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Value of Exercise Stress Echocardiography in Children with Hypertrophic Cardiomyopathy
Iqbal El Assaad1, Kimberlee Gauvreau1, Raheel Rizwan2
1Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts; Harvard Medical School, Boston, Massachusetts.
Insights
Exercise stress echocardiography (ESE) is safe and effective in identifying children with hypertrophic cardiomyopathy (HCM) at low risk for adverse cardiac events. This diagnostic tool helps stratify risk in pediatric HCM patients.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Imaging
- Genetic Heart Diseases
Background:
- Hypertrophic cardiomyopathy (HCM) is a significant concern in pediatric populations.
- Exercise stress echocardiography (ESE) is established in adults but understudied in children.
- Left ventricular outflow tract (LVOT) gradients are key indicators in HCM prognosis.
Purpose of the Study:
- To evaluate the safety and efficacy of ESE in pediatric patients with HCM.
- To determine the prognostic value of LVOT gradients during ESE in children.
- To identify risk stratification for adverse cardiac outcomes in pediatric HCM.
Main Methods:
- Retrospective review of 91 pediatric HCM patients undergoing ESE (2007-2018).
- Patients categorized by LVOT gradients: Group 1 (<30 mmHg rest/exercise), Group 2 (<30 mmHg rest, ≥30 mmHg exercise), Group 3 (≥30 mmHg rest/exercise).
- Composite endpoint included syncope, chest pain, ventricular arrhythmias, cardiac arrest, heart failure, or HCM-related death/transplant.
Main Results:
- ESE was safely performed in 91 children (median age 12 years).
- Group 3 patients showed a 5-fold higher risk of adverse clinical outcomes (HR=5.18, P=0.014).
- Group 2 showed a trend towards higher risk, but not statistically significant (HR=1.95, P=0.33).
Conclusions:
- ESE is a safe and effective diagnostic tool in pediatric HCM.
- It accurately identifies children at lowest risk for adverse cardiac events.
- LVOT gradient assessment during ESE aids in risk stratification for pediatric HCM patients.
Background:
Exercise stress echocardiography (ESE) is a valuable diagnostic and prognostic tool in adults with hypertrophic cardiomyopathy (HCM). Inducible and resting left ventricular outflow tract gradients are important predictors of heart failure. However, there are minimal data on the utility of this modality in children.
Methods:
Retrospective review of all pediatric HCM patients who underwent ESE at Boston Children's Hospital (January 2007-June 2018) was carried out. Patients were assigned to one of three categories based on left ventricular outflow tract gradients: group 1: <30 mm Hg at rest and exercise; group 2: <30 mm Hg at rest and ≥30 mm Hg with exercise; and group 3: ≥ 30 mm Hg at rest and exercise. Records were reviewed for earliest occurrence of composite endpoint of any one of the following: cardiac syncope, chest pain, nonsustained and sustained ventricular tachycardia, aborted cardiac arrest, heart failure class ≥ II, or HCM-related death/transplantation.
Results:
A total of 91 children (67% males) with median age 12 years (6-24 years) at first ESE and median left ventricle wall thickness of 20 mm formed the cohort. Median follow-up duration was 3 years. During ESE, only one child experienced an event and was resuscitated. Of the 91 children, 25 were classified as group 1, 40 as group 2, and 26 as group 3. Twenty-six patients met the composite endpoint, including two heart transplant, one aborted cardiac arrest, and one sudden cardiac death. Group 3 patients had a 5 times higher risk of developing symptoms and/or serious clinical outcome at any age (hazard ratio = 5.18; 95% CI, [1.39-19.2]; P = .014). During our short follow-up time, group 2 patients had a higher risk of outcome, but this did not achieve statistical significance (hazard ratio = 1.95; 95% CI, [0.5-7.6]; P = .33).
Conclusions:
In this large series of pediatric patients with HCM, ESE can be performed safely and served as an effective tool to identify the lowest risk patients for cardiac outcome.
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