HCM Risk-Kids

Estimates the individual probability of sudden cardiac death within five years in children with hypertrophic cardiomyopathy.

Validated age

1–16 years

Clinical variables

Enter the patient data

Fields marked with an asterisk are required. Results update automatically.

Demographics

Age*

The model is validated for patients aged 1–16 years.

years

Gender*

Weight*

Recorded at the time of clinical evaluation.

Echocardiography

LV maximal wall thickness*

Maximum LV wall thickness on transthoracic echocardiography at evaluation.

LVMWT Z-score*

Enter a Z-score generated using a validated pediatric reference method.

Left atrial diameter*

Measured by M-mode or 2D echocardiography in the parasternal long-axis plane.

LA diameter Z-score*

Enter a Z-score generated using a validated pediatric reference method.

Left ventricular outflow tract gradient*

Maximum gradient at rest or during Valsalva provocation.

mmHg

Arrhythmic and clinical history

Non-sustained ventricular tachycardia*

At least 3 consecutive ventricular beats at ≥120 beats/min lasting less than 30 seconds on ambulatory monitoring.

Unexplained syncope*

History of otherwise unexplained syncope at or before evaluation.

Model eligibility

These selections are not part of the mathematical equation but determine whether the model is applicable.

Previous sustained VT or ventricular fibrillation*

Syndromic or secondary HCM*

Includes metabolic disease, RASopathy syndromes and neuromuscular disorders.

The raw measurements are displayed for documentation. The risk equation uses the corresponding Z-scores.

Intended population

Children aged 1–16 years with primary hypertrophic cardiomyopathy and without a previous sustained ventricular arrhythmia.

Model predictors

Maximal wall thickness Z-score, left atrial diameter Z-score, LVOT gradient, NSVT and unexplained syncope.

Measurement note

Z-scores should be obtained using an appropriate and validated pediatric echocardiographic reference method.

Scientific reference

Norrish G, Ding T, Field E, Ziólkowska L, Olivotto I, Limongelli G, Anastasakis A, Weintraub R, Biagini E, Ragni L, Prendiville T, Duignan S, McLeod K, Ilina M, Fernández A, Bökenkamp R, Baban A, Kubuš P, Daubeney PEF, Sarquella-Brugada G, Cesar S, Marrone C, Bhole V, Medrano C, Uzun O, Brown E, Gran F, Castro FJ, Stuart G, Vignati G, Barriales-Villa R, Guereta LG, Adwani S, Linter K, Bharucha T, Garcia-Pavia P, Rasmussen TB, Calcagnino MM, Jones CB, De Wilde H, Toru-Kubo J, Felice T, Mogensen J, Mathur S, Reinhardt Z, O'Mahony C, Elliott PM, Omar RZ, Kaski JP. Development of a Novel Risk Prediction Model for Sudden Cardiac Death in Childhood Hypertrophic Cardiomyopathy (HCM Risk-Kids). JAMA Cardiol. 2019 Sep 1;4(9):918-927. doi: 10.1001/jamacardio.2019.2861. PMID: 31411652; PMCID: PMC6694401.