Introduction: The aim of this study was to evaluate the age at onset, clinical course, and patterns of left ventricular (LV) remodelling during follow-up in children and young patients with hypertrophic cardiomyopathy (HCM). Methods: We included consecutive patients with sarcomeric or non-syndromic HCM below 18 years old. Three pre-specified patterns of LV remodelling were assessed: maximal LV wall thickness (MLVWT) thickening; MLVWT thinning with preserved LV ejection fraction; and MLVWT thinning with progressive reduction in LV ejection fraction (hypokinetic end-stage evolution). Results: Fifty-three patients with sarcomeric/non-syndromic HCM (mean age 9.4 ± 5.5 years, 68% male) fulfilled the inclusion criteria. In total, 32 patients (60%) showed LV remodelling: 3 patients (6%) exhibited MLVWT thinning; 16 patients (30%) showed MLVWT thickening; and 13 patients (24%) progressed to hypokinetic end-stage HCM. Twenty-one patients (40%) had no LV remodelling during follow-up. In multivariate analysis, MLVWT was a predictor of the hypokinetic end-stage remodelling pattern during follow-up (OR 1.17 [95%CI 1.01-1.36] per 1 mm increase, p-value 0.043), regardless of sarcomeric variants and New York Heart Association class. Two patients with sarcomeric HCM, showing a pattern of MLVWT regression during childhood, experienced progression during adolescence. Conclusions: Different patterns of LV remodelling were observed in a cohort of children with sarcomeric/non-syndromic HCM. Interestingly, a pattern of progressive MLVWT thinning during childhood, with new progression of MLVWT during adolescence, was noted. A better understanding of the remodelling mechanisms in children with sarcomeric HCM may be relevant to defining the timing and possible efficacy of new targeted therapies in the preclinical stage of the disease.

Background: Fabry disease (FD) is a rare X-linked lysosomal storage disorder that commonly manifests cardiovascular complications. We aimed to assess the prevalence of FD in a Chinese population with left ventricular hypertrophy (LVH) whilst implementing a gender-specific screening approach. Methods: Patients with LVH, defined as a maximum thickness of the left ventricular septal/posterior wall ≥ 13 mm, were considered eligible. All patients with hypertrophic cardiomyopathy (HCM) were excluded. Plasma α-galactosidase (α-GLA) enzyme activity was assessed using a dried blood spot test. Males with low enzyme activity underwent genetic testing to confirm a diagnosis of FD whereas females were screened for both α-GLA and globotriaosylsphingosine concentration and underwent genetic analysis of the GLA gene only if testing positive for ≥1 parameter. Results: 426 unrelated patients (age = 64.6 ± 13.0 years; female: male = 113:313) were evaluated. FD was diagnosed in 3 unrelated patients (age = 69.0 ± 3.5 years, female: male = 1:2) and 1 related female subject (age = 43 years). Genetic analyses confirmed the late-onset cardiac variant GLA c.640-801G>A (n = 3) and the missense variant c.869T>C associated with classic FD (n = 1). Cardiac complications were the only significant findings associated with the late-onset c.640-801G>A mutation, manifesting as mild or severe concentric LVH. In contrast, the classic c.869T>C mutation FD exhibited multisystemic manifestations in addition to severe concentric LVH. Conclusions: The prevalence of FD is lower in Chinese patients with LVH when HCM is excluded. The pathological variant c.640-801G>A remains the most common cause of late-onset FD, while the detection of FD in females can be improved by utilizing a gender-specific screening method.

UNASSIGNED: Left ventricular hypertrophy (LVH) is a common consequence of hypertension and can lead to heart failure. The immune response plays an important role in hypertensive LVH; however, there is no comprehensive method to investigate the mechanistic relationships between immune response and hypertensive LVH or to find novel therapeutic targets. This study aimed to screen hub immune-related genes involved in hypertensive LVH as well as to explore immune target-based therapeutic drugs.
UNASSIGNED: RNA-sequencing data from a mouse model generated by angiotensin II infusion were subjected to weighted gene co-expression network analysis (WGCNA) to identify core expression modules. Machine learning algorithms were applied to screen immune-related LVH characteristic genes. Heart structures were evaluated by echocardiography and cardiac magnetic resonance imaging (CMRI). Validation of hub genes was conducted by RT-qPCR and western blot. Using the Connectivity Map database and molecular docking, potential small-molecule drugs were explored.
UNASSIGNED: A total of 1215 differentially expressed genes were obtained, most of which were significantly enriched in immunoregulation and collagen synthesis. WGCNA and multiple machine learning strategies uncovered six hub immune-related genes (Ankrd1, Birc5, Nuf2, C1qtnf6, Fcgr3, and Cdca3) that may accurately predict hypertensive LVH diagnosis. Immune analysis revealed that fibroblasts and macrophages were closely correlated with hypertensive LVH, and hub gene expression was significantly associated with these immune cells. A regulatory network of transcription factor-mRNA and a ceRNA network of miRNA-lncRNA was established. Notably, six hub immune-related genes were significantly increased in the hypertensive LVH model, which were positively linked to left ventricle wall thickness. Finally, 12 small-molecule compounds with the potential to reverse the high expression of hub genes were ruled out as potential therapeutic agents for hypertensive LVH.
UNASSIGNED: This study identified and validated six hub immune-related genes that may play essential roles in hypertensive LVH, providing new insights into the potential pathogenesis of cardiac remodeling and novel targets for medical interventions.

BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) is currently the most common liver disorder affecting about 25% of the global population. The causes of its development include poor diet, low physical activity, overweight, obesity, older age, diabetes, and lipid disorders. Non-alcoholic fatty liver disease is identified by some researchers as a hepatic manifestation of metabolic syndrome. It has been observed that patients with NAFLD have an increased risk of cardiovascular events, as well as a higher number of deaths from myocardial infarction compared to the general population.
METHODS: A retrospective analysis was conducted on the data of 237 patients diagnosed with hepatic steatosis, treated in the Department of Geriatrics at the University Clinical Hospital in Wrocław from 2019 to 2022, focusing on coexisting overweight, obesity, and concomitant diseases. Laboratory results and the degree of left ventricular muscle hypertrophy were analyzed. Parameters assessed by echocardiography, including interventricular septal thickness in diastole (IVSd), left ventricular posterior wall thickness in diastole (LVPWd), and IVSd + LVPWd/2, were used to evaluate left ventricular hypertrophy.
RESULTS: Data from 237 patients were analyzed: 79 men (age: 77.2±7.1 years) and 158 women (age: 78.4±7.7 years). Body mass index (BMI) values for men and women were 30.5±5.0 kg/m² and 31.9±5.6 kg/m², respectively. There was a positive correlation between BMI and the degree of left ventricular hypertrophy for the parameters IVSd (ρ = 0.36, p < 0.001), LVPWd (ρ = 0.36, p < 0.001), and IVSd + LVPWd/2 (ρ = 0.38, p < 0.001).
CONCLUSIONS: The study demonstrated a moderate positive correlation between BMI and the degree of left ventricular hypertrophy in patients diagnosed with hepatic steatosis. These findings indicate the necessity of actively searching for cardiovascular risk factors, including the evaluation of echocardiographic parameters in patients with NAFLD. Med Pr Work Health Saf. 2024;75(3).
UNASSIGNED: Niealkoholowa stłuszczeniowa choroba wątroby (non-alcoholic fatty liver disease – NAFLD) to obecnie najczęstsze schorzenie wątroby dotyczące ok. 25% populacji światowej. Za przyczyny jej rozwoju uznaje się nieprawidłową dietę, niską aktywność fizyczną, nadwagę i otyłość, starszy wiek, cukrzycę oraz zaburzenia lipidowe. Choroba jest uznawana przez część badaczy za wątrobową manifestację zespołu metabolicznego. Zaobserwowano, że u pacjentów z NAFLD zwiększone jest ryzyko zdarzeń sercowo-naczyniowych oraz liczba zgonów z powodu zawału mięśnia sercowego w porównaniu z populacją ogólną.
UNASSIGNED: Przeprowadzono retrospektywną analizę danych 237 pacjentów z rozpoznanym stłuszczeniem wątroby leczonych w Klinice Geriatrii Uniwersyteckiego Szpitala Klinicznego we Wrocławiu w latach 2019–2022 pod kątem współistnienia nadwagi, otyłości i chorób współistniejących. Przeanalizowano wyniki badań laboratoryjnych oraz stopień przerostu mięśnia lewej komory serca. Do oceny przerostu mięśnia użyto ocenianych w echokardiografii parametrów: grubość przegrody międzykomorowej w rozkurczu (interventricular septal thickness in diastole – IVSd), grubość późnorozkurczowa tylnej ściany lewej komory (left ventricular posterior wall thickness in diastole – LVPWd) i IVSd + LVPWd/2.
UNASSIGNED: Przeanalizowano dane 237 pacjentów: 79 mężczyzn (wiek: 77,2±7,1 roku) i 158 kobiet (wiek: 78,4±7,7 roku). Wartości wskaźnika masy ciała (body mass index – BMI) dla mężczyzn i kobiet wynosiły, odpowiednio, 30,5±5,0 kg/m2 oraz 31,9±5,6 kg/m2. Wykazano istnienie dodatniej korelacji między wielkością BMI a stopniem przerostu mięśnia lewej komory dla parametrów IVSd (ρ = 0,36, p < 0,001), LVPWd (ρ = 0,36, p < 0,001) i IVSd + LVPWd/2 (ρ = 0,38, p < 0,001).
UNASSIGNED: W badaniu wykazano dodatnią korelację o umiarkowanej sile między wielkością BMI a stopniem przerostu mięśnia lewej komory u chorych z rozpoznanym stłuszczeniem wątroby. Wyniki te wskazują na konieczność aktywnego poszukiwania czynników ryzyka chorób sercowo-naczyniowych, w tym oceny parametrów echokardiograficznych u pacjentów z NAFLD. Med Pr Work Health Saf. 2024;75(3).

Several studies suggested the utility of artificial intelligence (AI) in screening left ventricular hypertrophy (LVH). We hence conducted systematic review and meta-analysis comparing diagnostic accuracy of AI to Sokolow-Lyon\'s and Cornell\'s criteria. Our aim was to provide a comprehensive overview of the newly developed AI tools for diagnosing LVH. We searched MEDLINE, EMBASE, and Cochrane databases for relevant studies until May 2023. Included were observational studies evaluating AI\'s accuracy in LVH detection. The area under the receiver operating characteristic curves (ROC) and pooled sensitivities and specificities assessed AI\'s performance against standard criteria. A total of 66,479 participants, with and without LVH, were included. Use of AI was associated with improved diagnostic accuracy with summary ROC (SROC) of 0.87. Sokolow-Lyon\'s and Cornell\'s criteria had lower accuracy (0.68 and 0.60). AI had sensitivity and specificity of 69% and 87%. In comparison, Sokolow-Lyon\'s specificity was 92% with a sensitivity of 25%, while Cornell\'s specificity was 94% with a sensitivity of 19%. This indicating its superior diagnostic accuracy of AI based algorithm in LVH detection. Our study demonstrates that AI-based methods for diagnosing LVH exhibit higher diagnostic accuracy compared to conventional criteria, with notable increases in sensitivity. These findings contribute to the validation of AI as a promising tool for LVH detection.

Patients with chronic kidney disease (CKD) face a high risk of cardiovascular disease. Previous studies reported that endogenous thrombospondin 1 (TSP1) involves right ventricular remodeling and dysfunction. Here we show that a murine model of CKD increased myocardial TSP1 expression and produced left ventricular hypertrophy, fibrosis, and dysfunction. TSP1 knockout mice were protected from these features. In vitro, indoxyl sulfate is driving deleterious changes in cardiomyocyte through the TSP1. In patients with CKD, TSP1 and aryl hydrocarbon receptor were both differentially expressed in the myocardium. Our findings summon large clinical studies to confirm the translational role of TSP1 in patients with CKD.

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UNASSIGNED: To determine the factors associated with left ventricular diastolic dysfunction (LVDD) in adults residing in a region of the Andes in Peru.
UNASSIGNED: A case-control study was conducted on adults living at an altitude of more than 3000 meters in Peru. Cases consisted of patients diagnosed with LVDD through echocardiography, whereas controls were adults without LVDD, as confirmed by echocardiography.
UNASSIGNED: A total of 50 cases and 100 controls were included in the study. Among them, 38.7% had high blood pressure, and 41.3% were overweight. Upon adjusted analysis, age 60 or older (aOR: 4.06; 95%CI: 1.29-12.8), female sex (aOR: 2.24; 95%CI: 1.01-4.96) and left ventricular hypertrophy (aOR: 3.17; 95%CI: 1.41-7.17) were identified as statistically significant factors associated with LVDD.
UNASSIGNED: The risk of LVDD is associated with older adults, female gender, and left ventricular hypertrophy among individuals residing above 3000 meters altitude in a region of the Andes, in Peru.
UNASSIGNED: Determinar los factores asociados con la disfunción diastólica del ventrículo izquierdo (DDVI) en adultos de una región de los Andes, en Perú.
UNASSIGNED: Estudio de casos y controles en adultos residentes a más de 3000 metros de altitud en Perú. Los casos fueron pacientes adultos diagnosticados con DDVI por ecocardiografía, y los controles fueron adultos sin DDVI por ecocardiografía.
UNASSIGNED: Se incluyeron 50 casos y 100 controles. El 38.7% tuvieron hipertensión arterial y el 41.3% sobrepeso. En el análisis ajustado, la edad de 60 o más años (ORa: 4.06; IC95%: 1.29-12.8), el sexo femenino (ORa: 2.24; IC95%: 1.01-4.96) y la hipertrofia ventricular izquierda (ORa: 3.17; IC95%: 1.41-7.17) fueron factores estadísticamente significativos.
UNASSIGNED: El riesgo de DDVI estuvo asociado a los adultos mayores, las mujeres y los pacientes con hipertrofia ventricular izquierda que viven por encima de los 3000 metros de altitud en una región de los Andes, en Perú.

BACKGROUND: Left ventricular hypertrophy (LVH), including hypertensive LVH, hypertrophic cardiomyopathy (HCM) and cardiac amyloidosis (CA), is a commonly encountered condition in cardiology practice, presenting challenges in differential diagnosis. In this study, we aimed to investigate the importance of echocardiographic evaluation of the inferior vena cava (IVC) in distinguishing LVH subtypes including hypertensive LVH, HCM, and CA.
METHODS: In this retrospective study, patients with common causes of LVH including hypertensive LVH, HCM, and CA were included. The role of echocardiographic evaluation of IVC diameter and collapsibility in distinguishing these causes of LVH was assessed in conjunction with other echocardiographic, clinical, and imaging methods.
RESULTS: A total of 211 patients (45% HCM, 43% hypertensive heart disease, and 12% CA) were included in our study. Their mean age was 56.6 years and 62% of them were male. While mean IVC diameter was significantly dilated in CA patients (13.4 mm in hypertensive LVH, 16.0 mm in HCM, and 21.1 mm in CA, p < .001), its collapsibility was reduced (IVC collapsible in 95% of hypertensive patients, 72% of HCM patients, and 12% of CA patients, p < .001). In the analysis of diagnostic probabilities, the presence of both hypovoltage and IVC dilation is significant for CA patients. Although it is not statistically significant, the presence of IVC dilation along with atrial fibrillation supports the diagnosis of HCM.
CONCLUSIONS: In conclusion, although advances in imaging techniques facilitate the diagnosis of LVH, simple echocardiographic methods should never be overlooked. Our study supports the notion that IVC assessment could play an important role in the differential diagnosis of LVH.

BACKGROUND: This study explores the impact of intensive blood pressure (BP) control on left ventricular hypertrophy (LVH) incidence and evaluates the prognostic implications of LVH status (pre-existing/new-onset/persistent/regression) using Systolic Blood Pressure Intervention Trial (SPRINT) Electrocardiogram Data.
METHODS: Poisson regression was used to assess new-onset LVH and LVH regression rates. Multivariable-adjusted Cox proportional hazard models determined the risk of adverse cardiovascular events (ACE), a composite of myocardial infarction (MI), non-MI acute coronary syndrome, stroke, heart failure, or cardiovascular death, alongside safety adverse events.
RESULTS: In 8,016 participants, intensive BP control significantly reduced new-onset LVH (8.27 vs. 14.79 per 1000-person years; adjusted p<0.001) and increased LVH regression (14.89 vs. 11.89 per 1000-person years; adjusted p<0.001). Elevated ACE risk was notable in participants with pre-existing LVH [adjusted HR: 1.94 (95% CI: 1.25-2.99); p = 0.003], new-onset LVH [adjusted 1.74 (95% CI: 1.16-2.60); p = 0.007], and persistent LVH[adjusted HR: 1.96 (95% CI: 1.11-3.46); p = 0.020], compared to those without LVH. Intriguingly, LVH regression attenuated this risk increment [adjusted HR: 1.57 (95% CI: 0.98-2.53); p = 0.062]. Achieving a BP target of < 120/80 mmHg nullified the increased ACE risk in those with pre-existing LVH.
CONCLUSIONS: Intensive BP control is instrumental in both reducing the emergence of LVH and fostering its regression. Pre-existing, new-onset LVH and persistent LV remain a predictor of adverse cardiovascular prognosis, whereas LVH regression and achieving on-treatment BP < 120/80 mmHg in pre-existing LVH individuals may further mitigate residual cardiovascular risk.
BACKGROUND: URL: ClinicalTrials.gov Unique Identifier: NCT01206062.