Cardiovascular Magnetic Resonance-Based Tissue Characterization in Patients With Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a common hereditable cardiomyopathy that affects between 1:200 to 1:500 of the general population. The role of cardiovascular magnetic resonance (CMR) imaging in the management of HCM has expanded over the past 2 decades to become a key informant of risk in this patient population, delivering unique insights into tissue health and its influence on future outcomes. Numerous mature CMR-based techniques are clinically available for the interrogation of tissue health in patients with HCM, inclusive of contrast and noncontrast methods. Late gadolinium enhancement imaging remains a cornerstone technique for the identification and quantification of myocardial fibrosis with large cumulative evidence supporting value for the prediction of arrhythmic outcomes. T1 mapping delivers improved fidelity for fibrosis quantification through direct estimations of extracellular volume fraction but also offers potential for noncontrast surrogate assessments of tissue health. Water-sensitive imaging, inclusive of T2-weighted dark blood imaging and T2 mapping, have also shown preliminary potential for assisting in risk discrimination. Finally, emerging techniques, inclusive of innovative multiparametric methods, are expanding the utility of CMR to assist in the delivery of comprehensive tissue characterization toward the delivery of personalized HCM care. In this narrative review we summarize the contemporary landscape of CMR techniques aimed at characterizing tissue health in patients with HCM. The value of these respective techniques to identify patients at elevated risk of future cardiovascular outcomes are highlighted.

Contrast Echocardiography in Heart Failure: Update 2023.

PURPOSE OF REVIEW: The application of ultrasound-enhancing agents (contrast agents) has improved the accuracy and reproducibility of echocardiography. The review focuses on the currently approved and evolving indications for contrast echocardiography in patients with heart failure, specifically examining clinical studies conducted after the publication of the guidelines in 2017 and 2018. RECENT FINDINGS: The current ASE/EACVI recommendations for contrast echocardiography are based on its accuracy and reproducibility in comparison to non-enhanced echocardiography or other imaging modalities like cardiac MRI. However, tissue characterization remains limited with contrast echocardiography. During the last few years, several studies have demonstrated the clinical impact of using contrast agents on the management of patients with heart failure. There is growing evidence on the benefit of using contrast echocardiography in critically ill patients where echocardiography without contrast agents is often suboptimal and other imaging methods are less feasible. There is no risk of worsening renal function after the administration of ultrasound-enhancing agents, and these agents can be administered even in patients with end-stage renal disease. Contrast echocardiography has become a valuable tool for first-line imaging of patients with heart failure across the spectrum of patients with chronic heart failure to critically ill patients.

Outcomes of pregnancy in women with hypertrophic cardiomyopathy: A systematic review.

INTRODUCTION: Hypertrophic cardiomyopathy (HCM) is a genetic disorder that can be complicated by heart failure and sudden cardiac death. Pregnancy causes hemodynamic changes, which may be deleterious in patients with HCM. Existing cohort studies, analyzing maternal and fetal outcomes of pregnant HCM patients, are limited by small sample sizes. We performed a systematic review of maternal and fetal outcomes of pregnancy in patients with HCM. METHODS: We performed a literature search for studies reporting maternal or fetal outcomes in pregnant women with HCM. Primary outcomes included maternal death, stillbirth, and fetal death. Secondary maternal outcomes included both sustained and non-sustained ventricular tachycardia (VT), atrial fibrillation, heart failure (HF), syncope, cesarean delivery, and preeclampsia/eclampsia. The secondary fetal outcome was preterm birth. We used a random-effects model to determine pooled incidences of outcomes. RESULTS: We identified a total of 18 studies with 1624 pregnancies. The incidence of maternal death was 0.2%. The rates of sustained VT, any VT (including non-sustained), AF, HF, and syncope were 1% (0-1%), 6% (4-8%), 4% (2-6%), 5% (3-8%), and 9% (3-14%), respectively. Postpartum hemorrhage, preeclampsia/eclampsia, and cesarean section complicated 2% (1-4%), 4% (2-6%), and 43% (32-54%) of pregnancies, respectively. Neonatal death occurred in 0.2% of pregnancies. Stillbirth complicated 1% (95% CI, 0-3%) of pregnancies, whereas the incidence of preterm birth was 22% (95% CI, 18-25%). CONCLUSIONS: Women with HCM considering pregnancy can be reassured that the risk of maternal, fetal, or neonatal death is low. However, they are at risk of several non-fatal cardiac and pregnancy-related complications.

A Contemporary Review of the Effects of Exercise Training on Cardiac Structure and Function and Cardiovascular Risk Profile: Insights From Imaging.

Exercise is a commonly prescribed therapy for patients with established cardiovascular disease or those at high risk for de novo disease. Exercise-based, multidisciplinary programs have been associated with improved clinical outcomes post myocardial infarction and is now recommended for patients with cancer at elevated risk for cardiovascular complications. Imaging studies have documented numerous beneficial effects of exercise on cardiac structure and function, vascular function and more recently on the cardiovascular risk profile. In this contemporary review, we will discuss the effects of exercise training on imaging-derived cardiovascular outcomes. For cardiac imaging via echocardiography or magnetic resonance, we will review the effects of exercise on left ventricular function and remodeling in patients with established or at risk for cardiac disease (myocardial infarction, heart failure, cancer survivors), and the potential utility of exercise stress to assess cardiac reserve. Exercise training also has salient effects on vascular function and health including the attenuation of age-associated arterial stiffness and thickening as assessed by Doppler ultrasound. Finally, we will review recent data on the relationship between exercise training and regional adipose tissue deposition, an emerging marker of cardiovascular risk. Imaging provides comprehensive and accurate quantification of cardiac, vascular and cardiometabolic health, and may allow refinement of risk stratification in select patient populations. Future studies are needed to evaluate the clinical utility of novel imaging metrics following exercise training.

Plasma Exchange for Immune Checkpoint Inhibitor-Induced Myocarditis.

Immune checkpoint inhibitor therapy has been shown to improve outcomes across many types of malignancies. However, immune checkpoint inhibitor has been associated with several immune-related adverse events including myocarditis. We describe the case of a 69-year-old man with fulminant myocarditis likely due to pembrolizumab therapy, complicated by biventricular failure with cardiogenic shock. Because of deterioration in hemodynamic status refractory to conventional immunosuppression, therapeutic plasma exchange was performed, resulting in a rapid reduction of serum pembrolizumab levels, and marked clinical, radiological, and biochemical improvement. To our knowledge, this is the first reported case on the successful use of plasma exchange for pembrolizumab-associated fulminant myocarditis.

Il a été montré que le traitement par un inhibiteur du point de contrôle immunitaire améliore les résultats dans de nombreux types de cancer. Un inhibiteur du point de contrôle immunitaire a toutefois été associé à plusieurs effets indésirables d'origine immunologique, y compris la myocardite. Nous vous présentons le cas d'un homme de 69 ans ayant présenté une myocardite fulminante, probablement causée par un traitement par le pembrolizumab, compliquée par une insuffisance biventriculaire accompagnée d'un choc cardiogénique. En raison de la détérioration de l'état hémodynamique réfractaire à une immunosuppression classique, un échange plasmatique thérapeutique a été effectué, lequel a entraîné une réduction rapide des taux sériques de pembrolizumab, et une amélioration marquée sur les plans clinique, radiologique et biochimique. À notre connaissance, il s'agit du premier cas signalé dans lequel un échange plasmatique a été utilisé avec succès pour traiter une myocardite fulminante associée au pembrolizumab.

Cardiomyopathies and Genetic Testing in Heart Failure: Role in Defining Phenotype-Targeted Approaches and Management.

Cardiomyopathies represent an important cause of heart failure, often affecting young individuals, and have important implications for relatives. Genetic testing for cardiomyopathies is an established care pathway in contemporary cardiology practice. The primary cardiomyopathies where genetic testing is indicated are hypertrophic, dilated, arrhythmogenic, and restrictive cardiomyopathies, with left ventricular noncompaction as a variant phenotype. Early identification and initiation of therapies in patients with inherited cardiomyopathies allow for targeting asymptomatic and presymptomatic patients in stages A and B of the American College of Cardiology/American Heart Association classification of heart failure. The current approach for genetic testing uses gene panel-based testing with the ability to extend to whole-exome and whole-genome sequencing in rare instances. The central components of genetic testing include defining the genetic basis of the diagnosis, providing prognostic information, and the ability to screen and risk-stratify relatives. Genetic testing for cardiomyopathies should be coordinated by a multidisciplinary team including adult and pediatric cardiologists, genetic counsellors, and geneticists, with access to expertise in cardiac imaging and electrophysiology. A pragmatic approach for addressing genetic variants of uncertain significance is important. In this review, we highlight the indications for genetic testing in the various cardiomyopathies, the value of early diagnosis and treatment, family screening, and the care process involved in genetic counselling and testing.