Nuno Cardim1, Dulce Brito2, Luís Rocha Lopes3, António Freitas4, Carla Araújo5, Adriana Belo6, Lino Gonçalves7, Jorge Mimoso8, Iacopo Olivotto9, Perry Elliott10, Hugo Madeira11. 1. Hospital da Luz, Lisboa, Portugal; Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisboa, Portugal. Electronic address: cardimnuno@gmail.com. 2. Hospital de Santa Maria, Centro Hospitalar Lisboa Norte (CHLN), Centro Cardiovascular da Universidade de Lisboa (CCUL), Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal. 3. Barts Heart Centre, Barts Health NHS Trust; Institute of Cardiovascular Science, University College London, United Kingdom; Centro Cardiovascular da Universidade de Lisboa, Lisboa, Portugal. 4. Hospital Fernando da Fonseca, Amadora-Sintra, Portugal. 5. Centro Hospitalar de Trás-os-Montes e Alto Douro, EPE, Hospital de São Pedro, Vila Real, Portugal; Epidemiology Research Unit (EPIUnit), Instituto de Saúde Pública, Universidade of Porto (ISPUP), Porto, Portugal. 6. Sociedade Portuguesa de Cardiologia, Departamento de Bioestatística, Coimbra, Portugal. 7. Centro Hospitalar e Universitário de Coimbra-Hospital Geral; Faculdade de Medicina da Universidade de Coimbra, Coimbra, Portugal. 8. Centro Hospitalar do Algarve, Faro, Portugal. 9. Careggi University Hospital, Florence, Italy; Member of the Scientific Committee of PRo-HCM. 10. Barts Heart Centre, Barts Health NHS Trust / Institute of Cardiovascular Science, University College London, United Kingdom; Member of the Scientific Committee of PRo-HCM. 11. Centro Cardiovascular da Universidade de Lisboa, Lisboa, Portugal.
Abstract
INTRODUCTION: We 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. METHODS: A direct invitation to participate was sent to cardiology departments. Baseline and outcome data were collected. RESULTS: A 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. CONCLUSIONS: Contemporary 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.
INTRODUCTION: We 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. METHODS: A direct invitation to participate was sent to cardiology departments. Baseline and outcome data were collected. RESULTS: A 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. CONCLUSIONS: Contemporary 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.
Authors: Tiffany T S Ye; Qi Zhuang Siah; Benjamin Y Q Tan; Jamie S Y Ho; Nicholas L X Syn; Yao Hao Teo; Yao Neng Teo; James W Yip; Tiong-Cheng Yeo; Weiqin Lin; Raymond C C Wong; Ping Chai; Bernard Chan; Vijay Kumar Sharma; Leonard L L Yeo; Ching-Hui Sia Journal: J Thromb Thrombolysis Date: 2022-10-03 Impact factor: 5.221