TY - JOUR T1 - The Portuguese Registry of Hypertrophic Cardiomyopathy: Overall results JO - Revista Portuguesa de Cardiologia T2 - AU - Cardim,Nuno AU - Brito,Dulce AU - Rocha Lopes,Luís AU - Freitas,António AU - Araújo,Carla AU - Belo,Adriana AU - Gonçalves,Lino AU - Mimoso,Jorge AU - Olivotto,Iacopo AU - Elliott,Perry AU - Madeira,Hugo SN - 08702551 M3 - 10.1016/j.repc.2017.08.005 DO - 10.1016/j.repc.2017.08.005 UR - https://revportcardiol.org/pt-the-portuguese-registry-hypertrophic-cardiomyopathy-articulo-S0870255117305425 AB - IntroductionWe report the results of the Portuguese Registry of Hypertrophic Cardiomyopathy, an initiative that reflects the current spectrum of cardiology centers throughout the territory of Portugal. MethodsA direct invitation to participate was sent to cardiology departments. Baseline and outcome data were collected. ResultsA total of 29 centers participated and 1042 patients were recruited. Four centers recruited 49% of the patients, of whom 59% were male, and mean age at diagnosis was 53±16 years. Hypertrophic cardiomyopathy (HCM) was identified as familial in 33%. The major reason for diagnosis was symptoms (53%). HCM was obstructive in 35% of cases and genetic testing was performed in 51%. Invasive septal reduction therapy was offered to 8% (23% of obstructive patients). Most patients (84%) had an estimated five-year risk of sudden death of <6%. Thirteen percent received an implantable cardioverter-defibrillator. After a median follow-up of 3.3 years (interquartile range [P25-P75] 1.3-6.5 years), 31% were asymptomatic. All-cause mortality was 1.19%/year and cardiovascular mortality 0.65%/year. The incidence of heart failure-related death was 0.25%/year, of sudden cardiac death 0.22%/year and of stroke-related death 0.04%/year. Heart failure-related death plus heart transplantation occurred in 0.27%/year and sudden cardiac death plus equivalents occurred in 0.53%/year. ConclusionsContemporary HCM in Portugal is characterized by relatively advanced age at diagnosis, and a high proportion of invasive treatment of obstructive forms. Long-term mortality is low; heart failure is the most common cause of death followed by sudden cardiac death. However, the burden of morbidity remains considerable, emphasizing the need for disease-specific treatments that impact the natural history of the disease. ER -