title: Sudden death in hypertrophic cardiomyopathy: old risk factors re-assessed in a new model of maximalized follow-up.
abstract: OBJECTIVE
  in hypertrophic cardiomyopathy (HCM), the following five risk factors have a major role in the primary prevention of sudden death (SD): family history of SD (FHSD), syncope, massive wall thickness (MWTh) >30 mm, non-sustained ventricular tachycardia (nsVT) in Holter monitoring of electrocardiography, and abnormal blood pressure response to exercise (aBPRE). In HCM, as a genetic cardiac disease, the risk for SD may also exist from birth. The aim of the study was to compare the survival curves constructed for each of the five risk factors in a traditional follow-up model (started at the first presentation of a patient at the institution) and in a novel follow-up model (started at the date of birth). In an additional analysis, we compared the survival rate in three subgroups (without FHSD, with one SD, and with two or more SDs in a family).
  
  
  RESULTS
  a total of 1306 consecutive HCM patients (705 males, 601 females, mean age of 47 years, and 193 patients were <18 years) evaluated at 15 referral centres in Poland were enrolled in the study. In a novel method of follow-up, all the five risk factors confirmed its prognostic power (FHSD: P = 0.0007; nsVT: P < 0.0001; aBPRE: P = 0.0081; syncope: P < 0.0001; MWTh P> 0.0001), whereas in a traditional method, only four factors predicted SD (except aBPRE). In a novel model of follow-up, FHSD in a single episode starts to influence the prognosis with a delay to the fifth decade of life (P = 0.0007). Multiple FHSD appears to be a very powerful risk factor (P < 0.0001), predicting frequent SDs in childhood and adolescence.
  
  
  CONCLUSIONS
  the proposed concept of a lifelong calculated follow-up is a useful strategy in the risk stratification of SD. Multiple FHSD is a very ominous risk factor with strong impact, predicting frequent SD episodes in the early period of life.
authors.affiliation: 2nd Department of Cardiology, CMUJ, ul. Kopernika 17, 31-501 Kraków, Poland. dimitrow@mp.pl
authors.forename: Pawel Petkow
authors.initials: PP
authors.lastname: Dimitrow
authors.forename: Lidia
authors.initials: L
authors.lastname: Chojnowska
authors.forename: Tomasz
authors.initials: T
authors.lastname: Rudzinski
authors.forename: Walerian
authors.initials: W
authors.lastname: Piotrowski
authors.forename: Lidia
authors.initials: L
authors.lastname: Ziólkowska
authors.forename: Andrzej
authors.initials: A
authors.lastname: Wojtarowicz
authors.forename: Anna
authors.initials: A
authors.lastname: Wycisk
authors.forename: Alicja
authors.initials: A
authors.lastname: Dabrowska-Kugacka
authors.forename: Ewa
authors.initials: E
authors.lastname: Nowalany-Kozielska
authors.forename: Bozena
authors.initials: B
authors.lastname: Sobkowicz
authors.forename: Wojciech
authors.initials: W
authors.lastname: Wróbel
authors.forename: Janina
authors.initials: J
authors.lastname: Aleszewicz-Baranowska
authors.forename: Andrzej
authors.initials: A
authors.lastname: Rynkiewicz
authors.forename: Krystyna
authors.initials: K
authors.lastname: Loboz-Grudzien
authors.forename: Michal
authors.initials: M
authors.lastname: Marchel
authors.forename: Andrzej
authors.initials: A
authors.lastname: Wysokinski
mesh_terms: D000367:Age Factors; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications / Q000235:genetics / Q000401:mortality*; D016757:Death, Sudden, Cardiac / Q000453:epidemiology* / Q000209:etiology; D015444:Exercise / Q000502:physiology; D005260:Female; D006801:Humans; D006973:Hypertension / Q000209:etiology / Q000401:mortality; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011044:Poland / Q000453:epidemiology; D011379:Prognosis; D012307:Risk Factors; D013575:Syncope / Q000209:etiology / Q000401:mortality