Abnormal LV Research Articles

Evaluation of left ventricular systolic function in patients with iron deficiency anemia based on non-invasive left ventricular pressure–strain loops

BackgroundIron deficiency anemia (IDA) is a common health problem worldwide. The objective of this study was to noninvasively and quantitatively evaluate early changes in left ventricular systolic function in patients with IDA using the left ventricular press–strain loop (LV-PSL).MethodsSixty-two patients with IDA were selected and divided into two groups based on hemoglobin (Hb) concentration: Group B with Hb > 9 g/dL and group C with 6 g/dL < Hb < 9 g/dL. Thirty-three healthy individuals were used as the control (Group A). The global longitudinal strain (GLS), global work index (GWI), global constructive work (GCW), global waste work (GWW), global work efficiency (GWE) were derived using LV-PSL analysis. Receiver operating characteristic (ROC) curves were constructed for MW parameters to detect abnormal left ventricular systolic function in IDA patients.ResultsCompared to group A, GWI and GCW were reduced in group B (both P < 0.01). Compared with groups B and A, GLS, GWI, GCW and GWE, and E/A were all diminished, and GWW, LVEDV, LVESV, and E/mean e’ were all increased in group C (all P < 0.01). GLS was positively correlated with GWI, GCW, and GWE (r = 0.679, 0.681, and 0.447, all P < 0.01), and negatively associated with GWW (r = − 0.411, all P < 0.01). For GWI, area under the ROC curve (AUROC) was 0.783. The optimal GWI threshold for detecting abnormal LV systolic function in IDA was1763 mmHg%, with sensitivity of 0.71 and specificity of 0.78.ConclusionsLV-PSL allows noninvasive quantitative assessment of early impaired LV systolic function in IDA patients with preserved LV ejection fraction, and GWI has high sensitivity and specificity compared with other parameters.

A Deep Patient-Similarity Learning Framework for the Assessment of Diastolic Dysfunction in Elderly Patients.

Age-related changes in cardiac structure and function are well recognized and make the clinical determination of abnormal LV diastolic function (LVDD) particularly challenging in the elderly. We investigated whether a deep neural network (DeepNN) model[70] of LVDD, previously validated in a younger cohort, can be implemented in an older population to predict incident heart failure (HF). A previously developed DeepNN was tested on 5,596 older participants (66-90 years; 57% female; 20% black) from the Atherosclerosis Risk in Communities study. The association of DeepNN predictions with HF or all-cause death for the American College of Cardiology Foundation/American Heart Association Stage A/B (n = 4,054) and Stage C/D (n = 1,542) subgroups was assessed. The DeepNN-predicted High-Risk compared to the Low-Risk phenogroup demonstrated an increased incidence of HF and death for both Stage A/B and Stage C/D (log-rank p < 0.0001 for all). In multivariable analyses, the High-Risk phenogroup remained an independent predictor of HF and death in both Stages A/B (adjusted hazard ratio (HR) [95% confidence interval], 6.52[4.20-10.13] and 2.21(1.68-2.91), both p < 0.0001) and Stage C/D (6.51[4.06-10.44] and 1.03(1.00-1.06), both p < 0.0001) respectively. In addition, DeepNN showed incremental value over the 2016 ASE/EACVI guidelines (Net reclassification index, 0.5[CI:0.4-0.6], p < 0.001; C-statistic improvement, DeepNN [0.76] vs. ASE/EACVI [0.70], p < 0.001) overall and maintained across stage-groups. Despite training with a younger cohort, a deep patient-similarity-based learning framework for assessing LVDD provides a robust prediction of all-cause death and incident HF for older patients.

Right and left ventricular cardiac magnetic resonance imaging derived peak systolic strain is abnormal in children with myocarditis.

Cardiac Magnetic resonance (CMR) derived left ventricular longitudinal and circumferential strain is known to be abnormal in myocarditis. CMR strain is a useful additional tool that can identify subclinical myocardial involvement and may help with longitudinal follow-up. Right ventricular strain derived by CMR in children has not been studied. We sought to evaluate CMR derived biventricular strain in children with acute myocarditis. Children with acute myocarditis who underwent CMR between2016-2022 at our center were reviewed, this group included subjects with COVID-19 myocarditis. Children with no evidence of myocarditis served as controls Those with congenital heart disease and technically limited images for CMR strain analysis were excluded from final analysis. Biventricular longitudinal, circumferential, and radial peak systolic strains were derived using circle cvi42®. Data between cases and controls were compared using an independent sample t-test. One-way ANOVA with post hoc analysis was used to compare COVID-19, non-COVID myocarditis and controls. 38 myocarditis and 14 controls met inclusion criteria (mean age14.4 ± 3 years). All CMR derived peak strain values except for RV longitudinal strain were abnormal in myocarditis group. One-way ANOVA revealed that there was a statistically significant difference with abnormal RV and LV strain in COVID-19 myocarditis when compared to non-COVID-19 myocarditis and controls. CMR derived right and left ventricular peak systolic strain using traditionally acquired cine images were abnormal in children with acute myocarditis. All strain measurements were significantly abnormal in children with COVID-19 even when compared to non-COVID myocarditis.

Non-Invasive Risk Assessment and Prediction of Mortality in Patients Undergoing Coronary Artery Bypass Graft Surgery.

Objectives: Heart rate turbulence (HRT) and T-wave alternans (TWA), non-invasive markers of cardiac autonomic dysfunction, and ventricular repolarization abnormality, reportedly, predict the risk of cardiovascular death after myocardial infarction. We investigated whether pre-operative assessment of HRT and/or TWA could predict long-term mortality following coronary artery bypass graft (CABG) surgery. Methods: From May 2010 to December 2017, patients undergoing elective CABG and receiving 24 h ambulatory electrocardiogram monitoring 1 to 5 days prior to CABG surgery were prospectively enrolled. Pre-operative HRT and TWA were measured using a 24 h ambulatory electrocardiogram. The relative risk of cardiac or overall death was assessed according to abnormalities of HRT, TWA, or left ventricular ejection fraction (LV EF). Results: During the mean follow-up period of 4.6 ± 3.9 years, 40 adjudicated overall (5.9%/yr) and 5 cardiac deaths (0.9%/yr) occurred in 146 enrolled patients (64.9 ± 9.3 years; 108 males). Patients with abnormal HRT exhibited significantly higher relative risks of cardiac death (adjusted hazard ratio [HR] 24.9, 95% confidence interval [CI] 1.46-427) and all-cause death (adjusted HR 5.77, 95% CI 2.34-14.2) compared to those with normal HRT. Moreover, abnormal HRT plus abnormal TWA and LV EF < 50% was associated with a greater elevation in cardiac and overall mortality risk. The predictive role of abnormal HRT with/without abnormal TWA for all-cause death was likely more prominent in patients with mildly reduced (35 to 50%) or preserved (≥50%) LV EF. Abnormal HRT plus abnormal TWA and LV EF < 50% showed high negative predictive value in cardiac and overall mortality risk. Conclusions: Assessment of pre-operative HRT and/or TWA predicted mortality risk in patients undergoing elective CABG. Combined analysis of HRT, TWA, and LVEF enhanced the prognostic power. In particular, the predictive value of HRT was enhanced in patients with preserved or mid-range LV EF.

Left Ventricular Function in Recovered COVID 19 Patients Using 4-Dimension Echocardiography

Abstract Aim and Objectives The aim of the Work was to study the effect of COVID 19 on Left Ventricular Function and dimensions using 4D Echocardiography in recovered patients Patients and Methods This study is a pilot study on 30 patients who are recovered from COVID19 to study LV function using 4D Echocardiography. Results our study showed that 50% of our patients with COVID 19 had abnormal LV global longitudinal strain. Conclusion In patients with COVID-19 infection, we did full study 2D echocardiography in addition to GLS &amp; 4D echocardiography on the left ventricle and we concluded that LV global longitudinal strain was altered and it showed that 50% of our patients had abnormal LV global longitudinal strain by Speckle Tracking Echocardiography

Evaluation of Left Ventricular Diastolic Function Using a Tissue-Doppler Imaging Based Algorithm According to 2016 Ase/Eacvi Guidelines in Patients with Decompensated Liver Cirrhosis: A Prospective Observational Study

Abstract Background Cirrhosis is a late stage of scarring (fibrosis) of the liver caused by many forms of liver diseases and conditions, such as hepatitis and chronic alcoholism. The prevalence of cirrhosis has nearly doubled over the last decade, and the number of hospitalizations has similarly increased. Aim of the Work We aimed to correlate between the severity of liver cirrhosis using new MELD score and left ventricle diastolic dysfunction using tissue doppler imaging. Patients and Methods A prospective observational study in ICU of Theodor Bilharz Research Institute Hospital and Ain Shams University Hospitals for 6 months from June 2021 till end of November 2021 on hepatic patients who were admitted to ICU of Theodor Bilharz Research Institute Hospital and Ain Shams University Hospitals. Results In the studied group, the mean age was 53.13±9.44 years with males constituting more than half of them (57.8%). The mean MELD score was 24.08±6.01, and the mean left ventricular ejection fraction (LVEF %) was 60.43±5.49%. There were 5 patients (5.6%) with normal LV diastolic function and 85 patients (94.4%) with abnormal LV diastolic dysfunction. Out of the 90 patients, 5 patients were normal, 44 patients (48.9%) had a grade I LVDD, 28 patients (31.1%) had a grade II LVDD and 13 patients (14.4%) had a grade III LVDD. There was a highly statistically significant positive association between the grades of left ventricular diastolic dysfunction according to ALT, AST, T.Bilirubin, D.Bilirubin, INR and Creatinine. Additionally, there was a highly statistically significant negative association between grading of left ventricular diastolic dysfunction according to platelet count and sodium levels. Conclusion There was a statistically significant correlation found between the severity of liver cirrhosis represented by the new MELD score and the grade of left ventricular diastolic dysfunction. Therefore, in order to prepare the best post-procedural management of cirrhotic patients, echocardiography is required for all patients before any interventional procedure or liver transplantation regardless of the stage of cirrhosis. The mechanism of cardiac involvement in patients with cirrhosis seems to be complex and multifactorial.

Abnormal Left Ventricular Strain Correlates with Left Ventricular Dysfunction but not Aortic Pathology in Marfan Syndrome in Children.

Cardiomyopathy is a complication in adults with Marfan syndrome (MFS). Early recognition of MFS patients at high risk of cardiomyopathy could impact monitoring and treatment. Abnormal ventricular strain has been associated with impaired ventricular function among adults with MFS but remains understudied in children. We retrospectively analyzed a cohort of patients with MFS undergoing cardiac magnetic resonance imaging (CMR) performed in 2003-2018 at age < 19years. Correlations were evaluated between initial global circumferential strain (GCS) and global longitudinal strain (GLS) and the outcomes of left ventricular ejection fraction (LVEF), aortic root z-score, and vertebral artery tortuosity index corrected for height (VTI-h), all measured from CMR, using Spearman correlation. In those with serial CMR, the ability of ventricular strain to predict development of abnormal LVEF within a 5-year period was assessed. A total of 31 subjects were included (median age at initial CMR 13.5 years, Q1Q3 10.7-16.2 years), with 48% (n = 15) having LVEF < 55%. Worse GCS and worse GLS were associated with lower LVEF (ρ = - 0.629, p < 0.001 and ρ = - 0.411, p = 0.030, respectively). A clinical cutoff of GCS = - 34% predicted LVEF < 55% with sensitivity = 80% and specificity = 50%. Neither GCS nor GLS was associated with aortic root z-score (GCS: p = 0.524; GLS: p = 0.624) nor VTI-h (GCS: p = 0.949; GLS: p = 0.593). Of those with LVEF ≥ 55%, initial GCS and GLS did not differ between those with later normal versus abnormal LVEF (GCS: p = 0.505; GLS: p = 0.232). In this cohort, abnormal LV strain was associated with abnormal LVEF, but not with aortic dilation or low LVEF within the 5years post-CMR.

Segmental and global myocardial work in hypertensive patients with different left ventricular ejection fraction: what's the role of the apex played?

We investigated myocardial work in hypertension (HT) among patients with different left ventricular ejection fraction (LVEF) to analyze the contribution of segmental myocardial work to global myocardial work. 114 patients with HT were divided into 4 groups: HTsnEF ("supra-normal" EF, > 65%); HTnEF ("normal" EF, 60-65%); HTmEF (designed as "middle" EF, 50-60%, within an abnormal LV geometry); HTrEF (reduced EF, < 50%). Longitudinal strain (LS) and myocardial work indices were obtained by echocardiography: myocardial work index (MWI), constructive work (MCW), wasted work (MWW), myocardial work efficiency (MWE), and percentages of apical work were calculated (PApi-MWI, PApi-MCW). Global LS (GLS) and global MWE (GWE) decreased in HTmEF and HTrEF groups. Global MWI(GWI) and MCW(GCW) increased in HTsnEF and HTnEF groups, and subsequently decreased, particularly in HTrEF group (P < 0.05). GWW increased in all HT subgroups. All segmental MWI and MCW were elevated or preserved initially in HTsnEF and HTnEF groups, and subsequently decreased, obviously in basal and middle segments in HTrEF group (P < 0.05). All segmental MWW increased and MWE decreased in HTmEF and HTrEF groups (P < 0.05). PApi-MWI and PApi-MCW increased initially, and subsequently decreased in HTmEF group, and elevated significantly in HTrEF group. Correlation analysis revealed a close connection of GLS and myocardial work parameters with LVEF. Apical myocardial work increased at the early stages of hypertensive systolic dysfunction, as a compensatory mechanism. Segmental myocardial work analysis added value to explore the distribution of myocardial impairment.

Micro-RNA-532-3p: a potential target in CKD-HFpEF pathophysiology

Background: Chronic kidney disease (CKD), an independent risk factor for heart failure with preserved ejection fraction (HFpEF), is present in over 50% of CKD patients and accounts for over 50% of hospital admissions for HF. We showed in a novel swine model of CKD-HFpEF that cardiac abnormalities associate with upregulation of pro-inflammatory nuclear factor kappa B (NF-kB). NF-κB activation is controlled by its natural inhibitor IkBα, a protein encoded by the NFKBIA gene that prevents its nuclear translocation, but the exact mechanisms remain unknown. We hypothesize that micro-RNAs (miRNAs), small noncoding RNAs that regulate gene expression, downregulate cardiac NFKBIA and activates NF-κB contributing to cardiomyocyte injury in CKD-HFpEF. Methods: Unbiased cardiac miRNA-sequencing was performed in normal and CKD-HFpEF pigs (n=4/group, 2 males/2 females). Differentially expressed (fold change ≥2 and false discovery rate (FDR)≤0.05) miRNAs capable of targeting NFKBIA were identified using miRWalk 3.0 and Target Scan 8.0, further validated by qPCR. To study the impact of CKD-HFpEF on cardiac tissue, cardiomyocites (CM) were exposed for 48 hours to plasma from normal and CKD-HFpEF pigs (n=3 each) and cardiac injury (LDH release) evaluated, and selected miRNAs modulated (mimic, antagomiR). Results: All CKD-HFpEF pigs showed a significant reduction in renal hemodynamics and function compared to controls accompannied by left ventricular hypertrophy (LVH), abnormal diastolic relaxation and LV strain, and increased circulating ANP and BNP levels, underscoring successful developing of CKD-HFpEF.Unbiased miRNA-seq identified 402 miRNAs targeting NFKBIA, of which 13 were broadly conserved among vertebrates and only 4 expressed in pig’s cardiac tissue. Among them, only miR-532-3p was upregulated in myocardium of CKD-HFpEF pigs.Expression of miR-532-3p was also higher in CM exposed to CKD-LVDD plasma or miR-532-3p mimic, associated with down-regulation of NFKBIA and LDH release. Treatment with anti-miR-532-3p decreased miR-532-3p, increased NFKBIA expression, and reduced CM injury. Conclusions: These observations support a role of miR-532-3p in cardiac NF-kB activation in CKD-HFpEF. In turn, these proof-of-concept studies may assist to identify novel therapeutic targets and develop new interventions to protect the heart in CKD. NIH HL095638 (ARC), DK129240 (AE) This is the full abstract presented at the American Physiology Summit 2023 meeting and is only available in HTML format. There are no additional versions or additional content available for this abstract. Physiology was not involved in the peer review process.

Echocardiographic Features Beyond Ejection Fraction and Associated Outcomes in Patients With Heart Failure With Mildly Reduced or Preserved Ejection Fraction.

Heart failure (HF) guidelines recommend assessment of left ventricular ejection fraction (LVEF) to classify patients and guide therapy implementation. However, LVEF alone may be insufficient to adequately characterize patients with HF, especially those with mildly reduced or preserved LVEF. Recommendations on additional testing are lacking, and there are limited data on use of echocardiographic features beyond LVEF in patients with heart failure with mildly reduced or preserved LVEF. In patients with HF with mildly reduced or preserved LVEF identified in a large US health care system, the association of the following metrics with mortality was evaluated: LV global longitudinal strain (LV GLS>-16), left atrial volume index (>28 mL/m2), left ventricular hypertrophy (LVH), and E/e´>13 and e´<9. A multivariable model for mortality was constructed including age, sex, and key comorbidities followed by stepwise selection of echocardiographic features. Characteristics and outcomes of subgroups with normal versus abnormal LV GLS and LVEF were evaluated. Among 2337 patients with complete echocardiographic data assessed between 2017 and 2020, the following features were associated with all-cause mortality on univariate analysis over 3 years of follow-up: E/e´+e´, LV GLS, left atrial volume index (all P<0.01). In the multivariable model (C-index=0.65), only abnormal LV GLS was independently associated with all-cause mortality (HR, 1.35 [95% CI, 1.11-1.63]; P=0.002). Among patients with LVEF>55%, 498/1255 (40%) demonstrated abnormal LV GLS. Regardless of specific LVEF, patients with abnormal LV GLS demonstrated a higher burden of multiple comorbidities and higher event rates compared with patients with normal LV GLS. In a large, real-world HF with mildly reduced or preserved LVEF population, echocardiographic features, led by LV GLS, were associated with adverse outcomes irrespective of LVEF. A large proportion of patients demonstrate adverse myocardial function by LV GLS despite preserved LVEF and may represent a key cohort of interest for HF medical therapies and future clinical studies.

Relation of the left ventricular geometric patten to coronary artery disease in hypertensive patients using a 320-detector-row CT scanner

BackgroundIt was estimated that about 1.3 billion people were diagnosed to be hypertensive in 2015. All countries consistently show this high prevalence. Ischemic heart disease stands as the most common cause of systolic blood pressure-related deaths per year. Left ventricular hypertrophy determined by echocardiography can predict cardiovascular morbidity and mortality. The question of whether the LV geometric pattern has an additional prognostic value is still not clearly answered. Currently, coronary computed tomography is widely used in clinical practice with a great capability of simultaneous evaluation of the LV mass and the coronary arterial tree. Our study aims to examine the relationship between LV mass and geometry and coronary artery disease using an ECG-gated 320-detector- row CT scanner.ResultsTwo hundred ninety-eight hypertensive Egyptian individuals were enrolled in our study, the mean age was 57.5 ± 10.5, and males comprised 76.5% of the study population. The mean LV mass and LV mass index were 193 ± 60 gm and 95.2 ± 27.5 g/m2 respectively. One-fifth of the patient had CAD luminal stenosis ≥ 50%. Normal LV geometric pattern was observed in about 37% of the study population. About one-third of the patients showed concentric remodeling. Patients with increased LV mass index represented one-third of the study population with a greater percentage of the concentric hypertrophy pattern than the eccentric hypertrophy pattern. Patients with high CAD-RADS showed statistically significant higher LV mass, LV mass index, and septal wall thickness. Patients with high CAD-RADS showed a greater percentage of concentric and eccentric hypertrophy. The LV geometric pattern was the only independent predictor of the high CAD-RADS. The LV geometric patterns associated with high RADS ordered from the highest to the lowest, were concentric LVH, Eccentric LV, and concentric remodeling.ConclusionsLV geometric pattern is the only independent predictor of high CAD-RADS after adjustment for LV mass index and septal wall thickness. Among abnormal LV geometric patterns, concentric hypertrophy stands as the most important predictor of high CAD-RADS.

Assessment of left ventricular geometry in normotensive type II diabetic patients.

Abnormalities of glucose metabolism are associated with abnormal left ventricular geometry (LV) independent of atherosclerosis. Abnormal LV geometry, a predictor of premature cardiovascular events, indicates presence of subclinical target organ damages. Screening for abnormal LV geometry in diseases of abnormal glucose metabolism is desirable as part of their management protocol. To assess the left ventricular geometry in normotensive type II diabetic patients. Cross-sectional, descriptive, hospital-based study. One hundred normotensive type II diabetic patients drawn from the Endocrinology and Family Medicine Clinics of a tertiary hospital were age- and gender-matched with 100 apparently healthy controls. Participants meeting the criteria and informed consent proceeded for clinical evaluation, biochemical assessment, electrocardiography, and echocardiography using the American Society of Echocardiography guideline. Data were analyzed using the Statistical Package for Social Sciences [SPSS] version 25.0 (Chicago Illinois, USA). Mean age of study and control groups was (55.56 ± 9.89 versus 55.47 ± 10.7) years (χ2 = 0.062, P = 0.951). The mean duration of diabetes illness was 6.57 ± 6.26 years. Prevalence of abnormal LV geometry was 51% (study) versus 18% (control) FT, P < 0.001). Concentric remodeling was the predominant geometry in 36% of study versus 11% of controls, followed by eccentric hypertrophy in 11% (study) versus 4% (control) and concentric hypertrophy in 4% (study) versus 3% (control). Geometry was normal in 49% of study against 82% in the controls (FT, P < 0.001). Significant association existed between LV geometry and duration of diabetes (χ2 = 10.793, P = 0.005). Abnormal LV geometry is highly prevalent in normotensive diabetic patients.

PS-C14-8: SINGLE GRAFT AFTER MALFUNCTIONING STENT IN CORONARY ARTERY DISEASE WITH DIABETES AND HYPERTENSION

Introduction: A 65-year-old woman was sent to the Heart Center Hospital emergency room because she had a history of persistent chest pain that had gotten worse during demanding conditions and strenuous activities. She has had diabetes and hypertension for ten years and is on medication with reasonable control. She had an angiography two years ago due to related complications. When the patient was taken to the Cath Lab for the second angiography it was discovered that the cardiology interventionist had wrongly put the stent after the LAD proximal and left the LAD artery stenosis significantly untreated. All of the specific symptoms of complications were, of course, connected to the stenosis of the LAD. The stenosis of the LAD was about 90% in the third stage. Discussion: The patient had an EF of 50% a PAP of 29 and no severe Echo issues were instantly found. Her stenosis was rapidly worsening, the angle of the stent used in the angioplasty was the standard size for the LAD and the fibrosis in the LAD pathway had unusual anatomy, making the second angioplasty exceedingly dangerous. An open-heart procedure seemed to be the only option to prevent a potential arterial rupture in angiography since the stenosis site was severely injured and fibrosis had developed due to the stent. The left internal mammary was anastomosed to the LAD artery after the stent had been implanted in a patient undergoing open-heart surgery. And only one incision was made for the bypass surgery. Summary: To be clear bypass surgery was the only viable option in this complex situation because removing the stent could have badly disrupted the intimal layer of the artery, progressively increasing the likelihood of thrombosis. It should be emphasized that the left and right coronary arteries were in good shape, with standard size and runup. Given the significance of the LAD and that it supplies the heart with about 70% of its blood, myocardial infarction would have been quite likely in this case if there had been no surgery. Past History: DM.HTN. MI RECENT ECHO Finding EF: 50% Normal LV and RV the average size S/P recent PCI on LAD average LV size with mild systolic dysfunction LVEF 50 % abnormal septal motion GI LV diastolic dysfunction, average RV size with mild systolic dysfunction MILD MAC Mild to moderate MR Thickened and mildly calcified AV

Cardiac Phenotyping of SARS-CoV-2 in British Columbia: A Prospective Echo Study With Strain Imaging.

There is limited data on the residual echocardiographic findings including strain analysis among post-coronavirus disease (COVID) patients. The aim of our study is to prospectively phenotype post-COVID patients. All patients discharged following acute COVID infection were systematically followed in the post-COVID-19 Recovery Clinic at Vancouver General Hospital and St. Paul's Hospital. At 4-18 weeks post diagnosis, patients underwent comprehensive echocardiographic assessment. Left ventricular ejection fraction (LVEF) was assessed by 3D, 2D Biplane Simpson's, or visual estimate. LV global longitudinal strain (GLS) was measured using a vendor-independent 2D speckle-tracking software (TomTec). A total of 127 patients (53% female, mean age 58 years) were included in our analyses. At baseline, cardiac conditions were present in 58% of the patients (15% coronary artery disease, 4% heart failure, 44% hypertension, 10% atrial fibrillation) while the remainder were free of cardiac conditions. COVID-19 serious complications were present in 79% of the patients (76% pneumonia, 37% intensive care unit admission, 21% intubation, 1% myocarditis). Normal LVEF was seen in 96% of the cohort and 97% had normal right ventricular systolic function. A high proportion (53%) had abnormal LV GLS defined as < 18%. Average LV GLS of septal and inferior segments were lower compared to that of other segments. Among patients without pre-existing cardiac conditions, LVEF was abnormal in only 1.9%, but LV GLS was abnormal in 46% of the patients. Most post-COVID patients had normal LVEF at 4-18 weeks post diagnosis, but over half had abnormal LV GLS.

641 A CONTROVERSIAL CASE OF FABRY DISEASE: ADDING ANOTHER PIECE TO THE INTERPRETATION PUZZLE OF C.376A&amp;gt;G MISSENSE MUTATION, A PATHOGENIC VARIANT

Abstract Background The association between chronic neuropathic pain in childhood, left ventricular hypertrophy(LVH), early ischemic stroke and unexplained renal failure is suggestive of FD. However, because of the heterogeneity of the genetic substrate, different and more subtle phenotypic manifestations are possible as well as single organ involvement. Diagnostic criteria require an initial measurement of both alpha-galactosidase A (α-Gal-A) activity levels in leukocytes and globotriaosylsphingosine (LysoGb3, a glycosphingolipid derivative) concentration. Genetic testing is needed to confirm the diagnosis if α-Gal-A is decreased or absent and Lyso-Gb3 is increased. Case report A 35-year-old man was referred to our outpatient cardiology clinic at the beginning of summer 2022. He had a family history of sudden death, he had no cardiovascular risk factors and a history of severe acral pain during childhood. On medical background, he had undergone renal transplantation after two years of hemodialysis therapy due to nephroangiosclerosis and tubulointerstitial nephritis, identified in renal biopsy. On physical examination were visible neither angiokeratomas nor ocular manifestations. ECG showed LV hypertrophy and a transthoracic ECO confirmed LV concentric hypertrophy, a preserved LV ejection fraction and the presence of unexplained subtle LV systolic dysfunction due to an abnormal LV global longitudinal strain. Laboratory tests revealed normal serum creatinine and elevated NT-ProBNP levels. Unfortunately, a CMR examination was not performed because of the patient's claustrophobia. Genetic and enzymatic analyses were performed on the high suspicion of a cardiac storage disorder. The enzymatic activity of α-Gal-A and lyso-Gb3 concentration was within normal values, whereas genetic analysis detected a missense mutation (c.376A&amp;gt;G) in exon 3 causing an amino acid substitution (p.S126G). The suspicion of FD was confirmed. Discussion This is an interesting case with a diagnosis of FD that is controversial for several reasons. The c.376A&amp;gt;G is a rare mutation, found mainly in female patients with normal α-Gal-A and LysoGb3 levels ​​and associated with isolated renal failure. In addition, this variant is typically responsible for the late onset of the disease, occurring after 40 years of age2,8–11. Our patient is young and presents early cardiac and severe nephrological involvement, both typical clinical features of FD. A normal activity of α-Gal-A is consistent with the missense gene mutation: enzyme activity appears to be normal up to at least 90-95% reduction of function. To date, there is little evidence in the scientific literature of similar cases and the clinical presentations associated with this mutation are contradictory. Since 2002, c.376A&amp;gt;G has been known to be a pathogenetic variant of FD12, in 2010 it was associated to late-onset disease13. However, in the International Fabry Disease Genotype-Phenotype Database, it is considered a likely benign variant of FD2. Conclusion In this case report, we emphasize that high clinical suspicion should lead to more diagnostic evaluations, including genetic testing in the daily routine. Additional studies are needed to better understand the role of the c.376 A&amp;gt;G mutation in the FD.

Association of biomarkers and risk scores with subclinical left ventricular dysfunction in patients with type 2 diabetes mellitus.

Subclinical LV dysfunction (LVD) identifies heart failure (HF) risk in type 2 diabetes mellitus (T2DM). We sought the extent to which clinical scores (ARIC-HF, WATCH-DM), natriuretic peptides (NTpBNP) and troponin (hs-TnT) were associated with subclinical LV dysfunction (LVD). These associations could inform the ability of these tests to identify which patients should undergo echocardiography. Participants with T2DM were prospectively recruited from three community-based populations. ARIC-HF risk at 4years and WATCH-DM scores were calculated from clinical data. NTpBNP and hs-TnT were measured using an electro-chemiluminescence assay. All underwent a comprehensive echocardiogram. We calculated the sensitivity and specificity of clinical scores and biomarkers to identify abnormal global longitudinal strain (GLS ≥ -16%)), diastolic function (E/e' ≥ 14 or e' < 8cm/s), left atrial volume index (LAV > 34ml/m2) and LV hypertrophy (LV mass index > 88g/m2 (F) > 102g/m2(M)). Of 804 participants (median age 69years [inter-quartile range (IQR) 65-73], 36% female), clinical scores suggested significant HF risk (median ARIC-HF 8% [IQR 4-12]; WATCH-DM 10 points [IQR 8-12]), and the median NTpBNP was 50pg/mL [IQR 25-101] and hs-TnT 9.6pg/mL [IQR 6.8-13.6]. Abnormal GLS was present in 126 (17%), elevated E/e' in 114 (15%), impaired e' in 629 (78%), increased LAV in 351 (44%) and LV hypertrophy in 113 (14%). After adjustments for age, body-mass index, and renal function, each standard deviation increase in NTpBNP was associated with a GLS increase of 0.32 (p < 0.001) and hs-TnT increase by 0.26 (p < 0.001). Similar trends were observed with ARIC-HF (standardised β = 0.22, p < 0.001) and WATCH-DM (standardised β = 0.22, p < 0.001) in univariable analyses. However, none of the risk assessment tools provided satisfactory discrimination for abnormal GLS (AUC 63%), diastolic indices (e' AUC 54-61%) or LV mass (AUC 59-67%). At a sensitivity of 90%, there was an unacceptably low (< 50%) specificity. Although risk assessment based on clinical scores or biomarkers would be desirable to stratify HF risk in people with T2DM, they show a weak relationship with subclinical LVD.

The Echo Graphic Assessment of Inappropriate Left Ventricular Mass and Left Ventricular Hypertrophy in Patients with Diastolic Function

Objective: To evaluate the echo graphic assessment of inappropriate left ventricular mass and left ventricular hypertrophy in patients with diastolic function. Method: This cross sectional study was carried out at tertiary medical hospital where Two-dimensional echocardiography was performed on 200 patients with simple hypertension at a tertiary medical center. If a woman had an LVM index of more than 88 g/m2 of body-surface area, or if a man had an LVM index of more than 102 g/m2 of body-surface area, they were considered to have an unhealthy amount of LVM. Women with a septal wall thickness of more than 0/9 cm and males with a thickness of more than 1 cm are considered to have LVH. Early diastolic peak velocity (E) was compared to late diastolic peak velocity (A), deceleration time (DT), and early diastolic peak velocity (E′) were also assessed as echocardiographic parameters. Results: Patients' averaged systolic and diastolic blood pressure readings on the day of admission were 142.87 18.12 and 88.45 9.18 mmHg, respectively. Twenty-one percent of the individuals had an abnormal LV mass, with 5.6% having a mild abnormality and 5.6% having a severe abnormality. Patients with mild left ventricular hypertrophy had a higher mean age and body mass index (P 0.05). Patients with more severe ventricular hypertrophy had longer E/A ratios and longer deceleration times after controlling for age, gender, body mass index, and systolic and diastolic blood pressures. The mean body mass index (BMI) of subjects with severe was 33. 7 3.7 (P 0.001). Slightly different levels of diastolic dysfunction were associated with varying degrees of improper LV mass (P = 0.065). However, there was no correlation between E/A, E/E′, or deceleration time and excessive LV mass (P &gt; 0.05). The relationship between diastolic dysfunction and LV mass was analyzed using Spearman's Rank test (P = 0.025). Conclusion: While LVH is a strong predictor of diastolic dysfunction severity as measured .......

Abstract 12349: Can Biomarkers and Risk Scores Be Used to Select Patients With Type 2 Diabetes Mellitus for Testing for Subclinical Left Ventricular Dysfunction

Background: Subclinical LV dysfunction (LVD) identifies heart failure (HF) risk in type 2 diabetes mellitus (T2DM). Hypothesis: Clinical scores (ARIC HF and WATCH-DM) and NTpBNP can be used as screening tests to identify which people should undergo echo. Methods: 803 asymptomatic subjects (69 years [IQR 65-73], 36% female) with T2DM were prospectively recruited from three community-based populations. All underwent a comprehensive echo to identify abnormal global longitudinal strain (GLS &gt; -16%), diastolic function (E/e’ &gt; 14 or e’&lt;8cm/s), left atrial volume index (LAV&gt;34ml/m 2 ) or LV hypertrophy (LV mass index&gt;88g/m 2 (F)&gt;102g/m 2 (M)). ARIC HF risk at 4 years and WATCH-DM scores were calculated from clinical data. NTpBNP was measured using an electro-chemiluminescence assay. Results: In the cohort, 126 (17%) had an abnormal GLS, 16 (2%) elevated E/e’, 266 (33%) had impaired e’, 351 (44%) had increased LAV and 113 (14%) had LV hypertrophy. Median NTpBNP level was 50pg/mL [25-101]. Clinical scores corresponded to expected HF risk (ARIC-HF 8% [4-12]; WATCH-DM 10 points [8-12]). NTpBNP was weakly associated with GLS after adjustments for age, body-mass index, and renal function (β=0.004 [95% CI 0.003 to 0.004], p&lt;0.001). Similar trends were observed with ARIC HF (β=0.07 [0.05 to 0.09], p&lt;0.001) and WATCH-DM (β=0.19 [0.13 to 0.25], p&lt;0.001) in univariable analyses. None of the risk assessment tools provided a satisfactory AUC for detection of abnormal GLS, diastolic indices or LV mass (Table) ( p =0.804). Development of cut-points to optimize sensitivity (to 90%) led to an unacceptably low specificity (Table). Conclusion: Although risk assessment based on clinical scores or biomarkers would be desirable to stratify HF risk in people with T2DM, they show a weak relationship with subclinical LV dysfunction.

Prevalence of abnormal left ventricular geometry and predictors of left ventricular hypertrophy in patients with rheumatoid arthritis

Abstract Background Rheumatoid arthritis (RA) patients have an excess risk of cardiovascular morbidity and mortality. Left ventricular hypertrophy (LVH) is generally considered a target organ damage of hypertensive patients, but it is an overlooked condition in the RA. In addition, LVH is one-sixth prevalence in RA patients and reflects a greater risk of cardiovascular events. Purpose We aimed to study the prevalence of LVH in patients with RA without symptomatic cardiovascular disease and explore the associated factors of LVH. Methods A cross-sectional study was conducted among adult RA patients and a retrospectively review of echocardiography from the database in non-RA participants. LVH was defined as LV mass index greater than 115 g/m2 in males or 95 g/m2 in females. The prediction model for LVH was constructed by stepwise logistic regression. The prediction model's performance on the test data is evaluated using receiver operator characteristic (ROC) curves to predict the presence of LVH and optimal cut-off point determined by the Youden index. Results This study included 109 RA patients and 119 age- and sex-matched non-RA participants. RA participants showed a significantly greater CV risk factor profile; hypertension, diabetes, and dyslipidemia than non-RA participants. Fifty two of 109 (47.7%) RA patients had abnormal LV geometry; eccentric hypertrophy, concentric remodeling, and concentric hypertrophy were 8/109 (7.3%), 28/109 (25.7%), and 16/109 (14.7%), respectively. The prevalence of LVH in RA patients was comparable to non-RA participants 24/109 (22%) and 28/119 (23.5%), p=0.91, respectively. However, there was significantly higher relative wall thickness in RA than non-RA participants 0.4±0.1 and 0.3±0.1, p&amp;lt;0.001, respectively. The diagnostic performance of the score prediction [(0.025×systolic blood pressure) + (non-steroidal anti-inflammatory drug × (0 or 1)) + (0.141×Troponin T level) ≥3.948] was to predict LVH in RA patients showed 91% sensitivity and 39% specificity (AUC = 0.689, 95% CI 0.564–0.814). Conclusion RA was associated with higher abnormal LV geometry compared to the non-RA participant. LVH is not uncommon in RA patients, and we could consider it as a target organ damage despite no obvious cardiovascular risk factors. The risk predictor score may be applied for fundamental screening tool for LVH in RA patients before sending the patients for echocardiography. Funding Acknowledgement Type of funding sources: None.

Clinical and Laboratory Characteristics of the Multisystem Inflammatory Syndrome in Children: A Case Series of 75 Patients

Background: SARS-CoV-2 has been characterized since December 2019 as the etiology of severe pneumonia throughout the world. However, the majority of children and adolescents with the respective infection have mild COVID-19. In April 2020, a warning was issued by the National Health Service (NHS), based on which a multisystem inflammatory syndrome in children (MIS-C) could be associated with COVID-19, presenting with cardiovascular shock, fever, and hyperinflammation. The syndrome presents with fever and organ involvement but with no pathognomonic findings or diagnostic tests, while some of the manifestations are almost the same as those of Kawasaki disease. Objectives: Knowledge of clinical course, demographic data, treatment, and prognosis can contribute to the more efficient management of the patients and, consequently, a decrease in morbidity and mortality. Methods: Seventy-five patients &lt; 18 years from September 22, 2020, to March 10, 2021, in Namazi hospital, Shiraz, Iran, with a diagnosis as per MIS-C defined criteria, were recruited. Results: Median age of the patients was 6.2 years, and 58.6% were male. Of the patients, 46% had positive SARS-CoV-2 RT-PCR, antibody, or both. Thirty percent of the total patients reported contact with proven COVID-19 cases. The abdominal free fluid in 17 patients, hepatitis in one patient, and stasis in both kidneys of one patient were detected. Upon echocardiography on the first day, 77%, 48%, 21%, and one patient were with tricuspid regurgitation, mitral regurgitation, abnormal LV function, and myocarditis, respectively; however, after 5 - 7 days, the repeated echocardiography revealed 44% of patients with tricuspid regurgitation, 30% with mitral regurgitation, and 6% with abnormal LV function. For the treatment, 18% of the patients received inotropes, 60% ASA, 32% IVIG, 84% glucocorticoids, and 25.3% received furosemide. All of the patients received antibiotics as well. Finally, 97% of the patients were discharged alive, while two cases died. Conclusions: The results of this study suggest the importance of cardiac consultation along with early hospital care during the course of MIS-C in order to prevent the associated short-term and long-term complications.