Consultant Cardiologist "Blackouts” Dr Paul Venables Consultant Cardiologist Paul.Venables@ipswichhospital.nhs.uk

Blackouts Introduction NICE Clinical Guideline Red Flags Epidemiology Historic standard of care Key recommendations Practice points Red Flags Background on syncope NICE: Important cardiovascular documnt we ought to have a working knowledge of, aim to summarise the main points

Blackout: Definition Loss of consciousness Rapid onset Character: Loss of consciousness Rapid onset Transient/Short duration Spontaneous recovery TLoC (blackout) is a spontaneous loss of consciousness with complete recovery Syncope is a T-LOC due to transient global cerebral hypoperfusion TLOC: Most frequently cardiovascular, epilepsy, or more rarely Psychogenic or rare cause such as metabolic Syncope: characterized by rapid onset, short duration, and spontaneous complete recovery. Excludes TLOC caused by other mechanisms ie epilepsy/hypoglcaemia Important to consider these as part of a wider framework, because the history may not be claer cut for ex possible syncope in an elderly patient.

Epidemiology Increase with old age Peak in teenage² Incidence 2.6 -19.5/1000¹ Often unreported Affects half population during lifetime Incidence according to age and sex Graphic data based on Framingham Approximate around 1% of population annually Soteriades et al. NEJM 2002 Ganzeboom et al. J Cardiovasc Electrophysiol 2006

Blackout in the UK: Scale 4% ED attendances 550,000 per annum Primary care 6% Acute admissions >100,000 per annum¹ Average Length of stay 3.9 days Falls not included Finished consultant episodes: Syncope /collapse Staggering numbers Additionally many present to primary care- number difficult to dtermine Graph shows over 100K admission purely for syncope Hospital episode statistics : 44-266, 000 admissions PA, Massive bed use Not specified admission speciality 1 .NHS Health and Social care information centre (2009): Hospital episode statistics

Blackout: Causes Reflex syncope 56-73% Orthostatic Hypotension 1-10% Cardiac 6-37% Neurological 1-6% Unexplained 5-20% Psychogenic Rare

Survival after presentation Framingham data: Survival after presentation Underlines that The underlying cause of Syncope determines survival The 10% with Cardiac cause has significant mortality, over 10% even afetr a single year Green line is unknow: Hybrid of vasovagal and more malignant conditions Soteriades et al. NEJM 2002

Both Comprehensive systematic reviews of available evidence Expert Panel recommendations where evidence is poor NICE: Bottom-up approach: Algorithm designed to define appropriate pathways. Where necessary relate to the other available guidance Looking at the point of presentation ESC: Comprehensive document: Designed to give clarity about Classification of syncope, recommend strategies for diagnosis and management and risk stratification No going on to talk aboutthe NICE guideline over the next 10 minutes

NICE Clinical Guideline 109: Historic standard of care Range of clinicians Non-Standardised investigation: Underuse of ECG Overuse of EEG Poor Referral: Wrong speciality or unnecessary Delayed diagnosis: Dangerous conditions missed Inaccurrate: 20-30% “epilepsy” had cardiac cause¹ Inefficient: Resource implications Next 10 mins I’ll go on to talk about the NICE guidance We have seen that syncope is frequent cause of presentation to the NHS. This is a summary of how NICE felt Tloc was currently being managed Range of clinicians Data from neuro literature>74,000 people in UK treated for epilepsy without the condition£189 million economic cost Dangerous conditions: Overall mortality rates high: High Risk patient not identified eg ischaemic cardiomyopathyNICE Expert panel Innappropriate testing: Holter monitors used in low Fz setting of low yield of only 1-2% 1. NICE CG20 Epilepsy in adults and children 2004

NICE Clinical Guideline: Key priorities Define pathways: Algorithm Initial assessment: History ECG Risk stratification Appropriate referral for specialist assessment Importance of obtaining accurrate history and collateral history. Witness. Important for Primary care, Emergency settings. And of ECG: Recognition that abnormal ECG is an important maker of SCD

Initial assessment: History and examination Exact history of event: Personal and collateral history Posture, prodrome etc Previous blackout? Medical history and family history Medication Examination Vital signs Other cardiovascular and neurological signs +/- Lying and standing blood pressure

1.1.4.2 Red flags: Urgent assessment and treatment Refer for specialist cardiovascular assessment within 24 hours, patients with: ECG abnormality Heart failure TLoC during exertion Family history of SCD <40 or ICC New or unexplained breathlessness Heart murmur Consider referring >65 years and TLoC without prodromal symptoms Once TLOc confirmed the algorithm invilved a checklist of Red Flags There is evidence suggesting 6.1-11.5% major adverse events in allcomers presenting to ED with syncope Should have high sensitivity for picking up high risk patients, at the expense of being overly inclusive The GDG therefore decided to also take into account the significant univariate and multivariable predictors which they predicted poor short term outcomes. FH included as rare but serious >65 is predictor of poor outcome longer term, but included due to opinion of GDG

1.1.2.3 ECG “Red Flags" Use automated interpretation...... if not possible obtain expert opinion! Conduction abnormality Inappropriate persistent bradycardia Any ventricular arrhythmia (including ventricular ectopic beats) Long QT (corrected QT > 450 ms) and short QT (corrected QT < 350 ms) Brugada syndrome Ventricular pre-excitation Left or right ventricular hypertrophy Abnormal T wave inversion Pathological Q waves Atrial arrhythmia (sustained) Paced rhythm Inclusive list, to avoid missing high risk patients

Diagnosis based on the initial assessment: Diagnose uncomplicated faint when: There are features suggestive of uncomplicated faint such as: Posture Provoking factors (such as pain or a medical procedure) Prodromal symptoms Diagnose situational syncope when: Syncope is clearly and consistently provoked by straining during micturition or by coughing or swallowing Diagnose orthostatic hypotension when: The history is typical and Lying and standing blood pressure confirms postural drop There are no features that suggest an alternative diagnosis or red flag If Hx suggestive Provoking OH if typical Hx and postural drop is noted Appropriate advice and education in the first instance and review of medication

1.2.3 Referral for specialist cardiovascular assessment Refer all people with TLoC for a specialist cardiovascular assessment except: Uncomplicated faint Situational syncope Orthostatic hypotension Presentation is strongly suggestive of epileptic seizures Everyone other than this should be referred for specialist opinion: most to Cardiology, but Neuro in case of....

1.3.2 Diagnostic tests for different types of syncope Ambulatory ECG: Arrhythmic cause Unexplained cause (after CSM where appropriate) Exertional syncope: Initial investigations negative CSM If history suggestive or unexplained and >60yrs

Criteria to determine type of ambulatory ECG: TLoC at least several times a week: Holter monitoring If no TLoC occurs during monitor, offer external event recorder TLoC every 1–2 weeks: External event recorder Further TLoC outside the period of external event recording, offer an ILR TLoC less than once every 2 weeks: Offer an ILR Holter monitor should not usually be offered unless there is evidence of a conduction abnormality on the 12-lead ECG Effective

Implantable loop recorders Subcutaneous Patient and automatic activation Stores EGM Lasts 2 years Home monitoring

NICE: ILR vs “Conventional testing” Low diagnostic yield with external recorders Much better symptom–ECG correlation with ILR High initial cost May be more cost-effective 44 vs 10% Medtronic Farwell DJ, Freemantle N, and Sulke AN (2004) Use of implantable loop recorders in the diagnosis and management of syncope, European Heart Journal, 25(14):1257-63. Medtronic Krahn AD, Klein GJ, Yee R, Skanes AC, and REVEAL Investigators (2001) Predictive value of presyncope in patients monitored for assessment of syncope, American Heart Journal, 141(5):817-21. american Extrapolated Note: Possibly lower hospitalisation

Tilt Test Vasovagal syncope: Consider a tilt test only if: Recurrent and affecting their quality of life, or high risk of injury Pacing considered In patients with normal hearts and a suggestive history the pre-test probability is high and contribute little to diagnosis And in this group where the test is negative the most likely diagnosis is still VVS

Advice for patients: Condition appropriate advice Driving Safety at work

Summary: Practice points History and ECG: Red flags: Refer “urgently” Epilepsy: Refer to Neurology Unclear Cause: Refer to Cardiology Reflex syncope, Situational syncope and Orthostatic hypotension: Advise and Reassure Separate the wheat from the chaff

Summary: Loads more ECG’s: Robust diagnosis: ECG red flags Diagnose and treat severe cardiac conditions Avoid misdiagnosis, especially epilepsy

Blackouts: Clinical ECG’s not to be missed Sudden cardiac death Inherited cardiac conditions

80F unwitnessed fall ? blackout Reassure? b) Emergency admission? c) Refer to Specialist?

Complete Heart Block Class 1 indication for pacemaker

73M brief blackout standing in church Reassure? b) Emergency admission? c) Refer to Specialist?

Conduction system disease RBBB, left axis deviation 2a indication for pacemaker if other causes excluded

24 male with palpitations and blackout: Reassure? b) Emergency admission? c) Refer to Specialist?

Wolff-Parkinson-White syndrome (WPW) Common 0.1% prevalence AVRT Sudden Cardiac Death: - 0.15 – 0.25% per year Pre-excited AF VF induced by “R on T” Avoid AV Node blocking drugs - Short refractory period- Rapid conduction velocity - Short refractory period- Asymptomatic - Palpitation - SCD - Rapid conduction velocity AP’s are thin fibres of myocardial cells, permitting electrical communication between the atrium and ventricles extrinsic to the normal AV node. may occur at rates approaching 300bpm, with multiple R-on-T stimuli risking VF. 31

Pre-excited AF Fast Broad Irregular Can occur following adenosine for SVT: where SVT was AVRT as AF is sometimes started by adenosine. This may be distinguished from VT by recognising the characteristic QRS broadening pattern from the delta wave compared to the uniformly broad QRS during VT.

29M blackout playing football a) 24 hour tape? b) Refer to specialist? c) Admission?

Hypertrophic cardiomyopathy 1 in 500 people AD Three of the HCM-causing mutant genes predominate in frequency— i.e., beta-myosin heavy chain (the first identified), myosin binding protein C and cardiac troponin-T probably comprise more than one-half of the genotyped patients to date. 34

Typical electrocardiographic pattern of Brugada syndrome, with ST segment elevation in the right precordial leads, coved type. Na channel gene SCN5A ©2011 by BMJ Publishing Group Ltd and British Cardiovascular Society

Brugada Syndrome Gene defect of cardiac Sodium Ion-channels (SCN5A) Associated with SCD Development of VF ICD: Syncope aborted SCD EPS inducibility Identification of families

ECG with prolonged QT in a patient with long QT syndrome. ©2011 by BMJ Publishing Group Ltd and British Cardiovascular Society

Long QT-Syndrome QTc >450ms Genetic Ion-channel defect Syncope (seizures) and SCD Drugs

Triggered activity Dispersion of refractoriness After depolarisations in phase 3 or 4 Exceed threshold Trigger further depolarisation Torsades de points in LQTS

Precordial leads of an ECG from a 44-year-old woman recorded during regular sinus rhythm, with an epsilon wave (arrow) in leads V1–V. The ECG shows a right bundle branch block pattern.

Arrythmogenic Right Ventricular Cardiomyopathy (ARVC) Multiple gene defects: Desmosomes AD Syncope SCD Palpitation HF

Sudden cardiac death CHD Cardiomyopathy: Channelopathies: 80% Coronary Heart Disease CHD Cardiomyopathy: Hypertrophic cardiomyopathy Channelopathies: Long QT syndrome Brugada syndrome “Electrical” diseases: Wolff-Parkinson-White 5% Other* 15% Cardiomyopathy Incidence 0.1% gen pop, 5% prior MI, 15% LVEF <35%, 20% prior SCD. SCD is first and most common presentation of CHD American Heart Association. Heart Disease and Stroke Statistics—2003 Update. Dallas, Tex.: American Heart Association; 2002. 2 Adapted from Heikki et al. N Engl J Med, Vol. 345, No. 20, 2001. 3 Myerberg RJ. Heart Disease, A Textbook of Cardiovascular Medicine. 6th ed. P. 895. 42

Prevention of SCD >95% patient die after out of hospital cardiac arrest Identify patients at risk before it occurs ICD is proven to improve survival as a primary preventative treatment in: Ischaemic/non-ischaemic cardiomyopathy Inherited cardiac conditions

Sudden death syndromes: Technology Impact Diagnostic devices Pacemakers Implantable defibrillators Catheter Ablation Prevention of SCD Cure Stroke prevention Patient reassurance

Thankyou www.guidance.nice.org.uk/CG109 www.arrhythmiaalliance.org.uk www.dft.gov.uk/dvla/medical/ataglance.aspx Paul.Venables@ipswichhospital.nhs.uk