SATURDAY CLINICAL SATURDAY CLINICAL MEETINGMEETING

SATURDAY CLINICAL SATURDAY CLINICAL MEETINGMEETING

DEPARTMENT OF CARDIOLOGYDEPARTMENT OF CARDIOLOGY

1212THTH FEB 2005 FEB 2005

DETAILS OF THE CASE• 40 years old serving soldier• Resident of Rourkela• Non-smoker, non-diabetic, non-

hypertensive• Referred from CH(NC)• Date of admission – 24 Jan 2005• Informant – self• Reliability - good

PRESENTING COMPLAINTS

• Syncope

• Angina on exertion NYHA – III • Dyspnoea on exertion NYHA – III

6months

WHAT IS SYNCOPE ?WHAT IS SYNCOPE ?WHAT IS SYNCOPE ?WHAT IS SYNCOPE ?

SYNCOPE

• A transient loss of consciousness

• Spontaneous and full recovery

• Loss of postural tone

• No prolonged confusion

Discord in theEvaluation of Syncope

NeurologistNeurologist CardiologistCardiologist

SYNCOPE: CARDIAC ETIOLOGY

• OBSTRUCTION TO FLOW (3-11%)

– HOCM, AS, MS, myxoma

– PS, PE, Pulm HTN– Tamponade

• ARRHYTHMIAS (5-30%)

– Sick sinus, AV block

– VT, SVT

SYNCOPE: NONCARDIAC• Vasodepressor (1-29%)• Situational (1-8%)• Seizure• Psychogenic• Orthostatic (4-12%)• Drug-induced (2-9%)• Carotid sinus• Neuralgia• Neurologic (TIA, stroke, migraine)

HOW TO DISTINGUISH HOW TO DISTINGUISH

SEIZURES FROM SYNCOPE ?SEIZURES FROM SYNCOPE ?HOW TO DISTINGUISH HOW TO DISTINGUISH

SEIZURES FROM SYNCOPE ?SEIZURES FROM SYNCOPE ?

SEIZURE SYNCOPE

AURA + -

CYANOSIS + -

TONGUE-BITE + -

POST-ICTAL CONFUSION

+ -

POST-ICTAL AMNESIA

+ -

POST-ICTAL HEADACHE

+ -

WHAT ARE THE CAUSES WHAT ARE THE CAUSES OF CHEST PAIN WITH OF CHEST PAIN WITH

SYNCOPE ?SYNCOPE ?

WHAT ARE THE CAUSES WHAT ARE THE CAUSES OF CHEST PAIN WITH OF CHEST PAIN WITH

SYNCOPE ?SYNCOPE ?

CONDITION MECHANISMACUTE MYOCARDIAL INFARCTION LARGE AREA OF MYOCARDIUM

AT RISK, ARRHYTHMIAS

VASOSPASTIC ANGINA ARRHYTHMIAS(VT/VF)

ANGINA PECTORIS LARGE AREA OF MYOCARDIUM AT RISK, ARRHYTHMIAS

ACUTE PULMONARY EMBOLISM OBSTRUCTION TO CIRCULATION

PERICARDIAL TAMPONADE CARDIAC COMPRESSION

TENSION PNEUMOTHORAX CARDIORESPIRATORY EMBARRASSMENT

PLEURAL HEMORRHAGE CARDIORESPIRATORY EMBARRASSMENT

UPPER GE BLEED LOSS OF BLOOD

ACUTE PANCREATITIS HYPOVOLEMIA DUE TO CHEMICAL PERITOITIS, SEVERE PAIN

HISTORY • First episode of loss of consciousness while

running in August 2004• Duration : 2-3 seconds• Preceded by angina• Similar episodes since last 6 months• No associated h/o Palpitations Sphincter incontinence/tongue bite

• Patient presented with retrosternal

discomfort associated with breathlessness

initially during unaccustomed exercise

which progressed to discomfort while

doing routine activities

WHAT IS THE MECHANISM WHAT IS THE MECHANISM OF ANGINA IN HOCM ?OF ANGINA IN HOCM ?

WHAT IS THE MECHANISM WHAT IS THE MECHANISM OF ANGINA IN HOCM ?OF ANGINA IN HOCM ?

INCREASED MYOCARDIAL OXYGEN DEMAND

REDUCED MYOCARDIAL PERFUSION

MYOCARDIAL HYPERTROPHY SMALL VESSEL DISEASE

DIASTOLIC DYSFUNCTION ABNORMAL VASCULAR RESPONSES

MYOCYTE DISARRAY MYOCARDIAL BRIDGES

LEFT VENTRICULAR OUTFLOW OBSTRUCTION

INCREASED CORONARY VASCULAR RESISTANCE

ARRHYTHMIAS

• PAST HISTORY : Not significant

• FAMILY HISTORY : No h/o sudden cardiac death No h/o premature death

TREATMENT HISTORY

• Tab Atenolol 100mg 01 OD

• Tab Isoptin 40mg 01 TDS

• Symptomatic improvement with

medication

SUMMARY ON HISTORY• 40 years old serving soldier• No modifiable risk factors• No family h/o SCD• Presented with c/o syncope, AOE /

DOE III

CLINICAL EXAMINATION• Height 166cms• Weight 68.9 Kgs• Average built• Pulse 80/min, regular, bisferiens, no

radio-radial or radio-femoral delay• BP 130/90 mmHg

WHAT IS MEANT BY WHAT IS MEANT BY

BISFERIENS PULSE ?BISFERIENS PULSE ?

WHAT IS MEANT BY WHAT IS MEANT BY

BISFERIENS PULSE ?BISFERIENS PULSE ?

• BIS means twice

• FERIENS means beating

• It is a double-peaked arterial pulse, so there is a

mid-systolic dip

• Both tidal and percussion wave are appreciable

WHAT ARE THE CAUSES OF WHAT ARE THE CAUSES OF BISFERIENS PULSE?BISFERIENS PULSE?

WHAT ARE THE CAUSES OF WHAT ARE THE CAUSES OF BISFERIENS PULSE?BISFERIENS PULSE?

• Severe aortic regurgitation

• Aortic stenosis and regurgitation

• Hypertrophic obstructive

cardiomyopathy

• Hyperkinetic circulatory states

• Muscular exercise

GENERAL EXAMINATION

• Fever (-)• Pallor (-)• Cyanosis (-)• Clubbing (-)• Jugular venous pulse ‘a’ wave

prominent• Lymphadenopathy (-)

WHAT ARE THE CHANGES WHAT ARE THE CHANGES SEEN IN SEEN IN JVPJVP IN A CASE OF IN A CASE OF

HOCM ? WHY ?HOCM ? WHY ?

WHAT ARE THE CHANGES WHAT ARE THE CHANGES SEEN IN SEEN IN JVPJVP IN A CASE OF IN A CASE OF

HOCM ? WHY ?HOCM ? WHY ?

• Prominent ‘a’ waves are seen

• It indicates hypertrophy of

ventricular septum leading to lack of

compliance of right ventricle

CVS EXAMINATION• INSPECTION

Apex visible in 6th intercostal space 1cm outside MCL

Diffuse apical impulseNo precordial bulge

• PALPATIONDouble apical impulse felt S4+No thrillNo PSH

WHAT ARE THE CAUSES OF WHAT ARE THE CAUSES OF DOUBLE AND TRIPLE DOUBLE AND TRIPLE

APICAL IMPULSE ?APICAL IMPULSE ?

WHAT ARE THE CAUSES OF WHAT ARE THE CAUSES OF DOUBLE AND TRIPLE DOUBLE AND TRIPLE

APICAL IMPULSE ?APICAL IMPULSE ?

• Double apical impulse

Decreased LV compliance

Pre-systolic apical impulse due to forceful

atrial systole

• Triple apical impulse

Late systolic bulge of left ventricle that

occurs when heart is almost empty and is

in near iso-metric contraction

• AUSCULTATIONS1 normalS2 split – paradoxicalHarsh cresendo-decresendo mid-systolic murmur grade III/VI heard between apex and left sternal border not radiating to carotid or axillaMurmur increases with standing, exercise, Valsalva maneuver (strain)Decreases with squatting, isometric hand grip, Valsalva maneuver (overshoot)

WHAT ARE THE CAUSES OF WHAT ARE THE CAUSES OF PARADOXICAL SPLIT S2 ?PARADOXICAL SPLIT S2 ?

WHAT ARE THE CAUSES OF WHAT ARE THE CAUSES OF PARADOXICAL SPLIT S2 ?PARADOXICAL SPLIT S2 ?

MECHANISM CAUSESDELAYED ELECTRICAL ACTIVATION OF LV

LBBBRV PACINGRV ECTOPY

PROLONGED LV MECHANICAL SYSTOLE

SEVERE ASSEVERE SYSTEMIC HYPERTENSIONACUTE MYOCARDIAL INFARCTIONDURING AN EPISODE OF ANGINACARDIOMYOPATHYSEVERE ARLARGE PATENT DUCTUS ARTERIOSIS

INCREASE OF HANGOUT INTERVAL ON THE AORTIC SIDE

ANEURYSM OF ASCENDING AORTA POST-STENOTIC DILATION IN AS

EARLY PULMONIC CLOSURE

EARLY ELECTRICAL ACTIVATION OF RV AS IN TYPE B WPW SYNDROME. IN WPW SYNDROME, REVERSED SPLIT OCCURS ONLY WHEN THERE IS SIGNIFICANT PRE-EXCITATIONSEVERE TRICUSPID REGURGITATION

WHAT TYPE OF MURMURS WHAT TYPE OF MURMURS CAN BE HEARD IN HOCM ?CAN BE HEARD IN HOCM ?WHAT TYPE OF MURMURS WHAT TYPE OF MURMURS CAN BE HEARD IN HOCM ?CAN BE HEARD IN HOCM ?

• Harsh cresendo-decrescendo systolic

murmur

• Holosystolic murmur of mitral

regurgitation

• Diastolic rumbling murmur in patients

with severe MR

• Murmur of aortic regurgitation

• OTHER SYSTEM EXAMINATION : NAD

INVESTIGATION

HEMOGRAM WNL

RENAL PROFILE WNL

LIVER PROFILE WNL

INVESTIGATION

ECG NORMAL SINUS RHYTHMAXIS : (– 30°)LEFT ATRIAL ENLARGEMENTPOOR PROGRESSION OF R WAVELVH PRESENT LARGE NEGATIVE PRECORDIAL T WAVES

WHAT ARE THE ECG WHAT ARE THE ECG CHANGES FOUND IN HOCM ?CHANGES FOUND IN HOCM ?

WHAT ARE THE ECG WHAT ARE THE ECG CHANGES FOUND IN HOCM ?CHANGES FOUND IN HOCM ?

• ST-T wave abnormalities• QRS complexes, tallest in the

midprecordial leads• Prominent Q waves in precordial leads• Giant negative T waves• Evidence of LAE/RAE • Short PR interval• Prolongation of QTc• Features of WPW syndrome• Arrhythmias- VPCs, VT, SVT, AF

ECHO DIMENSIONS LA – 40mm IVS(D) – 27mm LVPW(D) – 10mm IVS(S) - 30mm LVPW(S) – 19mm LVEF – 77%ASH +GROUND GLASS APPEARANCE OF SEPTUMLVOT Δ RESTING 27 mmHgPOST-SORBITRATE 60 mmHg

HOLTER OCCASIONAL SVCS VPCS OF RBBB, LBBB MORPHOLOGYONE COUPLETONE RUN OF 3 BEATSNO SINUS PAUSEASYMPTOMATIC DURING RECORD

WHO ARE THE PATIENTS WHO ARE THE PATIENTS AT HIGH RISK FOR SCD ?AT HIGH RISK FOR SCD ?WHO ARE THE PATIENTS WHO ARE THE PATIENTS AT HIGH RISK FOR SCD ?AT HIGH RISK FOR SCD ?

COURSE OF EVENTS

FINAL DIAGNOSIS

A CASE OF HYPERTROPHIC OBSTRUCTIVE

CARDIOMYOPATHY, NYHA CLASS III, IN

NORMAL SINUS RHYTHM, WITH NO

EVIDENCE OF INFECTIVE ENDOCARDITIS

WITH HIGH RISK FOR SCD

MANAGEMENT PLAN

• PRIORITIES OF THERAPY

Alleviation of symptoms

Prevention of complications

Prevention of sudden cardiac death

Management of heart failure

TREATMENT• PHARMACOLOGICAL

Atenolol 100mg 1ODVerapamil 40mg 1TDS

• NON-PHARMACOLOGICALAlcohol septal ablation

IMPORTANT POINTS IMPORTANT POINTS TO REMEMBER TO REMEMBER ABOUT HOCMABOUT HOCM

IMPORTANT POINTS IMPORTANT POINTS TO REMEMBER TO REMEMBER ABOUT HOCMABOUT HOCM

• First described by Sir Russell Brock in 1958

• Eugene Braunwald described the unique hemodynamics

• Other terms used are idiopathic hypertrophic subaortic stenosis and muscular subaortic stenosis

• Defined as myocardial hypertrophy in absence of identifiable cause

• Prevalence is 1:500 to 1:1000• Autosomal dominant disease• Most common genetically

transmitted cardiovascular disease• Genetic basis first reported by

Seidman and Seidman (chromosome 14q11-12)

• Most common cause of SCD in young including trained athletes

• Common sites of ventricular involvement are septum, apex and mid-ventricle in decreasing order of frequency

• Obstruction present in 25% of cases

• Most characteristic is diastolic dysfunction rather than systolic

• Triad of dyspnea, angina, syncope• Most common symptom is dyspnea• Angina in 70-80% cases• MI in 15% cases• Syncope in 20%, over 50% have

pre-syncope

• B-blocker is the initial drug of choice• Advantages – decreased heart rate

response to exercise• Decreased outflow gradient with

exercise• Decreased oxygen demand• Improvement in diastolic filling

• Ca-channel blockers improves abnormal diastolic relaxation

• Disopyramide with negative inotropic effect decreases the gradient and improve symptoms

• Disopyramide added to B-blocker or Ca-blocker if symptoms persist

• Pacing of right ventricular apex reduces SAM

• Pacing or sensing atrium helps to maintain hemodynamic contribution of atrial contraction

• Used in patients with assd. sinus or AV node dysfunction

• Used in elderly patient with multiple co-morbidities who are at high risk for other therapeutic modalities

• Alcohol septal ablation is new therapeutic modality

• 100% alcohol is infused in first septal perforator artey

• Produces controlled MI of proximal septum

• Remodelling of basal septum region results in reduction of outflow obstruction