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 over time → becomes stiff and harder to fill → 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

Question 13

Management of stenotic valve

Answer 13

Aortic valve replacement
Via surgical valve prosthesis and trans catheter valve replacement

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, diffuse crackles on lung auscultation, bounding/corrigan pulse,pulmonary congestion

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 (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

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
Chronic:SLE, hypertrophic cardiomyopathy,scleroderma,drug related

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, serum creatinine up due to kidney damage, peripheral oedema, O2 sat decrease and tachypnoea, wheeze & crackles

• transthoracic echocardiography, ECG, CXR

Question 27

Possible investigation of mitral regurgitation

Answer 27

transthoracic echocardiography, ECG, CXR

Question 28

Management of mitral regurgitation

Answer 28

acute severe: replace and repair valve structures. 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

myocardial ischaemia, heart valve defects, familial/inherited, myocarditis, alcoholism, thyroid disease, autoimmune disorders, drug ingestion,pregnancy (perioartum cardiomyopathy)

• 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 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

dyspnoea & pulmonary congestion (crackles), cold extremities, displaced apex beat, fatigue, angina, peripheral oedema

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

S4, syncope, fatigue, angina, pulmonary congestion & oedema, systolic murmur (interventricular septum involvement narrows the outflow tract), sudden cardiac death

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

Question 42

Pathophysiology of restrictive cardiomyopathy

Answer 42

infiltrative cardiomyopathy = deposition of abnormal substances into heart wall tissue → endomyocardial fibrosis → ventricular wall stiffening → diastolic dysfunction → atrial enlargement → conduction abnormalities and diastolic heart failure due to restriction → eventually adverse remodelling and systolic dysfunction

Question 43

Signs and symptoms of restrictive cardiomyopathy

Answer 43

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)

Question 44

Management of restive cardiomyopathy

Answer 44

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

Question 45

Management of aortic regurgitation

Answer 45

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 46

Mitral stenosis aetiology

Answer 46

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 47

Investigation of mitral stenosis

Answer 47

ECG
Chest X ray
Trans thoracic echo

Question 48

Difference in signs of right heart and left heart failure

Answer 48

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