Management of hypertrophic cardiomyopathy.

2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy

2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines

Management of Hypertrophic Cardiomyopathy: JACC State-of-the-Art Review.

2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: A report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.

Highly reliable identification of adults with hypertrophic cardiomyopathy (HC) at risk for sudden death (SD) has been reported. A significant controversy remains, however, regarding the most reliable risk stratification methodology for children and adolescents with HC. The present study assesses the accuracy of SD prediction and prevention with prophylactic implantable cardioverter-defibrillators (ICDs) in young HC patients. The study group is comprised of 146 HC patients <20 years of age evaluated consecutively over 17 years with prospective risk stratification and ICD decision-making. We relied on ≥1 established individual risk markers considered major within each patient's clinical profile, based on an enhanced American College of Cardiology /American Heart Association (ACC/AHA) guidelines algorithm. Of the 60 largely asymptomatic patients implanted with primary prevention ICDs at age 15 ± 4 years, 9 (15%) experienced device therapy terminating potentially lethal ventricular tachyarrhythmias and restoring sinus rhythm at 19 ± 6 years (range 9 to 29), 5.1 ± 6.0 years after implant; 3 patients had multiple appropriate ICD discharges. The individual risk marker algorithm was associated with 100% sensitivity in predicting SD events (95%CI: 69, 100) and 63% specificity for identifying patients without events (95%CI: 54, 71). Of these patients with device therapy, massive left ventricular hypertrophy (absolute wall thickness ≥30 mm) was the most common predictor, present in 70% of patients either alone or in combination with other risk markers. Each of the 146 study patients have survived to date at 22 ± 5 years, including all 86 without ICD recommendations. In conclusion, an enhanced ACC/AHA risk stratification strategy, based on established individual risk markers, was highly reliable in prospectively predicting SD events in children and adolescents with HC, and preventing arrhythmia-based catastrophes in this susceptible high risk population.

Prediction and Prevention of Sudden Death in Young Patients (<20 years) With Hypertrophic Cardiomyopathy.

Accurate assessment of right atrial pressure (RAP) and pulmonary artery systolic pressure (PASP) is critical in the management of heart transplant recipients. The accuracy of echocardiography in estimating these pressures has been debated.

https://onlinelibrary.wiley.com/doi/pdfdirect/10.1002/clc.23835

Accuracy of echocardiographic estimations of right heart pressures in adult heart transplant recipients

Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease occurring in up to 1 in 200 individuals, with a diverse phenotypic expression and clinical course.1,2 Overall, HCM patients are at risk for a number of adverse diseaserelated events; however, contemporary treatments allow for favorable outcomes and extended survival utilizing surgical myectomy and selectively alcohol septal ablation for reversal of heart failure (HF) symptoms secondary to left ventricular outflow obstruction,1–3 sudden death prevention utilizing a mature risk stratification algorithm and primary prevention implantable cardioverter defibrillators,1,4,5 and improved survival and quality of life in nonobstructive endstage disease with HF treatments including transplant.6 In this regard, while 40% to 50% of HCM patients at dedicated HCM centers have 1 or more diseaserelated event, HCMrelated mortality is only 0.5%/year, and 90% to 95% of patients have no or mild (New York Heart Association class I or II) symptoms after treatment interventions.1–3

Thinking Outside the Heart to Treat Atrial Fibrillation in Hypertrophic Cardiomyopathy

<h2>Abstract</h2> The autonomic nervous system functions in a fine-tuned manner to dynamically modulate cardiac function during normal physiological state. Autonomic dysregulation in cardiac disease states such as myocardial infarction and heart failure alters this fine balance, which in turn promotes disease progression and arrhythmogenesis. Neuromodulatory interventions that aim to restore this balance at distinct levels of the cardiac neuraxis thus have been shown to be effective in the treatment of arrhythmias. This review first describes the anatomy of the cardiac autonomic nervous system and the pathological changes that occur with neural remodeling in the setting of scar and cardiomyopathy, followed by therapeutic interventions for neuraxial modulation of arrhythmias such as atrial fibrillation and ventricular tachyarrhythmias. 

Aadhavi Sridharan

Papers

Prediction and Prevention of Sudden Death in Young Patients (<20 years) With Hypertrophic Cardiomyopathy.

Accuracy of echocardiographic estimations of right heart pressures in adult heart transplant recipients

Thinking Outside the Heart to Treat Atrial Fibrillation in Hypertrophic Cardiomyopathy

Autonomic nervous system and arrhythmias in structural heart disease

Apple watch detected ventricular tachycardia in a pregnant patient