Cardim, N;
Brito, D;
Rocha Lopes, L;
Freitas, A;
Araújo, C;
Belo, A;
Gonçalves, L;
... participating centres; + view all
(2018)
The Portuguese Registry of Hypertrophic Cardiomyopathy: Overall results.
Revista Portuguesa de Cardiologia
, 37
(1)
pp. 1-10.
10.1016/j.repc.2017.08.005.
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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.
| Type: | Article |
|---|---|
| Title: | The Portuguese Registry of Hypertrophic Cardiomyopathy: Overall results |
| Location: | Portugal |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1016/j.repc.2017.08.005 |
| Publisher version: | http://dx.doi.org/10.1016/j.repc.2017.08.005 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | Hipertrofia ventricular esquerda, Hypertrophic cardiomyopathy, Left ventricular hypertrophy, Miocardiopatia hipertrófica, Outcome, Prognóstico, Registo, Registry |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science > Clinical Science |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10043345 |
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