Family History and Warning Symptoms Precede Sudden Cardiac Death in Arrhythmogenic Right Ventricular Cardiomyopathy (From A Nationwide Study in Sweden)

• Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiacdisease explaining about 4% of sudden cardiac death (SCD) cases among the youngin Sweden. The aim of this study was to describe the circumstances preceding SCDdue to ARVC in all victims <35 years of age who received an autopsy-confirmeddiagnosis of ARVC from January 1st, 2000 to December 31st, 2010 in Sweden (n=22).Data on demographics, medical and family history, circumstances of death, andanatomopathological findings were collected from several compulsory national healthregistries, clinical records, family interviews, and autopsy reports. Registry-based datawas compared with age-, sex- and geographically-matched population controls. Duringthe 6 months preceding SCD, 15 cases (68%) had experienced symptoms of cardiacorigin, mainly syncope or presyncope (54%), and chest discomfort (27%). Eight cases(36%) had sought medical care due to cardiac symptoms. The occurrence of hospitalvisits was significantly increased in cases compared with controls (OR 4.62 [1.35-15.8]). Ten cases (45%) had a family history of SCD. The most common activity at thetime of death was exercise (41%). Complete cardiac investigation was seldomperformed, only one case was diagnosed with ARVC before death. In conclusion, inthis nationwide study we observed a high prevalence of symptoms of cardiac origin,health-care utilization, and family history of SCD preceding SCD due to ARVC amongthe young. Increased awareness of these warning signals in the young is critical toimprove risk stratification and early disease detection.

Subject headings

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Kardiologi (hsv//swe)

MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Cardiac and Cardiovascular Systems (hsv//eng)

Keyword

Arrhythmogenic Right Ventricular Cardiomyopathy

symptoms

family history

sudden cardiac death

Kardiologi

Cardiology

Publication and Content Type

ref (subject category)

art (subject category)