequency and extend of cardiac pathology positively correlated with the detection of antiphospholipid antibodies. No such relationship was observed in patients with the presence of very high titers of antinuclear antibodies (anti-dsDNA). In conclusion, our results indicate that echocardiography is a useful method for assessment and monitoring cardiac involvement in the systemic autoimmune diseases.

Not all coronary events occur in young individuals with traditional risk factors. In recent years some authors have observed increased prevalence of elevated anti-phospholipid (aPL) antibodies in young patients with myocardial infarction. Also, thickening of the combined arterial intima-media thickness (IMT) of superficial vessels has been identified as an independent risk factor for both stroke and heart attack. The objective of our study was to assess possible association between aPL antibodies and carotid IM thickening in young survivors of myocardialIn a case control study we determined IgG and IgM antiphospholipid antibodies by enzyme-immunoassay, and IMT by ultrasonography in 50 male survivors of myocardial infarction under the age of 50, and compared them to 50 healthy controls.Elevated aPL antibody levels (IgG10 GPL; IgM20MPL) were detected in 12 of 50 patients (24%) with MI and in 3 of 50 controls (6%). The mean level of aPL antibodies was significantly higher in the patients than in the controls (IgG 9.15+/-3.53 vs 7.69+/-2.98 GMP, p=0.04 and IgM 18.46+/-7.61 vs 12.14+/-5.05 MPL, p0.01). Patients with MI had a significantly greater IMT than healthy controls (0.9 mm vs 0.6 mm; p0.01). There was a correlation between aPL and IM thickening (r=0.31; p=0.01). Among coronary risk factors only hypertension (r=0.28; p=0.01) and smoking (r=0.41; p=0.01) showed a relationship with IMT.The intima-media thickness of the carotid artery and elevated aPL antibodies are strongly associated with the risk of myocardial infarction in young patients.

Background Fibrosis of extracellular matrix (ECM) in dilated cardiomyopathy (DCM) corresponds to the myocardial over-production of various types of collagens. However, mechanism of this process is poorly understood. Objective To investigate whether enhanced metabolism of ECM occur in DCM. Methods Seventy consecutive DCM patients (pts) (48 ± 12.1 years, EF 24.4 ± 7.4 %) and 20 healthy volunteers were studied. Based on symptoms duration, pts were divided into new-onset (n = 35, 6 months) and chronic DCM (n = 35, >6 months). Markers of collagen type I and III synthesis-procollagen type I carboxy- and amino-terminal peptides (PICP and PINP) and procollagen type III carboxy- and amino-terminal peptides (PIIICP and PIIINP), collagen 1 (col-1), ECM metabolism controlling factors—tumor growth factor beta-1 (TGF1-β), connective tissue growth factor (CTGF), and ECM degradation enzymes—matrix metalloproteinases (MMP-2, MMP-9) and their tissue inhibitor (TIMP-1) were measured in serum. All pts underwent right ventricular endomyocardial biopsy to study ECM fibrosis. Results The presence of fibrosis was detected in 24 (34.3 %) pts and was more prevalent in chronic DCM [17 (48.6 %) vs. 7 (20 %), p