title: Risk of sudden death and outcome in patients with hypertrophic cardiomyopathy with benign presentation and without risk factors.
abstract: Patients with hypertrophic cardiomyopathy (HC) are reported to have a mortality rate of about 1.0% per year, and those patients without sudden death risk factors and with no or mild symptoms are generally considered to have a benign clinical presentation. However, the risk of sudden death and the outcome in this latter subgroup have not been investigated systematically and remain unresolved. We assessed the risk of sudden death and outcome in 653 consecutive patients with HC without risk factors and with no or mild symptoms. Over a median follow-up of 5.3 years, 35 patients (5.4%) died of HC-related causes. Mean age at death was 46 ± 20 years in patients who died suddenly and 66 ± 15 and 72 ± 9 years, respectively, in patients who died of heart failure or stroke. Event rate was 0.6% per year for sudden death, 0.2% per year for heart failure death, and 0.1% per year for stroke-related death. Sudden death risk was independently and inversely related to age, and risk of heart failure or stroke death was directly related to age (p = 0.020). At 10 years after the initial evaluation, sudden death risk was 5.9%, with sudden death rate being the lowest (0.3% per year) in patients with normal left atrial dimension (≤40 mm). In conclusion, in patients with HC without conventional risk factors and with no or mild symptoms, the risk of sudden death was not negligible, with an event rate of 0.6% per year. Heart failure and stroke-related death were less common and largely confined to older patients. These results underscore the need for a more accurate assessment of the sudden death risk in patients with HC.
authors.affiliation: Ente Ospedaliero Ospedali Galliera, Genoa, Italy. Electronic address: paolo.spirito@galliera.it.
authors.forename: Paolo
authors.initials: P
authors.lastname: Spirito
authors.affiliation: Dipartimento di Medicina Clinica e Molecolare, Università Sapienza, Roma, Italy.
authors.forename: Camillo
authors.initials: C
authors.lastname: Autore
authors.affiliation: Ente Ospedaliero Ospedali Galliera, Genoa, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Formisano
authors.affiliation: Dipartimento di Medicina Clinica e Molecolare, Università Sapienza, Roma, Italy.
authors.forename: Gabriele Egidy
authors.initials: GE
authors.lastname: Assenza
authors.affiliation: Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Alma Mater Studiorum, Università di Bologna, Bologna, Italy.
authors.forename: Elena
authors.initials: E
authors.lastname: Biagini
authors.affiliation: Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota.
authors.forename: Tammy S
authors.initials: TS
authors.lastname: Haas
authors.affiliation: A.O. Ordine Mauriziano di Torino, Torino, Italy.
authors.forename: Sergio
authors.initials: S
authors.lastname: Bongioanni
authors.affiliation: Agnes Ginges Centre for Molecular Cardiology, Centenary Institute, Sydney Medical School, University of Sydney and Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Christopher
authors.initials: C
authors.lastname: Semsarian
authors.affiliation: Ente Ospedaliero Ospedali Galliera, Genoa, Italy.
authors.forename: Emmanuela
authors.initials: E
authors.lastname: Devoto
authors.affiliation: Dipartimento di Medicina Clinica e Molecolare, Università Sapienza, Roma, Italy.
authors.forename: Beatrice
authors.initials: B
authors.lastname: Musumeci
authors.affiliation: Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Alma Mater Studiorum, Università di Bologna, Bologna, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Lai
authors.affiliation: Agnes Ginges Centre for Molecular Cardiology, Centenary Institute, Sydney Medical School, University of Sydney and Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Laura
authors.initials: L
authors.lastname: Yeates
authors.affiliation: A.O. Ordine Mauriziano di Torino, Torino, Italy.
authors.forename: Maria Rosa
authors.initials: MR
authors.lastname: Conte
authors.affiliation: Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Alma Mater Studiorum, Università di Bologna, Bologna, Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Rapezzi
authors.affiliation: Centro di Coordinamento Sperimentazioni Cliniche, Istituto Toscano Tumori/Azienda Ospedaliero-Universitaria Careggi, Firenze, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Boni
authors.affiliation: Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota.
authors.forename: Barry J
authors.initials: BJ
authors.lastname: Maron
mesh_terms: D000368:Aged; D000704:Analysis of Variance; D001281:Atrial Fibrillation / Q000401:mortality; D002312:Cardiomyopathy, Hypertrophic / Q000401:mortality*; D003645:Death, Sudden; D016757:Death, Sudden, Cardiac; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D012307:Risk Factors