European registry of cardiomyopathy patients publishes first data

30th September 2015

A registry set up to record the scale of cardiomyopathy in Europe and how well patients do has just published its first results.

The European cardiomyopathy registry, set up by the European Society of Cardiology (ESC), is collecting previously unobtainable comprehensive data on the disease in a bid to improve care services and treatments.

The ESC says that individually the different disorders are relatively uncommon, but together around three per 1,000 people in Europe have it, putting a substantial burden on the healthcare services of all European nations.

The first observational study looked at 1,115 patients added to the register between 1 December 2012 and 30 November 2013. They had four different types of cardiomyopathy: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM).

A total of 27 hospitals in 12 countries participated. The commonest cardiomyopathy was HCM (681 patients), followed by DCM (346), ARVC (59), and RCM (29); 423 patients (46.4% of those reported) had familial disease. There were more men for all the types except RCM.

Most patients (813) were in New York Heart Association heart function class I (no limitation of physical activity and ordinary physical activity did not cause undue fatigue, palpitation or shortness of breath) when put on the register.

But 139 (12.5%) reported loss of consciousness, most frequently in ARVC. Nearly half (45.5%) of patients had a cardiac MRI (magnetic resonance imaging), 117 (10.6%) had a heart biopsy, and 462 (41.4%) genetic testing with a disease-causing mutation reported in 236 people(51.1%). Just over 1,000 patients (92.0%) were on drug treatments; 316 (28.3%) had  an internal defibrillator (termed an implantable cardioverter defibrillator or ICD). The highest proportion of those with an ICD had ARVC.

The researchers, who included Professor Perry Elliott from the Barts Heart Centre in London and others doctors from France, Spain, Italy and Poland, said the data showed that the care patients needed was complex, requiring a large range of invasive and non-invasive investigations and the involvement of multidisciplinary teams of medical staff.

Treatment was equally multifaceted and patients were likely to need long-term follow-up in close liaison with expert centres.

The study has been published in the online European Heart Journal.