Moderate Left Ventricular Hypertrophy Research Articles

Prognostic Implications of Left Ventricular Hypertrophy and Mechanical Function in Fabry Disease: A Longitudinal Cohort Study

The prognostic value of different grades of left ventricular hypertrophy (LVH) and left ventricular (LV) mechanical function in Fabry disease is unclear. We aimed to evaluate the association between the severity of LVH, LV mechanical function, and clinical outcomes in Fabry disease. We conducted a retrospective cohort study from a single-center registry of adult patients with Fabry disease. Left ventricular mass index (LVMI) was measured by echocardiography. The severity of LVH was categorized by LVMI using the sex-specific cutoff values. Left ventricular mechanical function was measured as LV global longitudinal strain (GLS) by speckle-tracking analysis. The primary outcome was a composite of major adverse cardiovascular events (MACE) at 5years, including heart failure hospitalization, sustained ventricular tachycardia, acute ischemic stroke, and all-cause mortality. The study included 268 patients (age 50.4±15.4years, men 46.6%) with Fabry disease (83.2% IVS4+919G>A mutation), and 106 patients (39.6%) had LVH. Patients with mild, moderate, or severe LVH had 5-year MACE rates of 7.4%, 10%, and 30.5%, respectively (P<.001). Moreover, patients with impaired LV GLS (<14.1%) had a higher 5-year MACE rate than those with preserved LV GLS (32.1% vs 2.4%, P<.001). Severe LVH was an independent predictor of MACE compared with absence of LVH (adjusted hazard ratio, 12.73; 95% CI, 1.3-124.71; P=.03), after adjusting for age, sex, hypertension, hyperlipidemia, atrial fibrillation, renal function, average E/e', enzyme replacement therapy, and LV GLS. Patients with severe LVH and impaired LV GLS had the highest incidence for MACE (log-rank P<.05), irrespective of sex, genotypes, and whether receiving enzyme replacement therapy or not. Sex-specific grading of LVH by LVMI is practical for risk stratification in patients with Fabry disease, and impaired LV GLS further refines the prognostication.

Cholecalciferol, serum calcium and left ventricular hypertrophy in essential hypertension.

Objective: To assess the correlation of vitamin cholecalciferol and serum calcium with left ventricular hypertrophy (LVH) in essential hypertension patients presenting at a tertiary care hospital. Study Design: Cross Sectional Comparative study. Setting: Department of Cardiology and Medicine, Liaquat University of Medical and Health Sciences, Jamshoro, Sindh. Period: July 2019 to January 2021. Material &amp; Methods: 100 diagnosed LVH+ Essential Hypertenison cases and 100 diagnosed essential hypertension (controls) were selected through non- probability purposive sampling. Echocardiography, serum cholecalciferol, calcium, phosphate, parathyroid hormone (PTH), alkaline phosphatase (ALP), urea and creatinine were measured form sera. Devereux’s formula measured the LV thickness LVH. SPSS ver. 21.0 analyzed the descriptive statistics and compared the variables at 95% Confidence interval (P≤0.05). Results: Serum calcium, phosphate and parathyroid hormone were found elevated in LVH cases compared to control (P=0.0001). Cholecalciferol in LVH cases was low noted as 27.3±7.84 ng/dl compared to 39.5±0.43 ng/dl in controls (P=0.0001). Cholecalciferol in mild, moderate and severe LVH cases was descending order as 30.67±7.84, 24.76±7.72 and 14.96±6.12 mm respectively (P-0.0001). Serum cholecalciferol proves inverse association (r= -0.180) with LVH (p=0.003). Conclusion: The present study reports vitamin cholecalciferol deficiency increases the chances of left ventricular hypertrophy through increased secretion of parathyroid hormone that induces de – novo protein synthesis of ventricular myocardium.

Prognostic value of left ventricular hypertrophy in patients with myocardial fibrosis by ECV mapping on cardiac MRI

Abstract Background Cardiovascular magnetic resonance (CMR) can noninvasively identify myocardial fibrosis using extracellular volume (ECV) mapping. In patients with elevated ECV, and without hypertrophic cardiomyopathy (HCM) or cardiac amyloidosis, the prognostic significant of mild-to-moderate left ventricular hypertrophy (LVH) has not been well established. Purpose To determine if the presence of mild to moderate LVH has an impact on major adverse cardiovascular outcomes (MACE) in patients with elevated ECV without HCM or cardiac amyloidosis. Methods This was a retrospective cohort study assessing a group of patients referred for CMR between July 2019 and January 2023 to Mount Sinai Morningside. Patients with ECV&amp;gt;30% and left ventricular wall thickness &amp;lt;1.6 cm (to exclude patients with the possible diagnosis of HCM or cardiac amyloidosis) were considered for inclusion. Those with an established diagnosis of HCM or cardiac amyloidosis were also excluded. The LVH group comprised of patients with mild-to-moderate LVH, defined as a maximum wall thickness of 1.2-1.5 cm. The control group included patients without LVH (wall thickness &amp;lt;1.2 cm). Patients were further stratified by ECV, using the median value for our sample, as "high ECV" (≥35%) or "low ECV" (&amp;lt;35%). The outcome of interest was time-to-first MACE (all-cause death, or incident acute coronary syndrome, acute heart failure, or arrhythmia). Time-to-first MACE within 1 year was compared among groups using unadjusted Kaplan-Meier curves. Additional Cox regression analyses were performed to adjust for significant univariate analysis, including hypertension, hyperlipidemia, diabetes mellitus, use of beta-blocker, use of diuretic, and category of glomerular filtration rate. The results are presented as hazard ratios and 95% confidence intervals. All analyses were performed in SAS Enterprise using α&amp;lt;0.05. Results Out of the cohort, we identified 186 patients that met our selection criteria. The mean age was 61.1 ± 15.5 years, and 58.1% of patients were female. The median follow-up was 175 days in the control group and 115 days in the LVH group. There were 61 MACE (32.8%) in our sample. After adjusting for covariates, mild-to-moderate LVH was associated with 2.2 times the risk of MACE (95%CI 1.3 to 3.8; p=0.0047) at 1 year, compared to patients without LVH. A higher ECV (≥35%) was not significantly associated with worse outcomes within each study arm (p=0.3450). Kaplan Meier survival curves for patients with and without LVH are shown in Figure 1 (p=0.0002). Conclusions In patients with evidence of elevated ECV without underlying cardiac amyloidosis or hypertrophic cardiomyopathy, mild-to-moderate LVH is associated with 2.2 times the risk of MACE compared to normal left ventricular wall thickness.Figure 1

Abstract 18713: The Impact of ECG-AI Design Choices on Clinical Utility: A Deep Dive Into Left Ventricular Hypertrophy, Hypertrophic Cardiomyopathy, and Cardiac Amyloidosis

Introduction: Artificial intelligence applied to electrocardiograms (ECG-AI) enables early and efficient detection of cardiovascular disease. The technical and clinical impact of different approaches to designing ECG-AI remains under-explored. We consider left ventricular hypertrophy (LVH) and examine the impact of disease-specific labeling of minority etiologies of left ventricular wall thickening- hypertrophic cardiomyopathy (HCM) and cardiac amyloidosis (CA) - on ECG-AI performance. Hypothesis: ECG-AI designed to detect HCM or CA outperforms ECG-AI broadly trained to identify LVH due to differences in attention to key ECG features associated with HCM or CA. Methods: Independent neural networks were trained to identify HCM, CA, and LVH. HCM and CA patients were identified using the full depth of EHR data and validated via clinician adjudication (N = 3,025 and 4,251 patients, respectively). Moderate and severe LVH patients (N = 98,144) were identified using echocardiogram results and international LVH criteria. We compared the ability of disease-specific ECG-AI vs. LVH ECG-AI to detect HCM (or CA) by examining model performance within each of the three test sets. Results: 52.6% of HCM patients and 49.5% of CA patients met LVH criteria. Within the HCM test set, the AUROC, sensitivity, specificity, and positive predictive value (PPV) of the HCM ECG-AI were 0.94, 0.88, 0.85, and 60%, respectively, whereas the same for LVH ECG-AI were 0.83, 0.83, 0.64, and 37%. Within the CA test set, CA ECG-AI achieved AUROC, sensitivity, specificity, and PPV of 0.94, 0.81, 0.91, and 69%, respectively, whereas the same for LVH ECG-AI were 0.75, 0.73, 0.68, and 31%. ECGs positive for HCM exhibited higher P wave area, QRS deflection and R wave amplitude compared to LVH-positive ECGs (Cohen's D &gt; 0.75). ECGs positive for CA exhibited lower R wave amplitude/area, QRS deflection and QRS areas (Cohen's D &gt; 0.6). Conclusion: Models trained using carefully labeled HCM or CA identify distinct signals associated with the diseases, resulting in improved disease discrimination compared to a model trained to identify echocardiographic left ventricular thickening. Labeling choices should be communicated transparently to clinicians to ensure safe and effective use of ECG-AI.

Evolving Concept of Aortic Valve Replacement in Asymptomatic Patients With Severe Aortic Stenosis

BackgroundSurgical treatment of asymptomatic severe aortic stenosis (AS) has been gaining attention ever since the results of the Early Surgery Versus Conventional Treatment in Very Severe Aortic Stenosis (RECOVERY) and Aortic Valve replacement versus conservative treatment in Asymptomatic seveRe aortic stenosis (AVATAR) trials showed survival benefits with early surgical aortic valve replacement (SAVR). This study analyzed the long-term clinical and echocardiographic outcomes of SAVR in asymptomatic severe AS. MethodsBetween 2002 and 2020, 272 patients with asymptomatic severe AS and a left ventricular ejection fraction ≥50% underwent SAVR with or without concomitant aortic surgery and met the study criteria. The median follow-up was 8.5 years (interquartile range, 6-12.8 years), for a total of 2584 patient-years. The time course of the left ventricular mass index (LVMI) and the average E/E′ (ratio of the Doppler-derived E wave to the tissue Doppler-derived E′ wave) were assessed using 594 postoperative echocardiograms. The association of preoperative LVMI and average E/E′ with survival was assessed using Cox proportional hazards. ResultsThere was no operative mortality. On longitudinal analyses, LVMI improved in patients who presented with moderate or severe preoperative left ventricular hypertrophy (LVH). However, after the early decline in average E/E′, there was a late increase to greater than upper limit normal, particularly in patients with a preoperative average E/E′≥14. Postoperative survival was 100%, 94%, 84%, and 76% at 1, 5, 10, and 15 years, respectively, comparable to age- and sex-matched expected survival on the basis of the US general population. On adjusted Cox survival analysis, only moderate to severe LVH was associated with a survival penalty (hazard ratio], 2.32; 95% CI, 1.02-5.27; P = .045). ConclusionsIn asymptomatic patients with AS, SAVR restores survival and improves LVH, but patients with diastolic dysfunction are left with persistent dysfunction. Presentation with moderate or severe LVH at the time of surgery translated to a survival penalty. This observational study supports early SAVR in this population before development of LVH, although further investigation is needed.

In search of heart barometer—advantage of left atrial dimension over electrocardiographic indices of left ventricular hypertrophy in arterial hypertension

Aim: Left ventricular (LV) hypertrophy (LVH) is a common and relevant complication of arterial hypertension (AH) and 12-lead electrocardiogram (ECG) is widely used for its preliminary assessment. The aim of the study was to compare the correlations of four ECG-derived criteria of LVH and left atrial (LA) anteroposterior diameter with LVH assessed by echocardiography and expressed as left ventricular mass (LVM) index (LVMI) in search of the most accurate preliminary indicator of LVH. Methods: The study included 61 subjects with AH [age (year) 69 ± 10, 17 females] and 27 without AH, (age 40 ± 9, 10 females) evaluated with 12-lead ECG and transthoracic echocardiography (TTE). As the ECG-based criteria of LVH Sokolow-Lyon index (SLI), Cornell voltage (CV), Cornell product (CP), and Romhilt-Estes point score (RES) system were evaluated. The ECG indices and LA diameter were correlated with LVMI and correlations coefficients were compared. Results: Among ECG-LVH indicators SLI showed the closest correlation with LVMI [rank correlation coefficients (rho) = 0.38, P &lt; 0.0001], followed by CV and CP with rho = 0.33, P = 0.002 and rho = 0.32, P = 0.002, respectively, whereas RES did not correlate significantly with LVMI. The strongest correlation with LVMI was found for the LA diameter with rho = 0.73 and P &lt; 0.0001, showing an even stronger correlation in women—rho = 0.8 (P &lt; 0.0001) vs. rho = 0.65 (P &lt; 0.0001) in men. In the multivariate analysis, the LA was the only independent predictor of the increased LVMI with R2 = 0.52, P &lt; 0.0001. Conclusions: LA diameter outperformed significantly the ECG indices as far as the correlation with LVMI was concerned and emerged as the only independent predictor of mild and moderate LVH in hypertensive patients. Among the ECG criteria, the strongest correlation was shown for SLI, followed by CV and CP indices.

Simultaneous late-gadolinium enhancement and T1 mapping of fibrosis and a novel cell-based combination therapy in hypertensive mice

Fibrosis is a hallmark of chronic hypertension and disrupts the viability of human bone marrow-derived mesenchymal stromal cells (BM-MSCs) post-transplantation. This study thus, determined whether the anti-fibrotic drug, serelaxin (RLX), could enhance the therapeutic effects of BM-MSCs or BM-MSC-derived exosomes (BM-MSC-EXO) in hypertensive mice. Left ventricular (LV) fibrosis in particular was assessed using conventional histological staining and non-invasive cardiac magnetic resonance imaging (CMRI). CMRI was employed using a novel magnetisation prepared 2 rapid acquisition gradient echo (MP2RAGE) sequence to simultaneously perform late gadolinium enhancement imaging and T1 mapping. Adult male C57BL/6 mice were uninephrectomised, received deoxycorticosterone acetate and saline to drink (1 K/DOCA/salt) for 21 days, whilst control mice were given normal drinking water for the same time-period. On day 14 post-injury, subgroups of 1 K/DOCA/salt-hypertensive mice were treated with RLX alone or in combination with BM-MSCs or BM-MSC-EXO; or the mineralocorticoid receptor antagonist, spironolactone. At day 21 post-injury, LV and kidney histopathology was assessed, whilst LV fibrosis and function were additionally analysed by CMRI and echocardiography. 1 K/DOCA/salt-hypertensive mice developed kidney tubular injury, inflammation, fibrosis, and more moderate LV hypertrophy, fibrosis and diastolic dysfunction. RLX and BM-MSCs combined provided optimal protection against these pathologies and significantly reduced picrosirius red-stained organ fibrosis and MP2RAGE analysis of LV fibrosis. A significant correlation between MP2RAGE analysis and histologically-stained interstitial LV fibrosis was detected. It was concluded that the MP2RAGE sequence enhanced the non-invasive CMRI detection of LV fibrosis. Furthermore, combining RLX and BM-MSCs may represent a promising treatment option for hypertensive cardiorenal syndrome.

Abstract 15218: Hereditary Transthyretin Cardiac Amyloidosis in a Patient at High Risk for Plasma Cell Dyscrasias: A Complex Diagnostic Work-Up

Background: Cardiac amyloidosis (CA) is most commonly caused by the infiltration of misfolded amyloid transthyretin (ATTR) or light chain (AL) molecules in the myocardium. The workup for CA requires serum assessment to exclude the rapidly progressive AL subtype before imaging to assess the more common and indolent ATTR form. The diagnostic algorithm has the potential to become complex as in this case about a woman with Sjogren’s syndrome which predisposes to plasma cell dyscrasias. Case: A 68-year-old female with congenital long QT syndrome requiring implantable cardioverter-defibrillator (ICD), bilateral carpal tunnel, and recent diagnosis of Sjogren’s syndrome was found to have new systolic heart failure during workup for interstitial lung disease. Labs revealed pancytopenia, abnormal kappa/lambda ratio (2.25), and polyclonal gammopathy on immunofixation. Echocardiogram showed moderately reduced systolic function with an ejection fraction of 35-40% and moderate concentric left ventricular hypertrophy. Decision-making: Cardiology obtained cardiac magnetic resonance imaging (CMR) that returned indeterminate due to ICD artifact. Hematology performed a bone marrow biopsy that was negative for plasma cell dyscrasia, though stained positive for amyloid on congo red with mass spectrometry revealing the valine 142 isoleucine (Val142Ile) amino acid substitution consistent with hereditary ATTR-CA. To definitively assess for endomyocardial ATTR involvement the patient was sent for cardiac scintigraphy using 99mTc-labeled pyrophosphate which returned negative with a cardiac to contralateral lung uptake ratio of 1.001 and visual grade of 0. With suspicion still high, an endomyocardial biopsy was pursued and stained positive for congo red with mass spectrometry confirming hereditary Val142Ile ATTR-CA, allowing for tafamidis initiation. Conclusion: We present a complex diagnostic case of ATTR amyloid in a patient who is at high risk for plasma cell dyscrasias with an abnormal kappa/lambda ratio, non-diagnostic CMR, and negative nuclear scan. Despite significant advances in imaging techniques allowing for less invasive diagnostics, this case highlights the role of endomyocardial biopsy in complicated cases.

Abstract 15674: Utilization Rates of Follow-Up Testing With Pyp Scan and Cardiac Mri, and Clinical and Demographic Determinants of Their Use Among Patients With Echocardiographic Features of Cardiac Amyloidosis: A Population-Based Case Control Study

Introduction: Cardiac amyloidosis (CA) is an underdiagnosed cause of heart failure. Echocardiography provides an excellent screening tool for cardiac amyloidosis. For patients with echocardiographic findings suggestive of cardiac amyloidosis, it is unknown how frequently follow-up imaging such as 99mTc PYP-scan or cardiac MRI is obtained. Diagnosis rates after a suggestive echocardiogram as well as disparities in rates of follow-up imaging and subsequent CA diagnosis are also unknown. Methods: We extracted all index cardiac echocardiograms at our institutions of adult patients that were suggestive of a possible diagnosis of CA, which was defined as: moderate or worse left ventricular concentric hypertrophy plus grade II or grade III diastolic dysfunction or diastology could not be determined due to arrhythmia in those with atrial fibrillation. Patients with known diagnosis of amyloidosis were excluded. We determined which patients underwent further testing with 99m Tc-PYP scan or cardiac MRI. We performed a population based case control study in which we compared clinical and demographic factors between those who underwent follow-up testing with either PYP scan or cardiac MRI (cases) vs those who did not (controls). Results: Of 1348 echoes that met inclusion criteria, only 110 (8.2%) underwent PYP scan or cardiac MRI. Of those, 10.0 (11%) patients ultimately were diagnosed with CA. Between cases and controls, there was no difference in age, gender, or race/ethnicity. There were no differences in rates of HFrEF or HFpEF between cases and controls. Cases had lower rates of CKD (27% vs. 40%, p=0.01) and ESRD (8.2% vs 15%, p=0.03) and had higher rates of carpal tunnel disease (12% vs. 6%), p&lt;0.05). The rates of cardiologist ordered echocardiograms were similar between cases and controls. In terms of echocardiogram parameters, there were higher rates of grade III diastolic dysfunction (36.7% vs 20.4%, p=0.002) and more frequent rates of severe hypertrophy (36.7% vs. 21.7% p=,0.004)) among cases vs. controls. Conclusions: Among patients with echocardiographic features suggestive of CA, follow-up testing with 99m Tc -PYP scans and Cardiac MRI, and subsequent diagnosis with CA remain low.

The Role of Speckle Tracking for Differentiating Patients with Hypertensive Cardiac Hypertrophy from Athlete’s with Moderate Left Ventricular Hypertrophy

Hypertensive cardiac hypertrophy is a leading cause of morbidity and death, which motivates our study's focus on these issues. Conditions include heart attacks, irregular heartbeats, diastolic dysfunction, heart failure, and sudden cardiac death fall under this category. A concentric cardiac hypertrophy pattern, like that seen in hypertensive hypertrophy, is also seen in athletes who participate in intense strength training. On the other hand, unlike hypertension hypertrophy, which may have dire consequences, athletic hypertrophy is generally seen as a harmless physiologic response. The research aimed to use speckle tracking echocardiography to identify mild LVH in athletes and distinguish it from hypertensive LVH in patients. A total of 105 participants were included in this prospective case control study and were randomly assigned to one of three groups: 45 healthy, inactive people served as the "healthy group." Patients with hypertension-related LVH numbered 30, whereas athletes with concentric LVH numbered 30 in the athletes group. The results showed that there were no statistically significant differences (p>0.05) in age, height, or body mass index between the groups that were analysed. The mean body mass index was significantly different between the three groups (p= 0.005). Heart rate, systolic blood pressure, and diastolic blood pressure all differed significantly (p0.001) across the three groups. The heart rates of the healthy and athletic groups were found to be significantly lower than those of the hypertension group.

PULMONARY AND CORONARIES OF THE HEART SHOULD STAY APART: A CASE OF MULTIPLE CORONARY TO PULMONARY ARTERY FISTULAS

PULMONARY AND CORONARIES OF THE HEART SHOULD STAY APART: A CASE OF MULTIPLE CORONARY TO PULMONARY ARTERY FISTULAS

Improved evaluation of left ventricular hypertrophy using the spatial QRS-T angle by electrocardiography

Electrocardiographic (ECG) signs of left ventricular hypertrophy (LVH) lack sensitivity. The aim was to identify LVH based on an abnormal spatial peaks QRS-T angle, evaluate its diagnostic performance compared to conventional ECG criteria for LVH, and its prognostic performance. This was an observational study with four cohorts with a QRS duration < 120 ms. Based on healthy volunteers (n = 921), an abnormal spatial peaks QRS-T angle was defined as ≥ 40° for females and ≥ 55° for males. In other healthy volunteers (n = 461), the specificity of the QRS-T angle to detect LVH was 96% (females) and 98% (males). In patients with at least moderate LVH by cardiac imaging (n = 225), the QRS-T angle had a higher sensitivity than conventional ECG criteria (93–97% vs 13–56%, p < 0.001 for all). In clinical consecutive patients (n = 783), of those who did not have any LVH, 238/556 (43%) had an abnormal QRS-T angle. There was an association with hospitalization for heart failure or all-cause death in univariable and multivariable analysis. An abnormal QRS-T angle rarely occurred in healthy volunteers, was a mainstay of moderate or greater LVH, was common in clinical patients without LVH but with cardiac co-morbidities, and associated with outcomes.

Renal Transplantation in Pure Autonomic Failure.

Renal Transplantation in Pure Autonomic Failure.

P317 FATAL EVOLUTION IN A YOUNG WOMAN DUE TO AMYLOIDOSIS HEART FAILURE IN A RARE MULTIPLE MYELOMA DISEASE

Abstract Introduction Immunoglobulin (Ig) D (IgD) monoclonal gammopathy is a rare subtype of multiple myeloma (MM) associated with a worse prognosis. Ig light chains amyloidosis is a disorder characterized by extracellular deposition of Ig light chains in various tissues, leading to organ dysfunction. Case A 29–year–old woman was admitted for dyspnea and tachycardia for moderate efforts. Lab revealed hypogammaglobulinemia with a monoclonal IgD K paraprotein. There was an increase in K free chains, with a dramatic increase in K/λ free chains Ratio. Proteinuria was 1360 mg/24h with a free K chains Bence Jones Protein. An increase in values of Troponin–I (137,5 pg/mL) and NT–proBNP (12527 pg/mL) pointed towards heart involvement. Furthermore, the ECG showed low voltages in the peripheral leads. Echocardiogram showed a moderate concentric left ventricular hypertrophy with diffused myocardial speckled pattern, an ejection fraction (EF) of 53% and PAPs of 71 mmHg and pericardial effusion. Chest CT scan showed bilateral pleural effusion. Spine MRI and whole body PET/CT indicated areas of osteolytic lesions. Plasma cells infiltration (85% CD138, MUM1 and K chains positivity) was present at bone marrow biopsy. Amyloid deposition was detected in abdominal fat tissue sample. Total bone scintigraphy excluded a transthyretin heart deposition. Thus, amyloidosis associate to IgD MM was diagnosed. In few days heart failure worsened (hs–cTnI 156,6 pg/mL; NT–proBNP 26583 pg/mL, EF 48%,) and the patient began complaining non–productive cough, dyspnea, and columnar edema of the lower limbs. She was not eligible for bone marrow transplantation, so daratumumab, bortezomib, melphalan and prednisone were administered. Despite serological improvement after a short five days course of therapy, she worsened with a further reduction of EF (35%). Blood gas analysis showed hypoxemia and lactate increase quickly turned into a cardiogenic shock. She died by cardiac arrest, just three weeks after admission. Discussion The present case is remarkable for age and gender of the patient as well as the rapid onset and fast worsening of symptoms, which were related to secondary heart amyloid deposition. Symptoms associated to cardiac amyloidosis are expressions of right heart involvement. The early good results of the therapy did not turn the evolution of disease. The fatal and overwhelming progression of the myocardial involvement led to the patient’s death in less than one month.

Paroxysmal atrial fibrillation and the left heart structure and function in hypertensive patients

Objective. To identify the features of the left heart structure and function in patients with hypertension (HTN) and new onset paroxysmal atrial fibrillation (AF).Design and methods. We included HTN patients with left ventricular hypertrophy and valid 24-hour Holter monitoring carried out over the last month. Patients without AF paroxysms (age from 37 to 82 years, 38,4% men) were divided into two groups: the 1st group included 32 patients under 55 years of age, the 2nd group comprised 63 patients 55 years of age and older. Thirty-eight patients with AF paroxysms were included in the 3rd group (age from 56 to 74 years, men 28,9%). The parameters of the left heart structure and function, including the global longitudinal strain of the left ventricle and the left atrium strain in the reservoir and contraction phases, were assessed by echocardiography.Results. The average age of patients in the groups was 44,7 46,4 48,2 vs 61,9 63,7 65,4 vs 63,4 64,8 66,2 years (p &lt; 0,001), and the average duration of HTN was 3,2 4,35,6 vs 6,9 7,99,1 vs 5,7 6,98,1 years (p &lt; 0,001), respectively. The moderate and severe left ventricular hypertrophy and the diastolic dysfunction were found significantly less frequently in the 1st group than in other groups (5,0 12,528,1% vs 43,3 55,667,8% vs 36,8 52,668,5%, p &lt; 0,001 and 5,2 18,832,3% vs 51,6 63,575,4% vs 53,6 68,483,2%, p &lt; 0,001). In 1st group, the left atrial reservoir strain was higher than in the 2nd group, and in the 2nd group it was higher than in the 3rd group (24,7 26,3 28,0 vs 21,5 22,7 23,9 vs 17,8 19,2 20,5, p &lt; 0,001). The left atrial contraction strain did not differ in the 1st and 2nd groups and it was higher than in the 3rd group (13,8 14,9 16,0 and 12,9 13,6 14,2 vs 9,6 10,3 11,1, p &lt; 0,001). The multivariate logistic regression analysis showed that the left atrial strain in the contraction phase is a predictor of AF occurrence in HTN patients over 55 years old depends on. The chances of AF detecting in these patients increase by 3,122 9,21127,171 times (p &lt; 0,001), if the left atrial strain in the contraction phase is below 13%.Conclusions. The left atrial strain in HTN patients with paroxysmal AF are significantly lower than in patients without cardiac arrhythmias independently of age and the left ventricular state. The left atrial contraction strain ≤ 13% is a predictor of the detection of AF paroxysms during 24-hour Holter monitoring in HTN patients aged 55 years and older.

585 Natural history of left ventricular hypertrophy in infants of diabetic mothers

Abstract Aims This study sought to describe the characteristics and the natural course of left ventricular hypertrophy (LVH) in a well-characterized consecutive cohort of infants of diabetic mothers (IDMs). Methods and results Sixty consecutive IDMs with LVH have been retrospectively identified and enrolled in the study. All IDMs were evaluated at baseline and every 6 months until LV wall thickness regression, defined as the decrease of wall thickness measurement into the normal reference range for cardiac parameters (z-score &amp;gt; −2 and &amp;lt;2). A comprehensive assessment was performed in those patients with diagnostic markers suggestive of a different cause and/or without significant reduction of the LVH during follow-up. At 1-year follow-up, all IDMs showed a significant reduction of maximal wall thickness MWT [6.00 mm (IQR: 5.00–712) vs. 5.50 mm (IQR: 5.00–6.00), P-value &amp;lt;0.001; MWT-z-score: 4.86 (IQR: 3.93–7.61) vs. 1.72 (IQR: 1.08–2.85), P-value &amp;lt;0.001] compared to baseline, and all patients showed LV wall thickness regression or residual mild or moderate LVH (57%, 28%, and 12%, respectively), except two patients with persistent severe LVH, that after a comprehensive clinical-genetic assessment were diagnosed as Noonan syndrome with multiple lentigines. At multivariate analysis, MWT was negatively associated with LV wall thickness regression at 1-year follow-up [MWT-mm: OR: 0.48 (0.29–0.79), P-value = 0.004; MWT-z-score: OR: 0.71 (0.56–0.90), P-value = 0.004]. Conclusions LVH in IDMs represents a benign condition with complete regression during the first years of life. In those patients without LV wall thickness regression, combined with clinical markers suggesting a specific disease, a complete work-up is required for a definite diagnosis.

416 A very severe aortic stenosis secondary to radiation therapy for Hodgkin lymphoma: a case report

Abstract Aims Hodgkin lymphoma is a highly curable disease, with survival rates around 85% at 5 years, thanks to its treatment, mainly based on radiation therapy alone or in association with chemotherapy. However, radiation therapy carries the risk of long-term complications, mainly represented by secondary malignancies or cardiovascular diseases. Indeed, patients treated with radiation remain at increased risk of coronary artery disease, congestive heart failure, valve disease, pericardial disease and sudden death. In particular, radiation-induced valve disease has been reported in approximately 10% of treated patients, with the most common being aortic or mitral regurgitation, followed by aortic stenosis. We describe the case of a very severe aortic valve stenosis developed 35 years after radiotherapy for Hodgkin Lymphoma. Methods A 60-years-old woman with history of Hodgkin Lymphoma treated with radiotherapy 35 years ago, no cardiovascular risk factors, complained progressive fatigue and worsening dyspnoea. She was referred to our centre from a spoke centre for congestive heart failure caused by aortic stenosis. At her arrival, we performed a transthoracic echocardiography finding a very-severe aortic stenosis (mean gradient 97 mmHg, AVA 0.3 cm2), a moderate functional mitral regurgitation and an important hypertrophy suspected of infiltrative pattern. No coronary artery disease was found at coronary angiography. Furthermore, she underwent a cardiac magnetic resonance showing a moderate left ventricular dilatation and hypertrophy with areas of non-ischaemic late gadolinium enhancement, significant for myocardial fibrosis with no specific pattern of infiltration. She was treated with surgical aortic valve replacement with biological prosthesis (Inspiris Resilia 21) with good results at the discharge echocardiography. Discussion Cardiovascular toxicity is nowadays the most common non-malignancy cause of death in radio-treated cancer survivors. At the level of the valvular apparatus, radiation damage is expressed with fibrosis of the myocardium and of the valvular endothelium, leading to thickening and calcification especially of the left-sided valves. Radio-treated patients have a 50% cumulative incidence of cardiovascular diseases at 40 years after treatment, and a clinically-relevant valve disease is found in approximately 6% of radio-treated patients at 20 years. Conclusions Thus, an early identification of the disease in a subclinical state is crucial. Nevertheless, there is still no consensus on the timing and the modality of cardiovascular screening after radiation therapy. Moreover, an elevated cardiovascular risk profile was found to be directly related to cardiotoxicity; this underlines the importance of identification and aggressive treatment of cardiovascular risk factors. Radiation dose reduction and the use of shields may further reduce the incidence of late cardiovascular complications in these patients.

AVOIDING ANCHORING BIAS: DIAGNOSIS OF AL-AMYLOIDOSIS IN WORSENING HEART FAILURE AFTER CORONARY ARTERY BYPASS GRAFTING

TOPIC: Cardiovascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Amyloidosis is a systemic disease caused by deposition of misfolded protein in various tissues and organs. Early recognition of amyloidosis is necessary as cardiac dysfunction is the major determinant of survival(1). The diagnosis is often delayed given the non-specific symptoms at onset. Prognosis in amyloidosis is primarily dependent on the degree of cardiac involvement and, in AL amyloidosis, on circulating light-chain levels(2). CASE PRESENTATION: The patient is a 68-year-old male referred to our institution for assessment of worsening heart failure (HF). Four months prior, he began to experience exertional dyspnea along with jaw pain. Subsequent evaluation included lab work, chest X-ray and electrocardiogram demonstrating troponin elevation, pulmonary edema and complete heart block respectively. An echocardiogram(ECHO) demonstrated a severely reduced ejection fraction(EF). Temporary pacemaker was placed and a subsequent angiogram showed severe multi-vessel coronary artery disease(CAD). He underwent coronary artery bypass graft surgery. Post-operatively EF improved to 45%. Implantable loop recorder was placed due to concerns for post-operative atrial fibrillation. Three months later, he experienced a syncopal event attributed to ventricular tachycardia, and CRT-D was placed. ECHO revealed reduction of his EF (30%) with moderate concentric left ventricular hypertrophy(LVH). A repeat angiogram demonstrated patency of his bypass grafts. After referral to our institution, a repeat ECHO demonstrated findings concerning amyloidosis. Protein electrophoresis revealed monoclonal IgG gammopathy, and fat pad aspirate confirmed the diagnosis of AL amyloidosis. DISCUSSION: Although CAD is the most common cause of HF with reduced EF, other etiologies must be considered when symptoms cannot be explained solely by ischemia. Cardiac amyloidosis was suspected based on a constellation of clinical symptoms as well as suggestive ECHO findings(Biventricular concentric hypertrophy, restrictive filling pattern, Bull's eye appearance on LV strain). The diagnosis should be suspected in patients with HF who have unexplained LVH with no prior history of hypertension(3). Patients also present with syncopal events caused by conduction blocks, bradyarrhythmia, or in the case of our patient, ventricular tachyarrhythmias. Early diagnosis is important given the emerging therapies that can halt cardiac damage, potentially reducing the risk of mortality and allowing amyloid clearance and improved cardiac function. CONCLUSIONS: Anchoring bias is the tendency to stick with initial impressions even as new information becomes available. Clinicians must avoid this and consider alternative diagnoses when patients do not experience the anticipated outcome to a particular treatment. REFERENCE #1: 1. Fontana M. Banypersad SM. Treibel TA, et al. Differential myocyte responses in patients with cardiac transthyretin amyloidosis and light-chain amyloidosis: a cardiac MR imaging study. Radiology. 2015;277:388–97 REFERENCE #2: 2. Kumar S, Dispenzieri A, Lacy MQ, et al. Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements. J Clin Oncol 2012;30:989-95 REFERENCE #3: 3. Gertz MA. Comenzo R. Falk RH, et al. Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis, Tours, France, 18-22 April 2004. Am J Hematol. 2005;79:319–28 DISCLOSURES: no disclosure on file for Brian Hardaway; No relevant relationships by Michael Liu, source=Web Response No relevant relationships by Amith Reddy Seri, source=Web Response

Massive myocardial calcification: a heart of stone!

Abstract A 71-year-old female was admitted in the emergency room after effort-related syncope. She had past medical history of obesity and hypertension treated with lercanidipine. No relevant family history. Physical examination revealed systolic murmur (grade 2/6). ECG showed sinus rhythm and left bundle brunch block (Supplementary figure). Lab results were unremarkable including troponin I. Transthoracic echocardiography (TTE) revealed moderate left ventricular (LV) hypertrophy [septal thickness 14 mm (normal: 6–9mm)], extensive mitral annulus calcification (MAC) with exuberant myocardial calcification at the level of posterior leaflet that invaded adjacent LV walls, systolic anterior motion of the anterior mitral leaflet causing LV outflow tract (LVOT) obstruction (maximum gradient 34 mmHg) and moderate mitral regurgitation (MR) (Panel A). Exercise echocardiogram provoked LVOT gradient &amp;gt;100mmHg and severe MR. Continuous ECG monitoring during 13 days demonstrated no arrhythmic events. Cardiac CT showed multiple calcifications extending from mitral annulus to LV anterior and lateral walls and cardiac MRI was compatible with caseous MAC (Panels B-F). Coronary angiography showed 50% stenosis of mid left anterior descending artery. Normal thoracic CT and ACE, and negative IGRA excluded sarcoidosis and tuberculosis. Comprehensive study, including calcium metabolism and autoantibodies, excluded metabolic and inflammatory aetiologies of myocardial calcification, which was assumed as dystrophic in the context of MAC. No syncope occurred after bisoprolol 2.5 mg, oral rehydration and discontinuation of lercanidipine. However, rest and exercise TTE failed to show improvement of LVOT gradient or MR and patient remained on NYHA class II-III, despite maximal tolerated dose of bisoprolol (5 mg), being therefore referred to cardiac surgery.

Randomized controlled trial of perhexiline on regression of left ventricular hypertrophy in patients with symptomatic hypertrophic cardiomyopathy (RESOLVE-HCM trial)

The presence and extent of left ventricular hypertrophy (LVH) is a major determinant of symptoms in patients with hypertrophic cardiomyopathy (HCM). There is increasing evidence to suggest that myocardial energetic impairment represents a central mechanism leading to LVH in HCM. There is currently a significant unmet need for disease-modifying therapy that regresses LVH in HCM patients. Perhexiline, a potent carnitine palmitoyl transferase-1 (CPT-1) inhibitor, improves myocardial energetics in HCM, and has the potential to reduce LVH in HCM. The primary objective is to evaluate the effects of perhexiline treatment on the extent of LVH, in symptomatic HCM patients with at least moderate LVH. RESOLVE-HCM is a prospective, multicenter double-blind placebo-controlled randomized trial enrolling symptomatic HCM patients with at least moderate LVH. Sixty patients will be randomized to receive either perhexiline or matching placebo. The primary endpoint is change in LVH, assessed utilizing cardiovascular magnetic resonance (CMR) imaging, after 12-months treatment with perhexiline. RESOLVE-HCM will provide novel information on the utility of perhexiline in regression of LVH in symptomatic HCM patients. A positive result would lead to the design of a Phase 3 clinical trial addressing long-term effects of perhexiline on risk of heart failure and mortality in HCM patients.