1. Sudden Cardiac Death in Women Briain MacNeill Galway University Hospital Oct 6 th, 2012

2. Women and Heart Disease Myths and Truths MYTH: Most women die from cancer. TRUTH: Heart disease is the leading cause of death of women in North America and Europe. Nearly five times as many women will die from heart attacks alone this year than will die from breast cancer.

3. Women and Heart Disease Myths and Truths MYTH: Heart disease is a man’s problem. TRUTH: Since 1984, more women than men have died of heart disease each year. Women are 28% more likely than men to die within the first year after a heart attack.

4. Women and Heart Disease Myths and Truths MYTH: Only older women have heart disease. TRUTH: The rate of sudden cardiac death of women in their 30s and 40s is increasing much faster than in men their same age - rising 21 percent in the 1990s.

5. Women and Heart Disease Myths and Truths MYTH: Women and men with heart disease get the same care. TRUTH: Women are less likely to receive Aspirin, beta blockers, statins, ACE inhibitors and defibrillators. Men are 52% more likely to be referred for angiography

6. SUDDEN CARDIAC DEATH IN WOMEN WELL VISITS SPORTS CLEARANCE PALPITATIONS SYNCOPE FAMILY SCREENING CHEST PAIN

7. SUDDEN CARDIAC DEATH IN WOMEN WELL VISITS SPORTS CLEARANCE PALPITATIONS SYNCOPE FAMILY SCREENING CHEST PAIN

8. Well Visit Physical examination: BMI, Pulse and Blood pressure measurement Heart murmur (supine / sitting / standing) Peripheral Pulses Stigmata of Marfans Syndrome Personal History: Heart murmur Systemic hypertension Fatigability Syncope Exertional dyspnoea Exertional chest pain Family History: Premature sudden death Heart disease in relatives Cardiac Risk Profile Exercise Capacity Cardiac Symptoms Lipid Levels

9. The #1 Preventable Risk - Smoking A. 50% of heart attacks among women are due to smoking. Smokers tend to have their first heart attack 10 years earlier than nonsmokers. Smokers are 4-6x’s more likely to suffer a heart attack Women who smoke and take OCP’s increase their risk of heart disease 30x Smoking cessation was associated with a 36% reduction in mortality among patients with CHD

10. Obesity and Coronary Heart Disease Mortality Nurses’ Health Study: Women who never smoked Relative Risk of Coronary Heart Disease mortality Body Mass Index (kg/m 2 ) P<0.001 for trend Manson JR, et al. N Engl J Med. 1995;333:677-685.

11. Who to Treat Practice Prevention Low Risk Women <10%: Intervention is useful and effective: Lifestyle Interventions Smoking Cessation Physical Activity Heart Healthy Diet Weight Reduction Treat Individual CVD risk factors

12. Practice Prevention Intermediate Risk Women (10-20%):Smoking Cessation Physical Activity Heart Healthy Diet Weight Reduction Control BP and Lipids Class Ila- most scientific evidence favors this type of therapy: ASA Rx- as long as BP is controlled (hemorrhagic stroke) and low risk of GI bleed

13. Practice Prevention High Risk Women (>20%): Class I Smoking Cessation Physical Activity/cardiac rehab Heart Healthy Diet- DASH Diet Weight Reduction Control BP and Lipids- Statin ASA therapy Glycemic control in DM

14. Croi My Action – 1 year results

16. SUDDEN CARDIAC DEATH IN WOMEN WELL VISITS SPORTS CLEARANCE PALPITATIONS SYNCOPE FAMILY SCREENING CHEST PAIN

17. Chest Pain Algorithm

19. Not so straightforward Chest pain is the presenting symptom in <50% of women Almost half of MIs in women present with SOB, nausea, indigestion, fatigue and shoulder pain Atypical symptoms contribute to later presentation and higher rates of misdiagnosis. Women presenting with MI and CAD are more likely to be older, have a history of DM, HTN, Hyperlipids, CHF, and unstable angina than male counterparts. Women were less likely have an ECG, antianginal therapy or invasive mangaement. Women were less likely to enroll in cardiac rehabilitation after an MI or bypass surgery.

20. CHD Mortality in Younger Women Women under 65 suffer the highest relative CHD mortality

21. SUDDEN CARDIAC DEATH IN WOMEN WELL VISITS SPORTS CLEARANCE PALPITATIONS SYNCOPE FAMILY SCREENING CHEST PAIN

22. Palpitations Algorithm History, Physical, ECG Structural Heart Disease UnlikelyStructural Heart Disease Suspected ECHO, Holter, Cardiac ReviewLabs including TFTs, Drug Screen Daily Symptoms? 24 or 48 hour HolterEvent Monitor / Loop YesNo Palpitations During NSR Reassurance Consider alternatives NonVentricular Arrhythmia Treat Cause Routine cardiac evaluation Ventricular Arrhythmia Urgent Cardiac Review Angio, MRI, EP study

23. SUDDEN CARDIAC DEATH IN WOMEN WELL VISITS SPORTS CLEARANCE PALPITATIONS SYNCOPE FAMILY SCREENING CHEST PAIN

25. .Eur Heart J 2009;30:2631-2671

26. Cardiac Syncope Bradycardia SA Node Dysfunction AV Conduction Defect Medication Related Tachycardia Supraventricular Ventricular - Preserved LV - Reduced LV Hypotension Hypoperfusion Reflec Mediated Medication Related

27. SUDDEN CARDIAC DEATH IN WOMEN WELL VISITS SPORTS CLEARANCE PALPITATIONS SYNCOPE FAMILY SCREENING CHEST PAIN

28. Causes of SCD Over 35 yrs of age –Coronary Heart Disease Under 35 yrs –Cardiomyopathies –Congenital Heart Disease –‘Structurally Normal Heart’ (ion channel disorders, conduction disease) = SADS –Anomalous coronaries –Myocarditis

29. Hypertrophic cardiomyopathy (HOCM) Increased thickness of heart muscle Most common inherited cardiac disease Prevalence > 1 in 500 people carry gene >11000 in 32 counties 90% of cases thought to be inherited (runs in family) 10% ‘sporadic’ – pass on to their children? Approx 50% who inherit genetic change develop full- blown condition (‘incomplete penetrance’) Inheritance pattern Autosomal Dominant = 50% risk of inheriting gene if parent affected

30. HOCM Symptoms include : –Shortness of breath with exercise –chest pain (usually with exercise) –Diziness (at rest or with exercise) –blackouts –Palpitations –No symptoms Risk of sudden death ~ 1% per year Intensive exercise can increase risk Usually identifiable on ECG and Echo

31. Other Cardiomyopathies- Dilated May be inherited, much less common Other causes include viral illness, drugs, alcohol May cause shortness of breath, palpitations, blackout, sudden death ECG and echo usually identifies Other tests may be necessary Treatment: Medications, pacemakers and/or ICD Risk of SCD usually highest in those with poorest pump function, who usually have symptoms

32. Other Cardiomyopathies – Arrhythmogenic (ARVC or ARVD) Heart may become enlarged Scarring develops in heart Causes palpitations, dizzy spells, blackouts, shortness of breath, sudden death Often inherited May need several tests to diagnose –ECG, echo, Exercise test, Holter, Cardiac MRI Milder cases can be missed Treatment –Medications –Lifestyle modification –If high risk, recommend ICD

33. Other inherited conditions Marfan’s syndrome –Weakness of walls or large blood vessels –May be associated with tall stature and hyperflexibility, eye problems –Identified on physical exam, echo and X-ray scans Congenital heart disease –Abnormal development of cardiac structure(s) in the womb –Milder forms generally not life-threatening –< 10 % inherited, most occur spontaneously Mitral valve prolapse –1% of population have at least mild case –Severe cases may be associated with sudden death –May be over-estimated as cause of sudden death

34. Other conditions Valve disease –Usually causes a murmur –May cause reduction in exercise tolerance Anomalous coronaries –Anatomical variant in placement of blood vessels –Some may reduce blood supply during stress or exercise but most probably don’t cause problem and may be over-estimated as cause of SCD Myocarditis –Inflammation of heart muscle –Usually thought to follow viral infection –1/8 people with virus + fever have ECG change –Probably should avoid exercise during viral infection –Possible genetic predisposition to being affected by virus

35. Sudden Arrhythmic (Adult) Death Syndrome (SADS) ‘Diagnosis of exclusion’ - Electrical problem is cause of death, but no electrical activity after death so not detectable at post-mortem Sudden death occurs, and is consistent with cardiac rhythm disturbance, but post-mortem examination finds no abnormality If post-mortem not carefully done Structural cause of death may be missed Minor abnormalities may be incorrectly recorded as cause of sudden death True number of SCD which are actually due to SADS probably under-estimated

36. Electrical problems – ‘Channelopathies’ Electricity in heart is generated by pump channels in walls of each cell in heart –pump salts (Na, K, Ca) in and out of cell –Pump channel = ion channel If pump malfunctions (under or over-active) changes electrical activation of heart which causes electrical instability and increases chance of arrhythmia May not cause symptoms unless palpitations, dizzy episodes or blackouts Usually detectable on ECG (if looking for it) Different genes code for different pumps and mutations cause different conditions : –Long QT syndrome –Brugada Syndrome –Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) Not identifiable on PM Can be identified on ECG (+/- exercise test and rhythm monitor) in living 40% of families of those who die of SADS have inherited cause identified (mostly LQT syndrome and Brugada syndrome)

37. SUDDEN CARDIAC DEATH IN WOMEN WELL VISITS SPORTS CLEARANCE PALPITATIONS SYNCOPE FAMILY SCREENING CHEST PAIN

39. Piermario Morosini Fabrice Muamba

40. Etiology based on largest US data set 1)HCM – 36% 2)Coronary Anomalies 17% 3)Increased Cardiac Mass (possible HCM) 10% 4)Ruptured Aorta/Dissect 5% 5)Tunneled LAD 5% 6)Aortic Stenosis 5% 7)Myocarditis 3% 8)Dilated CM 3% 9)Idiopathic Myocdardial scarring 3% 10)Arrhythmogenic RV dysplasia 3% OTHERS… MVP CAD ASD Brugada Syndrome Commotio Cordis Complete heart block QT prolongation syndrome Ebstein’s anomaly Marfan’s Syndrome Wolff-Parkinson White Syndrome – WPW Ruptured AVM SAH

41. Sports Screening – Italian Protocol

42. Results of Screening in Veneto Italy

43. Conditions Screened

44. Piermario Morosini Fabrice Muamba

45. Will This Work in Ireland “We're taking this match awful seriously. We're training three times a week now, and some of the boys are off the beer since Tuesday.” Offaly hurler,In the week before a Leinster hurling final vs. Kilkenny. “The stopwatch has stopped. It's up to God and the referee now. The referee is Pat Horan. God is God.” Micheal O Muircheartaigh “Sean Og O'Hailpin... his father's from Fermanagh, his mother's from Fiji, neither a hurling stronghold.” Micheal O Muircheartaigh

47. Sudden Cardiac Death in Women Briain MacNeill Galway University Hospital Oct 6 th, 2012