sudden cardiac death-kopi - fysio.no · epidemiology marathon running: 1 deaths per 50 000 hours...

Jarle Jortveit, MD, Department of Cardiology, Sørlandet Hospital, Arendal, Norway

Sudden cardiac death in the young

Definition

"[Sudden] cardiac arrest is the sudden cessation of cardiac activity so that the victim becomes unresponsive, with no normal breathing and no signs of circulation. If corrective measures are not taken rapidly, this condition progresses to sudden death.»

2006 American College of Cardiology/American Heart Association/Heart Rhythm Society (ACC/AHA/HRS)

Epidemiology

❖ Wordwide

❖ 17 mill deaths/year related to cardiovascular diseases

❖ 25% sudden cardiac deaths

Epidemiology

❖ Ca. 15% of annual mortality

❖ Age dependent

Epidemiology

Kannel WB, Wilson PWF, D'Agostino RB, et al. Am Heart J 1998; 136:205.

Epidemiology

❖ Sudden cardiac death rate

❖ Men: 6.7 per 100 000 person-years

❖ Women: 1.4 per 100 000 person-years

Epidemiology

❖ The most frequent cause in young age

Bardai a, berdowski J, van der Werf C et al. Incidence, causes , and outcomes of out-of-hospital cardiac arrest in children

Etiology

❖ Ischemic heart disease - 70 % of all SCAs

❖ 6-10x increased risk of SCA

Sudden cardiac death in the young

Epidemiology

❖Male dominates (67%)❖Median age: 29 years❖The risk of SCD doubled every decade

Winkel et al. Nation wide study of SCD in persons aged 1-35 years. Eur Heart 2011

Epidemiology

❖ Witnessed deaths: 45%

❖ Out of hospital deaths: 89%

❖ During sleep: 34%

❖ During physical activity: 11%

❖ None with a toxicological explanation

Winkel et al. Nation wide study of SCD in persons aged 1-35 years. Eur Heart 2011.

Etiology❖ Ischemic heart disease

❖ Structural heart disease

❖ Cardiomyopathy❖ Hypertrophic cardiomyopathy (HCM)❖ Arrhythmogenic right ventricular

cardiomyopathy (ARVC)❖ Myocarditis❖ Congenital heart defects

❖ Coronary artery anomalies❖ Absence of structural heart disease

❖ Primary arrhythmogenic diseases❖ Long QT Syndrome

Hypertrophic cardiomyopathy

❖ LV wall thickness ≥ 15 mm

❖ Prevalence: 0,02 – 0,23%

❖ Autosomal dominant heritage

Arrhythmogenic right ventricular cardiomyopathy

❖ Fibrofatty infiltration most commonly of the right ventricular myocardium

❖ Prevalence 1 : 2-5000

❖ Autosomal dominant

❖ 20% 10-year mortality (SCD)❖ A long term follow up of 15 patients with arrhythmogenic right ventricular

dysplasia. Blomström-Lundqvist C, Sabel KG, Olsson SB. Br Heart J. 1987;58(5):477.

Congenital coronary artery abnormalities

❖ High-risk coronary anomalies are those in which the anomalous coronary artery makes an acute bend and courses between the pulmonary artery and aorta

Long QT syndrome

❖ Prevalence 1 : 2500-7000

❖ Autosomal dominant

❖ Potassium channel defect (LQT1 & LQT2) and sodium channel (LQT3) defect

❖ ca 25% normal QTc

Survival

The chain of survival

Physical activity related SCD

Epidemiology

❖Marathon running: 1 deaths per 50 000 hours ❖ Risk for sudden cardiac death associated with marathon running. Maron BJ, Poliac LC, Roberts WO. J Am Coll Cardiol. 1996;28(2):428.)

❖ 4 SCD in 215 413 marathon runners

❖ 3/4 coronary artery disease

❖Non-competitive jogging: 1 death per 396 000 hours

❖ Incidence of death during jogging in Rhode Island from 1975 through 1980. Thompson PD, Funk EJ, Carleton RA, Sturner WQ. JAMA. 1982;247(18):2535.

❖ 11/12 coronary heart disease

❖Cross-country skiing: 1 death per 607 000 hours

Young athletes (<35 years)

• Hypertrophic cardiomyopathy (HCM) – 36%

•Anomalous origin of a coronary artery – 17%• Myocarditis – 6%

• Arrhythmogenic right ventricular cardiomyopathy – 4%

• Mitral valve prolapse – 4%

• Aortic stenosis – 3%

United States: SCD among 1435 young competitive athletes Recommendations and considerations related to preparticipation screening for cardiovascular abnormalities in competitive athletes: 2007 update: a scientific statement from the American Heart Association Council on Nutrition, Physical Activity, and Metabolism: endorsed by the American College of Cardiology Foundation. Maron BJ, Thompson PD, Ackerman MJ, Balady G, Berger S, Cohen D, Dimeff R, Douglas PS, Glover DW, Hutter AM Jr, Krauss MD, Maron MS, Mitten MJ, Roberts WO, Puffer JC, American Heart Association Council on Nutrition, Physical Activity, and Metabolism. Circulation. 2007;115(12):1643.

Young athletes (<35 years)

❖ Arrhythmogenic right ventricular cardiomyopathy – 22%

❖ Coronary atherosclerosis – 18%❖ Anomalous origin of a coronary artery – 12%

❖ Mitral valve prolapse – 10%

❖ Myocarditis – 6%

❖ Hypertrophic cardiomyopathy – 2%

Italy: 49 athletes under age 35 with SCD

Screening for hypertrophic cardiomyopathy in young athletes. Corrado D, Basso C, Schiavon M, Thiene G N Engl J Med. 1998;339(6):364.

Screening?

❖ Low incidence

❖ Multiple times

❖ ECG poor sensitivity and specificity

❖ Unknown proportion false positives/false negatives

Screening!

❖ Family screening after SCD in the young

❖ 40-70% genetic cause

Congenital heart defects

CHD definition

❖ Structural abnormalities of the heart or intrathoracic vessels with functional or potentially functional significance

❖ Not cardiomyopahties

❖ Not primary arrhytmias

Epidemiology

❖ <1 % of all sudden cardiac deaths

❖ 0-2 years

❖ Congenital heart defects 84%

Incidence, causes, and survival trends from cardiovascular-related sudden cardiac arrest in children and young adults 0 to 35 years of age: a 30-year review. Meyer L, Stubbs B, Fahrenbruch C, Maeda C, Harmon K, Eisenberg M, Drezner J Circulation. 2012;126(11):1363

Epidemiology❖ The risk of SCD increases with age

(> 20 years of age)

❖ Highest risk with…

❖ Tetralogy of Fallot (ToF)

❖ Transposition of the great arteries (TGA)

❖ Senning/Mustard

❖ Left ventricle outflow obstructions (LVOTO)

Epidemiology

❖ The risk of SCD increases with age (> 20 years of age)

❖ Highest risk with…

❖ Univentricular hearts

Children with CHDs & SCD

❖ Few studies!

❖ Netherlands 1990-2001, Polderman et al, Internal Journal of Cardiology, 2004

❖ 150 sudden deaths in children with CHDs

❖ 33% < 1 year of age

❖ 72% surgery

❖ 76% cardiac sudden death

❖ Arrhythmias the main cause of death

Children with CHDs & SCD

❖ Canada 1993-2001, Sanatani et al, Congenital Heart Disease, 2006

❖ 80 children with sudden death and CHDs

❖ Arrhythmias 30%

Data sources❖ The Medical Births Registry of Norway

❖ Patients administrative systems in all hospitals in Norway (The cardiovascular Disease in Norway (CVDNOR))

❖ The Oslo University Hospital´s clinical registry for congenital heart defects (Berte)

❖ The Norwegian Cause of Death Registry

❖ Medical records at Oslo University Hospital

Study population

❖ 943 871 live born children in Norway 1994-2009

❖ 11 272 (1.2%) with CHD

❖ 23.7% severe CHD

Results

❖ 11 272 children with CHD

❖ Mean follow-up 10.6 years (+- 4.5 years)

❖ 19 sudden deaths

❖ 12 chromosomal aberrations and/or extracardiac malformations

❖ 5 with infection at the time of death

Results

❖7 sudden cardiac deaths❖Diagnoses

❖TGA x 2❖Tricuspid atresia x 2❖Ebstein´s anomaly❖TOF❖PDA

❖Cardiac surgery in 6

❖Arrhythmias

Results

❖ Estimated rate of non-operative sudden unexpected cardiac death in children 2-18 years old

❖ Severe CHDs: 25 per 100 000 person-years

❖ Non-severe CHDs: 1 per 100 000 person-years

Results

No death during physical activity!

Conclusions

❖The benefits of physical activity in children with CHDs outweigh the low risk of sudden unexpected death!

Conclusions

❖We do not find any support for restricting physical activities based on the risk of sudden unexpected death in most children with CHDs

sudden cardiac death-kopi - fysio.no · epidemiology marathon running: 1 deaths per 50 000 hours...

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