In children with HCM, pathogenic sarcomere mutations were very prevalent. Outcome was largely driven by severe diastolic dysfunction in the presence of comparable maximal LV wall thickness. Risk of LAE was greatest among patients with at least one thin filament mutation, which was associated with high prevalence of restrictive progression; whereas a negative genetic test predicted favourable outcome
Genetic basis of pediatric sarcomeric hypertrophic cardiomyopathy: impact on long term outcome / Maurizi, N; Passantino, S; Favilli, S; Girolami, F; Arretini, A; Targetti, M; Spaziani, G; De Simone, L; Pelo, E; Marchionni, N; Cecchi, F; Olivotto, I. - In: EUROPEAN HEART JOURNAL. - ISSN 0195-668X. - ELETTRONICO. - 38:(2017), pp. 945-945.
Genetic basis of pediatric sarcomeric hypertrophic cardiomyopathy: impact on long term outcome
Maurizi, N;Passantino, S;Favilli, S;Arretini, A;Targetti, M;Spaziani, G;De Simone, L;Pelo, E;Marchionni, N;Olivotto, I
2017
Abstract
In children with HCM, pathogenic sarcomere mutations were very prevalent. Outcome was largely driven by severe diastolic dysfunction in the presence of comparable maximal LV wall thickness. Risk of LAE was greatest among patients with at least one thin filament mutation, which was associated with high prevalence of restrictive progression; whereas a negative genetic test predicted favourable outcome
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