Publication
The Portuguese Registry of Hypertrophic Cardiomyopathy: Overall results
| dc.contributor.author | Cardim, N | |
| dc.contributor.author | Brito, D | |
| dc.contributor.author | Lopes, L | |
| dc.contributor.author | Freitas, A | |
| dc.contributor.author | Araújo, C | |
| dc.contributor.author | Belo, A, et al. | |
| dc.date.accessioned | 2019-12-23T14:28:07Z | |
| dc.date.available | 2019-12-23T14:28:07Z | |
| dc.date.issued | 2018 | |
| dc.description.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. | pt_PT |
| dc.description.version | info:eu-repo/semantics/publishedVersion | pt_PT |
| dc.identifier.citation | Rev Port Cardiol. 2018 Jan;37(1):1-10. | pt_PT |
| dc.identifier.doi | 10.1016/j.repc.2017.08.005 | pt_PT |
| dc.identifier.issn | 2174-2030 | |
| dc.identifier.uri | http://hdl.handle.net/10400.10/2377 | |
| dc.language.iso | eng | pt_PT |
| dc.peerreviewed | yes | pt_PT |
| dc.publisher | Elsevier | pt_PT |
| dc.relation.publisherversion | file:///U:/Downloads/1-s2.0-S0870255117305425-main.pdf | pt_PT |
| dc.subject | Hypertrophic cardiomyopathy | pt_PT |
| dc.subject | Registries | pt_PT |
| dc.subject | Portugal | pt_PT |
| dc.title | The Portuguese Registry of Hypertrophic Cardiomyopathy: Overall results | pt_PT |
| dc.type | journal article | |
| dspace.entity.type | Publication | |
| oaire.citation.conferencePlace | Lisboa | pt_PT |
| oaire.citation.title | Revista Portuguesa de Cardiología | pt_PT |
| rcaap.rights | openAccess | pt_PT |
| rcaap.type | article | pt_PT |