Structural Heart Disease 2

Question 1

Are ventricular and atrial septal defects congenital or acquired

Answer 1

Congenital

Question 2

what is coarctation of the aorta?

Answer 2

congenital narrowing of the aorta → pinching effect at curve point

Question 3

what is the tetralogy of Fallot?

Answer 3

ventricular septal defect, pulmonary stenosis, right ventricular hypertrophy and overriding aorta all occurring together (congenital)

Question 4

what is overriding aorta?

Answer 4

congenital heart defect → aorta placed over a ventricular septal defect → transports some deoxygenated blood from right ventricle to the rest of the body

Question 5

examples for valvular defects

Answer 5

mitral/aortic stenosis/regurgitation

Question 6

difference between stenosis and regurgitation?

Answer 6

stenosis = stiffening, can’t open fully. regurgitation = can’t close fully, leads to backflow

Question 7

aortic sclerosis vs aortic stenosis?

Answer 7

sclerosis precedes stenosis. = valve thickening but without flow restriction

Question 8

how to confirm aortic stenosis?

Answer 8

suspected by ejection systolic heart murmur, confirm by echocardiography,Doppler echo for pressure gradient

Question 9

Aortic stenosis risk factor

Answer 9

hypertension, high LDL levels, smoking, chronic kidney disease, elevated c reactive protein ,radiotherapy,old age,congenital bicuspid valves

Question 10

Causes of aortic stenosis

Answer 10

rheumatic heart disease, calcium build up, congenital heart diseases e.g. malformed valves

Question 11

consequences of stenotic valve → pathophysiology?

Answer 11

Degeneration (old age)/congenital malformed valves cause wear and tear of valves//untreated group A streptococcus untested causes antibodies to attack valves—>fibrosis and calcification of aortic valve—>
left ventricle has to contract harder → LV myocardial hypertrophy (concentric) over time → becomes stiff and harder to fill diastolic dysfunction → pressure overload backs up and causes left atrium dilation → lung pressure up and pulmonary congestion

Question 12

Symptoms of stenosis valve

Answer 12

syncope due to lack of blood flow to brain on exertion, dyspnoea & crackles on lung auscultation due to pulmonary congestion, angina on exertion due to less coronary artery flow (hypertrophic heart muscles and less perfusion
Ejection systolic murmur crescendo decrescendo

Question 13

Management of stenotic valve

Answer 13

Aortic valve replacement
Via surgical valve prosthesis and trans catheter valve replacement
For severe AR only
Currently surgical have mechanical and bio prosthetic valves available
For minimal surgery use bio prosthetic
For transcatheter use bio prosthetic

Question 14

what is aortic regurgitation + subtypes?

Answer 14

diastolic leakage of blood from aorta into ventricles. can be acute (sudden onset pulmonary oedema, hypotension, cardiogenic shock) or chronic (eventually congestive cardiac failure)
Occurs due to incompetence of valve leaflets resulting from intrinsic valve disease or dilation of aortic root

Question 15

Causes of aortic regurgitation

Answer 15

split into root dilatation and inflammation of valvular endocardium

rheumatic heart disease, aortic valve stenosis, infective endocarditis, congenital heart defects,congenital bicuspid valves

marfan’s syndrome (root dilatation), connective tissue disease (root dilatation),idiopathic,ankylosing spondylitis ,traumatic

Question 16

Pathophysiology of aortic regurgitation

Answer 16

Marfans (aortic root dilation)/untreated group A streptococcus/congeital (inflammation of valvular endothelium)—>valve leaflets close poorly when aortic pressure is higher than LV during diastole causing back flow of blood—>acute dilatation as increases in stroke volume or chronically LV dilated and eccentricity hypertrophies to accommodate increase in volume—>systolic heart failure as excessive stretching weakened myocardium

Question 17

Symptoms of aortic regurgitation

Answer 17

diastolic murmur, louder S3, angina on exertion and fatigue bounding/corrigan pulse,pulmonary congestion

ACUTE-cardio genetic shock tachycardia cyanosis pulmonary Odema and diastolic murmur

Chronic
Wide pulse pressure
Corrigan pulse

Question 18

Investigation of aortic regurgitation

Answer 18

echocardiography → can see presence and severity of aortic regurgitation

Question 19

Main cause of mitral stenosis

Answer 19

rheumatic fever (in developing countries)
Leads to pulmonary hypertension and right heart failure as disease progresses

Question 20

Mitral stenosis Pathophysiology

Answer 20

valve thickening due to fibrous deposition and calcification e.g. from inflammatory causes and chordae tendinae shortening → obstructed blood flow through mitral valve → impaired emptying of left atrium and filling of LV → more back pressure in left atrium and lungs, less cardiac output and stroke volume leading to congestive heart failure->increase in RV pressure leads to hyoertrophy of right ventricle so right sided heart failure

Question 21

Signs and symptoms of mitral stenosis

Answer 21

atrial fibrillation bc stretch of atrial conduction fibres, mid diastolic murmur opening snap (turbulent blood flow), right sided cardiogenic shock/congestive heart failure, dyspnoea,left atrial enlargement causes compression of surrounding structures
May see dysphagia,haemoptysis

Question 22

Management of mitral stenosis

Answer 22

if asymptomatic none needed, if symptomatic → diuretic, balloon valvotomy, valve replacement and b blockers
Severe asymptomatic. No therapy usually but can get adjuvants balloon valvotomy

Question 23

Mitral regurgitation

Answer 23

inappropriate backflow of left ventricle blood into left atrium

Question 24

Causes of mitral regurgitation

Answer 24

Acute:mitral valve prolapse, infective endocarditis, rheumatic heart disease,prosthetic mitral valve dysfunction , following valvular surgery
Chronic:SLE, hypertrophic cardiomyopathy,scleroderma,drug related,rheumatic heart disease

Question 25

Pathophysiology of mitral regurgitation

Answer 25

number of causes lead to structural defects in the mitral valve → improper closing → backflow and increase of volume and pressure in left atrium → increased volume pushed into LV in next diastole → LV dilation and decrease in function → decreased cardiac output = congestive heart failure + increased back pressure in lung vasculature = congestion

Question 26

Signs and symptoms of mitral regurgitation

Answer 26

holosystolic murmur, S3 heart sound, O2 sat decrease and tachypnoea, wheeze & crackles and frothy sputum Signs of CHF dyspnoea

Question 27

Possible investigation of mitral regurgitation

Answer 27

transthoracic echocardiography, ECG, CXR,cardiac mri/ct

Question 28

Management of mitral regurgitation

Answer 28

acute severe: replace and repair valve structures eg a prosthetic ring can be inserted to reshape the valve chronic asymptomatic nothing but chronic symptomatic = surgery

Question 29

types of cardiomyopathy?

Answer 29

dilated, hypertrophic, restrictive

Question 30

consequence of dilated cardiomyopathy?

Answer 30

ventricular dilatation and thin walls leads to systolic dysfunction and heart failure and hypokinesis

Question 31

Causes of dilated cardiomyopathy

Answer 31

Idiopathic Genetic Toxins alcohol chemo Pregnancy (peripartum cardiomyopathy) Viral infections (myocarditis) Tachycardia related cardiomyopathy Thyroid disease Muscular dystrophy

Question 32

Pathophysiology of dilated cardiomyopathy

Answer 32

inflammatory/toxic damage and death of myocytes → enlargement of LV without corresponding myocardial mass increase → gradual overdistention → systolic dysfunction → cardiac output down → volume overload and congestive heart failure

Question 33

Signs and symptoms of dilated cardiomyopathy

Answer 33

Echo shows dilated left ventricle with reduced systolic fraction and global hypokinesis Syncope A fib Palpitations Can lead to heart failure

Question 34

Management of dilated cardiomyopathy

Answer 34

consider underlying cause, then symptomatic treatment e.g. arrhythmias and thrombotic events, diet modification etc Use SGLT2 before any medication Heart failure therapy-ACEi,beta blockers,mineralcorticoid receptor agonists Diuretic for fluid overload Anticoagulant fir a fib Cardiac resynchronization therapy/cardioverter defibrillator or LVAD while waiting for transplant

Question 35

what is hypertrophic cardiomyopathy?

Answer 35

increase in LV wall thickness not solely explained by abnormal loading Caused by missense mutation in 1 of @0 genes that code for proteins of cardiac sarcomere

Question 36

Cause of hypertrophic cardiomyopathy

Answer 36

genetic → often autosomal dominant

Question 37

Pathophysiology of hypertrophic cardiomyopathy

Answer 37

thickening of LV myocardium, frequently involves interventricular septum causing disruption in left ventricular outflow tract→ disorganised myocytes possibly with diffuse interstitial/focal replacement fibrosis → signal conduction disrupted → ventricular arrhythmias → sudden cardiac death

Question 38

Signs and symptoms of hypertrophic cardiomyopathy

Answer 38

Ajoritt pt are asymptomatic but some may experience dyspnea Angina Syncope Death from, cardiac causes

Question 39

Management of hypertrophic cardiomyopathy

Answer 39

Based on symptoms If have atrial fibrillation:electrical/oharmalogical defibrillation,anticoagulant therapy Non obstructive and LVEF >50 then give beta blockers,calcium channel blockers such as verapamil or diltiazem,disopyramide (anti arrhythmic) or diuretic Or if <50 give b blocker,ACEi/ARB,MRA,diuretic if resistant then do heart transplant,cardiac resynchronisation,ventricular assist device,heart transplant If obstruction then b blocker,verapamil or dilitazem,disopyramide or cibenzoline or surgery,PTSMA,pacing therapy

Question 40

what is restrictive cardiomyopathy?

Answer 40

diagnosed if restrictive ventricular filling pattern is established

Question 41

Causes of restrictive cardiomyopathy

Answer 41

idiopathic, familial(troponin I or desmin mutation), various systemic disorders such as sarcoidosis,haemochomostasis,amyloidosis,fabrys,previous radiation,carcinoid syndrome,scleroderma,anthracycline toxicity *histopathology shows non caseating granulomas so most likely Y sarcoidosis*

Question 42

Signs and symptoms of restrictive cardiomyopathy

Answer 42

ascites and pitting oedema, S4, hepatomegaly, venous pressure up, easy bruising and weight loss (all mostly due to right heart failure from increased venous pressure),palpitations,sob, Biopsy’s may show non caseating granulomas

Question 43

Management of restive cardiomyopathy

Answer 43

heart failure medication e.g. ACE inhibitors, diuretics, aldosterone inhibitors antiarrhythmic therapy, pacemaker immunosuppresion via steroids, transplant

Question 44

Management of aortic regurgitation

Answer 44

Aortic valve replacement Acute AR is a medical emergency so replace valve immediately Asymptomatic pt with chronic severe AR give vasodilators therapy to improve haemodynamics reducing the need for aortic valve replacement Treat rheumatic fever and infective endocarditis

Question 45

Mitral stenosis aetiology

Answer 45

Rheumatic fever Carcinoid symptoms Use of ergot/sertogeneric drugs,SLE,mitral annular calcification due to ageing,amyloidosis,rheumatoid arthritis,whipple disease,congenital deformity of the valves cause wear RRAWCCUMS

Question 46

Investigation of mitral stenosis

Answer 46

ECG Chest X ray Trans thoracic echo

Question 47

Difference in signs of right heart and left heart failure

Answer 47

Right heart -peripheral Oedema eg leg swelling and raised jugular venous pressure Left heart failure-pulmonary Oedema

Question 48

Mitral regurgitation aetiology

Answer 48

Acute Rheumatic heart disease Infective endocarditis Mitral valve prolpase Prosthetic mitral valve dysfunction After valvular surgery Chrinuc Rheumatic heart disease SLE scleroderma Hypertrophic cardiomyopathy Drug related

Question 49

Infective endocarditis

Answer 49

Infection of the endocardium which affects the heart valves Bacteria enter blood stream and form vegetation (bacteria platelets and fibrin) Most commonly due to streptococcus

Question 50

How do you diagnose infective endocarditis/symptoms

Answer 50

Revere Malaise Sweats Unexplained weight loss New heart murmur Anaemia Raised infection marker Echo shows vegetation abcess valve perforation or dehiscence of prosthetic valve Regurgitation of affected valve often seen Transoesophegal ech has a higher sensitivity than transthroacic

Question 51

Features of heart decompensation

Answer 51

Shortness of breath Frequent coughing Swelling of legs and abdomen Fatigue Raised JVP lung cracked and odema May also have vascular and emboli phenomena *stroke janeway lesion,splinter/conjunctival haemorrhages* Immunological phenomena *oslers nodes or roth spots* Decompensation is where heart can’t maintain adequate circulation

Question 52

What part of the heart does infective endocarditis affect

Answer 52

Endocardium esp valves Aortic most commonly affected Aortic>mitral>right sided valves Bacteria will attach to places where more damage is present thus occurs more frequently at sites of turbulent flow such as valves

Question 53

How are IV drug users affected by infective endocarditis

Answer 53

Increased risk as bloodstream is exposed to bacteria More common in those that are immunosuppressed or have congenital heart defects Dental surgery increases the risk as well

Question 54

RHD vs rheumatic fever

Answer 54

Rheumatic fever is temporary inflammation caused by group A streptococcus This affects the connective tissue in the heart and else where RHD is a permanent condition following RF, there’s a lag between the two from 7-30 years

Question 55

What is the most common valve dysfunction by rheumatic heart disease

Answer 55

Mitral stenosis Can affect aortic valve causing regurgitation and then stenosis Can also affect tricuspid valve

Question 56

Mitral stenosis clinical signs and symptoms

Answer 56

Fluid overload Peripheral odema Malar flush Hoarse voice due to compression of left recurrent laryngeal nerve by dilated left atrium Loud s2 RV heave Haemoptysis fatigue SOB paroxysmal nocturnal dyspnoea palpitations Palpitations due to a fib

Question 57

Jones criteria

Answer 57

Used to RHD **evidence of preceding group A streptococcal infection** Major criteria Carditis Arthritis Chorea Erythema marginatum Subcutaneous nodule Minor criteria Polarthralgia Fever >38.5 Elevated CRP/ESR Prolonged PR interval Need 2 major criteria OR 1 major and 2 minor OR 3 minor

Question 58

Pathognomic features of rheumatic heart valves

Answer 58

Leaflet or chordal thickening Prolapse of leaflet Excessive leaflet tip motion during systole *due to initial inflammatory process of acute rheumatic fever affecting connective tissue of valves*

Question 59

Mitral stenosis management for rhd

Answer 59

Valve commissurotomy (separation of fused commisured which is where leaflets meet) Valve replacement Manage complications eg heart failure and a fib use vit k antagonist If there is no annular calcification then we can replace valve easily but if there is delay until pt becomes severely symptomatic

Question 60

When do we do PMC Percutaneous mitral commisuratomy

Answer 60

If there is moderate to severe mitral stenosis MVA <1.5 In symptomatic patients eg fatigue No significant mitral regurgitation Done when there no annular calcification then

Question 61

Dukes criteria for infective endocarditis

Answer 61

Major criteria Persistent positive blood culture ECHO:vegetation,dehiscence of prosthetic valve abcess New valvular regurgitation murmur Coxiella burnetti infection Minor criteria Predisposing heart condition or IV drug use Fever greater than 38 Vascular:embolism to organs and Braun Immunological:glomeruloneohritis,oslers node,roth spots Postive blood cultures that don’t meet specific criteria **definitive endocarditis ** 2 major criteria 1 major 3 minor 5 minor + gram stain or culture from surgery or autopsy **possible endocarditis** 1 major and >1 minor 3 minor **rejected endocarditis** Resolution after less than 4 days abx No evidence of infection after surgery Define or possible criteria not met