Primary Heart Research Articles

Expanding the clinical and immunological phenotypes of PAX1-deficient SCID and CID patients

Paired box 1 (PAX1) deficiency has been reported in a small number of patients diagnosed with otofaciocervical syndrome type 2 (OFCS2). We described six new patients who demonstrated variable clinical penetrance. Reduced transcriptional activity of pathogenic variants confirmed partial or complete PAX1 deficiency. Thymic aplasia and hypoplasia were associated with impaired T cell immunity. Corrective treatment was required in 4/6 patients. Hematopoietic stem cell transplantation resulted in poor immune reconstitution with absent naïve T cells, contrasting with the superior recovery of T cell immunity after thymus transplantation. Normal ex vivo differentiation of PAX1-deficient CD34+ cells into mature T cells demonstrated the absence of a hematopoietic cell-intrinsic defect. New overlapping features with DiGeorge syndrome included primary hypoparathyroidism (n = 5) and congenital heart defects (n = 2), in line with PAX1 expression during early embryogenesis. Our results highlight new features of PAX1 deficiency, which are relevant to improving early diagnosis and identifying patients requiring corrective treatment.

Клиническое наблюдение пациентки с первичным гипопаратиреозом, неконтролируемой гипокальциемией и сердечной недостаточностью

Клиническое наблюдение пациентки с первичным гипопаратиреозом, неконтролируемой гипокальциемией и сердечной недостаточностью

Chronic inflammatory effects of in vivo irradiation of the murine heart on endothelial cells mimic mechanisms involved in atherosclerosis

PurposeRadiotherapy is a major pillar in the treatment of solid tumors including breast cancer. However, epidemiological studies have revealed an increase in cardiac diseases approximately a decade after exposure of the thorax to ionizing irradiation, which might be related to vascular inflammation. Therefore, chronic inflammatory effects were examined in primary heart and lung endothelial cells (ECs) of mice after local heart irradiation.MethodsLong-lasting effects on primary ECs of the heart and lung were studied 20–50 weeks after local irradiation of the heart of mice (8 and 16 Gy) in vivo by multiparameter flow cytometry using antibodies directed against cell surface markers related to proliferation, stemness, lipid metabolism, and inflammation, and compared to those induced by occlusion of the left anterior descending coronary artery.ResultsIn vivo irradiation of the complete heart caused long-lasting persistent upregulation of inflammatory (HCAM, ICAM‑1, VCAM-1), proliferation (CD105), and lipid (CD36) markers on primary heart ECs and an upregulation of ICAM‑1 and VCAM‑1 on primary ECs of the partially irradiated lung lobe. An artificially induced heart infarction induces similar effects with respect to inflammatory markers, albeit in a shorter time period.ConclusionThe long-lasting upregulation of prominent inflammatory markers on primary heart and lung ECs suggests that local heart irradiation induces chronic inflammation in the microvasculature of the heart and partially irradiated lung that leads to cardiac injury which might be related to altered lipid metabolism in the heart.

Prognostic Impact of Mitral Regurgitation Before and After Transcatheter Aortic Valve Replacement in Patients With Severe Low-Flow, Low-Gradient Aortic Stenosis.

Background There is little evidence about the prognostic role of mitral regurgitation (MR) in patients with low-flow, low-gradient aortic stenosis undergoing transcatheter aortic valve replacement (TAVR). The aim of this study was to assess the prevalence and outcome implications of MR severity in patients with low-flow, low-gradient aortic stenosis undergoing TAVR, and to evaluate whether MR improvement after TAVR could influence clinical outcome. Methods and Results This study included consecutive patients with low-flow, low-gradient aortic stenosis undergoing TAVR at 2 Italian high-volume centers. The study population was categorized according to the baseline MR severity and to the presence of MR improvement at discharge. The primary outcome was the composite of all-cause death and hospitalization for worsening heart failure up to 1 year. The study included 268 patients; 57 (21%) patients showed MR >2+. Patients with MR >2+ showed a lower 1-year survival free from the primary outcome (P<0.001), all-cause death (P<0.001), and heart failure hospitalization (P<0.001) compared with patients with MR ≤2+. At multivariable analysis, baseline MR >2+ was an independent predictor of the primary outcome (P<0.001). Among patients with baseline MR >2+, MR improvement was reported in 24 (44%) cases after TAVR. The persistence of MR was associated with a significantly reduced survival free from the primary outcome, all-cause death, and heart failure hospitalization up to 1 year. Conclusions In this study, the presence of moderately severe to severe MR in patients with low-flow, low-gradient aortic stenosis undergoing TAVR portends a worse clinical outcome at 1 year. TAVR may improve MR severity in nearly half of the patients, resulting in a potential outcome benefit after discharge.

A primary multiple pleomorphic rhabdomyosarcoma of the heart in an adult dog

BackgroundHeart tumors are rare in dogs. They can be benign or malignant. Clinical signs depend primarily on the location of the tumor and its effect on blood flow.Case presentationAn eleven-year-old crossbreed male dog lethargic and anorectic for previous 3 days was presented to the veterinary clinic. The focused ultrasound assessment with sonograms in trauma (FAST) revealed multiple tumors in the heart which were then confirmed in echocardiographic examination performed by a veterinary cardiologist. Due to the poor general condition and grave prognosis, the dog was humanely euthanized. The autopsy revealed numerous intracardiac tumors in all four heart chambers. No proliferative changes were found in other organs either in thoracic or abdominal cavity. Immunohistochemical examination was performed using formalin-fixed, paraffin-embedded tissue from heart masses. The antibodies against myoglobin, desmin, smooth muscle actin, vimentin, CD34, S100, and pan-cytokeratin (AE1/AE3) were used. Microscopically, the tumor was composed of fascicles of spindle-shaped cells with pale eosinophilic cytoplasm with round, oval, and focally elongated nuclei and one or two prominent nucleoli. The tumor cells showed strong diffuse cytoplasmic immunopositivity for myoglobin and vimentin and focal staining for desmin. Immunostainings for smooth muscle actin-SMA, CD34, pan-cytokeratin, S-100 protein were negative. The immunohistochemical staining pattern confirmed rhabdomyosarcoma.ConclusionsThis is the first description of the primary multiple heart rhabdomyosarcoma in a dog.

A review of stem cell therapy in ischemic heart disease, where are we now?

Background: Regenerative therapies to rejuvenate the heart have a significant appeal for researchers. Preliminary findings from pre-clinical studies suggest that bone marrow cells may have reparative and regenerative effects on heart muscles, creating a ripe area for research. Many generations of stem cells used in pre-clinical and early clinical studies have shown promising but variable results. Objective: The current review article discusses the dilemmas in applying stem cell therapy to cardiovascular diseases and possible strategies to make it feasible. Methods: The field of regenerative therapies continued to progress with second-generation cells, third-generation cells, combination cell therapy, and the use of cell products alone. Research showed promising positive results in multiple randomized phases 1, 2, and 3 clinical trials in addition to numerous meta-analyses. The gaps in knowledge included stem-cell sources, their delivery routes, dosing, types of cells, and the indicated cardiac conditions. Results: The results from the latest randomized clinical trials, namely the Dream-HF, showed improved left ventricle function, symptoms, and overall survival. Studied patient populations include post-myocardial infarction (MI), ischemic/non-ischemic cardiomyopathy, intractable/microvascular angina, and cardiac surgery for congenital and valvular heart disease. The phase 3 DREAM-HF trial did not meet the primary heart failure endpoint of reducing hospital admission. Still, it showed a clinically significant reduction in major adverse cardiovascular events (MACE), including recurrent MI and stroke, by 60%. A 60% reduction in cardiovascular mortality and a 79% reduction in cardiovascular mortality in patients with evidence of inflammation (high CRP). The latter finding suggests a more anti-inflammatory effect. This effect was much higher than that observed in the PARADIGM-HF trial, which showed a 20% relative risk reduction in cardiovascular mortality. By combining the results of the DREAM-HF trial with MSC-HF, ixCELL-DCM, CONCERT-HF, and REGENERATE-DCM, the potential for clinical applications of cell therapy is promising. Conclusion: There is a promising role for cell therapy in the management of cardiovascular diseases. Results of trials in the setting of heart failure are more encouraging in both ischemic and non-ischemic cardiomyopathy. This is in contrast with acute myocardial infarction, where the results have been variable. Amongst all the various cell types tested MSCs show the most significant promise for treating HF.

Recurrent episodes of Takotsubo cardiomyopathy in systemic sclerosis

Systemic sclerosis is an autoimmune disease characterized by fibrosis and small vessel vasculopathy, which affects various organ systems, such as the heart. Takotsubo cardiomyopathy is a transient cardiomyopathy in reaction to an emotional or physical trigger. There may be clinical and pathogenetic overlap between Takotsubo cardiomyopathy and primary systemic sclerosis heart disease, and some patients with systemic sclerosis have been diagnosed with recurrent Takotsubo cardiomyopathy. Our large systemic sclerosis clinical cohort was reviewed to identify cases diagnosed with Takotsubo cardiomyopathy. The clinical features, laboratory and imaging results were reviewed and evaluated to perform a comparison between cases. We identified five patients with systemic sclerosis, all female (age 68.6 ± 5.7 years), who were diagnosed with Takotsubo cardiomyopathy. Two of these patients had recurrent episodes: one case with a history of multiple episodes and the other with one recurrence. Typical features included repolarization abnormalities on the electrocardiogram and transient left ventricular dysfunction observed using echocardiography or cardiac magnetic resonance imaging. Our findings build upon previous reports and observations that systemic sclerosis may cause Takotsubo cardiomyopathy. To our knowledge, this is the largest case series of Takotsubo syndrome in patients with systemic sclerosis. This association may provide novel insights into the aetiopathogenesis of Takotsubo cardiomyopathy as part of primary systemic sclerosis heart involvement.

Abstract P3102: Sex Differences In Outcomes Among Heart Failure Hospitalizations

Background: Both men and women have a comparable overall lifetime chance of developing heart failure, but there are clear sex differences in the nature of this condition that are both significant and yet underexplored. In this study, we investigate gender differences in clinical characteristics and in-hospital outcomes of heart failure hospitalizations using a large national database. Methods: This study is a retrospective analysis of National Inpatient Sample data, 2016-2019. Hospitalizations ≥41 years of age, admitted for heart failure were included in the study and stratified by sex. The outcomes of the study were in-hospital mortality rate, prolonged hospital length of stay, mechanical ventilation, mechanical circulatory support, vasopressors use, and adverse disposition. Propensity score matching with conditional logistic regression analysis was done to compare outcomes between males and females. Results: A total of 4,704,684 primary heart failure hospitalizations were included for the analysis. Of these 2,447,784 (52.0%) were males and 2,256,899 (48.0%) were females. In-hospital mortality ( P &lt;0.001), mechanical ventilation ( P &lt;0.001), mechanical circulatory support ( P &lt;0.001), and vasopressors use ( P &lt;0.001) were significantly lower among females, while prolonged length of stay ( P &lt;0.001) and disposition other than home ( P &lt;0.001) were higher among females. Conditional logistics regression analysis showed that females had significantly lower odds for mortality (aOR, 0.92, 95% CI: 0.89-0.94), mechanical ventilation (aOR, 0.89, 95% CI: 0.86-0.92), mechanical circulatory support (aOR, 0.54, 95% CI: 0.50-0.58), vasopressor use (aOR, 0.70, 95% CI: 0.66-0.74), and significantly higher odds for prolonged length of stay (aOR, 1.05, 95% CI: 1.04-1.06) and disposition other than home (aOR, 1.32, 95% CI: 1.31-1.34) Conclusion: Results of our study show that there are significant differences in outcomes between males and females hospitalized for heart failure. Further research is required to delineate how sex affects several aspects of heart failure, including epidemiology, risk factors, pathogenesis, response to medication, and eventually outcomes.

Abstract P3156: Activated Fibroblast-Derived Exosomal Mir-216a-5p Promotes Endothelial Dysfunction By Targeting PGM5 In Diabetic Heart

Background: Diabetic patients are more susceptible to heart failure (HF). High blood glucose level in diabetic patients eventually triggers the body's inflammatory response and causes cardiac fibroblast activation, endothelial cell dysfunction (ECD), and, ultimately, HF. Previously, we have shown that activated fibroblast-mediated ECD leads to HF. However, the molecular mechanism of fibroblast-induced ECD in diabetics is not yet well defined. Therefore, we hypothesized that “myofibroblast in the diabetic heart secretes exosomes loaded with antiangiogenic/profibrotic factors, which impair EC function.” Methods: Exosomes were isolated from diabetic mice plasma and fibroblast conditioned media by ultracentrifugation and characterized by nanosight &amp; electron microscopy. We cultured mouse primary heart endothelial cells in EC growth media. ECs were treated with exosomes derived from fibroblasts (treated with either 25mM glucose, 500nM Angiotensin II (AngII) or both) for 48 hr. Mannitol (25mM) served as control. Pathway-based miRNA array was screened with exosome-derived miR, and targets were predicted bioinformatically for selected miR. Results: Glu-AngII cotreatment significantly activated fibroblasts as shown by qPCR (Col1α, and FN expression) and western blot (pSmad2/Smad2, p-p38/p38). Interestingly, exosomes derived from activated fibroblasts significantly induced ECD (eNOS, VEGF, CD31 genes, and proteins expression). We further checked the effect of diabetic mice plasma exosomes on ECs function. We found significantly impaired endothelial function, as shown by Matrigel tube formation and Boyden chamber migration assays. Micro RNA array and qPCR data showed that miR-216a-5p was highly upregulated in exosomes derived from FBs cotreated with Glu-AngII. Pathway-based analysis revealed that PGM5 could be a potential target in miR-216-induced ECD. Interestingly, miR-216a-5p inhibition significantly rescued diabetes-induced PGM5 inhibition and improved endothelial function Conclusions: Taken together, this study demonstrates that fibroblast in the diabetic heart releases miR-216a-5p through exosomes, which promote endothelial dysfunction via the miR-216a-5p/PGM5 axis.

Resveratrol prevents Ang II-induced cardiac hypertrophy by inhibition of NF-κB signaling

BackgroundPathological cardiac hypertrophy is a hallmark of various cardiovascular diseases (CVD) including chronic heart failure (HF) and an important target for the treatment of these diseases. Aberrant activation of Angiotensin II (Ang II)/AT1R signaling pathway is one of the main triggers of cardiac hypertrophy, which further gives rise to excessive inflammation that is mediated by the key transcription factor NF-κB. Resveratrol (REV) is a natural polyphenol with multiple anti-inflammatory and anti-oxidative effects, however the ability of REV in preventing Ang II-induced cardiac hypertrophy in combination with NF-κB signaling activation remains unclear. MethodsMurine models of cardiac hypertrophy was conducted via implantation of Ang II osmotic pumps. Primary neonatal rat cardiomyocyte and heart tissues were examined to determine the effect and underlying mechanism of REV in preventing Ang II-induced cardiac hypertrophy. ResultsAdministrations of REV significantly prevented Ang II-induced cardiac hypertrophy, as well as robustly attenuated Ang II-induced cardiac fibrosis, and cardiac dysfunction. Furthermore, REV not only directly prevented Ang II/AT1R signal transductions, but also prevented Ang II-induced expressions of pro-inflammatory cytokines and activation of NF-κB signaling pathway. ConclusionsOur study provides important new mechanistic insight into the cardioprotective effects of REV in preventing Ang II-induced cardiac hypertrophy via inhibiting adverse NF-κB signaling activation. Our findings further suggest the therapeutic potential of REV as a promising drug for the treatment of cardiac hypertrophy and heart failure.

Left Ventricular Assist Device explantation using a new double patch technique.

There are several surgical approaches for explantation of an LVAD after recovery of cardiac function. Thus, remaining ventricular assist device components may bear significant risks of infection or thrombosis. We hereby report our technique and two-center experience with explantation of LVADs using a new double patch technique. From March 2019-April 2021, 5 patients underwent LVAD explantation after myocardial recovery (HVAD, n = 2; HeartMate 3, n = 3). Mean patient age was 50.3 years (100% male) and mean time on LVAD was 23.1 ± 20.8 months. Primary heart failure aetiology was dilated cardiomyopathy (n = 4) and myocarditis (n = 1).LVAD explantation was performed using a median sternotomy and cardiopulmonary bypass. The LVAD was stopped, and the outflow graft clamped. The outflow graft was ligated and sutured close to the aortic anastomosis. The driveline was clipped and removed. Under induced fibrillation, the attachment of the LVAD was released from the apical cuff and the LVAD was removed. A round pericardial patch was fixed from the inner of the ventricle. This already seals the apex of the heart. An additional Goretex patch is continuously sutured epicardially over the suture ring. The 5 cases showed technically uncomplicated explantation of the LVADs. During the follow-up of mean 16.4 ± 16.9 months, survival of 100% was observed. There were no bleeding complications or thromboembolic events during follow-up. LVAD explantation with the double patch technique is feasible and safe. This technique allows discontinuation of anticoagulation. There was a 30-day survival of 100%. Further studies are needed to provide better evidence for LVAD explantation and long-term follow-up.

Association of Chronic Renal Insufficiency with Inhospital Outcomes in Primary Heart Failure Hospitalizations (Insights from the National Inpatient Sample 2004 to 2018)

Chronic kidney disease (CKD) is a major co-morbidity in patients with heart failure (HF). There are limited contemporary data characterizing the clinical profile, inhospital outcomes, and resource use in patients hospitalized for HF with co-morbid CKD. We utilized a nationally representative population to address the knowledge gap. We examined the National Inpatient Sample 2004 to 2018 database to study the co-morbid profile, in-hospital mortality, clinical resource utilization, healthcare cost, and length of stay (LOS) in primary adult HF hospitalizations stratified by presence versus absence of a diagnosis codes of CKD. There were a total of 16,050,301 adult hospitalizations with a primary HF diagnosis from January 1, 2004, to December 31, 2018. Of these, 428,175 (33.81%) had CKD; 1,110,778 (6.92%) had end-stage kidney disease (ESKD); and 9,511,348 (59.25%) had no diagnosis of CKD. Patients with hospitalizations for HF with ESKD were younger (mean age 65.4 years) compared with those without ESKD. In multivariable analysis, those with CKD had higher odds of inhospital mortality (2.82% vs 3.57%, adjusted odds ratio [aOR] 1.30, confidence interval [CI] 1.28 to 1.26, p<0.001), cardiogenic shock (1.01% vs 1.79% aOR 2.00, CI 1.95 to 2.05, p<0.001), and the need for mechanical circulatory support (0.4% vs 0.5%, aOR 1.51, 1.44 to 1.57, p<0.001) compared with those without CKD. In multivariable analysis, those with ESKD had higher odds of inhospital mortality (2.82% vs 3.84%, aOR 2.07, CI 2.01 to 2.12, p<0.001), need for invasive mechanical ventilation use (2.04% vs 3.94%, aOR 1.79, CI 1.75 to 1.84, p<0.001), cardiac arrest (0.72% vs 1.54%, aOR 2.09, CI 2.00 to 2.17, p<0.001), longer LOS (Adjusted mean difference 1.48, 1.44 to 1.53, p<0.001) and higher inflation-adjusted cost (Adjusted mean difference 3,411.63, CI 3,238.35 to 3,584.91, p<0.001) compared with those without CKD. CKD and ESKD affected about 40.7% of all primary HF hospitalizations from 2004 to 2018. The inhospital mortality, clinical complications, LOS, and inflation-adjusted cost were higher in hospitalized patients with ESKD compared with patients with and without CKD. In addition, compared with those without CKD, hospitalized patients with CKD had higher inhospital mortality, clinical complications, LOS, and inflation-adjusted cost compared with patients with no diagnosis of CKD.

Hypertrophic cardiomyopathy in the structure of infiltrative diseases in children

Introduction. In 2006, the American Heart Association identified two main groups of cardiomyopathies (CM) as primary and secondary, referring to the primary CM heart diseases of genetic, acquired or mixed etiology, and to the secondary — pathological involvement of the myocardium as a part of a systemic pathology.&#x0D; Aim: to determine the most common phenocopies of hypertrophic CM (HCM) in children, due to the accumulation of pathological substances in the myocardium and present their differences.&#x0D; Materials and methods. Instrumental diagnostic methods (echocardiography, electrocardiography, 24-hour Holter ECG monitoring), laboratory tests (N-terminal propeptide of natriuretic hormone, creatine phosphokinase, creatine phosphokinase-MB, lactate dehydrogenase, aspartate aminotransferase, alanine aminotransferase, lactate, ammonia), and molecular genetic tests were used. &#x0D; Results. Nucleotide variants in non-sarcomeric genes causing myocardial hypertrophy were identified in one hundred four (39%) patients: infiltrative diseases with heart damage were diagnosed in 46 cases, syndromes from the RAS-pathy group were diagnosed in 58 cases. Patients with storage diseases included 12 children with Pompe disease, 2 cases with PRKAG2 syndrome, 11 cases had Danon disease, 15 — Corey–Forbes disease, and 6 — Friedreich ataxia. Adverse events were reported in group of patients with Pompe disease (9 deaths), and with Danon’s disease (2 deaths). &#x0D; Conclusion. The phenocopy varieties of HCM in children are represented by a wide variety of genetic variants and often by diseases from the group of glycogen metabolism disorders, fatty acid oxidation disorders, and mitochondrial diseases. Identification of the genetic causes of ventricular myocardial hypertrophy in children is the key to early diagnosis of rare diseases, timely and adequate treatment, as well as predicting the course and outcome of the disease.

Is myocardial fibrosis in patients with non-ischemic dilated cardiomyopathy associated with the whole-heart myocardial mechanics?

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Non-ischemic dilated cardiomyopathy (NIDCM) is a severe form of primary myocardial disease related with progressive heart failure (HF) and a poor prognosis. In patients with NIDCM, myocardial fibrosis (MF) is associated to adverse cardiovascular outcomes and should be evaluated quickly in each case of NIDCM [1,2]. Purpose To evaluate the relationships between the whole-heart myocardial strain parameters and presence of late gadolinium enhancement (LGE) in a prospective cohort of NIDCM patients. Methods The study group consisted of 101 patients with NIDCM who were referred for a cardiovascular magnetic resonance (CMR) and speckle tracking echocardiography examination. We analyzed MF by LGE, and the whole-heart (both ventricles and atria) myocardial mechanics were evaluated by speckle tracking. All patients underwent a comprehensive clinical evaluation, laboratory test assessment, electrocardiogram, Holter monitoring. The exclusion criteria were ischemic coronary disease, primary valvular heart disease, chronic kidney disease (eGFR &amp;lt;30 ml/min/1.73 m2), under the age of 18, a poor echocardiographic or CMR image quality, inflammatory myocardial disease, previous pulmonary embolism, peripartum cardiomyopathy and toxic damage. Results The study group was divided into two groups according to the presence of MF fibrosis. The presence of MF was associated to worse of the whole-heart myocardial strain parameters. Both ventricles or atria were more dilated in the group with MF. The strongest relationships were detected between presence of MF and mechanical strain parameters of the left side of the heart (left ventricle ejection fraction (LVEF) (r=0.662, 25 p&amp;lt;0.001), left ventricle global circumferential strain (LV GCS) (r=-0.609, p&amp;lt;0.001), left ventricle global longitudinal strain (LV GLS) (r=-0.586, p&amp;lt;0.001), left atrial reservoir strain (LASr) (r=0.588, p&amp;lt;0.001). Logistic regression analysis revealed that the last three mentioned strain parameters were independently associated with presence of MF. Conclusions In patients with NIDCM, the whole-heart myocardial mechanics are worse in patients‘ presence of MF. LV GLS, LV GCS, and LARs are the best 2D echocardiographic mechanical strain parameters to prognosticate the presence of MF.

Serum markers in systemic sclerosis with cardiac involvement.

Cardiac involvement in systemic sclerosis is a common clinical entity that may range from subclinical to life-threatening complications. The classification of cardiac involvement may be expressed as either primary or secondary involvement. Primary systemic sclerosis heart involvement (SSc-pHI) refers to cardiac pathologies primarily ascribed to systemic sclerosis rather than concomitant conditions like ischemic heart disease and pulmonary hypertension. The timely recognition of cardiac involvement holds significant clinical relevance. Therefore, numerous screening or diagnostic tools have been evaluated to forecast the likelihood of cardiac involvement, particularly in the absence of clinically evident cardiac symptoms. Of these modalities, serum biomarkers are often preferred due to their expeditiousness and non-invasive nature. Hence, the crucial goal of this narrative review is to review serum biomarkers that can be a valuable or promising tool in diagnosing cardiac involvement, especially SSc-pHI, in the early stages or predicting disease prognosis.

Prognostic impact of precipitated cardiac decompensation in symptomatic heart failure with reduced ejection fraction and severe secondary mitral regurgitation

Our aim was to assess the distribution of primary (with no trigger) and secondary (with a decompensation trigger) heart failure events in a severe heart failure population and their association with 2-year all-cause mortality in the Mitra.Fr study. We included 304 patients with symptomatic heart failure, and severe mitral regurgitation and guideline directed medical therapy randomized to medical therapy alone or medical therapy with percutaneous mitral valve repair. According to the follow-up, we defined 3 categories of events: follow-up without any heart failure event, at least 1 decompensation starting with a primary heart failure decompensation or starting with a precipitated secondary heart failure event. The primary outcome was 2-years all-cause mortality. A total of 179 patients (59 %) had at least 1 heart failure decompensation within 24-months of follow-up. 129 heart failure decompensations (72%) were a first primary heart failure and 50 (28%) were a first secondary decompensation. Finally, 30 patients had both types of decompensations but these were not taken into account for the comparison of primary and secondary decompensations. Primary decompensations were 3-times more frequent than secondary decompensations, but the mean number of heart failure decompensations was similar in the "Primary heart failure group" compared to the "Secondary heart failure group": (1.94 ± 1.39 vs 1.80 ± 1.07 respectively; P=.480). Compared to patients without heart failure decompensation, patients with "Only primary decompensation" or with "Only secondary decompensation" had a significantly increased risk of death (HR=4.87, 95% CI [2.86, 8.32] and 2.68 95%CI [1.64, 4.37] respectively). All-cause mortality, was not significantly different between these 2 type of decompensations (HR=1.82, 95% CI [0.93, 3.58]; P=.082), but each additional heart failure recurrence was associated with a significant increase in mortality risk (HR = 1.27, 95% CI [1.08; 1.50]; P=.005). In heart failure with reduced ejection fraction and severe secondary mitral regurgitation patients, primary heart failure decompensations were 3-times more frequent compared to precipitated decompensations with a nonsignificant trend in increased risk of all-cause mortality. Our results fail to support the differentiation between primary and secondary decompensations as they seem to portend the same outcome impact.

КРУПНАЯ ФИБРОМА ЛЕВОГО ЖЕЛУДОЧКА СЕРДЦА У РЕБЕНКА

Introduction. Fibroma is considered as benign solitary tumor, which is the second most common among primary heart tumors in children. It rarely starts to regresses, contributes to the formation of hemodynamic disorders and congestive heart failure, as well as life-threatening arrhythmias, and also can lead to the development of sudden death. Aim. Informing physicians about a rare cardiac fibroma in children on the example of a clinical case. Material and methods. Describing a clinical case of patient K., 7 months old, who was referred to the cardiac surgery department for a planned operation to remove a left ventricular mass, which was diagnosed prenatally at 32 weeks. Results and discussion. A dynamic observation was carried out, during which, according to echocardiography and magnetic resonance imaging, an increase in the tumor from 35x25 mm to 70x35 mm and the formation of mitral stenosis were noted. The average pressure gradient in the mitral valve increased from 2 to 11 mm Hg there was a limitation of the mobility of its valves. A partial resection of the tumor was performed. Morphological and immunohistochemical studies confirmed the diagnosis of fibroma. The postoperative period proceeded well. Conclusion. Cardiac fibromas in children, regardless to the clinical course of the disease, require long-term monitoring of patients with an electrocardiological study, echocardiography and magnetic resonance imaging method, and if there is a threat of complications, surgical removal of the tumor.

Regularities of structural organisation of the heart myxomas in patients with Karney complex

Myxomas of the heart are one of the most common primary heart tumours, which most often develop in the left atrium, are characterised by a benign nature and no relapses after surgical removal of the tumour. Karney complex is an autosomal dominant disease characterised by numerous tumours, in particular, myxomas of the heart. Insufficient coverage of this pathology in the literature leads to an erroneous diagnosis, progression of the disease and inadequate treatment. The purpose of the study was to determine the morphological features of heart myxomas in patients with Karney complex. Using light microscopy, a morphological examination of the heart myxomas was performed, which were removed during 7 operations. Histological sections were made from the operating material, which were stained with hematoxylin and eosin, according to Van Gieson, fuchselin, and Masson's Trichrome Stain in the Zerbino-Lukasevich modification. A comparison of the group of heart myxomas of patients with the Karney complex (16 tumours) with a group with myxomas of the heart that occur sporadically (278 tumours) was made to identify morphological features. It was established that myxomas of the heart are multiple, they were detected with the same frequency in the right and left chambers of the heart. It was generalised that in myxomas of the heart, signs of both alteration and high proliferative activity of endotheliocytes and fibrous connective tissue cells are noted. Secondary myxomas of the heart in patients with the Karney complex have the same structural organisation as primary tumours, but most often they are not relapses, more often they are multiple and do not have a predominant localisation in the left atrium. The analysis established an identical cellular composition of both groups of heart myxomas, which suggests the same source of tumour growth, but the presence of more complex cell formations in sporadic myxomas and the predominance of single cells in Karney heart myxomas indicate the existence of differences in their morphogenesis

Trends in unhealthy lifestyle factors in US NHANES respondents with cardiovascular disease for the period between 1999 and 2018.

To examine national trends in unhealthy lifestyle factors among adults with cardiovascular disease (CVD) in the United States (US) between 1999 and 2018. We analyzed data from National Health and Nutrition Examination Survey (NHANES), a nationally representative survey of participants with CVD who were aged ≥20 years, which was conducted between 1999 and 2000 and 2017-2018. CVD was defined as a self-report of congestive heart failure, coronary heart disease, angina, heart attack, or stroke. The prevalence rate of each unhealthy lifestyle factor was calculated among adults with CVD for each of the 2-year cycle surveys. Regression analyses were used to assess the impact of sociodemographic characteristics (age, sex, race/ethnicity, family income, education level, marital status, and employment status). The final sample included 5610 NHANES respondents with CVD. The prevalence rate of their current smoking status remained stable among respondents with CVD between 1999 and 2000 and 2017-2018. During the same period, there was a decreasing trend in the age-adjusted prevalence rate of poor diet [primary American Heart Association (AHA) score <20; 47.5% (37.9%-57.0%) to 37.5% (25.7%-49.3%), p < 0.01]. Physical inactivity marginally increased before decreasing, with no statistical significance. The prevalence rate of sedentary behavior increased from 2007 to 2014 but subsequently returned to its original level in 2018 with no statistical significance. The age-adjusted prevalence rate of obesity increased from 32% (27.2%-36.8%) in 1999-2000 to 47.9% (39.9%-55.8%) in 2017-2018 (p < 0.001). The age-adjusted prevalence rate of depression increased from 7% (4.2%-9.9%) in 1999-2000 to 13.9% (10.2%-17.6%) in 2017-2018 (p = 0.056). Trends in mean for each unhealthy lifestyle factor were similar after adjustment for age. We found that respondents who had low education and income levels were at a higher risk of being exposed to unhealthy lifestyle factors (i.e., smoking, poor diet, and physical inactivity) than those who had high education and income levels. There is a significant reduction in the prevalence rate of poor diet among US adults with CVD between 1999 and 2018, while the prevalence rate of obesity showed increasing trends over this period. The prevalence rate of current smoking status, sedentary behavior, and depression was either stable or showed an insignificant increase. These findings suggest that there is an urgent need for health policy interventions targeting unhealthy lifestyles among adults with CVD.

Heart Transplant Waitlist Outcomes and Wait Time by Center Volume in the Pre-2018 Allocation Change Era.

Although the transplant outcomes of centers are heavily monitored and compared, with a particular link between posttransplant outcomes and center volume demonstrated, little data exist comparing waitlist outcomes. Here, we explored waitlist outcomes by transplant center volume. We performed a retrospective analysis of adults listed for primary heart transplantation (HTx) from 2008 to 2018 using the United Network for Organ Sharing database. Transplant centers were split into low (<10 HTx/year), medium (10-30 HTx/year), and high (>30 HTx/year) volume, and waitlist outcomes were compared. Of the 35,190 patients included in our study, 23,726 (67.4%) underwent HTx, 4,915 (14.0%) died or deteriorated before receiving HTx, 1,356 (3.9%) were delisted due to recovery, and 1,336 (3.8%) underwent left ventricular assist device (LVAD) implantation. High-volume centers had higher rates of survival to transplant (71.3% vs. 60.6% for low-volume centers and 64.9% for medium-volume centers), and low rates of death or deterioration (12.6% vs. 14.6% for low-volume centers and 15.1% for medium-volume centers). Listing at a low-volume center was independently associated with death or delisting before HTx (HR 1.18, p = 0.007), whereas listing at a high-volume center (HR 0.86; p < 0.001) and prelisting LVAD (HR 0.67, p < 0.001) were protective. Death or delisting before HTx was lowest for patients listed in higher volume centers.