Wall Of Left Ventricle Research Articles

Abstract 16825: Brahma Related Gene-1 (BRG1) Protein Mediates Helpful Compensatory Response to Retard Development of Heart Failure

Background: Chromatin remodeling can modulate transcription by changing gene accessibility. Brahma Related Gene-1 (Brg1) protein, which is an adenosine triphosphate catalytic subunit that supports chromatin remodeling, has been implicated in development of hypertrophic cardiomyopathy. However, contribution of Brg1 to the development of heart failure (HF) has not been clearly elucidated. Hypothesis: We hypothesize that Brg1 mediates compensatory response to cardiac stress; therefore, loss of Brg1 function will accelerate development of HF. Methods: The myosin light chain-2 Cre/loxP system was used to produce cardiac myocyte specific deletion of Brg1(-/-) mouse model with floxed littermates as control. Results: At baseline, Brg1(-/-) hearts showed mildly reduced left ventricular ejection fraction (LVEF). Aging to 12 months caused LV dilation and further reduction of LVEF in Brg1(-/-). We used trans-aortic constriction (TAC) for pressure overload challenge and chose similarly severe TAC induced pressure gradient as indicated by similarly fast peak flow velocities across the constriction. At 2-weeks post-TAC, Brg1(-/-) hearts exhibited severely reduced LVEF, severely dilated LV, and decreased ability to thicken LV wall during systole. In contrast, control hearts did not dilate and maintained its LVEF with increased LV wall thickness. Thus, Brg1 deletion greatly diminished the heart’s ability to compensate for aging and pressure challenge, thereby accelerating development of heart failure with reduced ejection fraction (HFrEF). Separately, RNA sequence analyses of 12-months aged hearts showed Brg1 deletion caused upregulation of 810 genes including HF associated long noncoding RNA XIST and down regulation of 50 genes, while 3-months old hearts were not significantly different. Conclusion: Brg1 mediates compensatory responses that are helpful to the heart to prevent development of HF; therefore, it is a potential treatment mechanism for HF.

Abstract 19192: Deciphering the Cardioprotective Role of Ufm1 Modification in Cardiac Remodeling

UFM1 (ubiquitin-fold modifier 1) is a novel Ub-like protein that covalently modifies protein substrates via a highly conserved E1 (UBA5)-E2 (UFC1)-E3 (UFL1) enzymatic cascade. UFM1 modification (ufmylation) controls multiple pathophysiological events and is implicated in various human diseases. However, the significance of ufmylation in cardiac health remains poorly understood. We have previously reported that ufmylation is upregulated in compensated, hypertrophic mouse hearts but decreased in failing human hearts. Inhibition of ufmylation by targeted ablation of the E3 UFM1 ligase 1 (UFL1) in mouse hearts resulted in dilated cardiomyopathy under baseline and stress conditions. Targeted deletion of a putative UFM1 target, UFBP1, also evokes endoplasmic reticulum (ER) stress, leading to dilated cardiomyopathy While these exciting data reveal the pivotal role of ufmylation in the maintenance of ER/cardiac homeostasis, it remains unclear whether ufmylation of cardiac proteins is required for heart function and if so, how ufmylation regulates cardiac function. To determine the role of UFM1 modification in cardiomyocytes we have generated cardiac specific UFM1 CKO mice. The UFM1 CKO mice developed dilated cardiomyopathy at resting condition, as indicated by left ventricle (LV) wall thinning, chamber dilatation and impaired contractility at 4 months of age, which were further exacerbated by 6 months. To test the hypothesis that UFM1 protects the heart by modifying cardiac proteins we replenished UFM1-deficient mice with WT and conjugation deficient UFM1 via AAV9. Restoration of WT-UFM1 expression, but not the conjugation-deficient mutant, attenuated cardiac dysfunction of UFM1CKO hearts. It appears that UFM1 modification constraints pathological cardiac remodeling by maintaining ER homeostasis. Taken together, our data show that Ufmylation is a critical post-translational modification that regulates cardiac homeostasis.

Abstract 13013: Predictors of Mortality in Elderly Patients Admitted With Takotsubo Cardiomyopathy and a History of Depression

Introduction: Takotsubo cardiomyopathy(TC) (broken heart syndrome or stress-induced cardiomyopathy) is usually a catecholamine-driven weakening of the left ventricle wall motion due to severe emotional or physical stress. It presents as an acute onset of reversible symptoms resembling acute coronary syndrome without occluded coronary arteries. As emotional stressors often trigger the condition, we aim to evaluate factors that can cause hospital death following admission for TC among elderly patients with depression. Methods: Our study group assessed mortality risk factors in patients ages ≥60 years with a diagnosis of TC and depression from the 2016-2020 National Inpatient Sample. Results: There were a total of 26375 elderly patients who had a diagnosis of TC. Amongst them, 25225 (95.6%) did not survive their hospitalization. After adjusting for variables, we found several factors that increased the odds of all-cause mortality in these patients, such as cirrhosis (aOR 2.093, p<0.01), peripheral vascular disease (PVD)(aOR 1.319, p=0.017), and events of Acute Kidney Injury (AKI)(aOR 3.072, p<0.01). Meanwhile, Females (vs. Males, aOR 0.597, p<0.01), the presence of hypertension(aOR 0.854, p=0.029), lipid disorder (aOR 0.696, p<0.01), smoking (aOR0.74, p<0.01), history of stroke (aOR 0.612, p<0.01) or history of myocardial infarction (aOR 0.536, p<0.01) showed lower odds of mortality among elderly patients admitted with TC. No disparities based on race or insurance forms were observed (Table 1). Conclusions: Our analysis found that patients ages ≥ 60 years with TC had poorer outcomes if they had cirrhosis, PVD, or AKI. While reduced odds of death were seen in females and patients with specific comorbidities such as smoking, hypertension, lipid disorder, history of stroke, or myocardial infarction, further studies, adjusting for the length of diagnosis and the medications being used, should be encouraged to expand and understand our results better.

Abstract 12972: Trained Innate Immunity Through Toll-Like Receptor 4/Interferon Signaling Establishes Cardioprotection in a Stress-Induced Cardiomyopathy Model

Introduction: Although extensively studied, the mechanisms responsible for the development of cardioprotection following preconditioning stimuli are not known. Our group showed that isoproterenol (ISO) mediated cardiac injury induces potent cardioprotection against a second ISO challenge for up to 5 weeks. The lack of an immune response after the second ISO dose and the durability of protection suggested that trained innate immunity might represent a novel mechanism for cardioprotection. Hypothesis: We hypothesized that ISO confers cardioprotection through trained immunity via Toll-like receptor 4 (TLR4) signaling that is activated by damage-associated molecular patterns released by ISO-induced cell necrosis. Methods: Wild-type C57BL/6J mice were intraperitoneally injected with TLR agonists or diluent (saline), and then 300 mg/kg ISO a week later. Mice were assessed before and after receiving ISO via serum cardiac troponin analysis, flow cytometry, and 2-D echocardiography. Results: The TLR4 agonist lipopolysaccharide (LPS), but not TLR1/2 or TLR3 agonists, induced cardioprotection as shown by decreased troponin I release (p<0.01), reduced cardiac neutrophil influx (p<0.05), and decreased left ventricle wall motion abnormalities (p<0.05) following ISO injury compared to saline treated controls . RNA sequencing identified interferon signaling as commonly upregulated (p<0.05) in mouse hearts a week after LPS or ISO pre-treatment. Blocking type I/II interferon receptors partially abolished LPS-induced cardioprotection, while pre-treatment with recombinant interferon β+γ conferred cardioprotection as shown by decreased troponin I release post-ISO (p<0.01). Given that β-glucan reverses the epigenetic tolerizing effects of LPS in immune cells, we treated mice with β-glucan following LPS pre-treatment and observed that the cardioprotection to ISO was abolished. Conclusions: Viewed together this study shows for the first time that TLR4/interferon signaling confers cardioprotection against ISO-induced injury. Our findings further suggest that this cardioprotective response may be secondary to TLR4 mediated epigenetic changes that dampen immune responses following tissue injury, consistent with trained innate immunity.

Abstract 13381: Syncope as a Presentation of Waldenstrom Macroglobulinemia

Syncope as defined by ACC/AHA is defined as a symptom that presents with an abrupt, transient, complete loss of consciousness, associated with inability to maintain postural tone with rapid and spontaneous recovery without clinical features of other non-syncope causes of loss of consciousness, such as seizure, antecedent head trauma, or apparent loss of consciousness. Syncope is a common complaint treated in the emergency department and a major reason for hospital admissions. A rare etiology to this common symptom is Waldenstrom macroglobulinemia. Case: A 49-year-old man presented to the emergency department, referred for hypercalcemia. On review of records, the patient was noticed to have been seen previously in emergency visits for two episodes of syncope and recurrent anemia. The laboratory studies at the time of admission were remarkable for hemoglobin of 7.3, WBC of8.32, hematocrit of 28.5, MCV 77.2, platelets of 124,000. Complete metabolic panel showed a total protein of 13.1, calcium of 11.2 with an albumin of 1.6 and corrected sodium of 117. EKG demonstrated a sinus rhythm with left atrial enlargement and nonspecific T wave abnormalities. Echocardiogram demonstrated a LVEF of 60-65% with normal LV wall motion, posterior wall thickness, and septal wall thickness with grade 1 diastolic dysfunction. CT of the head was unremarkable. Further work up for recurrent microcytic anemia. Bone marrow biopsy was obtained which demonstrated a Waldenstrom macroglobulinemia. Further follow up revealed that the patient has not had any other syncopal episodes since initiation of treatment. Conclusion: Although current literature suggests a broad differential diagnosis for the symptom of syncope, Waldenström macroglobulinemia remains a rare etiology of syncope with an estimated incidence of 1000 to 1500 cases diagnosed per year. Waldenstrom macroglobulinemia can cause hyperviscosity syndrome in about 30 percent of the population with Waldentroms, which when severe and coupled with anemia and plasma volume expansion has been reported as arare cause of syncope. Syncope evaluation should be detailed with history and physical examination and hyper-viscosity syndromes, a medical emergency, should be considered as differential diagnosis of syncope.

Abstract 12798: Cardiovascular Function and Prognostic Relevance of Cardiac Cachexia in Patients With Cancer; a Systematic Review and Meta Analysis

Introduction: Cardiac cachexia (CC), a severe muscle-wasting condition prevalent in advanced cancers, poses significant implications for heart health. Hypothesis: To evaluate the specific alterations in clinical and imaging parameters and assess their prognostic value. Methods: A systematic search was conducted on PubMed, Cochrane, and Embase, covering articles available until June 1, 2023. The outcomes were pooled using a random-effects model, and mean differences (MD) with 95% confidence intervals (CI). Results: Our analysis included a total of 873 patients (670 cancer patients and 203 controls) from five studies. Cancer patients exhibited a significant reduction in LV Ejection Fraction [MD, 2.61 (95% CI: 0.29 to 4.93), I2=82.05%, p=0.03], LV Mass [MD, 26.59 (95% CI: 13.57 to 39.61), I2=0.00%, p<0.001], Left Ventricular Mass Index for height [MD, 8.028 (95%CI: 4.257-11.800), I2=0.00%, p<0.001] and LV Mass Index for Body Surface Area [MD, 9.684 (95%CI: 4.233-15.135), I2=0.00%, p=0.0005] for cancer patients. However, no statistically significant differences were found in Right Ventricular Wall Thickness [MD, -0.065 (95%CI: -0.246 to 0.115), I2=9.88%, p=0.4770], LV Wall Thickness [MD, 0.511 (95% CI: -1.027 to 2.049), I2=78.48%, p=0.5150], Intraventricular Septal thickness [MD, 0.420 (95% CI: -0.804 to 1.644), I2=76.71%, p=0.50], and LV End-Diastolic Volume Index [MD, -0.186 (95% CI: -7.222 to 6.849), I2=74.09%, p=0.9586]. Qualitative analysis: CC causes remodeling and hence, alters cardiac parameters. It is associated with chemo- and radiotherapy, and tumor progression. Furthermore, it is also linked to increased all-cause mortality. Conclusions: The presence of CC is associated with notable changes in various parameters, detectable through routine imaging techniques. Screening for these alterations may hold clinical importance in guiding the selection of appropriate cancer regimens for affected patients.

Abstract 17104: Characterization of a Novel, Clinically Relevant Mouse Model of Peripartum Cardiomyopathy

Pregnancy requires significantly increased cardiac output (~30%) achieved through peripheral vasodilation and increased stroke volume, due to increased end-diastolic volume. Most women resolve these physiological changes within 6 months of delivery; however, some women progress to heart failure late in pregnancy or in the immediate post-partum period. Peri-partum cardiomyopathy (PPCM) is a rare condition of largely unknown etiology which, at its most-severe and life-threatening, requires cardiac transplantation. Pre-eclampsia occurs at much higher rates in the PPCM population (~40%) compared with the generally pregnant population (~2-5%). We have previously shown that transgenic mice expressing the human-specific isoform of the TBXA2R gene (TPβ) spontaneously develop pre-eclampsia accompanied by all the associated hallmarks expected from the human disease. We now show TPβ transgenic (TPβ-Tg) mice are a model of PPCM. Using trans-thoracic echocardiographic imaging, TPβ-Tg mice had no basal phenotype; however, unlike C57/Bl6J (WT) mice that resolved the pregnancy-induced cardiac remodeling, TPβ-Tg mice had exaggerated cardiac dilatation at term (E18.5; P<0.01) which increased in severity over the subsequent 6 months (P<0.005). In addition, TP-Tg mice exhibited reduced ejection fraction (22±13% vs 67% in WT), significant LV wall thinning (1.12±0.16 mm vs 1.6±0.17 mm WT), and loss of LV mass (66.55±9 mg vs 95±11mg WT). Analysis of myocardium of human PPCM patients undergoing heart transplant (n=12) compared to healthy myocardium from non-diseased donors (n=42) by RT-PCR and IHC showed highly expressed TPβ in PPCM patients. In human PPCM samples, there was a significant (p<0.01) dysregulation of genes associated with circadian rhythm as determined by RNAseq analysis. Quantitative PCR analysis of hearts from non-pregnant (WT=8; TPβ-Tg=4) and pregnant (WT=10; TPβ-Tg=13) mice found similar dysregulation of multiple genes associated with circadian rhythm compared to WT mice (p<0.01). This indicates that PPCM may be associated with failure to terminate CLOCK signaling as a result of TPβ activation. Our results support dysregulation of TBXA2R splicing as part of the pathogenesis of PPCM and identify new therapeutic targets for this condition.

Abstract 14137: Stress-Induced Cardiomyopathy After Uncomplicated Permanent Pacemaker Implantation

Stress-induced cardiomyopathy (SCMP) after permanent pacemaker (PPM) implantation is rare. Here, we report an additional case which was initially misdiagnosed as another disease due to atypical symptoms after PPM implantation. Case presentation: A 79-year-old woman with dizziness and dyspnea for one month visited Cardiology Department. She had a history of hypertension, dyslipidemia, diabetes-mellitus and stage IV chronic kidney disease. We confirmed complete atrioventricular block. Left ventricular ejection fraction (LVEF) was normal. PPM implantation was successfully performed without complications. The next day she felt very well with no symptoms. On the second day, she suddenly developed a fever and complained severe left neck pain. She had cough, sputum and general weakness without chest pain. C-reactive protein level increased and systolic blood pressure was decreased. We started antibiotics considering infection. We also checked implantation site, but there were no signs of infection. On the third day, echocardiography showed decreased LVEF (30%), akinesia of apical and medium segments of LV wall with preserved kinetics of basal segments without pericardial effusion (Figure 1). There was no significant stenosis on coronary angiography and we confirmed SCMP. After few days, her blood pressure and symptoms were improved. We prescribed sacubitril-valsartan, bisoprolol and furosemide. At 10 days after implantation, she was discharged with improved symptoms. Conclusion: Our case emphasizes that even successful implantation of PPM without complications can be a potential risk of SCMP. In the absence of typical cardiac symptoms after PPM implantation, a poor general condition may also be considered as a sign of SCMP. Therefore, it is necessary to monitor the patient for at least three more days after PPM implantation.

Abstract 12088: Left Ventricular Non-Compaction, Coronary Artery Fistula, and Infective Endocarditis: A Rare Combination

Description of Case: A 48-year-old man underwent echocardiography for multifocal subacute infarctions found on brain MRI. No intracardiac shunts were found, but prominent LV trabeculae raised suspicion of LV non-compaction (LVNC). LV ejection fraction was 53%, and LV volume was within upper-normal range. Cardiac MRI confirmed these findings, although non-compacted:compacted myocardium ratio was 1.9. (Panel A-B) A right coronary artery to LV fistula was also diagnosed. (Panel C-D) The patient was followed-up uneventfully, but 10 months later was admitted after a generalized seizure episode. Brain MRI detected multiple new hemorrhagic infarcts, and echocardiography revealed a 10.8mmх10.1mm mobile mass and high-speed diastolic flow near the basolateral LV wall. (Panel E-F) Gemella morbillorum was identified on two blood cultures. The patient underwent surgery for fistula closure and vegetation resection. Vegetation pathology and culture however was negative for bacterial growth. Paroxysmal atrial fibrillation was also detected, before discharge with full recovery. Discussion: LVNC and coronary artery fistula (CAF) are both caused by arrest in embryonic myocardial compaction. Despite a hypothetical common origin, reports of co-occurrence are extremely rare. This is the first to report the association of a mild form of LVNC and a single large CAF. This is also the first to report infective endocarditis due to CAF by G. morbillorum . G. morbillorum infective endocarditis is rare, subacute in course, and causes significant destruction. The extensive, prolonged embolism found here has not been reported in other cases of CAF-associated infective endocarditis and may be due to G. morbillorum .

Abstract 12135: Quality of Life in Early-Stage Hypertrophic Cardiomyopathy: A Secondary Analysis of the VANISH Trial

Background: The VANISH trial (Valsartan for Attenuating Disease Evolution in Early Sarcomeric Hypertrophic Cardiomyopathy) showed valsartan improves LV remodeling in early-stage HCM, but its effects on quality of life (QOL) are unknown. A better understanding of QOL may impact patient wellness. Aims: Describe baseline QOL in early-stage HCM. Assess the impact of valsartan on QOL in early-stage HCM. Methods: We evaluated QOL using age-adjusted versions of the Pediatric Quality of Life Inventory in VANISH participants (ages 8-45): 166 with early-stage HCM, 34 with subclinical HCM (sarcomere variant present but normal LV wall thickness/no HCM diagnosis). Demographic, imaging, CPET, and biomarker correlates of baseline QOL were studied. The impact of valsartan on QOL was analyzed in the early-stage HCM cohort. Results: Participants with early-stage HCM reported good baseline QOL, better than an earlier published healthy validation cohort but diminished compared to subclinical participants (mean overall/physical/psychosocial scores 85/87/84 for early-stage, 90/92/89 subclinical, 82/84/81 earlier validation cohort). Race and ethnicity influenced QOL, with non-white and Hispanic participants reporting lower scores (Table 1). Valsartan improved physical QOL (p=0.03), with suggestion of differential effect by sex and pubertal status, but limited power to detect effect modification (Figure 1). Conclusion: Baseline QOL is lower in patients with early-stage HCM compared to subclinical participants despite NYHA I status. Non-white and Hispanic participants and those with lower baseline peak VO 2 may have lower quality of life. Valsartan treatment improved physical QOL.

Abstract 14213: A Retrospective Natural History Study of Danon Disease

Introduction: Danon disease (DD) is a rare X-linked dominant cardioskeletal myopathy caused by mutations in the lysosome-associated membrane protein-2 (LAMP2) gene. The purpose of this study is to characterize disease progression and outcomes in males and females with DD. Methods: Data were from the International Danon Disease Registry that includes patients from the United States and Europe consented between 2005 and 2022. Records were obtained from the first episode of care to the date of enrollment and included demographics, clinical characteristics and outcomes. Chi square and Wilcoxon testing were used for cross-sectional and mixed effects models used for longitudinal analyses. Data were censored after TXP or LVAD. Results: This study included 112 patients with a median follow up of 6.8 yrs (IQR: 2.5-12.8 yrs). Demographics and outcomes are in Table 1. Males were more likely to experience the combined heart failure outcome. At least mild ventricular dysfunction occurred in 30% of patients, and almost all patients had LV hypertrophy. Regarding arrhythmias, WPW and CIED placement were common. Over time, LVEF decreased in both sexes and LV wall thickness did not increase, although LV mass increased in males only. BNP, CPK and AST did not change. [Table 2] Conclusions: DD is a multisystem and progressive disease with potential for cardiac arrhythmias, progressive hypertrophy and ventricular dysfunction. Currently, there are no disease modifying therapies for DD, although gene therapy trials are underway. Understanding the natural history of DD is critical for informing patient selection of therapies, including heart transplantation and new gene modifying therapies.

Use of the intrusion angle to describe the radial orientation of local cardiomyocytes in the left ventricle.

The projected transverse angle and the nonprojected intrusion angle can be used to describe the radial orientation of local cardiomyocytes in the left ventricle wall, although to date their descriptive relevance has not been demonstrated. This paper compares the evolution of the transverse angle and the intrusion angle in five left ventricle wall samples, and investigates in more detail their respective behaviors when the nonprojected helical angle varies. We show that the intrusion angle avoids the "projection" effect, and contrary to the transverse angle, it remains stable whatever the values taken by the nonprojected helical angle, even when this approaches 90°. The intrusion angle is the better choice, rather than the transverse angle, in describing the radial orientation of local cardiomyocytes. Furthermore, the oscillation of the intrusion angle in the samples is assessed, whose results indicate that the intrusion angle's oscillation amplitude and period are regional and related to the local tissue architecture.

Echocardiographic assessment of cardiac function abnormalities and related risk factors in Thai overweight and obese children.

Childhood obesity has become a global concern, with its prevalence steadily increasing over the past decade. This condition negatively impacts the cardiovascular system, increasing the risk of morbidity and mortality in adulthood. This study aimed to identify cardiac function abnormalities and related risk factors among overweight and obese Thai children. A cross-sectional observational study of 70 children with body mass index (BMI) >1 standard deviation above the mean for Thai children was conducted at Naresuan University Hospital. Body fat percentage (%Fat) was measured, and standard transthoracic echocardiography was performed. Metabolic profiles were collected from medical records. The thickness and diameter of the Left ventricle (LV) wall, including the LV posterior wall, LV internal diameter, and interventricular septum (IVS), were significantly increased in both systolic and diastolic phases in obese children. LV concentric hypertrophy and an abnormal E/E' ratio were found in 27% and 34.3% of participants, respectively. Right ventricular systolic dysfunction indicated by abnormal tricuspid annular plane systolic excursion values was found in 55.7% of all participants. In addition, 27.1% of all obese children had pulmonary hypertension. Significant differences in BMI and %Fat were detected between children with abnormal and normal IVS diastolic (IVSd) (P = 0.016). Our univariate and multivariate correlation analyses revealed a significant positive association between abnormal IVSd and %Fat, with an odd ratio (OR) of 1.13 (95% confidence interval [CI]: 1.01-1.27; P = 0.047) and an adjusted OR of 1.17 (95% CI: 1.01-1.36; P = 0.04). Cardiac function abnormalities in childhood obesity exhibit a significant positive correlation with BMI and various cardiac dimensions, including ventricular wall thickness. One important related risk factor for increased IVS thickness is %Fat. Therefore, multidisciplinary management of obesity should be initiated as early as possible to prevent future cardiovascular morbidity and mortality.

Myocardial perfusion segmentation and partitioning methods in personalized models of coronary blood flow

Abstract In this work we present methods and algorithms for construction of a personalized model of coronary haemodynamics based on computed tomography images. This model provides estimations of fractional flow reserve, coronary flow reserve, and instantaneous wave-free ratio taking into account transmural perfusion ratio indices obtained from perfusion images. The presented pipeline consists of the following steps: aorta segmentation, left ventricle wall segmentation, coronary arteries segmentation, construction of 1D network of vessels, partitioning of left ventricle wall, and personalization of the model parameters. We focus on a new technique, which generates specific perfusion zones and computes transmural perfusion ratio according to the quality of available medical images with a limited number of visible terminal coronary vessels. Numerical experiments show that accurate evaluation of stenosis before precutaneous coronary intervention should take into account both fractional flow reserve indices and myocardial perfusion, as well as other indices, in order to avoid misdiagnosis. The presented model provides better understanding of the background of clinical recommendations for possible surgical treatment of a stenosed coronary artery.

ECG/echo indexes in the diagnostic approach to amyloid cardiomyopathy: A head-to-head comparison from the AC-TIVE study

Background and aimsThe discordance between QRS voltages on electrocardiogram (ECG) and left ventricle (LV) wall thickness (LVWT) on echocardiogram (echo) is a recognized red flag (RF) of amyloid cardiomyopathy (AC) and can be measured by specific indexes. No head-to-head comparison of different ECG/echo indexes among subjects with echocardiographic suspicion of AC has yet been undertaken. The study aimed at evaluating the performance and the incremental diagnostic value of different ECG/echo indexes in this subset of patients. MethodsElectrocardiograms of subjects with LV hypertrophy, preserved ejection fraction and ≥ 1 echocardiographic RF of AC participating in the AC-TIVE study, an Italian prospective multicenter study, were independently analyzed by two cardiologists. Low QRS voltages and 8 different ECG/echo indexes were evaluated. Cohort specific cut-offs were computed. ResultsAmong 170 patients, 55 (32 %) were diagnosed with AC. Combination of low QRS voltages with interventricular septum ≥ 1,6 cm was the most specific (specificity 100 %, positive predictive value 100 %) ECG/echo index, while the ratio between the sum of all QRS voltages and LVWT <7,8 was the most sensitive and accurate (sensitivity 94 %, negative predictive value 97 %, accuracy 82 %). When the latter index was added to a model using easily-accessible clinical variables, the diagnostic accuracy for AC greatly increased (AUC from 0,84 to 0,95; p = 0,007). ConclusionsAmong patients with non-dilated hypertrophic ventricles with normal ejection fraction and echocardiographic RF of AC, easily-measurable ECG/echo indexes, mainly when added to few clinical variables, can help the physician orient second level investigations. External validation of the results is warranted.

A Giant Calcified Amorphous Tumor in the Left Ventricle Presentingwith Dyspnea

Introduction: Calcified amorphous tumor (CAT) is a non-neoplastic cardiac tumor, frequently presenting as a a mass with variable sizes. Scare cases of this phenomenon have been reported so far, so its natural history and therapeutic options are still unclear. In fact, CAT diagnosing relies on pathological findings where a definite diagnosis is not reachable until the mass is removed. So, CAT is a retrospectively diagnosed following surgical intervention. Case Presentation Case Presentation: A 72-year-old man was referred for evaluation due to complaints of dyspnea. Initial coronary examinations yielded normal findings. In echocardiography, a 56 × 34 mm mass was seen in the posterior left ventricle wall, along with severe mitral stenosis and moderate to severe mitral regurgitation. The patient subsequently underwent cardiac surgery, during which the mass was completely removed through the left atrium and mitral valve. Then the patient underwent biological heart valve replacement. Post-surgery echocardiography demonstrated the absence of any residual mass, and no complications were reported during the six months follow-up period. Conclusions: The symptoms and complications of CAT during imaging manifest as a calcified mass. Due to the diagnostic reliance on pathological evidence, surgical treatment is the preferred option for excluding any malignancy and resolve and prevent further complications. Follow-up with echocardiography is advised for monitoring.

Abdominal compression as motion management for stereotactic radiotherapy of ventricular tachycardia

Background and purposeStereotactic body radiotherapy (SBRT) has emerged as a promising treatment for patients with ventricular tachycardia (VT) who do not respond to standard treatments. However, the management of respiratory motion during treatment remains a challenge. This study aimed to investigate the effect of abdominal compression (AC) on respiratory induced motion in the heart. Materials and methodsA patient cohort of 18 lung cancer patients was utilized, where two four-dimensional computed tomography (4DCT) scans were performed for each patient, one with and one without AC. The patient setup consisted of an AC plate together with a stereotactic body frame. The left coronary artery, the left anterior descending artery, the lateral wall of the left ventricle, the heart apex, the carina, and the right and left diaphragm were delineated in max expiration and max inspiration phases in both 4DCT scans. The center of mass shift from expiration to inspiration phase was determined to assess the AC’s impact on respiratory motion. ResultsA significant reduction in motion in the superior-inferior direction was found for all heart structures when AC was used. The median respiratory motion of the heart structures decreased by approximately 1–3 mm with AC in the superior-inferior direction, and approximately 60% of the patients had a motion reduction ≥3 mm in the left ventricle wall. ConclusionThese findings suggest that AC has the potential to improve the motion management of SBRT for VT patients, by reducing the respiratory induced motion in the heart.

Adjustment of acquisition arc in cardiac malposition during myocardial perfusion SPECT imaging: computer simulation based on deterministic modeling

ObjectivesTo simulate cardiac malpositions, leftward and rightward shift and dextrocardia, and also to compare distribution of activity of septal and lateral walls of left ventricle acquired in standard acquisition arc and after relevant adjustment. MethodsIn this study, digital phantoms with cardiac malpositions are designed and procedure of acquisition of scan in standard arc (from right anterior oblique to left posterior oblique) and adjusted acquisition arc is simulated. The three situations of malposition including leftward and rightward shift and dextrocardia are considered. For all types, acquisition is conducted in standard and then adjusted arcs (from anterior to posterior and also from right to left for leftward and rightward shifts, respectively, and for dextrocardia, from left anterior oblique to right posterior oblique). All obtained projections are reconstructed using the algorithm of filtered back projection. During forward projection to obtain sinograms, radiation attenuation is also modeled by incorporation of a simplified transmission map to emission map. The resulting tomographic slices of the LV (septum, apex, and lateral wall) are presented visually and are compared by plotting intensity profiles of the walls. Finally, normalized error images are also computed. All the computations are performed in MATLAB software package. ResultsIn transverse slice, septum and lateral wall are attenuated progressively from apex, which is closer to the camera, to the base in similar fashion. In tomographic slices of standard acquisition arc, the septum shows remarkably higher activity compared to lateral wall. However, after adjustment, both seems equally intense and progressively being attenuated from apex to base, similar to that found in phantom with normally positioned heart. Likewise, for the phantom with rightward shift, when the scanning was done in standard arc, the septum is more intense than the lateral wall. And similarly, adjustment of the arc renders both walls equally intense. In dextrocardia, level of attenuation of basal parts of septum and lateral wall is higher in 360° arc compared to adjusted 180° arc. ConclusionAdjustment of acquisition arc exerts perceptible changes in distribution of activity over LV walls which are more compatible with normally positioned heart.

Cardiac hydatidosis complicated by vascular involvement: a case report

BackgroundHydatid disease is a parasitic infection which is endemic in multiple regions across the globe including Indian subcontinent. Although it commonly affects the liver and other solid organs, isolated involvement of the heart is relatively uncommon. Cardiac hydatidosis can be confidently diagnosed on echocardiography, but cross-sectional imaging by computed tomography (CT) or magnetic resonance imaging (MRI) provides accurate information about its location, extent, and multiplicity. Multiple case studies of cardiac hydatid disease have been reported in the literature. However, cardiac hydatidosis with extension into pulmonary vasculature has not been reported commonly. This case report highlights this rare association, with stress upon the altered surgical management.Case presentationA 50-year-old patient presented with atypical chest pain to the cardiology department of a tertiary care hospital where he underwent multiple blood tests as well as electrocardiography (ECG) and echocardiography as part of the routine examination. Echocardiography revealed a large, multicystic mass invading the posterolateral wall of left ventricle (LV) with no internal vascularity and was provisionally diagnosed as hydatid disease. Subsequent CT study confirmed a large multicystic lesion with the presence of another smaller lesion invading the left atrial appendage (LAA). In order to evaluate the vascular and mediastinal invasion, cardiac MRI was then performed which revealed invasion of right pulmonary artery (PA), right superior pulmonary vein (PV) as well as compression of superior vena cava (SVC) by the smaller lesion. A final diagnosis of cardiac hydatidosis with invasion into right PA and right superior PV was made. Treatment plan for the patient changed, who then underwent an additional reconstruction of right PA and right superior PV after cyst removal.ConclusionsCardiac hydatid disease is a rare but potentially lethal condition, which tends to show slow growth and local invasion. The diversity of findings in such rare cases makes individual surgical approach extremely valuable with preoperative radiological imaging becoming indispensable for correct localization and appropriate planning. Our case report highlights rare association of cardiac hydatidosis with invasion of both pulmonary artery and vein, which eventually altered the surgical management of patient.

Energetics of Cardiac Blood Flow in Hypertrophic Cardiomyopathy through Individualized Computational Modeling.

Hypertrophic cardiomyopathy (HCM) is a congenital heart disease characterized by thickening of the heart's left ventricle (LV) wall that can lead to cardiac dysfunction and heart failure. Ventricular wall thickening affects the motion of cardiac walls and blood flow within the heart. Because abnormal cardiac blood flow in turn could lead to detrimental remodeling of heart walls, aberrant ventricular flow patterns could exacerbate HCM progression. How blood flow patterns are affected by hypertrophy and inter-patient variability is not known. To address this gap in knowledge, we present here strategies to generate personalized computational fluid dynamics (CFD) models of the heart LV from patient cardiac magnetic resonance (cMR) images. We performed simulations of CFD LV models from three cases (one normal, two HCM). CFD computations solved for blood flow velocities, from which flow patterns and the energetics of flow within the LV were quantified. We found that, compared to a normal heart, HCM hearts exhibit anomalous flow patterns and a mismatch in the timing of energy transfer from the LV wall to blood flow, as well as changes in kinetic energy flow patterns. While our results are preliminary, our presented methodology holds promise for in-depth analysis of HCM patient hemodynamics in clinical practice.