Rev Port Cardiol. 2018 Jan;37(1):1-10. doi: 10.1016/j.repc.2017.08.005. Epub 2018 Jan 19.

The Portuguese Registry of Hypertrophic Cardiomyopathy: Overall results.

[Article in English, Portuguese]

Cardim N¹, Brito D², Rocha Lopes L³, Freitas A⁴, Araújo C⁵, Belo A⁶, Gonçalves L⁷, Mimoso J⁸, Olivotto I⁹, Elliott P¹⁰, Madeira H¹¹; participating centres.

Author information

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.

KEYWORDS:

Hipertrofia ventricular esquerda; Hypertrophic cardiomyopathy; Left ventricular hypertrophy; Miocardiopatia hipertrófica; Outcome; Prognóstico; Registo; Registry