DOI: 10.3897/bgcardio.31.e175420

Authors: I. Bayraktarova, I. Petrova, T. Doychinova, B. Georgiev

Abstract: Hypertrophic cardiomyopathy is one of the most common hereditary cardiomyopathies in daily practice. The different phenotypic manifestations and hemodynamic features often make diagnosis and therapy a clinical challenge. Diagnosis and imaging follow-up of cardiomyopathies are becoming increasingly multimodal. Despite the new imaging capabilities, echocardiography continues to play a key role in the primary differential diagnostic process, in the follow-up of patients, regardless of the therapeutic approach taken, and in the screening of relatives. Therefore, good knowledge of echocardiographic methods for assessment in these patients, of practical features and possible errors during the examination, is an essential prerequisite for high quality care. Precise systematic measurements and descriptions of the fi nding in each patient are the basis of good follow-up, adequate management planning and reassessment of therapy, and of seamless team care for the patient by various specialists. Confl icting or inconsistent imaging fi ndings, discrepancies in imaging fi ndings and clinical presentation, and the need for specialized therapy are valid reasons for referring the patient for a staged evaluation to an expert center for HCM.

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DOI: 10.3897/bgcardio.31.e164002

Authors: V. K. Singh, Shalabh Agarwal, Prithpal Singh Matreja

Abstract: Background: HDL is decisive for reverse cholesterol transport, enabling the removal of cholesterol from macrophages in atherosclerotic plaques. Although HDL-C has long been allied to CVS protection, recent evidence suggests that CEC may more accurately reflect HDL’s functional efficacy. However, studies exploring the relationship between CEC and CAD risk have produced inconsistent results. Objectives: The association between CEC and CAD risk was assessed, along with its potential to predict MACE, including cardiac mortality, all-cause mortality, and non-fatal MI, in this systematic review and meta-analysis. Material and methods: A comprehensive search of PubMed, Scopus, Web of Science, and The Cochrane Library was conducted to identify studies published up to January 2025. Observational studies comparing CEC levels between individuals with and without CAD were included. Results: Twenty-three studies met the inclusion criteria. The pooled SMD of – 0.40 (95% CI: -0.53−-0.26), with a p-value < 0.0001, revealed significantly lower CEC levels in CAD patients compared to non-CAD individuals. Higher CEC was strongly allied with a reduced risk of CAD – OR = 0.57; 95% CI: 0.48−0.67, P < 0.00001, and a pooled risk ratio (RR) of 0.64; 95% CI: 0.48−0.86, p = 0.003. Impaired CEC was associated with an increased risk of cardiac mortality – OR = 3.94; 95% CI: 2.63−5.90, p < 0.00001, and all-cause mortality – OR = 2.84; 95% CI: 2.01−4.00, p < 0.00001. However, insignificant association was found between CEC and non-fatal MI (OR = 3.47; 95% CI: 0.41−29.22, p = 0.25). Conclusion: This meta-analysis underscores the probability of CEC as a biomarker for the assessment of CVS risk. Higher CEC levels are linked to a reduced risk of CAD, cardiac mortality, and all-cause mortality, but no significant relationship was observed with non-fatal MI. Future research should prioritize standardizing CEC measurement methods and investigating its therapeutic potential for preventing atherosclerotic CVS disease.

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DOI: 10.3897/bgcardio.31.e171913

Authors: D. Maneva, L. Balabanski, M. Atanasoska, S. Y. Staykova, Е. Filipov, I. Bradinova, I. Tasheva, N. Marinov, R. Vazharova, D. Toncheva

Abstract: Hereditary cardiomyopathies are mostly autosomal dominant disorders that affect cardiac muscle structure and function, often leading to arrhythmias, heart failure, or sudden cardiac death. We report two brothers (aged 41 and 38), who presented with inherited cardiomyopathy and a family history of suspected cardiac disease in their father. The older sibling was referred for genetic evaluation due to clinical symptoms suggestive of cardiomyopathy. Whole genome sequencing (WGS), followed by targeted analysis of cardiomyopathy-associated genes, identifi ed a pathogenic heterozygous variant in the FLNC gene (c.7384+1G>T) located at the canonical donor splice site. The fi nding was consistent with the patient’s phenotype. In conjunction with the current clinical guidelines, an implantable cardioverter-defi brillator (ICD) was implanted as primary prevention of sudden cardiac death (SCD). Cascade genetic screening identifi ed the same FLNC variant in his younger, asymptomatic brother. He was enrolled in a structured surveillance program with personalized lifestyle recommendations. This case illustrates the critical role of WGS in establishing a defi nitive molecular diagnosis, informing clinical decision-making, and enabling timely intervention and clinical management. It also underscores the importance of family-based screening in inherited cardiomyopathies, even when the family history is limited or unconfi rmed. Integration of genomic tools into cardiology practice can enhance early detection, improve outcomes, and facilitate precision medicine strategies in affected families.

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DOI: 10.3897/bgcardio.31.e152709

Authors: H. Nough, M. Dehghanifirouzaadi, A. Nough, R. Sadeghi, N. Yoshany

Abstract: Background and Objective: Coronary artery ectasia (CAE), a diffuse or focal dilatation of coronary arteries, remains poorly understood. While the neutrophil-to-lymphocyte ratio (NLR) is a well-established infl ammatory marker in cardiovascular diseases, its diagnostic role in CAE is unclear. This study aimed to evaluate the diagnostic utility of NLR in CAE and explore its association with disease severity. Material and Methods: In this case-control study, 115 patients (28 CAE, 87 controls) admitted to Shahid Sadoughi Hospital (2012–2020) were analyzed. Demographic data, medical history, and complete blood count (CBC)-derived NLR were compared using SPSS and appropriate statistical tests. Results: The mean NLR did not differ signifi cantly between CAE (0.79 ± 2.01) and control groups (1.46 ± 2.07) (P = 0.830), nor by age, sex, or ectasia extent. However, NLR was lower in three-vessel involvement (0.49 ± 1.66) versus single-vessel involvement (1.04 ± 2.32) (P = 0.036). Conclusion: NLR lacks diagnostic value for CAE but may correlate with disease burden, as evidenced by reduced NLR in multi-vessel involvement. Further research should investigate NLR’s pathophysiological role and alternative infl ammatory markers in CAE.

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DOI: 10.3897/bgcardio.31.e149169

Authors: M. Dimova, M. Gospodinova, K, Genova, E. Paskalev, V. Velchev

Abstract: Anderson-Fabry disease is a hereditary, X-linked disorder with multiorgan impairment. The debut of the disease is usually in childhood, the clinical course and the prognosis are determined by the degree of the heart, kidney and brain dysfunction. Cardiac involvement includes the development of hypertrophic cardiomyopathy, conduction disorders and less common - valvular disease. Among patients with hypertrophic cardiomyopathy, about 0.5-1% are due to Anderson-Fabry disease. We present a clinical case of a man diagnosed with Anderson-Fabry disease, the clinical suspicion of which was raised based on rapid progression of renal disease, lead to end-stage kidney failure, cerebrovascular disease, and hypertrophic cardiomyopathy. The patient underwent a successful kidney transplantation, and systemic treatment with enzyme replacement therapy, but despite this, the cardiac involvement progressed, necessitating the placement of an ICD and alcohol septal ablation of the first septal branch due to a severe subvalvular gradient.

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DOI: 10.3897/bgcardio.30.e141268

Authors: H. Varnaliyska, R. Stoycheva, A. Popova, D. Dimitrov, A. Osmanov, V. Groudeva, D. Trendafilova

Abstract: We present a clinical case of a patient with hypertrophic cardiomyopathy (HCM) characterized by an inverted septal curvature phenotype, accompanied by angina and presyncope symptoms. Patients with this type of presentation remain a diagnostic and therapeutic challenge due to their association with midventricular obstruction, apical aneurysm formation, and an increased risk of arrhythmias and sudden cardiac death (SCD). Their treatment and follow-up require a multidisciplinary approach to improve quality of life and prognosis. Various imaging modalities were applied in this case, confirming the presence of severe left ventricular hypertrophy, posterior wall hypertrophy of the right ventricle, and a midventricular gradient. Magnetic resonance imaging (MRI) demonstrated fibrosis involving approximately 25% of the myocardium. The patient was stratified as high-risk patient for SCD. To improve the patient’s quality of life and prognosis, pharmacological therapy was optimized, and an implantable cardioverter-defibrillator (ICD) was placed.

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DOI: 10.3897/bgcardio.30.e141099

Authors: N. Spasova, E. Kinova, R. Savova, K. Genova, M. Garcheva, A. Goudev

Abstract: Transthyretin amyloidosis (ATTR) is a systemic disease characterized by the deposition of amyloid fi brils in various tissues, leading to progressive neurological manifestations and the development of cardiomyopathy, necessitating timely diagnosis and treatment. This article presents a clinical case of a 74-year-old patient with hereditary transthyretin amyloidosis (ATTRv), diagnosed using non-invasive imaging methods – echocardiography, magnetic resonance imaging, and single-photon emission computed tomography (SPECT-CT) – and confi rmed through molecular genetic analysis revealing a mutation in the TTR gene. The main clinical features of the disease, imaging fi ndings, and therapeutic strategies in the context of the presented case are discussed.

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DOI: 10.3897/bgcardio.30.e140680

Authors: A. Borizanova-Petkova, D. Somleva, E. Kinova, V. Koleva, Y. Zdravkova, A. Goudev

Abstract: Systemic lupus erythematosus is a chronic autoimmune disorder affecting predominantly young women. Cardiac involvement in systemic lupus erythematosus is common and the typical manifestations are pericarditis, myocarditis, valvular disease, coronary artery disease, cardiac arrhythmias, and congestive heart failure. Clinical lupus myocarditis typically occurs early during lupus, in the context of high disease activity. It may present as an acute illness or have a chronic course with the development of cardiomyopathy. We present a clinical case of young patient who developed a severe dilated cardiomyopathy as a fi rst manifestation of systemic lupus erythematosus.

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DOI: 10.3897/bgcardio.30.e140563

Authors: A. Borizanova-Petkova, E. Kinova, N. Spasova, D. Somleva, A. Goudev

Abstract: Background: The prevalence of obesity and hypertension among healthcare professionals has been previously described. Both diseases are known risk factors for atrial cardiomyopathy (ACM), a new clinical entity with potential to be a determinant of heart failure, atrial fi brillation and thromboembolic complications. In this perspective, early preventive strategy should be initiated in this special population. Aim: To defi ne ACM in middle-aged women healthcare professionals. Material and methods: Hundred and fi ve middle-aged women healthcare professionals, separated in 3 groups: with central obesity (n = 50,47%), obesity and hypertension (n = 24,23%), healthy control (n = 31,30%), underwent echocardiographic examination with volumetric and speckle-tracking analysis. Results: There were signifi cant differences in LA volumes between 3 groups. LA phasic function was signifi cantly reduced, and LA stiffness index was signifi cantly increased in obese groups compared to control. Higher body mass index was associated with increased LA volume index (p < 0.001, r = 0.56) and with reduced Peak atrial longitudinal strain (p < 0.001, r = -0.59) and Peak atrial contractile strain (p < 0.001, r= -0.56). Conclusion: Postmenopausal women, healthcare professionals with obesity and hypertension are at risk for asymptomatic ACM. Further investigations are needed to confi rm this concept.

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DOI: 10.3897/bgcardio.30.e143367

Authors: D.P. Amukti, L.M. Irham, S. Surono, R. El Khair, W. Adikusuma, R. Satria, B.D. Purwanto, S. Khairi, R. Chong

Abstract: Atherosclerosis (AS) a chronic cardiovascular disease, poses a major threat to human health and remains one of the leading causes of mortality among the elderly. Genetic factors have long been recognized as contributors to the predisposition to heart and vascular diseases, with several studies suggesting that specifi c genetic variants may infl uence AS risk. This study employs a bibliometric analysis to explore scientifi c literature related to AS throughusing genomic approaches, base on Scopus data spanning 1983 to 2024. A total of 1,702 studies related to AS research employing genomic approaches were identifi ed, comprising 1,137 research articles, 445 review articles, and 120 documents in other categories. The fi ndings reveal a marked rise in interest in regarding genomic approaches to AS, particularly since 2017, with the United States, China, and the United Kingdom leading in research output. International collaborations are also prevalent, with the United States contributing the most publications. Although contributions from Indonesian authors remain limited, there is signifi cant potential for future involvement. Notably, the most cited article, authored by Naghavi et al. in 2003, focusing on genomics in AS. Overall, the bibliometric analysis provides valuable insights into research trends and advancements in the study of atherosclerosis through genomic approaches.

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DOI: 10.3897/bgcardio.30.e140717

Authors: G. Tomova-Lyutakova, I. Lyutakov, P. Kalaydzhiev, E. Kinova

Abstract: In a world where metabolic disorders such as non-alcoholic fatty liver disease (NAFLD/NASH) and chronic heart failure (CHF) steadily increase, the limitations of current therapies become even more apparent. Lifestyle changes have been the primary approach to metabolic disorders. However, the new use of glucagon-like peptide-1 receptor agonists (GLP1RA) and sodium-glucose cotransporter-2 (SGLT2) – inhibitors seem to present a breakthrough in improving the outcome of metabolic disorders in diabetic and non-diabetic patients. In the current review, we focused on studies with non-diabetic metabolic disorders. The results underlined the hepatoprotective effect of GLP1RAs on the histological resolution of NASH/NAFLD and improved serum liver enzyme. GLP1RAs, especially Liraglutide, also seem benefi cial in reducing stool frequency in patients suffering from bile acid malabsorption (BAM). SGLT2 inhibitors improve cardiovascular and renal outcomes and decrease the hospitalization rate for heart failure, regardless of the diabetes status. GLP1RA reduce body mass and infl ammation . In conclusion, the most up-to-date studies on the respective topics show that GLP1RAs and SGLT2 inhibitors have promising systemic benefi cial effects on patients with metabolic disorders with or without type 2 diabetes mellitus.

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DOI: 10.3897/bgcardio.30.e140741

Authors: R. Savova, E. Kinova, A. Goudev

Abstract: Cardiac amyloidosis, once considered a rare disease, is a severe and progressive cardiomyopathy characterized by extracellular deposition of misfolded proteins in the ventricular myocardium. Early diagnosis of cardiac amyloidosis is essential for improving patient prognosis, as it allows for a broader range of therapeutic options that can enhance outcomes and/or prevent potentially irreversible loss of physical function and quality of life. Current data suggest that a majority of patients do not get a timely diagnosis. Patients with early-stage CA do not exhibit reduced global left ventricular systolic function but may develop symptoms of heart failure with preserved ejection fraction (HFpEF), with CA considered as one of the overlooked etiologies of HFpEF in the elderly. Cardiac amyloidosis is a progressive disease with a poor prognosis if left untreated. The average life expectancy of untreated patients with AL cardiac amyloidosis is less than 6 months, while that of patients with ATTR-CA is 2.5-3.5 years. Therefore, early clinical identifi cation is essential.

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DOI: 10.3897/bgcardio.30.e127924

Authors: L. Bardarska, K. Genova, Z. Shomanova, T. Chamova, I. Tournev, M. Gospodinova, A. Kaneva

Abstract: Mitochondrial diseases are rare and heterogeneous conditions frequently affecting the heart. They are caused by mutations in nuclear or mitochondrial DNA with maternal inheritance or de novo occurrence. We present a case of 13-year-old girl with malignant mitochondrial cardiomyopathy. First presentation of the disease is with tamponade and atrial fibrillation. Cardiac imaging shows non-specific changes of the left ventricle that cannot be explained by any cardiomyopathy: hypertrophy, dilation, hypertrabeculation. These findings raised the suspicion for mitochondrial cardiomyopathy. The patient is examined by neurologist due to multisystem character of the disease. Electromyography reveals myopathy. Serum lactate and creatine phosphokinase are elevated. Acute inflammation is excluded with endomyocardial biopsy which also discovers complex III deficiency. Genetic testing identified likely pathogenic mutation in mitochondrial DNA with maternal inheritance. Two years after initial presentation patient was referred for cardiac transplantation due to fast progression of the disease. Mitochondrial cardiomyopathies have variable clinical manifestation which impedes the diagnosis and makes impossible the creation of unified criteria. The disease often affects various organs with high oxygen demand. Multiparametric approach and multidisciplinary team are recommended to increase diagnostic accuracy.

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DOI: 10.3897/bgcardio.30.e126760

Authors: Z. Shomanova, H. Welp, R. Schmitz, J.R. Sindermann

Abstract: The follow-up of pregnant patients with cardiomyopathies is a challenge that every cardiologist will be facing sooner or later. We present the first case of a patient with restrictive cardiomyopathy and Alpha-B-crystallinopathy (distal myopathy type), who was followed up during two pregnancies. Cardiac follow-ups were carried out by short-term clinical status monitoring, echocardiography, ECG monitoring and NT-proBNP. At the same time, foetal ultrasound scans should be performed. Follow-up requires constant communication between the cardiologist and the gynaecologist. Any decision concerning the mother or the foetus should be made by consensus between both teams. Despite good follow-up, pregnancy in such complex patients remains a high risk due to heart. Heart failure and neuromuscular symptoms can worsen during or after pregnancy.

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DOI: 10.3897/bgcardio.30.e127156

Authors: P. Angelova, N. Stoyanov, V. Velchev, S. Atemin, M. Sleptsova, T. Todorov, D. Gencheva, M. Gospodinova, D. Pechilkov, A Dasheva, T. Tchamova, A. Taneva, I. Tournev, V. Mitev, A. Todorova

Abstract: Introduction: Cardiomyopathies are a clinically and genetically heterogeneous group of diseases, that are associated with significant morbidity and mortality. The aim of the present study is to clarify the molecular-genetic characteristics of cardiomyopathies in patients in Bulgaria. Material and methods: In the present study, targeted analysis of an expanded panel of 242 genes, associated with cardiomyopathy, and an additional panel of 20 genes, associated with hereditary amyloidosis, was performed in a total of 20 Bulgarian patients, diagnosed with cardiomyopathy, as follows: 12 patients with hypertrophic cardiomyopathy (HCM), including 1 pediatric patient, 6 patients with dilated cardiomyopathy (DCM), of whom 2 pediatric patients, and 2 patients with restrictive cardiomyopathy (RCM). Family segregation analyses were performed by direct Sanger sequencing. Results: Genetic findings were present in 90% of the patients. Pathogenic/likely pathogenic variants were found in 12 of the patients (60%), while genetic findings related to the clinical symptoms were not detected in the RCM patients (10%). Approximately 1/3 of the patients had a family history of sudden cardiac death or cardiomyopathy. Pathogenic/likely pathogenic variants were found in 55% of HCM patients with no family history, in ~67% of HCM patients with family history or with sporadic DCM, and in 100% of DCM patients with a positive family history. A respectively 2,5 and 4-fold higher frequency of truncating variants was found in the study group compared to the reports of around 10% in the literature, both in patients with HCM, and in patients with DCM. Pathogenic/likely pathogenic variants in the MYBPC3 gene (71%) were found with the highest frequency in HCM, while DCM is characterized by a diverse genetic profile, and genetic findings in the NDUFB11 and TAZ genes were associated with severe clinical presentation in pediatric patients in the first postnatal days. Results of segregation analyses were reported in 6 of the affected families. Conclusion: The data from the present study supports the importance of conducted genetic testing and medical-genetic counseling in patients and affected families with cardiomyopathy in Bulgaria

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DOI: 10.3897/bgcardio.30.e127340

Authors: S. Stefanov, N. Stoyanov, N. Koleva, D. Mikova, A. Yordanov, Z. Pavlova, E. Kinova, I. Petrova, L. Demirevska, T. Todorov, T. Chamova, M. Garcheva, A. Kundurdjiev, A. Todorova, I. Tournev, Y. Palashev, I. Daskalov, M. Dimova, I. Gruev, Y. Yotov, A. Goudev, V. Velchev, Mariana Gospodinova

Abstract: Introduction: Transthyretin amyloid cardiomyopathy (ATTR-CM) is a severe progressive disease, more common than previously expected. The main objectives were to evaluate hereditary and wild type ATTR-CM frequency and clinical manifestations. Material and methods: One hundred seventy-eight patients at mean age 68 ± 13 years (129 males), referred by cardiologists with suspected cardiac amyloidosis (left ventricular wall thickness ≥ 12 mm and red flags) were evaluated. Pyrophosphate scintigraphy (99mTc-PYP) grade 2-3 myocardial uptake in the absence of monoclonal protein confirmed the diagnosis. Genetic test differentiated variant from wild type ATTR-CM. Endomyocardial biopsy with amyloid typing was performed in two patients. Results: Cardiac amyloidosis was diagnosed in 69 patients: light chain in 24 (13%) and ATTR-CM in 45 patients (25%). Wild type (ATTRwt) was found in 21 (12%) male patients at mean age 81 ± 6 years and variant type (ATTRv) in 24 patients (13%) at mean age 58 ± 7 years (15 males). In all ATTRv-CM patients some degree of peripheral polyneuropathy was found. The most common clinical red flags at diagnosis in both ATTRv and ATTRwt were heart failure in all patients, atrial fibrillation (16; 36%), pacemaker (5; 11%), and carpal tunnel syndrome (8; 18%). Severe aortic stenosis (4; 19%), spinal stenosis (2;10%), biceps tendon rupture (1; 5%) were characteristic for the wild type. At follow up, four patients needed a pacemaker for high degree AV block, six patients developed new onset atrial fibrillation, seventeen patients needed either initiation or an increase of the dose of loop diuretic, another 7 patients were hospitalized for heart failure exacerbation. Eight patients (18%) died, 7 (33%) with ATTRwt-CM and 1 (4%) with ATTRv-CM, all with advanced heart failure, 4 with concomitant severe aortic stenosis. Conclusions: This study provides the first nationwide estimates of the frequency and the clinical manifestations of ATTR-CM. Confirming the severity of the disease and poor prognosis, a low threshold for screening is needed for early diagnosis and timely treatment.

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DOI: 10.3897/bgcardio.30.e128810

Authors: T. Boneva, V. Groudeva, K. Karamfiloff

Abstract: Myocardial interstitial fibrosis (MIF) occurs in several ischemic and non-ischemic cardiomyopathies and is associated with left ventricular dysfunction and progression to heart failure (HF). MIF exists in two types – reparative and reactive with different origin and molecular mechanisms. Although MIF arises mainly because of alterations in collagen turnover leading to collagen fiber accumulation, there are also qualitative changes in collagen fibers in cardiac diseases. There are several studies for cellular and molecular mechanisms and his role in cardiac function, biomarkers and potential target therapies.

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DOI: 10.3897/bgcardio.30.e128352

Authors: V. Groudeva, А. Partenova

Abstract: Hypertrophic cardiomyopathy (HCM) is one of the most common form of cardiomyopathies. It is characterized by primary hypertrophy, disorganization, and fibrosis of the myocardium. These morphological characteristics determine the applicability and significance of magnetic resonance imaging in the evaluation of these patients. Cardiac magnetic resonance helps the diagnostic process by identifying hypertrophic segments, which are less accessible by echocardiography ensures more precise measurements of wall thickness and differentiates HCM from other causes of left ventricular hypertrophy. The method allows for not only qualitative but also quantitive evaluation of focal and diffuse fibrosis which plays an important role in evaluation of patients’ prognosis and follow up. The aim of this article is to review the role of cardiac magnetic resonance in the diagnosis of HCM, demonstrating different forms and their typical morphological features, as well as to demonstrate the role of the method in the differential diagnosis of myocardial hypertrophy.

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DOI: 10.3897/bgcardio.30.e127290

Authors: N. Koleva, Y. Yotov, K. Dzhinsov, I. Gruev, M. Gospodinova

Abstract: The 2023 European Society of Cardiology guidelines for the management of cardiomyopathies (CM) provide practical recommendations for diagnosis and treatment. They emphasize on the need for a systematic clinical evaluation beginning with clinical suspicion of CM and using a multiparametric approach that leads to classification of CM into one of five distinct phenotypes. А cardiomyopathy mindset, together with a multidisciplinary approach are crucial. Further investigations, including advanced imaging and genetic testing, are needed to make a phenotype-based etiological diagnosis. Detection of pathogenic genetic variants allows risk stratification of sudden cardiac death, where new recommendations are available regarding primary prevention. Therapeutic options are expanded with the approval of a new class of drugs for the treatment of symptomatic hypertrophic cardiomyopathy with left ventricular outflow tract obstruction. Specific exercise recommendations are given and reproductive issues in patients with CM are addressed. The lack of reimbursement by the National Health Insurance Fund (NHIF) of genetic testing and the severely limited reimbursement of some imaging methods are a significant problem in Bulgaria, which leads to difficulties in implementing the new guidelines.

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DOI: 10.3897/bgcardio.30.e134498

Authors: Mariana Gospodinova, Kameliya Genova

Abstract:

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DOI: 10.3897/bgcardio.30.e117188

Authors: N. Dragnev, V. Valkov, N. Kalvachev

Abstract: We are presenting a clinical case of a 53-year-old woman with a sudden decline of the general condition during a stressful moment, respiratory arrest requiring intubation and intensive care unit transfer. Due to the clinical evidence of acute left-sided heart failure, ECG and echocardiographic evidence of acute coronary syndrome, invasive coronary angiography with left ventriculography was performed, demonstrating a non-occlusive coronary artery disease with signifi cant LAD stenosis and left ventricular kinetics indicative of a stress-induced cardiomyopathy. In the course of the treatment, the patient regained consciousness with favourable dynamics in the ECG, echocardiographic and laboratory fi ndings. A follow-up coronary angiography and intervention of the signifi cant LAD stenosis at a later stage were discussed with the patient.

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DOI: 10.3897/bgcardio.29.e113063

Authors: M. Peneva, H. Baychev, I. Baltov, L. Abujamus, Ts. Doychinova, H. Nikolov, V. Baycheva, H. Mateev, E. Trendafilova

Abstract: Takotsubo cardiomyopathy or broken heart syndrome is a stress-induced cardiomyopathy with clinical, laboratory and ECG resemblance to acute coronary syndrome. Major differences stem from transient segmental wall motion abnormalities beyond the territory of a single coronary artery and the lack of obstructive coronary disease during angiography. Overall the condition is considered as a benign one with a favourable outcome, however there are reports of complicated clinical course by cardiogenic shock, apical thrombus, death and arrhythmias. The latter are reported to occur in 13% of the cases with ventricular arrhythmias (ventricular tachycardia, ventricular fi brillation, torsades de pointes) as a leading fi nding and in minor percentage – high-grade AV block. Despite advances in our knowledge and experience there are still no strict guidelines for the management of conduction disorders. We present the case of a 78-year old lady with an apical variant of Takotsubo, complicated by syncope, complete AV block, new left bundle branch block, paroxysmal atrial fi brillation, ventricular tachycardia and implantation of a permanent pacemaker. During the hospitalization she developed acute cardiac failure with echocardiographic evidence of left ventricular systolic dysfunction and an apical thrombus. The presented case demonstrates the complexity of the condition and the necessity of strict follow-up and timely therapy.

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DOI: 10.3897/bgcardio.29.e105075

Authors: S. Kanuri, P. J. Sirrkay

Abstract: Takutsubo, or stress cardiomyopathy (TCM) is one of the important cardiovascular disorders encountered during the COVID-19 pandemic. We performed a PubMed search of relevant articles and presented this review which included epidemiology, etiopathogenesis, diagnosis and treatment of this clinical disorder. Takutsubo is usually more common in women than men. COVID-19 infection or vaccination can incite severe emotional disorders such as anxiety and depression, which flames up impaired neural networks in the limbic system. This stirs up disorganized regulation of autonomic nervous system with predominance and excessive firing of sympathetic nervous system to the ventricular myocardium. Moreover, direct invasion and systemic effects of COVID-19 infection including hormonal influences, autoimmunity, cytokine storm and neighboring infections might also play a significant role in the manifestation of this disorder. It commonly presents signs and symptoms of left ventricular dysfunction. Although most cases are undergoing remission within a few weeks, complications such as LV outflow tract obstruction, thromboembolism and arrhythmias were also reported. Since clinical symptoms are non-specific, a high degree of clinical suspicion is warranted particularly with the co-existing COVID-19 infections. Clinicians often leaned upon battery of tests including ECG, echocardiography and CMR to rule out myocarditis and coronary artery disease. Supportive management including treatment of heart failure and any associated arrhythmias and thromboembolism. Recurrences are common, but the treatment of underlying psychiatric disorders, including relaxation techniques, is the key strategy to avoid future occurrences.

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DOI: 10.3897/bgcardio.28.e93473

Authors: B. Kunev, V. Vasilev, G. Vladimirov, V. Karabinov

Abstract: We present a clinical case of a 70-year-old woman who was urgently admitted to the Department of Neurology with threatening status epilepticus from simple motor seizures in the right half of the face and right hand. Computed tomography of the head visualized a calcified extra axial round formation in the left posterofrontal, possibly a meningioma. On admission and during the symptomatic attacks, dynamic ECG changes were recorded with negative T waves precordially, as well as a dynamic increase in serum troponin. Echocardiography revealed hypokinesia involving the middle segments of the left ventricle. Coronary angiography was performed with no evidence of coronary disease. From the ventriculography hypokinesia of middle segments was found and measured EF 47%. Complex therapy with valproate, dexamethasone, mannitol and clonazepam, levetiracetam, acetizal, a statin and a beta-blocker was initiated. After controlling the epileptic seizures and diagnostic work-up of the cardiovascular status, the patient was referred for neurosurgical treatment of the tumor formation.

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DOI: 10.3897/bgcardio.28.e97285

Authors: K. Genova, D. Kostova-Lefterova

Abstract: Cardiac magnetic resonance tomography is widely used method in the diagnosis of cardiovascular diseases. In the last decade, new techniques have been developed to obtain quantitative parameters of myocardial changes. T1 and T2 mapping are part of the routine CMRT protocol and allow direct quantification of T1 and T2 relaxation times in the myocardium as well as the calculation of extracellular volume. These are important biomarkers both for the diagnosis of various myocardial diseases and for monitoring treatment follow-up and determining prognosis. The purpose of this article is to provide a brief overview of the basic principles of the T1 and T2 mapping techniques, as well as their main applications in different types of cardiomyopathies.

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DOI: 10.3897/bgcardio.28.e97338

Authors: A. Partenova, D. Kostova-Lefterova, K. Genova

Abstract: In recent decades, cardiac magnetic resonance imaging (CMR) has been established as a valuable tool in the diagnosis of patients with or at risk of heart failure. With its ability to characterize tissue changes in the myocardium, CMR can provide detailed and clinically useful information about the type and severity of cardiac damage. The method is not only important for differentiation of ischemic from non-ischemic cardiomyopathy, but also contributes to the correct diagnosis of non-ischemic cardiomyopathy subtypes. It is also widely accepted as a reference standard for the quantification of myocardial mass, volumes and ejection fraction. The purpose of this article is to review the role of CMR in the diagnosis and treatment of non-ischemic cardiomyopathies.

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DOI: 10.3897/bgcardio.28.e97065

Authors: D. Kostova-Lefterova, A. Partenova

Abstract: Cardiac magnetic resonance tomography (CMRT) is a method of high diagnostic value in the assessment of cardiac vitality through the application of various perfusion techniques, as well as in the evaluation and diagnosis of early myocardial ischemic changes. The aim of this article is to review the different technical aspects of CMRT. A major problem in CMRT studies is the deterioration of image quality due to the presence of motion artefacts. This necessitates the development of ECG-gated and respiratory triggered or breath-holding techniques and their implementation in practice. In contrast to most other applications of MRT, the planes used in CMRT are defined with respect to the orientation of the heart so that they are parallel and orthogonal to the cardiac axes. Two main groups of sequences are used in CMRT: 1) to determine morphology, function, and blood flow and 2) to provide good tissue contrast of the heart. Technological development of the method has not stopped, and techniques and pulse sequences continue to be developed to improve the diagnostic capabilities of CMRT. Through proper planning, a thorough understanding of the cardiac planes, and appropriate selection of technical parameters for the respective sequences depending on the clinical finding being addressed, a successful performance of each CMRT study is achieved.

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DOI: 10.3897/bgcardio.28.e79582

Authors: H. Baychev, E. Kostadinova, A. Partenova, K. Genova, T. Shalganov

Abstract: Myocardial infarction with non-obstructive coronary arteries (MINOCA) is a syndrome of diverse etiology and unclear pathogenesis, with an incidence of 5-15% and varying prognosis. Cardiac magnetic resonance imaging (MRI) is playing an increasing role in the diagnosis of MINOCA and in distinguishing the causes that led to it, while being at the same time an important predictor of prognosis in these patients. We present a 40-year-old man with clinical, laboratory and instrumental data for acute coronary syndrome complicated by acute heart failure. The invasive assessment ruled out obstructive coronary heart disease as well as Takotsubo cardiomyopathy. MINOCA and myocarditis were discussed in the differential diagnostic plan. To differentiate them, cardiac MRI was performed, which confi rmed the diagnosis of „myocardial infarction with non-obstructive coronary arteries“.

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DOI: 10.3897/bgcardio.28.e80076

Authors: Zornitsa Vassileva, Anna Kaneva, Anna Dasheva, Kameliya Genova

Abstract: Cardiac involvement by COVID-19 in children occurs most often as a part of the multisystem infl ammatory syndrome by children (MIS-C) and rarely as an isolated fi nding; affected children are predominantly older males. We present retrospective analysis data of 10 children with myocardial involvement within COVID-19, who were admitted at the Pediatric Cardiology Department of the National Heart Hospital – Sofi a. The main clinical symptoms were fever, heart failure, and gastrointestinal complaints, and the typical laboratory constellation included pronounced leukocytosis with extreme granulocytosis, signifi cant elevation of infl ammatory markers, increased serum troponin levels, and serologic evidence of contact with SARS-CoV2. Chest X-ray showed cardiomegaly and pulmonary hypervolemia; ECG changes were diverse and included abnormal repolarization and rhythm and conduction disturbances. Echocardiography revealed left ventricular dilation with depressed contractility, and cardiac MRI demonstrated myocardial edema and necrosis. Following immunomodulatory treatment, rapid recovery was observed. However, in most cases, the MRI changes persisted 6 months after the onset of symptoms, which makes the long-term prognosis unclear. We have reviewed our results considering the recent publications in the international databases regarding cardiac involvement by COVID-19 in children.

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DOI: 10.3897/bgcardio.28.e81214

Authors: Kalina Ganeva, Petar Shivachev

Abstract: Thalassemias are a group of inherited disorders, that require regular lifelong blood transfusions, which are vital for the normal development and life of the patients. With time, complications occur due to iron deposition in internal organs with subsequent chronic hemochromatosis development and predominantly affecting the heart, the liver and the endocrine glands. Despite the absence of clinical symptoms in young patients, enough data are supporting the fact that early myocardial iron deposition occurs. This necessitates the introduction of suffi ciently reliable methods and techniques for the detection and follow-up of early heart disorders before the onset of symptoms. Evaluation of myocardial iron deposition by the T2* MRI technique is established as a reliable, non-invasive method with good reproducibility and a low percentage of variability. Echocardiography as a method does not give information concerning the iron deposition in the heart but is easily applicable, safe and widespread for screening and follow up of patients. The use of new technique such as longitudinal strain makes the method almost comparable for the assessment of early subclinical myocardial damage. Although still in the fi eld of research, the identifi cation of specifi c miRNAs associated with cardiac stroke, fi brosis and remodeling is emerging as a new, potential biomarker for myocardial damage that could support the overall cardiovascular assessment of patients. This review will focus on current manifestations of cardiac complications in young patients with beta-thalassemia major, as well as the noninvasive methods for assessing cardiac function described above.

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DOI: 10.3897/bgcardio.28.e81454

Authors: Petar Shivachev, Kalina Ganeva, Darina Krumova, Miglena Georgieva, Petar Petrov

Abstract: Anorexia nervosa is an eating disorder with a multisystemic nature, affecting female adolescents predominantly. The disease is potentially fatal with cardiac complications being the leading cause. We present a clinical case of a 16-year-old girl with anorexia nervosa after a mental trauma, hospitalized in our Clinic in an extremely severe, pre comatose state with weight 26 kg, BSA – 1.12 m2 and BMI – 10.4. On the background of severe abnormalities in homeostasis, bradycardia with left ventricular extrasystoles in bigeminy and episodes of tachycardia with a frequency of 238/min. Echocardiography revealed reduce in the heart size and the left ventricular muscle mass, MI – I + degree and a small, apical pericardial effusion. During the echocardiographic follow-up, there was signifi cant hypertrophy of the left ventricle with obstruction in the outfl ow tract, with a reduction in ventricular size, without signifi cant change in left ventricular muscle mass. As a result of long-term, comprehensive treatment, with the participation of a team of specialists, the patient achieved true behavioural criticality, improved food tolerance, weight gain up to 46 kg with BMI - 18.4 and normalization of cardiac anatomy. Cardiac complications of anorexia nervosa are common, varied, and potentially lethal and require targeted cardiac monitoring and treatment. Left ventricular hypertrophy with obstruction in the ventricular outfl ow tract, fi rst described in 2006, is a rare but potentially fatal cardiac complication in patients with the disease.

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DOI: 10.3897/bgcardio.27.e76255

Authors: Monika Shumkova, Kiril Karamfiloff, Raya Ivanova, Dobrin Vassilev

Abstract: Invasive hemodynamic assessment of cardiac diseases has become an important diagnostic tool in recent decades. Non-invasive methods are the main techniques used to assess cardiac function, due to their wider availability. Cardiac catheterization is useful when there are diagnostic problems that cannot be solved with routine methods. Cardiac catheterization should be individualized according to the specific problems of the patient and based on the results from non-invasive methods. Invasive diagnostics is used in the assessment of patients with various cardiovascular diseases, including cardiomyopathies. In this review, we consider the role of cardiac catheterization, its advantages and disadvantages as part of the overall assessment of patients with cardiomyopathies.

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DOI: 10.3897/bgcardio.27.e76672

Authors: Monika Shumkova, Kiril Karamfiloff, Raya Ivanova, Kristina Stoyanova, Dobrin Vassilev

Abstract: Cardiomyopathies are a heterogeneous group of diseases. The main pathogenetic mechanism is myocardial damage due to genetic mutations. Cardiomyopathies are one of the leading causes of heart failure, sudden cardiac death, and life-threatening arrhythmias. Certain factors associated with poor prognosis determined the prognosis in this group of patients. Survival in different types of cardiomyopathies depends on the time of diagnosis and initial treatment. The types of cardiomyopathies discussed in this review are hypertrophic cardiomyopathy, dilative cardiomyopathy, restrictive cardiomyopathy, left ventricle non-compaction, and arrhythmogenic right ventricular cardiomyopathy.

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DOI: 10.3897/bgcardio.27.e72412

Authors: Mohamed Dardari, Alexandrina Nastasa, Corneliu Iorgulescu, Stefan Bogdan, Vlad Bataila, Radu Vatasescu

Abstract: Objective. Radiofrequency catheter ablation is an effective treatment option for cardiac arrhythmias including complex and ventricular arrhythmias. Remote magnetic catheter navigation (RMN) has been developed as a novel way of approach aiming to improve outcome and reduce complication rate, and reduce radiation exposure for both operator and patient. Our aim was to compare success and complication rate in patients with or without severely reduced left ventricular ejection fraction (LVEF). Methods. We retrospectively analyzed all the patients (n = 98) which have undergone RMN in our center between 2015-2021. No selection criteria for RMN procedure have been applied. All clinical and paraclinical, as well as procedural data were collected. Patients were divided into two groups, with or without severely reduced LVEF ≤ 35%. CARTO system was used for 3D electroanatomic mapping. RMN was done using Niobe ES system and an open-irrigated magnetic ablation catheter. Success rate was defi ned by complete elimination of clinical arrhythmia. Non-inducibility following ablation was assessed in all patients presenting with any type of ventricular arrhythmia other than premature ventricular contractions. Testing for inducibility was done by ventricular programmed pacing with up to four extra-stimuli. The statistical analysis was performed using SPSS software. P-value < 0.05 was considered signifi cant. Results. Successful ablation with complete elimination of the clinical arrhythmia was achieved in 92.3% of the patients with severely reduced LVEF and in 88.1% of patients with LVEF > 35% (p = 0.73). Overall minor complication rate was 2.04% with spontaneous resolution. No major complications were reported. Non-inducibility was achieved in 56.4% of the patients with LVEF ≤ 35% and in 79.2% of the patients with LVEF >35% (p = 0.023). Conclusion. Radiofrequency catheter ablation using RMN is effective and safe regardless of the presence or not of a severely reduced LVEF.

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DOI: 10.3897/bgcardio.27.e67571

Authors: Damyan Boychev, Naidenka Zlatareva, Ivo Petrov

Abstract: The coronavirus disease 2019 (COVID-19) pandemic has affected health and economies around the globe at an unprecedented scale. Since the fi rst registered case of Covid-19 in December of 2019 until May 2021, more than 167 mil people have been infected and more than 3.5 mil have died. Patients with cardiovascular disease are one of the most affected groups. First, because cardiovascular disease, for example, stable angina or past myocardial infarction, weakens system’s abilities of dealing with stress due to infl ammation. Secondly, because COVID-19 is associated with multiple different mechanisms of cardiovascular injury. Developing COVID-19 related cardiovascular complications is associated with increased morbidity and mortality. The goal of this review is to present the known up to this moment mechanisms of cardiovascular injury and complications after COVID-19.

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DOI: 10.3897/bgcardio.26.e58048

Authors: Galina Zlatancheva, Dobrin Vassilev, Raya Ivanova, Marusya Genadieva

Abstract: Cardiac injury is a common condition among patients hospitalized with COVID-19, and it is associated with a higher risk of in-hospital mortality. Since the first data analyses in China, elevated cardiac troponin has been noted in a substantial proportion of patients, implicating myocardial injury as a possible pathogenic mechanism contributing to severe illness and mortality. Physicians need to pay heed to the possibility of myocarditis in cases of COVID-19. We present a first possible SARS-CoV-2 associated myocarditis case, confirmed by autopsy examination. The overlap with acute coronary syndrome, acute decompensated heart failure should be taken into consideration in the current state to COVID-19 epidemics.

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DOI: 10.3897/bgcardio.26.e52296

Authors: Nikolay Marinov, Iana Simova, Ivo Petrov

Abstract: Myocarditis is an inflammatory disease of the myocardium, with heterogeneous etiology.  Although rare, myocarditis can be a complication of the influenza infection. In the majority of cases, viral myocarditis is a self-limiting infection passing without permanent changes in the myocardium. We present a clinical case of a 30-year-old woman with acute heart failure in the course of respiratory infection accompanied by severe systolic dysfunction (left ventricular (LV) ejection fraction (EF) 16%). The patient was treated according to the current guideline recommendations for the treatment of acute and chronic heart failure with the conjunction of immunomodulating and metabolic therapy. During hospitalization from microbial and viral testing, acute-phase antibodies to parainfluenza virus were identified. Following the treatment, a complete resolution of heart failure symptoms and restoration of LVEF to baseline (40%) was observed. Timely initiation of treatment in myocarditis, followed by good clinical course and regression of LV systolic dysfunction, in some cases may cancel invasive procedures such as endomyocardial biopsy.

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DOI: 10.3897/bgcardio.26.e53407

Authors: Mariana Gospodinova, Elena Kinova, Iana Simova, Yoto Yotov, Marina Garcheva, Galina Kirova, Kamelia Genova, Albena Todorova, Stayko Sarafov, Ivailo Tournev, Mariya Tokmakova, Vasil Velchev

Abstract: Transthyretin cardiac amyloidosis is a restrictive cardiomyopathy ((ATTR-CM), caused by an extracellular deposition of insoluble amyloid fibrils in the myocardium. It is a life threatening disease with life expectancy of 2 to 6 years after diagnosis. There are two types – hereditary and wild type. Recent data reveal that the wild type ATTR-CM is a common cause of heart failure with preserved ejection fraction, especially in elderly men. Hereditary ATTR amyloidosis is not so rare in Bulgaria. Five different mutations have been diagnosed, the most common being p.Glu89Gln, identified in 62 unrelated families with 117 patients and 72 mutation carriers. ATTR-CM diagnosis is often delayed or even missed, however its early recognition has become very important as a new drug, which is a transthyretin stabilizer is now available and other drugs are under development. Updated knowledge about the clinical presentation, diagnostic algorithm, available and future therapeutic options for ATTR-CM are a prerequisite for an early identification, timely treatment and better prognosis of the affected patients. The diagnosis requires a multidisciplinary approach with the participation of experienced specialists, multimodality imaging, well equipped histopathological and genetic laboratories. Establishing centres of expertise could improve the management of the patients with ATTR-CM.

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