A Validated Model for Sudden Cardiac Death Risk Prediction in Pediatric Hypertrophic Cardiomyopathy

A Validated Model for Sudden Cardiac Death Risk Prediction in Pediatric Hypertrophic Cardiomyopathy.

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Item Type: Article
Status: Published
Official URL: https://doi.org/10.1161/CIRCULATIONAHA.120.047235
Journal or Publication Title: Circulation
Volume: 142
Number: 3
Page Range: pp. 217-229
Date: 2020
Divisions: Molecular Cardiology
Depositing User: General Admin
Identification Number: 10.1161/CIRCULATIONAHA.120.047235
ISSN: 0009-7322
Date Deposited: 04 Jan 2021 01:12
Abstract:

Background:
Hypertrophic cardiomyopathy is the leading cause of sudden cardiac death (SCD) in children and young adults. Our objective was to develop and validate a SCD risk prediction model in pediatric hypertrophic cardiomyopathy to guide SCD prevention strategies.

Methods:
In an international multicenter observational cohort study, phenotype-positive patients with isolated hypertrophic cardiomyopathy <18 years of age at diagnosis were eligible. The primary outcome variable was the time from diagnosis to a composite of SCD events at 5-year follow-up: SCD, resuscitated sudden cardiac arrest, and aborted SCD, that is, appropriate shock following primary prevention implantable cardioverter defibrillators. Competing risk models with cause-specific hazard regression were used to identify and quantify clinical and genetic factors associated with SCD. The cause-specific regression model was implemented using boosting, and tuned with 10 repeated 4-fold cross-validations. The final model was fitted using all data with the tuned hyperparameter value that maximizes the c-statistic, and its performance was characterized by using the c-statistic for competing risk models. The final model was validated in an independent external cohort (SHaRe [Sarcomeric Human Cardiomyopathy Registry], n=285).

Results:
Overall, 572 patients met eligibility criteria with 2855 patient-years of follow-up. The 5-year cumulative proportion of SCD events was 9.1% (14 SCD, 25 resuscitated sudden cardiac arrests, and 14 aborted SCD). Risk predictors included age at diagnosis, documented nonsustained ventricular tachycardia, unexplained syncope, septal diameter z-score, left ventricular posterior wall diameter z score, left atrial diameter z score, peak left ventricular outflow tract gradient, and presence of a pathogenic variant. Unlike in adults, left ventricular outflow tract gradient had an inverse association, and family history of SCD had no association with SCD. Clinical and clinical/genetic models were developed to predict 5-year freedom from SCD. Both models adequately discriminated between patients with and without SCD events with a c-statistic of 0.75 and 0.76, respectively, and demonstrated good agreement between predicted and observed events in the primary and validation cohorts (validation c-statistic 0.71 and 0.72, respectively).

Conclusion:
Our study provides a validated SCD risk prediction model with >70% prediction accuracy and incorporates risk factors that are unique to pediatric hypertrophic cardiomyopathy. An individualized risk prediction model has the potential to improve the application of clinical practice guidelines and shared decision making for implantable cardioverter defibrillator insertion.

Creators:
Creators
Email
Miron, Anastasia
UNSPECIFIED
Lafreniere-Roula, Myriam
UNSPECIFIED
Steve Fan, Chun-Po
UNSPECIFIED
Armstrong, Katey R.
UNSPECIFIED
Dragulescu, Andreea
UNSPECIFIED
Papaz, Tanya
UNSPECIFIED
Manlhiot, Cedric
UNSPECIFIED
Kaufman, Beth
UNSPECIFIED
Butts, Ryan J.
UNSPECIFIED
Gardin, Letizia
UNSPECIFIED
Stephenson, Elizabeth A.
UNSPECIFIED
Howard, Taylor S.
UNSPECIFIED
Aziz, Pete F.
UNSPECIFIED
Balaji, Seshadri
UNSPECIFIED
Ladouceur, Virginie Beauséjour
UNSPECIFIED
Benson, Lee N.
UNSPECIFIED
Colan, Steven D.
UNSPECIFIED
Godown, Justin
UNSPECIFIED
Henderson, Heather T.
UNSPECIFIED
Ingles, Jodie
UNSPECIFIED
Jeewa, Aamir
UNSPECIFIED
Jefferies, John L.
UNSPECIFIED
Lal, Ashwin K.
UNSPECIFIED
Mathew, Jacob
UNSPECIFIED
Jean-St-Michel, Emilie
UNSPECIFIED
Michels, Michelle
UNSPECIFIED
Nakano, Stephanie J.
UNSPECIFIED
Olivotto, Iacopo
UNSPECIFIED
Parent, John J.
UNSPECIFIED
Pereira, Alexandre C.
UNSPECIFIED
Semsarian, Christopher
UNSPECIFIED
Whitehill, Robert D.
UNSPECIFIED
Wittekind, Samuel G.
UNSPECIFIED
Russell, Mark W.
UNSPECIFIED
Conway, Jennifer
UNSPECIFIED
Richmond, Marc E.
UNSPECIFIED
Villa, Chet
UNSPECIFIED
Weintraub, Robert G.
UNSPECIFIED
Rossano, Joseph W.
UNSPECIFIED
Kantor, Paul F.
UNSPECIFIED
Ho, Carolyn Y.
UNSPECIFIED
Mital, Seema
UNSPECIFIED
Last Modified: 04 Jan 2021 01:12
URI: https://eprints.centenary.org.au/id/eprint/796

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