Value of Exercise Stress Echocardiography in Children with Hypertrophic Cardiomyopathy

Assaad, Iqbal El; Rizwan, Raheel; Margossian, Renée; Colan, Steven D.; Chen, Ming Hui

doi:10.1016/j.echo.2020.01.020

Cited by 24 publications

(11 citation statements)

References 17 publications

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“…Symptoms in non-obstructive disease are usually secondary to impaired diastolic function or myocardial ischaemia caused by increased LV mass. Exercise echocardiography can be a useful investigation if symptoms are suggestive of LVOT obstruction to elicit latent obstructive disease (74), which has been reported in up to two thirds of children with sarcomeric disease. Treatment is largely empirical and includes beta blockers and calcium channel blockers.…”

Section: Symptoms In the Absence Of Lvot Obstructionmentioning

confidence: 99%

Childhood Hypertrophic Cardiomyopathy: A Disease of the Cardiac Sarcomere

2021

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Hypertrophic cardiomyopathy is the second most common cause of cardiomyopathy presenting during childhood and whilst its underlying aetiology is variable, the majority of disease is caused by sarcomeric protein gene variants. Sarcomeric disease can present at any age with highly variable disease phenotype, progression and outcomes. The majority have good childhood-outcomes with reported 5-year survival rates above 80%. However, childhood onset disease is associated with considerable life-long morbidity and mortality, including a higher SCD rate during childhood than seen in adults. Management is currently focused on relieving symptoms and preventing disease-related complications, but the possibility of future disease-modifying therapies offers an exciting opportunity to modulate disease expression and outcomes in these young patients.

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Section: Symptoms In the Absence Of Lvot Obstructionmentioning

confidence: 99%

Childhood Hypertrophic Cardiomyopathy: A Disease of the Cardiac Sarcomere

2021

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“…The utility of exercise stress tests for evaluating arrhythmias and stress echocardiography to detect exercise-induced or exacerbated outflow tract obstruction is well documented. 102 Periodic cardiac magnetic resonance imaging evaluation of late gadolinium enhancement as evidence of myocardial fibrosis is recommended, although the frequency of testing and the threshold level of fibrosis that represents a risk factor for sudden death remain uncertain in pediatrics given the limited outcome data. Monitoring for the development of restrictive physiology is based on assessment of left atrial size and Doppler assessment of pulmonary and tricuspid regurgitant velocities, with right-sided heart catheterization for patients with findings suggestive of pulmonary or right ventricular hypertension.…”

Section: Stages Of Cardiomyopathy In Childrenmentioning

confidence: 99%

Treatment Strategies for Cardiomyopathy in Children: A Scientific Statement From the American Heart Association

Bogle¹,

Colan²,

Miyamoto³

et al. 2023

Circulation

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This scientific statement from the American Heart Association focuses on treatment strategies and modalities for cardiomyopathy (heart muscle disease) in children and serves as a companion scientific statement for the recent statement on the classification and diagnosis of cardiomyopathy in children. We propose that the foundation of treatment of pediatric cardiomyopathies is based on these principles applied as personalized therapy for children with cardiomyopathy: (1) identification of the specific cardiac pathophysiology; (2) determination of the root cause of the cardiomyopathy so that, if applicable, cause-specific treatment can occur (precision medicine); and (3) application of therapies based on the associated clinical milieu of the patient. These clinical milieus include patients at risk for developing cardiomyopathy (cardiomyopathy phenotype negative), asymptomatic patients with cardiomyopathy (phenotype positive), patients with symptomatic cardiomyopathy, and patients with end-stage cardiomyopathy. This scientific statement focuses primarily on the most frequent phenotypes, dilated and hypertrophic, that occur in children. Other less frequent cardiomyopathies, including left ventricular noncompaction, restrictive cardiomyopathy, and arrhythmogenic cardiomyopathy, are discussed in less detail. Suggestions are based on previous clinical and investigational experience, extrapolating therapies for cardiomyopathies in adults to children and noting the problems and challenges that have arisen in this experience. These likely underscore the increasingly apparent differences in pathogenesis and even pathophysiology in childhood cardiomyopathies compared with adult disease. These differences will likely affect the utility of some adult therapy strategies. Therefore, special emphasis has been placed on cause-specific therapies in children for prevention and attenuation of their cardiomyopathy in addition to symptomatic treatments. Current investigational strategies and treatments not in wide clinical practice, including future direction for investigational management strategies, trial designs, and collaborative networks, are also discussed because they have the potential to further refine and improve the health and outcomes of children with cardiomyopathy in the future.

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“…It can be used as a tool to identify low risk patients. 123 Recommendations and Key Points 1-In symptomatic patients, if a peak instantaneous gradient $50 mm Hg is not obtained, then provocative maneuvers should be pursued. (Figure 20)…”

Section: Provoking Obstructionmentioning

confidence: 99%