AIMS: Cardiac resynchronization therapy (CRT) is guideline recommended for the treatment of symptomatic heart failure (HF) with reduced left ventricular ejection fraction and prolonged QRS. However, patients with common comorbidities, such as persistent/permanent atrial fibrillation (AF), are often under-represented in clinical trials. METHODS: The Strategic Management to Optimize Response to Cardiac Resynchronization Therapy (SMART) registry (NCT03075215) was a global, multicentre, registry that enrolled de novo CRT implants, or upgrade from pacemaker or implantable cardioverter defibrillator to CRT-defibrillator (CRT-D), using a quadripolar left ventricular lead in real-world clinical practice. The primary endpoint was CRT response between baseline and 12 month follow-up defined as a clinical composite score (CCS) consisting of all-cause mortality, HF-associated hospitalization, New York Heart Association (NYHA) class and quality of life global assessment. RESULTS: The registry enrolled 2035 patients, of which 1558 had completed CCS outcomes at 12 months. The patient cohort was 33.0% female, mean age at enrolment was 67.5 ± 10.4 years and the mean left ventricular ejection fraction was 29.6 ± 7.9%. Notably, there was a high prevalence of mildly symptomatic patients (NYHA class I/II 51.3%), non-left bundle branch block (LBBB) morphology (38.0%), AF (37.2%) and diabetes mellitus (34.7%) at baseline. CCS at 12 months improved in 58.9% (n = 917) of patients; 20.1% (n = 313) of patients stabilized and 21.0% (n = 328) worsened. Several patient characteristics were associated with a lower likelihood of response to CRT including older age, ischaemic aetiology, renal dysfunction, AF, non-LBBB morphology and diabetes. Higher HF hospitalization (P < 0.001) and all-cause mortality (P < 0.001) were observed in patients with AF. These patients also had lower percentages of ventricular pacing than patients in sinus rhythm at baseline and follow-up (P < 0.001, both). A further association between AF and non-LBBB was observed with 81.4% of AF non-LBBB patients experiencing an HF hospitalization compared with 92.5% of non-AF LBBB patients (P < 0.001). Mortality between subgroups was also statistically significant (P = 0.019). CONCLUSIONS: This large, global registry enrolled a CRT-D population with higher incidence of comorbidities that have been historically underrepresented in clinical trials and provides new insight into factors influencing response to CRT. As defined by CCS, 58.9% of patients improved and 20.1% stabilized. Patients with AF had particularly worse clinical outcomes, higher HF hospitalization and mortality rates and lower percentages of ventricular pacing. High incidence of HF hospitalization in patients with AF and non-LBBB in this real-world cohort suggests that ablation may play an important role in increasing future CRT response rates.

• MeSH
• Global Health MeSH
• Ventricular Function, Left * physiology   MeSH
• Quality of Life * MeSH
• Middle Aged MeSH
• Humans MeSH
• Follow-Up Studies MeSH
• Registries * MeSH
• Aged MeSH
• Cardiac Resynchronization Therapy * methods   MeSH
• Heart Failure * therapy   physiopathology   mortality   MeSH
• Stroke Volume * physiology   MeSH
• Treatment Outcome MeSH
• Check Tag
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Multicenter Study MeSH

Tato kazuistika zdůrazňuje význam multimodálního přístupu, personalizovaných léčebných intervencí a nepříznivou prognózu infarktu myokardu bez významného postižení koronárních tepen (myocardial infarction with non-obstructive coronary arteries, MINOCA) u mladého pacienta s antifosfolipidovým syndromem (antiphospholipid syndrome, APS). Na oddělení urgentního příjmu byl přivezen 39letý muž s diagnózou infarktu myokardu s elevacemi úseku ST (STEMI) ve třídě III Killipovy klasifikace. Angiografické vyšetření neprokázalo přítomnost obstrukčního postižení epikardu koronárních tepen, což si vyžádalo další vyšetření srdce magnetickou rezonancí (MR). Toto vyšetření odhalilo jizvy po infarktu myokardu na řadě míst na tepnách a těžkou dysfunkci levé komory. U pacienta došlo k rozvoji srdečního selhání se sníženou ejekční frakcí, a proto mu byl subkutánně implantován kardioverter-defibrilátor.

This case report highlights the importance of a multimodal approach, tailored therapeutic interven- tions, and the poor prognosis of myocardial infarction with non-obstructive coronary arteries (MINOCA) in a young patient with antiphospholipid syndrome (APS). A 39-year-old man was admitted with a diagnosis of a ST elevation myocardial infarction (STEMI), in Killip class III. Angiography showed absence of obstructive epicardial coronary artery disease prompting further evaluation with cardiac magnetic resonance imaging (MRI). It revealed infarction scars in multiple arterial territories and severe left ventricular dysfunction. The patient evolved with heart failure with reduced ejection fraction and a subcutaneous implantable cardioverter-defibrillator was implanted.

Anderson-Fabry disease (AFD) is a rare genetic disease with X-linked transmission characterized by a defect in the enzyme alpha-galactosidase A, which impairs glycosphingolipid metabolism and leads to an excessive storage of globotriaosylceramide (Gb3) within lysosomes. AFD involves renal, cardiac, vascular, and nervous systems and is mainly observed in male patients with onset in childhood, although cardiac manifestation is often shown in adults. AFD cardiomyopathy is caused by the accumulation of Gb3 within myocytes first showed by left ventricular hypertrophy and diastolic dysfunction, leading to restrictive cardiomyopathy and systolic heart failure with biventricular involvement. The diagnosis of AFD cardiomyopathy may be insidious in the first stages and requires accurate differential diagnosis with other cardiomyopathies with hypertrophic phenotype. However, it is fundamental to promptly initiate specific therapies that have shown promising results, particularly for early treatment. A careful integration between clinical evaluation, genetic tests, and cardiac imaging is required to diagnose AFD with cardiac involvement. Basic and advanced echocardiography, cardiac magnetic resonance, and nuclear imaging may offer pivotal information for early diagnosis (Graphical Abstract), and the management of these patients is often limited to centres with high expertise in the field. This clinical consensus statement, developed by experts from the European Society of Cardiology (ESC) Working Group on Myocardial and Pericardial Diseases and the European Association of Cardiovascular Imaging of the ESC, aims to provide practical advice for all clinicians regarding the use of multimodality imaging to simplify the diagnostic evaluation, prognostic stratification, and management of cardiac involvement in AFD.

• MeSH
• Early Diagnosis MeSH
• Diagnosis, Differential MeSH
• Child MeSH
• Adult MeSH
• Echocardiography methods   standards   MeSH
• Fabry Disease * diagnostic imaging   therapy   MeSH
• Consensus MeSH
• Humans MeSH
• Multimodal Imaging * methods   standards   MeSH
• Check Tag
• Child MeSH
• Adult MeSH
• Humans MeSH
• Male MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Consensus Development Conference MeSH

BACKGROUND: Long-term outcomes following percutaneous coronary intervention (PCI) or coronary artery bypass grafting (CABG) might be changing because of improved techniques and better medical therapy. This final prespecified analysis of the Fractional Flow Reserve (FFR) versus Angiography for Multivessel Evaluation (FAME) 3 trial aimed to reassess their comparative effectiveness at 5 years. METHODS: FAME 3 was a multicentre, randomised trial comparing FFR-guided PCI using current-generation zotarolimus-eluting stents versus CABG in patients with three-vessel coronary artery disease not involving the left main coronary artery. 48 hospitals in Europe, USA and Canada, Australia, and Asia participated in the trial. Patients (aged ≥21 years with no cardiogenic shock, no recent ST segment elevation myocardial infarction, no severe left ventricular dysfunction, and no previous CABG) were randomly assigned to either PCI or CABG using a web-based system. At 1 year, FFR-guided PCI did not meet the prespecified threshold for non-inferiority for the outcome of death, stroke, myocardial infarction, or repeat revascularisation versus CABG. The primary endpoint for this intention-to-treat analysis was the 5-year incidence of the prespecified composite outcome of death, stroke, or myocardial infarction. The trial was registered at ClinicalTrials.gov, NCT02100722, and is completed; this is the final report. FINDINGS: Between Aug 25, 2014 and Nov 28, 2019, 757 of 1500 participants were assigned to PCI and 743 to CABG. 5-year follow-up was achieved in 724 (96%) patients assigned to PCI and 696 (94%) assigned to CABG. At 5 years, there was no significant difference in the composite of death, stroke, or myocardial infarction between the two groups, with 119 (16%) events in the PCI group and 101 (14%) in the CABG group (hazard ratio 1·16 [95% CI 0·89-1·52]; p=0·27). There were no differences in the rates of death (53 [7%] vs 51 [7%]; 0·99 [0·67-1·46]) or stroke (14 [2%] vs 21 [3%], 0·65 [0·33-1·28]), but myocardial infarction was higher in the PCI group than in the CABG group (60 [8%] vs 38 [5%], 1·57 [1·04-2·36]), as was repeat revascularisation (112 [16%] vs 55 [8%], 2·02 [1·46-2·79]). INTERPRETATION: At the 5-year follow-up, there was no significant difference in a composite outcome of death, stroke, or myocardial infarction after FFR-guided PCI versus CABG, although myocardial infarction and repeat revascularisation were higher with PCI. These results provide contemporary evidence to allow improved shared decision making between physicians and patients. FUNDING: Medtronic and Abbott Vascular.

• MeSH
• Fractional Flow Reserve, Myocardial * MeSH
• Myocardial Infarction epidemiology   MeSH
• Coronary Angiography MeSH
• Percutaneous Coronary Intervention * methods   MeSH
• Coronary Artery Bypass * methods   MeSH
• Middle Aged MeSH
• Humans MeSH
• Follow-Up Studies MeSH
• Coronary Artery Disease * surgery   mortality   MeSH
• Aged MeSH
• Sirolimus analogs & derivatives   administration & dosage   MeSH
• Drug-Eluting Stents MeSH
• Treatment Outcome MeSH
• Check Tag
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Multicenter Study MeSH
• Research Support, Non-U.S. Gov't MeSH
• Randomized Controlled Trial MeSH
• Comparative Study MeSH

Stresová kardiomyopatie je klinický syndrom, při kterém dochází k rozvoji myokardiální dysfunkce v reakci na stres. Častým spouštěčem bývá neurologické onemocnění, nejčastěji netraumatické subarachnoidální krvácení a ischemická CMP. Rozvoj stresové kardiomyopatie může být u těchto pacientů příčinou arteriální hypotenze, arytmií nebo akutního srdečního selhání. I přes reverzibilitu onemocnění je zvláště její sekundární forma nebezpečná pro riziko rozvoje závažných komplikací. Kauzální léčba se zaměřuje na eliminaci příčiny, další terapie je symptomatická, vedená echokardiografickým nálezem. Cílem tohoto přehledového článku je shrnutí dosavadních znalostí ohledně stresové kardiomyopatie se zaměřením na recentní postupy v diagnostice a terapii a zdůraznění jejich odlišností u pacientů s neurologickým onemocněním.

Stress cardiomyopathy stands for a clinical syndrome characterized by the onset of myocardial dysfunction caused by stressful event. A common trigger is neurological disease, most commonly non-traumatic subarachnoid hemorrhage and ischemic stroke. The development of stress cardiomyopathy may cause arterial hypotension, arrhythmias, or acute heart failure in these patients. Despite the reversibility of the disease, its secondary form is particularly dangerous because of the risk of developing serious complications. Causal treatment focuses on eliminating the cause; further therapy is symptomatic, guided by echocardiographic findings. The aim of this review article is to summarize the current knowledge regarding stress cardiomyopathy focusing on up- -to-date diagnostics and treatment and highlight their differences in patients with neurological disease.

• MeSH
• Acute Coronary Syndrome etiology   MeSH
• Stroke complications   MeSH
• Humans MeSH
• Risk Factors MeSH
• Subarachnoid Hemorrhage complications   MeSH
• Takotsubo Cardiomyopathy * diagnosis   etiology   complications   therapy   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Research Support, Non-U.S. Gov't MeSH
• Review MeSH

Fibroblast growth factor 21 (FGF21), a metabolic hormone with pleiotropic effects, is beneficial for various cardiac disorders. However, FGF21's role in heart failure with preserved ejection fraction (HFpEF) remains unclear. Here, we show that elevated circulating FGF21 levels are negatively associated with cardiac diastolic function in patients with HFpEF. Global or adipose FGF21 deficiency exacerbates cardiac diastolic dysfunction and damage in high-fat diet (HFD) plus N[w]-nitro-L-arginine methyl ester (L-NAME)-induced HFpEF mice, whereas these effects are notably reversed by FGF21 replenishment. Mechanistically, FGF21 enhances the production of adiponectin (APN), which in turn indirectly acts on cardiomyocytes, or FGF21 directly targets cardiomyocytes, to negatively regulate pyruvate dehydrogenase kinase 4 (PDK4) production by activating PI3K/AKT signals, then promoting mitochondrial bioenergetics. Additionally, APN deletion strikingly abrogates FGF21's protective effects against HFpEF, while genetic PDK4 inactivation markedly mitigates HFpEF in mice. Thus, FGF21 protects against HFpEF via fine-tuning the multiorgan crosstalk among the adipose, liver, and heart.

• MeSH
• Adiponectin * metabolism   genetics   MeSH
• Diet, High-Fat * adverse effects   MeSH
• Energy Metabolism * drug effects   MeSH
• Fibroblast Growth Factors * metabolism   genetics   MeSH
• Phosphatidylinositol 3-Kinases metabolism   MeSH
• Myocytes, Cardiac * metabolism   drug effects   MeSH
• Humans MeSH
• Mice, Inbred C57BL MeSH
• Mice, Knockout MeSH
• Mice MeSH
• Pyruvate Dehydrogenase Acetyl-Transferring Kinase * metabolism   genetics   MeSH
• Proto-Oncogene Proteins c-akt metabolism   MeSH
• Signal Transduction MeSH
• Mitochondria, Heart * metabolism   drug effects   MeSH
• Heart Failure * metabolism   prevention & control   genetics   MeSH
• Stroke Volume drug effects   MeSH
• Adipose Tissue metabolism   MeSH
• Animals MeSH
• Check Tag
• Humans MeSH
• Male MeSH
• Mice MeSH
• Animals MeSH
• Publication type
• Journal Article MeSH

BACKGROUND: Right ventricular dysfunction (RVD) is common in patients with heart failure with reduced ejection fraction, and it is associated with poor prognosis. However, no biomarker reflecting RVD is available for routine clinical use. METHODS: Proteomic analysis of myocardium from the left ventricle and right ventricle (RV) of patients with heart failure with reduced ejection fraction with (n=10) and without RVD (n=10) who underwent heart transplantation was performed. Concentrations of 2 ECM (extracellular matrix) proteins with the highest myocardial upregulation in RVD, FMOD (fibromodulin) and FBLN5 (fibulin-5), were assayed in the blood and tested in a separate cohort of patients with heart failure with reduced ejection fraction (n=232) to test for the association of the 2 proteins with RV function and long-term outcomes. RESULTS: Multivariable linear regression revealed that plasma concentrations of both FMOD and FBLN5 were significantly associated with RV function regardless of the RV function assessment method. No association of FMOD or FBLN5 with left ventricular dysfunction, cardiac index, body mass index, diabetes status, or kidney function was found. Plasma levels of FMOD and FBLN5 were significantly associated with patient outcomes (P=0.005; P=0.004). Area under the curve analysis showed that the addition of FBLN5 or FMOD to RV function assessment had a significantly higher area under the curve after 4 years of follow-up (0.653 and 0.631, respectively) compared with RV function alone (0.570; P<0.05 for both). Similarly, the combination of MAGGIC (Meta-Analysis Global Group in Chronic Heart Failure) score, FBLN5, and FMOD had a significantly larger area under the curve (0.669) than the combination of MAGGIC score+RVD grade (0.572; P=0.02). The Kaplan-Meier analysis demonstrated that patients with the elevation of both FMOD and FBLN5 (ie, FMOD >64 ng/mL and FMOD >27 ng/mL) had a worse prognosis than those with the elevation of either FBLN5 or FMOD (P=0.03) demonstrating the additive prognostic value of both proteins. CONCLUSIONS: Our study proposes that circulating levels of FMOD and FBLN5 may serve as new biomarkers of RVD in patients with heart failure with reduced ejection fraction.

• MeSH
• Biomarkers * blood   MeSH
• Extracellular Matrix Proteins blood   metabolism   MeSH
• Fibromodulin * MeSH
• Ventricular Function, Left physiology   MeSH
• Ventricular Function, Right physiology   MeSH
• Middle Aged MeSH
• Humans MeSH
• Prognosis MeSH
• Calcium-Binding Proteins blood   metabolism   MeSH
• Proteomics * methods   MeSH
• Aged MeSH
• Heart Ventricles physiopathology   metabolism   MeSH
• Heart Failure * physiopathology   metabolism   blood   MeSH
• Stroke Volume * physiology   MeSH
• Heart Transplantation MeSH
• Check Tag
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH

Hypertrophic cardiomyopathy (HCM) caused by autosomal-dominant mutations in genes coding for structural sarcomeric proteins, is the most common inherited heart disease. HCM is associated with myocardial hypertrophy, fibrosis and ventricular dysfunction. Hypoxia-inducible transcription factor-1α (Hif-1α) is the central master regulators of cellular hypoxia response and associated with HCM. Yet its exact role remains to be elucidated. Therefore, the effect of a cardiomyocyte-specific Hif-1a knockout (cHif1aKO) was studied in an established α-MHC719/+ HCM mouse model that exhibits the classical features of human HCM. The results show that Hif-1α protein and HIF targets were upregulated in left ventricular tissue of α-MHC719/+ mice. Cardiomyocyte-specific abolishment of Hif-1a blunted the disease phenotype, as evidenced by decreased left ventricular wall thickness, reduced myocardial fibrosis, disordered SRX/DRX state and ROS production. cHif1aKO induced normalization of pro-hypertrophic and pro-fibrotic left ventricular remodeling signaling evidenced on whole transcriptome and proteomics analysis in α-MHC719/+ mice. Proteomics of serum samples from patients with early onset HCM revealed significant modulation of HIF. These results demonstrate that HIF signaling is involved in mouse and human HCM pathogenesis. Cardiomyocyte-specific knockout of Hif-1a attenuates disease phenotype in the mouse model. Targeting Hif-1α might serve as a therapeutic option to mitigate HCM disease progression.

• MeSH
• Hypoxia-Inducible Factor 1, alpha Subunit * metabolism   genetics   MeSH
• Fibrosis MeSH
• Cardiomyopathy, Hypertrophic * metabolism   genetics   pathology   MeSH
• Myocytes, Cardiac * metabolism   pathology   MeSH
• Humans MeSH
• Disease Models, Animal * MeSH
• Mice, Knockout * MeSH
• Mice MeSH
• Sarcomeres * metabolism   MeSH
• Signal Transduction MeSH
• Animals MeSH
• Check Tag
• Humans MeSH
• Male MeSH
• Mice MeSH
• Animals MeSH
• Publication type
• Journal Article MeSH
• Research Support, Non-U.S. Gov't MeSH

BACKGROUND: Growth differentiation factor (GDF)-15 is a pleiotropic cytokine that is associated with appetite-suppressing effects and weight loss in patients with malignancy. OBJECTIVES: This study aims to investigate the relationships between GDF-15 levels, anorexia, cachexia, and clinical outcomes in patients with advanced heart failure with reduced ejection fraction (HFrEF). METHODS: In this observational, retrospective analysis, a total of 344 patients with advanced HFrEF (age 58 ± 10 years, 85% male, 67% NYHA functional class III), underwent clinical and echocardiographic examination, body composition evaluation by skinfolds and dual-energy x-ray absorptiometry, circulating metabolite assessment, Minnesota Living with Heart Failure Questionnaire, and right heart catheterization. RESULTS: The median GDF-15 level was 1,503 ng/L (Q1-Q3: 955-2,332 ng/L) (reference range: <1,200 ng/L). Higher GDF-15 levels were associated with more prevalent anorexia and cachexia. Patients with higher GDF-15 had increased circulating free fatty acids and beta-hydroxybutyrate, lower albumin, cholesterol, and insulin/glucagon ratio, consistent with a catabolic state. Patients with higher GDF-15 had worse congestion and more severe right ventricular dysfunction. In multivariable Cox analysis, elevated GDF-15 was independently associated with risk of the combined endpoint of death, urgent transplantation, or left ventricular assist device implantation, even after adjusting for coexisting anorexia and cachexia (T3 vs T1 HR: 2.31 [95% CI: 1.47-3.66]; P < 0.001). CONCLUSIONS: In patients with advanced HFrEF, elevated circulating GDF-15 levels are associated with a higher prevalence of anorexia and cachexia, right ventricular dysfunction, and congestion, as well as an independently increased risk of adverse events. Further studies are warranted to determine whether therapies altering GDF-15 signaling pathways can affect metabolic status and clinical outcomes in advanced HFrEF.

• MeSH
• Weight Loss * MeSH
• Cachexia * etiology   MeSH
• Middle Aged MeSH
• Humans MeSH
• Anorexia * etiology   MeSH
• Retrospective Studies MeSH
• Growth Differentiation Factor 15 * blood   MeSH
• Aged MeSH
• Heart Failure * complications   physiopathology   blood   MeSH
• Stroke Volume physiology   MeSH
• Check Tag
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Observational Study MeSH

Izolovaná srdeční sarkoidóza je vzácné onemocnění, které je často obtížně detekovatelné vzhledem k nízké senzitivitě endomyokardiální biopsie (EMB), a proto obvykle vyžaduje multimodální vyšetření pomocí magnetické rezonance srdce (CMR) a/nebo pozitronové emisní tomografie – výpočetní tomografie (PET-CT) s aplikací 18F fl uordeoxyglukózy (FDG). Klinická manifestace onemocnění zahrnuje pokročilou atrioventrikulární blokádu, maligní komorové arytmie a městnavé srdeční selhání. Cílem práce je ukázat úskalí při diagnostice klinicky izolované sarkoidózy srdce u mladého muže po resuscitaci pro maligní komorovou arytmii. V kontextu tohoto případu poskytneme přehled současných znalostí managementu srdeční sarkoidózy. Popis případu: Osmadvacetiletý muž bez významnějších přidružených onemocnění byl přijat na naše pracoviště po krátké resuscitaci pro mimonemocniční zástavu oběhu při fi brilaci komor v únoru 2022. Na vstupním EKG byl přítomen sinusový rytmus s kompletní atrioventrikulární blokádou a morfologií komplexu QRS charakteru blokády levého Tawarova raménka. Následně byla dokumentována těžká neischemická systolická dysfunkce nedilatované levé komory s ejekční frakcí 25 %. EMB ukázala pouze nespecifický nález. Pro přetrvávající atrioventrikulární blokádu III. stupně byl pacientovi implantován kardioverter-defibrilátor s možností biventrikulární stimulace. Magnetickou rezonanci srdce jsme neindikovali z bezpečnostních důvodů u pacienta dependentního na kardiostimulaci a také jsme očekávali horší kvalitu vyšetření při artefaktech z implantovaných elektrod. V březnu 2022 bylo provedeno PET-CT s aplikací FDG, nicméně nález na srdci byl nediagnostický vzhledem k nedostatečné supresi metabolismu cukrů. Byly však zastiženy tři avidní uzliny v mediastinu. Pro trvající podezření na srdeční sarkoidózu bylo provedeno kontrolní PET-CT v červenci 2022, kdy se již zdařila suprese metabolismu glukózy v myokardu. Byl dokumentován nepoměr mezi fokálně zvýšenou akumulací FDG v oblasti anteroapikální, septální a bazální a výpadkem perfuze, který je charakteristický pro pokročilejší stadia zánětlivých srdečních onemocnění. Současně byla zaznamenána progrese lymfadenopatie na obou stranách bránice. Bronchoskopie a transbronchiální biopsie přinesly negativní nález. Opakovaná EMB verifi kovala sarkoidózu myokardu. V době první manifestace onemocnění se tedy jednalo o klinicky izolovanou srdeční sarkoidózu. Na základě tohoto nálezu byla zahájena imunosupresivní léčba kortikoidy a azathioprinem, kterou pacient toleroval bez komplikací. Postupně došlo ke zlepšení ejekční frakce levé komory, která v září 2023 dosáhla 45 %. Pacient zůstává během sledování klinicky stabilní ve funkční třídě II, bez intervencí přístroje pro komorové tachykardie. Od března 2023 přestal být pacient dependentní na kardiostimulaci. Závěr: Na diagnózu srdeční sarkoidózy je třeba myslet u pacientů s neischemickou dysfunkcí levé komory, zvláště v přítomnosti aneurysmat levé nebo pravé komory. Podezření zvyšuje přítomnost pokročilejší atrio- ventrikulární blokády nebo výskyt maligních komorových arytmií. Diagnostika je zvláště obtížná u izolované srdeční sarkoidózy, kde je obvykle nutno aplikovat kombinaci zobrazovacích metod, včetně CMR a PET-CT, případně opakovat EMB k dosažení správné diagnózy. Ta je důležitá jak z prognostických, tak i terapeutických důvodů.

Isolated cardiac sarcoidosis is a rare disease with a challenging diagnostic process reflecting a low sensitivity of endomyocardial biopsy (EMB). Therefore, it often requires a multimodal imaging using cardiac magnetic resonance imaging (CMR) and/or positron emission tomography (PET-CT) with administration of 18Ffluordeoxyglucose (FDG). Its clinical manifestation includes advanced atrioventricular block, ventricular tachyar- rhythmias and congestive heart failure. We aimed to illustrate pitfalls in the diagnosis of clinically isolated cardiac sarcoidosis in a young male with an aborted cardiac arrest due to ventricular fibrillation. This case inspired us to provide an updated review of the management of cardiac sarcoidosis. Case description: A 28-year-old male without any comorbidities was admitted to our department after an aborted out-of-hospital cardiac arrest due to ventricular fibrillation in February 2022. His first electrocardiogram showed sinus rhythm and a complete atrioventricular block with left bundle branch block QRS morphology. Echocardiogram and coronary angiography revealed severe non-ischemic systolic dysfunction of the non-dilated left ventricle with an ejection fraction of 25%. Findings in EMB specimens were non-specific. A biventricular cardioverter-defibrillator was implanted due to a persistent complete atrioventricular block. Cardiac magnetic resonance imaging was not done from the safety reasons due to dependency of the subject on cardiac pacing and expected poor quality due to artefacts from the implanted electrodes. In March 2022, PET-CT with administration of FDG was performed. Unfortunately, the examination was not diagnostic due to an incomplete suppression of the metabolism of carbohydrates. However, three avid lymphatic nodes were detected in mediastinum. Repeated PET-CT was performed in July 2022 with successful suppression of the metabolism of carbohydrates. The examination revealed a mismatch between focally increased accumulation of FDG and absent perfusion in anteroapical, septal, and basal segments, which is a typical finding for an inflammatory cardiac disease. In addition, there was an obvious progression of lymphadenopathy both above and under the diaphragm. Subsequently, we performed a repeated EMB, which verified cardiac sarcoidosis. This suggested a diagnosis of isolated cardiac sarcoidosis at the time of disease manifestation. These results enabled to start immunosuppression with corticosteroids and azathioprine, which was tolerated without complications. A gradual improvement in left ventricular ejection fraction up to 45% was observed and documented in September 2023. The patient remains clinically stable in the functional class II, without interventions from the implanted cardioverter-defibrillator. Since March 2023, the patient is not dependent on cardiac pacing. Conclusion: The diagnosis of cardiac sarcoidosis should be considered in patients with non-ischemic dysfunction of the left ventricle, especially if accompanied with aneurysms of cardiac ventricles. The suspicion rises in the presence of atrioventricular blocks or occurrence of ventricular tachyarrhythmias. The difficult diagnosis of isolated cardiac sarcoidosis can be established in selected cases by multimodal imaging including CMR and PET-CT, or repeate

• MeSH
• Atrioventricular Block etiology   MeSH
• Azathioprine administration & dosage   MeSH
• Biopsy methods   MeSH
• Adult MeSH
• Ventricular Dysfunction, Left etiology   MeSH
• Humans MeSH
• Heart Diseases diagnosis   epidemiology   drug therapy   pathology   MeSH
• Positron Emission Tomography Computed Tomography methods   MeSH
• Prednisone administration & dosage   MeSH
• Sarcoidosis * diagnosis   epidemiology   drug therapy   pathology   MeSH
• Out-of-Hospital Cardiac Arrest * etiology   MeSH
• Check Tag
• Adult MeSH
• Humans MeSH
• Male MeSH
• Publication type
• Case Reports MeSH
• Research Support, Non-U.S. Gov't MeSH
• Review MeSH