Understanding Patterns of Service Use and Resilience Among High-Risk Children

Introduction: The most efficient risk stratification algorithms are expected to deliver robust and indefectible identification of high-risk children with hypertrophic cardiomyopathy (HCM). Here we compare algorithms for risk stratification in primary prevention in HCM children and investigate whether novel indices of biatrial performance improve these algorithms. Methods and Results: The endpoints were defined as sudden cardiac death, resuscitated cardiac arrest, or appropriate implantable cardioverter-defibrillator discharge. We examined the prognostic utility of classic American College of Cardiology/American Heart Association (ACC/AHA) risk factors, the novel HCM Risk-Kids score and the combination of these with indices of biatrial dynamics. The study consisted of 55 HCM children (mean age 12.5 ± 4.6 years, 69.1% males); seven had endpoints (four deaths, three appropriate ICD discharges). A strong trend (DeLong p = 0.08) was observed towards better endpoint identification performance of the HCM Risk-Kids Model compared to the ACC/AHA strategy. Adding the atrial conduit function component significantly improved the prediction capabilities of the AHA/ACC Model (DeLong p = 0.01) and HCM Risk-Kids algorithm (DeLong p = 0.04). Conclusions: The new HCM Risk-Kids individualised algorithm and score was capable of identifying high-risk children with very good accuracy. The inclusion of one of the atrial dynamic indices improved both risk stratification strategies.

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Interscallop separations of the posterior mitral valve leaflet: a solution to the ‘borderline RHD’ conundrum?

Open Heart ◽

10.1136/openhrt-2020-001452 ◽

2020 ◽

Vol 7 (2) ◽

pp. e001452

Author(s):

Luke David Hunter ◽

Mark Monaghan ◽

Guy Lloyd ◽

Carl Lombard ◽

Alfonso Jan Kemp Pecoraro ◽

...

Keyword(s):

High Risk ◽

Mitral Valve ◽

South African ◽

Underlying Mechanism ◽

Low Risk ◽

Cross Sectional ◽

Mitral Valve Leaflet ◽

Echocardiographic Study ◽

Sole Criterion ◽

High Risk Children

ObjectiveThe World Heart Federation (WHF) criteria incorporate a Doppler-based system to differentiate between ‘physiological’ and ‘pathological’ mitral regurgitation (MR)—a sole criterion sufficient for the diagnosis of WHF ‘borderline’ rheumatic heart disease (RHD). We have identified that interscallop separations (ISS) of the posterior mitral valve (MV) leaflet, can give rise to pathological MR in an otherwise-normal MV. We aimed to establish and compare the prevalence of ISS-related MR among South African children at high and low risk for RHD.MethodsA prospective cross-sectional echocardiographic study of 759 school children (aged 13–18) was performed. Cases with MR≥1.5 cm underwent a second comprehensive study to determine the prevalence of RHD according to the WHF guideline and establish the underlying mechanism of MR.ResultsOf 400 high-risk children, two met criteria for ‘definite RHD’ (5 per 1000 (95% CI 1.4 to 18.0); p=0.5) and 11 for ‘borderline RHD’ (27.5 per 1000 (95% CI 15.4 to 48.6)). Of 359 low-risk children, 14 met criteria for borderline RHD (39 per 1000 (95% CI 23.4 to 64.4)). Comprehensive echocardiography identified an underlying ISS as the mechanism of isolated pathological MR in 10 (83.3%) high-risk children and 11 low-risk children (78.5%; p>0.99).ConclusionsISS are a ubiquitous finding among South African schoolchildren from all risk profiles and are regularly identified as the underlying mechanism of WHF pathological MR in borderline RHD cases. A detailed MV assessment with an emphasis on ascertaining the underlying mechanism of dysfunction could reduce the reported numbers of screened cases misclassified as borderline RHD.

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