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Pulmonary hypertension (PH) in patients with heart failure (HF) contributes to a poorer prognosis. However, in those with dilated cardiomyopathy (DCM), the true prevalence and role of PH is unclear. Therefore, this study aimed to analyze the profile of DCM patients at various levels of PH risk, determined via echocardiography, and its impact on outcomes. The 502 DCM in- and out-patient records were retrospectively analyzed. Information on patient status was gathered after 45.9 &plusmn, 31.3 months. Patients were divided into 3 PH-risk groups based on results from echocardiography measurements: low (L, n = 239, 47.6%), intermediate (I, n = 153, 30.5%), and high (H, n = 110, 21.9%). Symptom duration, atrial fibrillation, ventricular tachyarrhythmia, ejection fraction, right atrial area, and moderate or severe mitral regurgitation were found to be independently associated with PH risk. During the follow-up period, 83 (16.5%) DCM patients died: 29 (12.1%) in L, 31 (20.3%) in I, and 23 (20.9%) in H. L-patients had a significantly lower risk of all-cause death (L to H: HR 0.55 (95%CI 0.32&ndash, 0.98), p = 0.01), while no differences in prognosis were found between I and H. In conclusion, over one in five DCM patients had a high PH risk, and low PH risk was associated with better prognoses.

Background: Fibrosis of the extracellular matrix (ECM) in dilated cardiomyopathy (DCM) is common and compromises both systolic and diastolic function. The aim of this study was to investigate the kinetics of ECM fibrosis markers over a 12 month follow-up in patients with DCM based on the severity of diastolic dysfunction (DD). Methods: Seventy consecutive DCM patients (48 ± 12.1 years, ejection fraction 24.4 ± 7.4%) were included in the study. The grade of DD was determined using the ASE/EACVI algorithm. Markers of ECM fibrosis were measured at baseline and at 3 and 12 month follow-ups: collagen type I and III (PICP, PINP, PIIICP, PIIINP), transforming growth factor beta-1 (TGF1-b), connective tissue growth factor (CTGF) and galectin-3 were measured. Results: Patients were divided into three groups according to DD severity: 30 patients with grade I, 18 with grade II and 22 with grade III of DD. Levels of PICP, PINP were increased over a 12-month period, while PIIINP decreased and PIIICP unchanged. Levels of TGF1-b decreased from the 3 to the 12-month points in grade I and II DD, and in grade III they remained unchanged. Levels of CTGF decreased over 12 months in grade III DD but were unchanged in grades I and II. Galectin-3 levels remained the same over all observation periods, irrespective of DD grade. Conclusions: Regardless of the DD grade, markers of collagen type I synthesis increased, markers of collagen type III decreased. Levels of TGF and CTGF had a tendency to decrease. Galectin-3 was revealed not to be a marker discriminating the severity of DD.

Aims The hybrid technique of single-photon emission tomography and computed tomography with technetium99m-hexamethylpropyleneamine oxime–labelled leucocytes (99mTc-HMPAO-SPECT/CT) is an emerging diagnostic technique in patients with cardiac device-related infective endocarditis (CDRIE). This prospective study assessed the 99mTc-HMPAO-SPECT/CT diagnostic profile and its added value to the modified Duke criteria (mDuke) in CDRIE diagnostic work-up. Methods and results The study examined 103 consecutive patients with suspected CDRIE, who underwent 99mTc-HMPAO-SPECT/CT. Diagnostic accuracy was calculated based on a final clinical CDRIE diagnosis, including microbiology, echocardiography, and a 6-month follow-up. Subsequently, we compared the diagnostic value of the initial mDuke classification with a classification including 99mTc-HMPAO-SPECT/CT positive results as an additional major CDRIE criterion: mDuke-SPECT/CT. Overall, CDRIE was diagnosed in 31 (31%) patients, whereas 35 (34%) 99mTc-HMPAO-SPECT/CT were positive. 99mTc-HMPAO-SPECT/CT was characterized by 86% accuracy, 0.69 Cohen’s kappa coefficient, 84% sensitivity, 88% specificity, 93% negative, and 74% positive predictive values. The original mDuke displayed 83% accuracy, 0.52 kappa, whereas mDuke-SPECT/CT had 88% accuracy, and 0.73 kappa. Compared with mDuke, mDuke-SPECT/CT showed significantly higher sensitivity (87% vs. 48%, P Conclusion In patients with CDRIE, 99mTc-HMPAO-SPECT/CT provides high diagnostic accuracy, whereas a negative scan excludes CDRIE with high probability. Inclusion of 99mTc-HMPAO-SPECT/CT into mDuke diagnostic criteria yields significantly higher sensitivity and a reduction in possible CDRIE diagnoses.

Background: Atrial fibrillation (AF) is the most common arrhythmia in patients with dilated cardiomyopathy (DCM). However, the epidemiology as well as clinical and prognostic significance of AF in DCM are poorly defined. Aims: We aimed to assess the impact and prognostic value of AF in DCM as well as to investigate the concept of AF‑induced DCM. Methods: Hospital records of 285 patients with DCM from 2012 to 2018 with follow-up were analyzed. Results: Atrial fibrillation was present in 89 patients (31%). They were older, more frequently male, hadhigher body mass index, New York Heart Association class, heart rate (HR), creatinine levels, and larger atria (all P < 0.05) than patients without AF. During follow‑up (mean [SD], 35 [24] months), death occurred in 20 of the 82 available patients with AF and 22 of the 188 patients without AF (24% and 12%, respectively; P = 0.007). Atrial fibrillation was independently associated with a worse outcome (hazard ratio, 2.4; 95% CI, 1.3–4.3) and was found to be the major cause of DCM in 21 patients (24%). The diagnostic accuracy of the most optimal predictive model for AF‑induced DCM was 0.935 (95% CI, 0.903–0.967). Despite numerical differences, survival was similar in DCM patients with and without AF (P = 0.15). Conclusions: Almost one‑third of patients with DCM had AF. Most of the parameters analyzed differed between patients with and without AF, and AF was found to be an independent prognostic factor of DCM. One‑fourth of patients with DCM and AF met the diagnostic criteria for AF‑induced DCM.

Infective endocarditis (IE) is a life-threatening disease, establishing a diagnosis is often challenging. The aim of this prospective study was to evaluate and compare the diagnostic performance of the combined use of single photon emission tomography and computed tomography with technetium99m-hexamethylpropyleneamineoxime—labeled leukocytes (99mTc-HMPAO-SPECT/CT) with transthoracic echocardiography (TTE) in patients with suspected IE. We enrolled 40 consecutive patients (12 females, 28 males, mean age: 58.6 ± 18) with suspected IE in the years 2015–2016. All patients underwent clinical evaluation, TTE and 99mTc-HMPAO-SPECT/CT for the assessment of lesions typical for IE. Scans were evaluated for the presence and location of increased radioactivity foci, corresponding to the accumulation of radiolabeled leukocytes in inflammatory lesions. After 6 months, the patients were re-evaluated clinically and with TTE. Final IE diagnosis was established in 14 (35%) patients. Lesions typical for IE were shown in 28 (70%) TTEs and 16 (40%) 99mTc-HMPAO-SPECT/CTs. The latter tests were characterized by 90% accuracy, 93% sensitivity, 88% specificity, 96% negative predictive value (NPV), 81% positive predictive value (PPV). TTE demonstrated 60% accuracy, 93% sensitivity, 42% specificity, 92% NPV, and 46% PPV. 99mTc-HMPAO-SPECT/CT was characterized by a lower number of false-positive results compared to TTE (3 vs. 15). In patients with suspected IE, 99mTc-HMPAO-SPECT/CT yields a smaller number of false-positive results, significantly higher diagnostic accuracy, specificity and PPV than TTE. It helps to differentiate IE infectious and sterile echocardiographic lesions and reduces by 27% the number of misdiagnosed IE classified in the ‘possible IE’ category by modified Duke Criteria.

The systemic amyloidosis are diseases induced by misfolded proteins. These insoluble proteins deposit in extracellular space. Infiltration the heart by amyloid can result in progressive diastolic and systolic dysfunction and restrictive cardiomyopathy phenotype – left ventricle hypertrophy and stiffness. More than 20 different precursor proteins have the propensity to form amyloid fibrils. One of the most common amyloid infiltrating the heart is transthyretin amyloid (ATTR) - mostly inherited disease. ATTR is generally considered a mainly neurological disease, but it is phenotypically heterogeneous and the clinical spectrum of the disease varies widely, which makes the diagnosis a real challenge. Although, the early diagnosis improve the prognosis, especially due to new drug introduced in ATTR - tafamidis. In this article we would like to present the case series of transthyretin amyloidosis, which was diagnosed by heart scintigraphy or genetic testing.

Hypertrophic cardiomyopathy (HCM) is a primary disease of the myocardium that is defined by the presence of regional (more frequent) or global myocardial hypertrophy, which usually results in functional cardiac impairment. We present a case of a 34-year-old man with apical HCM who was admitted to the cardiology department due to cardiac hypertrophy in the echocardiogram. The management of HCM patients is directed at heart failure treatment and decreasing left ventricular outflow tract or intraventricular gradient, if present. According to the patient’s calculated 5-year mortality risk, assessed using the HCM Risk-SCD Calculator, indications for implantation of an implantable cardioverter defibrillator were evaluated. JRCD 2017; 3 (5): 180–183

Background: The dynamics of the extracellular matrix (ECM) fibrosis process in dilated cardiomyopathy (DCM) may be assessed non-invasively by means of serum markers of fibrosis. Aim: To explore the kinetics of serum markers of fibrosis during a 12-month follow-up in DCM. Methods: We included 70 consecutive DCM patients (pts) (48 ± 12.1 years, EF 24.4 ± 7.4%) with new-onset (n = 35, duration 6 months). Markers of collagen type I and III synthesis – procollagens type I and III carboxy- and amino-terminal peptides (PICP, PINP, PIIICP, PIIINP), and ECM metabolism controlling factors – tumor growth factor beta-1 (TGF1-β), and connective tissue growth factor (CTGF) – were measured in serum at baseline, and at 3- and 12-month follow-up. All pts underwent endomyocardial biopsy to determine the presence and extent of ECM fibrosis. Results: Markers of collagen type I synthesis (PICP and PINP) were almost homogenously increased over the 3- and 12-month period, whereas PIIINP values decreased and PIIICP levels were unchanged in new-onset and chronic DCM, and in pts with and without ECM fibrosis. Both TGF-β and CTGF levels decreased over the observation period. Kinetics of serum markers of collagen synthesis and fibrosis controlling factors did not differ between DCM pts categorized according to disease duration and fibrosis status. Conclusions: The kinetics of collagen type I and III synthesis in DCM move in opposite directions, with production of collagen type I consistently increasing, and the synthesis of collagen type III decreasing. Levels of TGF and CTGF, which are proven fibrosis-stimulating factors, had a tendency to decrease. Regardless of disease duration or fibrosis status, the kinetics of serum markers of collagen synthesis, TGF and CTGF were similar in DCM. A better understanding of the kinetics of serum markers of fibrosis in DCM may help to develop more tailored therapeutic approaches to fibrosis.

Left ventricular reverse remodeling (LVRR) is reported in dilated cardiomyopathy (DCM) patients (pts). However, numerous definitions of LVRR exist. Measurements of serum markers of fibrosis provide insight into myocardial fibrosis. The relationship between LVRR and fibrosis is poorly understood. From July 2014 until October 2015, we included 63 consecutive DCM pts (48 ± 12.1 years, EF 24.4 ± 7.4%) with completed baseline and 3-month follow-up echocardiograms. LVRR was assessed on the basis of four differing definitions. Procollagens type I and III carboxy- and amino-terminal peptides (PICP, PINP, PIIICP, and PIIINP), collagen 1, ostepontin, tumor growth factor beta-1, connective tissue growth factor, and matrix metalloproteinases (MMP-2, MMP-9), and their tissue inhibitor (TIMP-1) were measured in serum. In addition, all pts underwent right ventricular endomyocardial biopsy. Depending on the definition chosen, LVRR could be diagnosed in between 14.3 and 50.8% pts. Regardless of the LVRR definition used, the frequency of LVRR was similar in fibrosis negative and positive DCM. Minor differences of markers of fibrosis were detected between pts with and without LVRR. For every LVRR definition, adjusted and unadjusted models were constructed to evaluate the predictive value of serum fibrosis parameters. Only an increase of TIMP-1 by 1 ng/ml was found to independently increase the probability of LVRR by 0.016%. The choice of a particular definition of LVRR determines the final diagnosis, and this has a profound impact on subsequent management. LVRR is unrelated to biopsy-detected ECM fibrosis. Serum markers of fibrosis are only weakly related to LVRR, and are not of use in the prediction of LVRR.

Cardiovascular diseases in pregnancy are the most common causes of maternal mortality in developed world and an important cause of heart failure, stroke, and arrhythmia. Cardiac disease complicates 0.4–4% of all pregnancies, and arrhythmias are among the most common cardiac complications [1]. In some cases, pregnancy triggers exacerbations of pre-existing arrhythmias, whereas in others it may manifest for the first time [2]. A prior history of arrhythmias or structural heart disease and family history of sudden death are the factors that matter to risk of tachyarrhythmias during pregnancy [3]. Due to extraordinary ethical considerations in pregnant women, there are only a few randomized studies and little data on efficacy or safety of antiarrhythmic drugs applied during pregnancy [4]. Therefore, much of clinical care is guided by general knowledge about hemodynamic changes in pregnant women, universal principles of treatment of arrhythmias and finally by gained experience in that area. Multi-disciplinary approach remains the overriding principle in management of pregnant woman with arrhythmias, including collaboration with gynecological and obstetric center. JRCD 2016; 2 (6): 177–180

Brugada syndrome is an autosomal dominant genetic disease with variable expression characterized by abnormal electrocardiographic findings – right bundle branch block and ST-segment elevation in the anterior precordial leads, without evidence of structural heart disease. Individuals affected by the disease have an increased risk of ventricular tachyarrhythmias and sudden cardiac death (SCD). We present a case of a 33-year old man who was admitted to cardiology department after sudden cardiac arrest due to ventricular fibrillation. Evaluation for a structural heart disease was negative and routine tests did not reveal the cause of cardiac arrest. Retrospective evaluation of the patient electrocardiograms showed changes consistent with Brugada type 2 pattern that were variable over time. Performed pharmacological challenge with sodium channel blocker – ajmaline – provided an ambiguous result. Nevertheless, the patient was qualified for an implantable cardioverter defibrillator implantation as a secondary SCD prophylaxis. He underwent the procedure without complications and was discharged home in good general condition without symptoms.

To validate the modified World Health Organization (mWHO) risk classification in advanced and emerging countries, and to identify additional risk factors for cardiac events during pregnancy.The ongoing prospective worldwide Registry Of Pregnancy And Cardiac disease (ROPAC) included 2742 pregnant women (mean age ± standard deviation, 29.2 ± 5.5 years) with established cardiac disease: 1827 from advanced countries and 915 from emerging countries. In patients from advanced countries, congenital heart disease was the most prevalent diagnosis (70%) while in emerging countries valvular heart disease was more common (55%). A cardiac event occurred in 566 patients (20.6%) during pregnancy: 234 (12.8%) in advanced countries and 332 (36.3%) in emerging countries. The mWHO classification had a moderate performance to discriminate between women with and without cardiac events (c-statistic 0.711 and 95% confidence interval (CI) 0.686-0.735). However, its performance in advanced countries (0.726) was better than in emerging countries (0.633). The best performance was found in patients with acquired heart disease from developed countries (0.712). Pre-pregnancy signs of heart failure and, in advanced countries, atrial fibrillation and no previous cardiac intervention added prognostic value to the mWHO classification, with a c-statistic of 0.751 (95% CI 0.715-0.786) in advanced countries and of 0.724 (95% CI 0.691-0.758) in emerging countries.The mWHO risk classification is a useful tool for predicting cardiac events during pregnancy in women with established cardiac disease in advanced countries, but seems less effective in emerging countries. Data on pre-pregnancy cardiac condition including signs of heart failure and atrial fibrillation, may help to improve preconception counselling in advanced and emerging countries.

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

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

Recent data indicate that substantial proportion of cardiomyopathy patients have in fact more than one phenotype, the phenomenon termed as overlap or mixed cardiomyopathy. The molecular mechanisms and pathology as well as clinical management and prognosis of overlap cardiomyopathy is largely unknown and speculative. We report the case of 51-year-old man who was admitted due to progressive intolerance of physical activity with concomitant shortness of breath. He underwent complex cardiological studies, including invasive examinations and endomyocardial biopsy. However, despite exhaustive diagnostic work-up, the final diagnosis is still not one-hundred percent certain, and we eventually diagnosed him with overlap cardiomyopathy as most probably two phenotypes of hypertrophic and restrictive cardiomyopathy coexist. Perhaps, cardiomyopathies are far too complex entities to be easily labeled with one or another term. Accumulated data on the clinical course of various cardiomyopathies provide numerous evidence on the continuum rather than onceforever diagnosis. JRCD 2014; 1 (6): 21–28

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

The mediastinal tumors are rare but potentially fatal diseases. Teratomas are derived from multipotent germ-line cells. In majority of cases mediastinal tumours are incidentally detected on chest X ray. We present 19-year-old patient with recurrent pericarditis recurrent pericarditis. After comprehensive diagnostic process it was figured out that the reason for recurrent pericarditis was large mediastinal teratoma. Once the correct diagnosis has been made, the patient could have been completely cured. JRCD 2014; 1 (6): 29–32