Obstruction in Hypertrophic Cardiomyopathy: Many Faces.

Hypertrophic cardiomyopathy (HCM), the most common inherited cardiomyopathy, exhibits left ventricular hypertrophy not secondary to other causes, with varied phenotypic expression. Enhanced actin-myosin interaction underlies excessive myocardial contraction, frequently resulting in dynamic obstruction within the left ventricle. Left ventricular outflow tract obstruction, occurring at rest or with provocation in 75% of HCM patients, portends adverse prognosis, contributes to symptoms, and is frequently a therapeutic target. Transthoracic echocardiography plays a crucial role in the screening, initial diagnosis, management, and risk stratification of HCM. Herein, we explore echocardiographic evaluation of HCM, emphasizing Doppler assessment for obstruction. Echocardiography informs management strategies through noninvasive hemodynamic assessment, which is frequently obtained with various provocative maneuvers. Recognition of obstructive HCM phenotypes and associated anatomical abnormalities guides therapeutic decision-making. Doppler echocardiography allows monitoring of therapeutic responses, whether it be medical therapies (including cardiac myosin inhibitor therapy) or septal reduction therapies, including surgical myectomy and alcohol septal ablation. This article discusses the hemodynamics of obstruction and practical application of Doppler assessment in HCM. In addition, it provides a visual atlas of obstruction in HCM, including high-quality figures and complementary videos that illustrate the many facets of dynamic obstruction.

Progression of Mild Mitral Annulus Calcification to Mitral Valve Dysfunction and Impact on Mortality.

BACKGROUND: Mitral annulus calcification (MAC) represents a degenerative process resulting in calcium deposition in the mitral valve apparatus. Mitral annulus calcification is associated with adverse clinical outcomes. We sought to examine the long-term significance of mild MAC and its relationship to subsequent mitral valve dysfunction (MVD) and mortality in patients without MVD on the initial echocardiogram. METHODS: A total of 1,420 patients with mild MAC and no MVD at baseline and 1 or more follow-up echocardiograms at least 1 year after the baseline echocardiogram were included in the analysis. For patients with >1 echocardiogram during follow-up, the last echocardiogram was used. The same criteria were used to identify 6,496 patients without MAC. Mitral valve dysfunction was defined as mitral regurgitation (MR) and/or mitral stenosis (MS) of moderate or greater severity. Mixed disease was defined as the concurrent presence of both moderate or greater MS and MR. The primary end point was development of MVD, and the secondary end point was all-cause mortality. RESULTS: For patients with mild MAC, age was 74 ± 10 years and 528 (37%) were female. Over a median follow-up of 4.7 (interquartile range, 2.7-6.9) years, 215 patients with mild MAC developed MVD, including MR in 170 (79%), MS in 37 (17%), and mixed disease in 8 (4%). In a multivariable regression model compared to patients without MAC, the presence of mild MAC was independently associated with increased mortality (hazard ratio = 1.43; 95% CI 1.24, 1.66; P < .001). Kaplan-Meier 4-year survival rates were 80% and 90% for patients with mild MAC and no MAC, respectively. CONCLUSIONS: Mild MAC observed on transthoracic echocardiography is an important clinical finding with prognostic implications for both valvular function and mortality.

Clinical Outcomes Among Immunotherapy-Treated Patients With Primary Cardiac Soft Tissue Sarcomas: A Multicenter Retrospective Study.

Background: Primary cardiac soft tissue sarcomas (CSTS) affect young adults, with dismal outcomes. Objectives: The aim of this study was to investigate the clinical outcomes of patients with CSTS receiving immune checkpoint inhibitors (ICIs). Methods: A retrospective, multi-institutional cohort study was conducted among patients with CSTS between 2015 and 2022. The patients were treated with ICI-based regimens. The Kaplan-Meier method was used to estimate overall survival (OS) and progression-free survival (PFS). Objective response rates were determined according to Response Evaluation Criteria in Solid Tumors version 1.1. Treatment-related adverse events were graded per the Common Terminology Criteria for Adverse Events version 5.0. Results: Among 24 patients with CSTS, 17 (70.8%) were White, and 13 (54.2%) were male. Eight patients (33.3%) had angiosarcoma. At the time of ICI treatment, 18 patients (75.0%) had metastatic CSTS, and 4 (16.7%) had locally advanced disease. ICIs were administered as the first-line therapy in 6 patients (25.0%) and as the second-line therapy or beyond in 18 patients (75.0%). For the 18 patients with available response data, objective response rate was 11.1% (n = 2 of 18). The median PFS and median OS in advanced and metastatic CSTS (n = 22) were 5.7 months (95% CI: 2.8-13.3 months) and 14.9 months (95% CI: 5.7-23.7 months), respectively. The median PFS and OS were significantly shorter in patients with cardiac angiosarcomas than in those with nonangiosarcoma CSTS: median PFS was 1.7 vs 11 months, respectively (P < 0.0001), and median OS was 3.0 vs 24.0 months, respectively (P = 0.008). Any grade treatment-related adverse events occurred exclusively in the 15 patients with nonangiosarcoma CSTS (n = 7 [46.7%]), of which 6 (40.0%) were grade ≥3. Conclusions: Although ICIs demonstrate modest activity in CSTS, durable benefit was observed in a subset of patients with nonangiosarcoma, albeit with higher toxicity.

Case Report: A myxoma with a far reach.

A 73-year-old woman presented to the emergency department with a syncopal episode and a history of dizzy spells. A transthoracic echocardiogram demonstrated a large left atrial mass extending into the right upper pulmonary veins. Subsequently, cardiac magnetic resonance imaging and coronary computed tomography angiography with three-dimensional reconstruction and printing of the heart and mass were performed, which demonstrated a high index of suspicion for an atypical left atrial myxoma. The mass was excised robotically, and the pathology report confirmed a diagnosis of myxoma.

Outcomes of transcatheter aortic valve replacement in patients with and without amyloidosis: A nationwide analysis from the United States.

BACKGROUND: Amyloidosis is a common comorbidity in elderly patients with aortic stenosis (AS) referred for transcatheter aortic valve replacement (TAVR). This study aims to assess the impact of amyloidosis on the clinical outcomes of TAVR. METHODS: This is a retrospective study of the National Inpatient Sample database that identified adult patients (≥18 years) with AS hospitalized for TAVR from 2016 through 2020 to compare outcomes in those with versus without amyloidosis. Our primary outcome was in-hospital mortality. Secondary outcomes included procedural complications, hospital length of stay (LOS), and total costs. TAVR trends in both cohorts were also evaluated. RESULTS: The total cohort included 304,710 patients with AS undergoing TAVR, of whom 410 had amyloidosis. Over the study period, TAVR trends increased significantly in patients with and without amyloidosis (both ptrend < 0.01). Patients with amyloidosis were more likely to be older males with atrial fibrillation/flutter, congestive heart failure, renal disease, and dementia compared to non-amyloidosis patients. After adjustment for baseline characteristics, patients with amyloidosis had similar odds of in-hospital mortality (adjusted odds ratio [aOR] 1.66, 95 % confidence interval [CI] 0.34-3.63), heart block (aOR 1.33, 95 % CI 0.84-2.10), permanent pacemaker insertion (aOR 0.67, 95 % CI 0.27-1.66), stroke (aOR 0.90, 95 % CI 0.32-3.13), acute kidney injury, major bleeding, blood transfusion, vascular complications, in addition to similar LOS (p = 0.21) and total costs (p = 0.18) compared to patients without amyloidosis. CONCLUSION: In patients with AS undergoing TAVR, comorbid amyloidosis is associated with similar in-hospital mortality and procedural complications compared to patients without amyloidosis.

Detection of Early Myocardial Dysfunction by Imaging Biomarkers in Cancer Patients Undergoing Photon Beam vs. Proton Beam Radiotherapy: A Prospective Study.

1. Background: We sought to determine acute and subacute changes in cardiac function after proton beam (PBT) and photon beam (PhT) radiotherapy (RT) using conventional and two-dimensional speckle tracking echocardiography (2D-STE) in patients with malignant breast and thoracic tumors. 2. Methods: Between March 2016 and March 2017, 70 patients with breast or thoracic cancer were prospectively enrolled and underwent transthoracic echocardiography with comprehensive strain analysis at pretreatment, mid-treatment, end of treatment, and 3 months after RT. 3. Results: PBT was used to treat 44 patients; PhT 26 patients. Mean ± SD age was 55 ± 12 years; most patients (93%) were women. The median (interquartile range) of the mean heart dose was lower in the PBT than the PhT group (47 [27-79] vs. 217 [120-596] cGy, respectively; p < 0.001). Ejection fraction did not change in either group. Only the PhT group had reduced systolic tissue Doppler velocities at 3 months. 2D-STE showed changes in endocardial and epicardial longitudinal, radial, and circumferential early diastolic strain rate (SRe) in patients undergoing PhT (global longitudinal SRe, pretreatment vs. end of treatment (p = 0.04); global circumferential SRe, pretreatment vs. at 3-month follow-up (p = 0.003); global radial SRe, pretreatment vs. at 3-month follow-up (p = 0.02) for endocardial values). Epicardial strain values decreased significantly only in patients treated with PhT. Patients in the PhT group had a significant decrease in epicardial global longitudinal systolic strain rate (GLSRs) (epicardial GLSRs, at baseline vs. at end of treatment [p = 0.009]) and in GCSRe and GRSRe (epicardial GCSRe, at baseline vs. at 3-month follow-up (p = 0.02); epicardial GRSRe, at baseline vs. at 3-month follow-up (p = 0.03)) during treatment and follow-up. No changes on 2D-STE were detected in the PBT group. 4. Conclusions: Patients who underwent PhT but not PBT had reduced tissue Doppler velocities and SRe values during follow-up, suggesting early myocardial relaxation abnormalities. PBT shows promise as a cardiac-sparing RT technology.

Ultrasound Enhancing Agents with Transthoracic Echocardiography for Maximal Wall Thickness in Hypertrophic Cardiomyopathy.

Objectives: To determine whether ultrasound enhancing agent (UEA) changes maximal wall thickness (WT) in hypertrophic cardiomyopathy (HCM), and if it improves correlation with magnetic resonance imaging (MRI). Patients and Methods: A total of 107 patients with HCM were prospectively enrolled at a single tertiary referral center between July 10, 2014, and August 31, 2017, and underwent transthoracic echocardiography (TTE) with and without UEA and MRI. Maximal WT measurements were compared, and variability among the 3 modalities was evaluated using a simple linear regression analysis and paired t tests and Bland-Altman plots. Interobserver variability for each technique was assessed. Results: Most (63%) of cardiac imagers found UEA helpful in determining maximal WT by TTE, with 49% reporting change in WT. Of 52 patients where UEA changed WT measurement, 32 (62%) reported an increase and 20 (38%) reported a decrease in WT. The UEA did not alter the median discrepancy in WT between MRI and TTE. However, where UEA increased reported WT, the difference between MRI and TTE improved in 79% of cases (P=.001) from 2.0-0.5mm. In those with scar on MRI, UEA improved agreement of WT between TTE and MRI compared with that of TTE without UEA (79% vs 39%; P=.011). Interclass correlation coefficient for WT for TTE without UEA, with UEA, and MRI was 0.84; (95% CI, 0.61-0.92), 0.88; (95%CI, 0.82-0.92), and 0.97; (95%CI, 0.96-0.98), respectively. Conclusion: Although use of UEA did not eliminate differences in WT discrepancy between modalities, the addition of UEA to TTE aided in WT determination and improved correlation with MRI in those with greater WT and in all patients with myocardial scars.

Cardiac Masses Discovered by Echocardiogram; What to Do Next?

Cardiac tumors are rare conditions, typically diagnosed on autopsy, but with the advancement of imaging techniques they are now encountered more frequently in clinical practice. Echocardiography is often the initial method of investigation for cardiac masses and provides a quick and valuable springboard for their characterization. While some cardiac masses can be readily identified by echocardiography alone, several require incorporation of multiple data points to reach diagnostic certainty. Herein, we will provide an overview of the main clinical, diagnostic, and therapeutic characteristics of cardiac masses within the framework of their location.

Outcomes of Transcatheter Aortic Valve Implantation in Nonagenarians and Octogenarians (Analysis from the National Inpatient Sample Database).

Risks among nonagenarian (age ≥90 years) and octogenarian (age 80 to 89 years) patients who underwent transcatheter aortic valve implantation (TAVI) compared with clinically similar septuagenarian (age 70 to 79 years) patients remain unclear. This study aimed to assess the outcomes of TAVI in nonagenarians and octogenarians compared with septuagenarians. We conducted a retrospective cohort study using the National Inpatient Sample database to identify patients aged ≥70 years hospitalized for TAVI from 2016 to 2020 and to compare outcomes in nonagenarians and octogenarians versus septuagenarians. The primary outcome was in-hospital mortality. Secondary outcomes included procedural complications, length of stay (LOS), and total costs. The trends in in-hospital outcomes were evaluated. Results were adjusted for demographic/clinical factors. The total cohort included 263,325 patients hospitalized for TAVI, of whom 11.9% were nonagenarians, 51.1% octogenarians, and 37.0% septuagenarians. After adjustment, nonagenarians and octogenarians had higher odds of in-hospital mortality (adjusted odds ratio 1.80, 95% confidence interval 1.34 to 2.41 for nonagenarians; adjusted odds ratio 1.65, 95% confidence interval 1.35 to 2.01 for octogenarians), heart block, permanent pacemaker insertion, stroke, major bleeding, blood transfusion, and palliative care consultation than septuagenarians (all p <0.01). LOS was longer and the total costs were higher for nonagenarians and octogenarians (both p <0.01). Over the study period, in-hospital mortality decreased in nonagenarians (ptrend = 0.04), and major bleeding, permanent pacemaker insertion, LOS, and costs decreased in all patients aged ≥70 years (ptrend <0.01). In conclusion, nonagenarians and octogenarians who underwent TAVI have higher rates of mortality and procedure-related complications than clinically similar septuagenarians. Further research is necessary to optimize outcomes in this frail population.

Cardiotoxicities of Novel Therapies in Hematological Malignancies: Monoclonal Antibodies and Enzyme Inhibitors.

Monoclonal antibodies (mAB) selectively target leukemia surface antigens and work by either blocking cell surface receptors or triggering the target cell's destruction. Similarly, enzyme inhibitors bind to complex molecular platforms and induce downstream mechanisms that trigger cell death. These are used in a variety of hematologic malignancies. Yet, they also elicit severe immune-mediated reactions as biological agents that require careful monitoring. Cardiovascular effects include cardiomyopathy, ventricular dysfunction, cardiac arrest, and acute coronary syndrome. While there have been scattered reviews of mAB and enzyme inhibitors, a consolidated resource regarding their cardiovascular risk profile is lacking. We provide general recommendations for initial screening and serial monitoring based on the literature.

Cardiotoxicities of Novel Therapies in Hematologic Malignancies: Chimeric Antigen Receptor T-Cell Therapy and Bispecific T-Cell Engager Therapy.

The field of malignant hematology is transforming with novel immunotherapeutic approaches. Unfortunately, quality of life, treatment efficacy, and life expectancy are negatively affected by cardiotoxic side effects of treatment. To date, the exact mechanism and incidence of cardiotoxicity associated with these therapies is unclear. These events are believed to be triggered or occur concurrently with cytokine release syndrome. Furthermore, there are no formal guidelines to provide evaluation, treatment, and surveillance. We aim to synthesize available literature with updates on the cardiotoxic effects of novel therapies used in malignant hematologic disorders, with a focus on chimeric antigen receptor T-cell therapy and bispecific T-cell engager therapy, along with a proposed algorithm that may guide pretreatment evaluation, monitoring during treatment, and post-treatment surveillance.

Racial and Ethnic Disparities in the Use and Outcomes of Transcatheter Mitral Valve Replacement: Analysis From the National Inpatient Sample Database.

Background Racial and ethnic disparities in outcomes exist following many cardiac procedures. Transcatheter mitral valve replacement (TMVR) has grown as an alternative to mitral valve surgery for patients at high surgical risk. The outcomes of TMVR by race and ethnicity are unknown. We aimed to evaluate racial and ethnic disparities in the outcomes of TMVR. Methods and Results We analyzed the National Inpatient Sample database from 2016 to 2020 to identify hospitalizations for TMVR. Racial and ethnic disparities in TMVR outcomes were determined using logistic regression models. Between 2016 and 2020, 5005 hospitalizations for TMVR were identified, composed of 3840 (76.7%) White race, 505 (10.1%) Black race, 315 (6.3%) Hispanic ethnicity, and 345 (6.9%) from other races (Asian, Pacific Islander, American Indian or Alaska Native, Other). Compared with other racial and ethnic groups, Black patients were significantly younger and more likely to be women (both P<0.01). There were no significant differences between White, Black, and Hispanic patients in in-hospital mortality (5.2% versus 5.0% versus <3.5%; P=0.89) and procedural complications, including heart block (P=0.91), permanent pacemaker (P=0.49), prosthetic valve dysfunction (P=0.45), stroke (P=0.37), acute kidney injury (P=0.32), major bleeding (P=0.23), and blood transfusion (P=0.92), even after adjustment for baseline characteristics. Adjusted vascular complications were higher in Black compared with White patients (P=0.03). Trend analysis revealed a significant increase in TMVR in all racial and ethnic groups from 2016 to 2020 (Ptrend<0.05). Conclusions Between 2016 and 2020, Black and Hispanic patients undergoing TMVR had similar in-hospital outcomes compared with White patients, except for higher vascular complications in Black patients. Further comparative studies of TMVR in clinically similar White patients and other racial and ethnic groups are warranted to confirm our findings.

Treatment of Highly Angulated Lesions Using SuperCross Microcatheter.

Unusually angulated coronary anatomy can decrease the success rate of coronary interventions by hindering successful wiring and equipment delivery. Additionally, due to the technical challenges involved, there is increased risk for complications such as perforations, dissections, stent loss, and equipment entrapment. In this case series, we illustrate the advantages of using angulated microcatheters to facilitate successful treatment of such patients in various clinical scenarios.

Sodium-Glucose Cotransporter-2 Inhibitors in Heart Failure With Mildly Reduced or Preserved Ejection Fraction: A Systematic Review and Meta-Analysis of Randomized Controlled Trials.

BACKGROUND: Sodium-glucose cotransporter-2 inhibitors (SGLT2i) reduce the risk of heart failure (HF) hospitalizations and cardiovascular mortality among patients with HF and left ventricular ejection fraction (LVEF) ≤40%. There is emerging evidence of the benefits of SGLT2i in HF patients with a higher LVEF (>40%). We aimed to evaluate the benefits of SGLT2i in different subgroups of patients with HF and LVEF >40%. METHODS: We searched PubMed, EMBASE, clinicaltrials.gov, Cochrane, and Google Scholar for randomized controlled trials (RCTs) comparing outcomes of SGLT2i vs placebo in patients with HF and LVEF >40%. The hazard ratios (HRs) and 95% confidence intervals (CIs) in each study were used for the meta-analysis. The primary composite outcome (PCO) was HF hospitalization or cardiovascular mortality. Secondary outcomes included HF hospitalization, cardiovascular mortality, and all-cause mortality. RESULTS: Six RCTs with 15,989 patients were included (median follow-up = 27.3 months, 40.8% females). In patients with HF and LVEF >40%, SGLT2i were associated with significantly lower PCO compared to placebo (HR 0.80; 95% CI 0.74-0.86; P < 0.001). This was consistent across 10 of 13 subgroups examined, including LVEF. SGLT2i also reduced HF hospitalization but not cardiovascular or all-cause mortality. Patients <65 years old, from racial minorities, or from Asia receiving SGLT2i did not demonstrate a significant reduction in PCO. CONCLUSIONS: SGLT2i significantly reduce the combined risk of HF hospitalization or cardiovascular mortality among patients with HF and LVEF >40%. However, younger patients, racial minorities, and patients from Asia did not demonstrate such a reduction. Further research is necessary to identify the reasons for such disparities.