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Regional variability of longitudinal strain (LS) has been previously described with echocardiography in patients with cardiac amyloidosis (CA), however, the reason for this variability is not completely evident. We sought to describe regional patterns in LS using feature-tracking software applied to cardiovascular magnetic resonance (CMR) cine images in patients with CA, hypertrophic cardiomyopathy (HCM), and Anderson-Fabry's disease (AFD) and to relate these patterns to the distribution of late gadolinium enhancement (LGE). Patients with CA (n = 45) were compared to LV mass indexed matched patients with HCM (n = 19) and AFD (n = 19). Peak systolic LS measurements were obtained using Velocity Vector Imaging (VVI) software on CMR cine images. A relative regional LS ratio (RRSR) was calculated as the ratio of the average of the apical segmental LS divided by the sum of the average basal and mid-ventricular segmental LS. LGE was quantified for the basal, mid, and apical segments using a threshold of 5SD above remote myocardium. A regional LGE ratio was calculated similar to RRSR. Patients with CA had significantly had worse global LS (−15.7 ± 4.6%) than those with HCM (−18.0 ± 4.6%, p = 0.046) and AFD (−21.9 ± 5.1%, p < 0.001). The RRSR was higher in patients with CA (1.00 ± 0.31) than in AFD (0.79 ± 0.24; p = 0.018) but not HCM (0.84 ± 0.32; p = 0.114). In CA, a regional difference in LGE burden was noted, with lower LGE in the apex (31.5 ± 19.1%) compared to the mid (38.2 ± 19.0%) and basal (53.7 ± 22.7%; p < 0.001 for both) segments. The regional LGE ratio was not significantly different between patients with CA (0.33 ± 0.15) and AFD (0.47 ± 0.58; p = 0.14) but lower compared to those with HCM (0.72 ± 0.43; p < 0.0001). LGE percentage showed a significant impact on LS (p < 0.0001), with a 0.9% decrease in absolute LS for every 10% increase in LGE percentage. The presence of marked “relative apical sparing” of LS along with a significant reduction in global LS seen in patients with CA on CMR cine analysis may provide an additional tool to differentiate CA from other cause of LVH. The concomitant presence of a base to apex gradient in quantitative LGE burden suggests that the regional strain gradient may be at least partially explained by the burden of amyloid deposition and fibrosis.

Undetected in many patients, hypertrophic cardiomyopathy (HCM) is a widespread genetic heart disorder. Conventional diagnosis is based on physiological metrics such as blood pressure, imaging techniques, and genetic testing. Detection of HCM is crucial for proper follow‐up, family screening, early treatment, and risk stratification to prevent sudden cardiac death. Therefore, there is an unmet need for fast and reliable diagnostic methods. This study introduces an innovative approach for the noninvasive, rapid, and accurate diagnosis of HCM by detecting patterns of volatile organic compounds (VOCs) in the patient's breath. Breath from 157 volunteers is collected on sorbent tubes and analyzed using a two‐step approach, gas chromatography‐mass spectrometry (GC‐MS), and a developed nano‐based sensor array. Initially, statistically significant differences in VOC patterns among sampled groups are identified using GC‐MS. Then, the sensor array is used to differentiate between HCM patients and controls, resulting in the testing set, with 92.9% accuracy, 75% specificity, and 94.7% sensitivity. The sensors can further classify subcategories of HCM with >70.3% accuracy for all cases in the testing set. These findings show the applicability of the sensors setup and suggest that VOCs may be a promising noninvasive and real‐time HCM diagnosis option. The use of sensor technologies for the diagnosis of hypertrophic cardiomyopathy (HCM) by identification of volatile organic compounds (VOCs) in breath samples. By combining gas chromatography‐mass spectrometry (GC‐MS) and electronic nose (eNose) platforms, specific VOC patterns associated with HCM are detected and analyzed. Results reveal distinct metabolic signatures, supporting sensor‐based, non‐invasive diagnostic approaches for early and accurate detection of HCM.

Hypertrophic cardiomyopathy is a common genetic disorder with a prevalence of 1:500 in the general population. Amongst a varied spectrum of clinical presentations, the most feared complication of this cardiac disorder is sudden cardiac death. Although only a minority of patients with hypertrophic cardiomyopathy who suffer sudden cardiac death or resuscitated cardiac arrest do so during exercise, strenuous physical activity is regarded as an important trigger for these tragic outcomes. Furthermore, during exercise, patients with hypertrophic cardiomyopathy may develop augmentation of left ventricular outflow tract obstruction, myocardial ischemia, diastolic dysfunction and/or inappropriate vasodilation in non-exercising vascular beds. This in turn may lead to exertional dyspnea, chest pain or syncope. Accordingly, patients with hypertrophic cardiomyopathy are disqualified from competitive sports and in many cases are recommended to avoid strenuous physical activity of any kind. Nevertheless, avoidance of physical activity comes with a price. The positive effects of regular exercise have been extensively reported to convey a wide range of benefits including reduced cardiovascular events, weight reduction and improved wellbeing. Therefore, finding the right exercise level that will offer some of the benefits of physical activity without increasing the risk of sudden cardiac death is of utmost importance. In this review, we discuss the current evidence for and against exercise in this patient population and review national guideline recommendations. We also propose alternative fitness strategies including a novel fitness program implemented by our hypertrophic cardiomyopathy center which may be of particular usefulness for hypertrophic cardiomyopathy patients.

Nonsustained ventricular tachycardia (NSVT), defined as ≥3 consecutive ventricular beats at ≥120 beats/min lasting <30 seconds, is an independent predictor of sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HC). Current guidelines recommend 24- to 48-hour Holter monitoring as part of SCD risk stratification. We sought to assess the difference in diagnostic yield of 14-day Holter monitoring compared to 24-48 hours for the detection of NSVT and to assess the prevalence and characteristics of NSVT in patients with HC with prolonged monitoring. We retrospectively analyzed the 14-day Holter monitors of 77 patients with HC from May 2014 to March 2016. Number of episodes and maximal length and rate on each day were recorded. NSVT was detected in 75.3% of patients during 14-day Holter monitoring. The median number of runs was 2 (range 0 to 26 runs). The median number of beats of the longest run was 10.5 (range 3 to 68 beats) with a mean maximum rate of 159.5 ± 20.8.4 beats/min (range 102 to 203 beats/min). First episodes of NSVT were detected throughout the 14 days, with only 22.5% and 44.8% of the episodes captured within the first 24 and 48 hours of monitoring, respectively. In conclusion, prolonged Holter monitoring revealed ≥1 episode of NSVT in 75% of patients with HC of which <50% were detected within the first 48 hours. Hence, prolonged Holter monitoring may be superior for SCD risk stratification in HC. However, the high prevalence of NSVT in this population may limit its utility in evaluating the risk for SCD of the individual patient.

Background. Right ventricular (RV) dysfunction during cancer therapy related cardiotoxicity and its prognostic implications have not been examined. Aim. We sought to determine the incidence and prognostic value of RV dysfunction at time of LV defined cardiotoxicity. Methods. We retrospectively identified 30 HER2+ female patients with breast cancer treated with trastuzumab (± anthracycline) who developed cardiotoxicity and had a diagnostic quality transthoracic echocardiography. LV ejection fraction (LVEF), RV fractional area change (RV FAC), and peak systolic longitudinal strain (for both LV and RV) were measured on echocardiograms at the time of cardiotoxicity and during follow-up. Thirty age balanced precancer therapy and HER2+ breast cancer patients were used as controls. Results. In the 30 patients with cardiotoxicity (mean ± SD age 54 ± 12 years) RV FAC was significantly lower (42 ± 7 versus 47 ± 6%, P=0.01) compared to controls. RV dysfunction defined by global longitudinal strain (GLS < −20.3%) was seen in 40% (n=12). During follow-up in 16 out of 30 patients (23 ± 15 months), there was persistent LV dysfunction (EF < 55%) in 69% (n=11). Concomitant RV dysfunction at the time of LV cardiotoxicity was associated with reduced recovery of LVEF during follow-up although this was not statistically significant. Conclusion. RV dysfunction at the time of LV cardiotoxicity is frequent in patients with breast cancer receiving trastuzumab therapy. Despite appropriate management, LV dysfunction persisted in the majority at follow-up. The prognostic value of RV dysfunction at the time of cardiotoxicity warrants further investigation.

Scar quantification on cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) images is important in risk stratifying patients with hypertrophic cardiomyopathy (HCM) due to the importance of scar burden in predicting clinical outcomes. We aimed to develop a machine learning (ML) model that contours left ventricular (LV) endo- and epicardial borders and quantifies CMR LGE images from HCM patients.We retrospectively studied 2557 unprocessed images from 307 HCM patients followed at the University Health Network (Canada) and Tufts Medical Center (USA). LGE images were manually segmented by two experts using two different software packages. Using 6SD LGE intensity cutoff as the gold standard, a 2-dimensional convolutional neural network (CNN) was trained on 80% and tested on the remaining 20% of the data. Model performance was evaluated using the Dice Similarity Coefficient (DSC), Bland-Altman, and Pearson’s correlation. The 6SD model DSC scores were good to excellent at 0.91 ± 0.04, 0.83 ± 0.03, and 0.64 ± 0.09 for the LV endocardium, epicardium, and scar segmentation, respectively. The bias and limits of agreement for the percentage of LGE to LV mass were low (-0.53 ± 2.71%), and correlation high (r = 0.92). This fully automated interpretable ML algorithm allows rapid and accurate scar quantification from CMR LGE images. This program does not require manual image pre-processing, and was trained with multiple experts and software, increasing its generalizability.

In particular, Doug Wigle developed the crucial principle of dynamic left ventricular outflow tract obstruction in hypertrophic cardiomyopathy, which we take for granted now, but which represents the foundation for surgical intervention to relieve outflow obstruction, reverse heart failure, and restore severely symptomatic patients to normal activity and lifestyle, as well as extended longevity. In this regard, we can remember Doug by his seminal moment in the landmark Great Obstruction Debate in 1966 in New York City at the annual American Heart Association meeting where the very principle of outflow obstruction was under attack.

Left atrial (LA) volumes are known to be increased in hypertrophic cardiomyopathy (HCM) and are a predictor of adverse outcome. In addition, LA function is impaired and is presumed to be due to left ventricular (LV) diastolic dysfunction as a result of hypertrophy and myocardial fibrosis. In the current study, we assess the incremental effect of outflow tract obstruction (and concomitant mitral regurgitation) on LA function as assessed by LA strain. Patients with HCM (50 obstructive, 50 nonobstructive) were compared to 50 normal controls. A subset of obstructive patients who had undergone septal myectomy was also studied. Utilising feature-tracking software applied to cardiovascular magnetic resonance images, LA volumes and functional parameters were calculated. LA volumes were significantly elevated and LA ejection fraction and strain were significantly reduced in patients with HCM compared with controls and were significantly more affected in patients with obstruction. LA volumes and function were significantly improved after septal myectomy. LVOT obstruction and mitral regurgitation appear to further impair LA mechanics. Septal myectomy results in a significant reduction in LA volumes, paralleled by an improvement in function.

The effects of left ventricular (LV) afterload on longitudinal versus circumferential ventricular mechanics are largely unknown. Our objective was to examine changes in LV deformation before and early after aortic valve replacement (AVR) in patients with severe aortic valve stenosis (AS). Paired echocardiographic studies before and early (7 ± 3 days) after AVR were analyzed in 45 patients (age 67 ± 12 years, 49% men) with severe AS and normal LV ejection fraction without segmental wall motion abnormalities. Longitudinal myocardial function was assessed from 3 apical views (average of 18 segments). Circumferential function was assessed at mid and apical levels (averaging 6 segments per view). Strain, strain rate (SR), and LV twist (relative rotation of the mid and apex) were measured using 2-dimensional strain software. Early post-AVR, (1) LV size and LV ejection fraction did not change; (2) longitudinal systolic strain, which was lower than normal before AVR, increased (−12.8 ± 1.7 to −15.9 ± 2.2, P < .05), whereas mid-LV circumferential strain, which was higher than normal, decreased (−27.0 ± 5.1 to −22.3 ± 4.9, P < .05); (3) longitudinal early diastolic SR increased (0.6 ± 0.1 to 0.7 ± 0.2, P < .05), whereas mid-LV circumferential diastolic SR decreased (1.2 ± 0.5 to 1.0 ± 0.3, P < .05); and (4) LV twist increased (3.7° ± 2.1° to 6.1° ± 2.9°, P < .05). Aortic valve stenosis causes differential changes in longitudinal and circumferential mechanics that partially normalize after AVR. These findings provide new insights into the mechanical adaptation of the LV to chronic afterload elevation and its response to unloading.

Hypertrophic cardiomyopathy, a disorder occurring in approximately 1 of every 500 people, causes a broad spectrum of pathological findings and clinical manifestations. 1 , 2 Early observations 2 , 3 emphasized the morphologic features of this disease (e.g., marked septal hypertrophy and subaortic obstruction) and its unfavorable natural history (e.g., progressive symptoms, serious arrhythmias, heart failure, and sudden death). Today the anatomical and clinical expression of the disease is recognized to encompass a wider range of phenotypes, including mild or focal hypertrophy, limited symptoms, and a good prognosis. Molecular genetic studies of familial hypertrophic cardiomyopathy have demonstrated that this autosomal dominant condition is caused . . .