Congenital Heart Disease + Valvular Heart Disease Flashcards
What does Left to right shunt and right to left shunt mean
NOTE:
- The left atrium receives oxygenated blood from the lungs to the rest of the body - SYSTEMIC CIRCULATION
- The right atrium receives deoxygenated blood returning from other parts of the body, and transports to lungs - PULMONARY CIRCULATION
- A left-to-right shunt allows the oxygenated, pulmonary venous blood to return directly to the lungs rather than being pumped to the body.
- A right-to-left shunt allows the deoxygenated, systemic venous return to bypass the lungs and return to the body without becoming oxygenated
What congenital heart disease is down syndrome associated with?
VSD - ventricular septal defect
AVSD - atrioventricular septal defect - ostium primum ASD (MOST COMMON IN DS)
Endocardial cushion defect: Ostiurn primum defects (15%-20% of cases) are located in the
lowest portion of the atrial septum; they are commonly associated with a cleft mitral valve, ventricular septal defect, and subaortic stenosis, a collection of abnormalities termed endocardial
cushion detect.
What congenital heart disease is associated with Di George (22q deletion)?
great artery malformation
tetralogy of fallot
What congenital heart disease is associated with lithium
Ebstein anomaly (tricuspid valve is in the wrong position and the valve’s leaflets are the incorrect shape) , TV anomaly
What congenital heart disease is associated with rubella?
Rubella - peripheral PA (pulmonary artery) stenosis
PDA
What defect is common in adults presenting with Eisenmenger syndrome?
VSD or PDA
Shunt Volumes
Qp/Qs ratio = 1:1
Qp/Qs >1
Qp/Qs < 1
The ratio of total pulmonary blood flow to total systemic blood flow, the Qp/Qs ratio, is a useful tool for quantifying the net shunt.
- A Qp/Qs ratio of 1:1 is normal and usually indicates that there is no shunting.
- A Qp/Qs ratio of >1:1 indicates that pulmonary flow exceeds systemic flow and defines a net left-to-right shunt.
- A Qp/Qs ratio of < 1:1 indicates a net right-to-left shunt.
ASD (atrial septal defect)
- Clinical Features
- Physical Exam
- Communication between atria, left and right shunt
- Ostium secundum (75%) are most common, ostium primum (15-20%)
Clinical Features
- Fatigue
- Exertional dyspnoea
- Atrial fibrillation
- PARADOXICAL EMBOLISM
Physical Exam
- Jugular venous distension
- Parasternal heave
- Mild systolic ejection murmur over LEFT sternal edge
- FIXED SPLITTING of S2
Can present with pulmonary HTN (rare)
Summary
- FIXED S2 SPLITTING
- Small atrial septic defect (Qp:Qs < 1.5) with no associated symptoms or right heart enlargement can be followed clinically
- SYMPTOMS AND RIGHT SIDED HART CHAMBER ENLARGEMENT = ATRIAL SEPTAL DEFECT CLOSURE
Symptoms: Platypnea-orthodeoxia (P-O) syndrome is an under-diagnosed condition characterized by dyspnea and deoxygenation when changing from a recumbent to an upright position (worse when upright). It is usually caused by increased right-to-left shunting of blood on assuming an upright position, with normal pressure in the right atrium
What examination finding is found in aortic stenosis?
Aetiology
Aetiology
- > 70yo: degenerative
- <70yo: bicuspid, rheumatic
Clinical: - Crescendo decrescendo ejection systolic murmur over right sternal murmur radiating to carotids - May progress to biventricular failure Paradoxically split S2 Single S2
Progression
- Angina
- Syncope
- Failure
TTE
Mean Gradient > 40
Aortic valve area < 1cm
Dobutamine stress echo can be done to determine pseudo AS vs true AS
Ventral septal defect murmur
- Most common at birth but frequency decrease by adulthood because of spontaneous closure
- Most commonly type - perimembranous
Clinical Features
Loud holosytolic murmur located at the left sternal border and often obliterates the S2 and may be palpable
- Small VSD do not cause left enlargement or PAH
- A moderate sized VSD with moderate left to right shunt may cause left ventricular volume overload and PAH
- Worsening PAH results in Eisenmenger syndrome and right to left shunt reversal
- Can present with symptoms of heart failure
Murmur, HF (dilated LV), endocarditis, cyanosis (pulmonary HT)
Closure when Qp:Qs > 2 and evidence of left ventricular volume overload or a history of endocarditis.
Large VSD with right to left shunt reversal and PAH (Eisenmenger syndrome) should not be closed as closure will result in clinical deterioration
During ventricular contraction, or systole, some of the blood from the left ventricle leaks into the right ventricle, passes through the lungs and reenters the left ventricle via the pulmonary veins and left atrium. This has two net effects. First, the circuitous refluxing of blood causes volume overload on the left ventricle. Second, because the left ventricle normally has a much higher systolic pressure (~120 mmHg) than the right ventricle (~20 mmHg), the leakage of blood into the right ventricle therefore elevates right ventricular pressure and volume, causing pulmonary hypertension with its associated symptoms.
In serious cases, the pulmonary arterial pressure can reach levels that equal the systemic pressure. This reverses the left to right shunt, so that blood then flows from the right ventricle into the left ventricle, resulting in cyanosis, as blood is by-passing the lungs for oxygenation.
This effect is more noticeable in patients with larger defects, who may present with breathlessness, poor feeding and failure to thrive in infancy. Patients with smaller defects may be asymptomatic. Four different septal defects exist, with perimembranous most common, outlet, atrioventricular, and muscular less commonly.
Patent ductus arteriosus
- Patent ductus arteriosus (PDA) is the persistence of the arterial duct that connects the aorta and the pulmonary artery in the fetus.
- Maternal rubella and neonatal prematurity predispose to PDA.
- Continuous murmur that envelops the S2 - a continuous “machinery” murmur heard beneath the left clavicle
- Bounding pulses and wide pulse pressure may be noted
- A large PDA causes a large left to right shunt and if unrepaired can cause PAH with eventual shunt reversal from right to left (Eisenmenger syndrome)
- Characteristic feature of Eisenmenger PDA is differential cyanosis/clubbing - cyanosis and clubbing that affects the feet but not the hands owing to desaturating blood reaching the lower part of the body preferentially (differential cyanosis)
Tx
- Closure of PDA is indicated for left sided cardiac chamber enlargement in the absence of severe PAH
- A large PDA with severe PAH and shunt reversal should be observed as closure can be detrimental
- VERY HIGH RISK for endocarditis - intervention in almost all cases
Pulmonary valve stenosis
- Usually isolated congenital cardiac lesion causing obstruction to right ventricular outflow
- Associated with Noonan syndrome
Noonan syndrome, an autosomal dominant disorder, is often associated with isolated pulmonary valve stenosis or other congenital cardiac defects. Additional features of Noonan syndrome include short stature, variable intellectual impairment, unique facial features, neck webbing, and hypertelorism
- Ejection systolic murmur (crescendo-decrescendo), heard at the left sternal border
- R ventricular S1 heard in severe pulmonary valve stenosis
Severe if peak gradient > 60mm Hg - pulmonary balloon valvuoplasty
Treatment of pulmonary valve stenosis
- Pulmonary balloon valvuloplasty
- Complications: increased risk for pulmonary valve regurgitation
What is aortic coarctation associated with?
- 50% of patients with aortic coarctation have bicuspid aortic valve
- Turner syndrome
Others:
- Aortic valve stenosis
- Parachute mitral valve
- VSD
- Cerebral artery aneurysms
Aortic coarctation pathophysiology
Pathophysiology: Aortic coarctation is a discrete aortic narrowing, usually located just beyond the LEFT SUBCLAVIAN artery, causing hypertension proximal and reduced blood pressure distal to the aortic narrowing
Physical examination findings of aortic coarctation
Treatment + complications
- Upper extremity HTN and reduced BP and pulse amplitude in low extremities
- Radial-femoral delay
- Systolic murmur in left infraclavicular region or over the back
- Aortic coarctation + bicuspid aortic valve = ejection click or systolic murmur, S4 often audible
CXR: figure 3 sign - dilatation of the aorta above and below the coarctation. Dilatation of intercostal arteries = rib notching
Treatment: when systolic peak pressure gradient > 20
Angioplasty/stenting
Main side effect after repair is hypertension
Tetralogy of fallot
It consists of 4 main defects:
- Pulmonary stenosis: narrowing of the exit from the right ventricle
- Ventricular septal defect
- Right ventricular hypertrophy: thickening of the right ventricular muscle
- Overriding aorta which allows blood from both ventricles to enter the aorta
Most common cyanotic congenital cardiac lesion
- ~15% with TOF has 2211q.2 chromosome microdeletion (Di George)
- Also seen in Down Syndrome
Complications post tetralogy of fallot repair
- Pulmonary regurgitation
- Can lead to RV enlargement/dysfunction then cause tricuspid regurgitation secondary to pulmonary regurgitation
Examination findings of aortic regurgitation
- Collapsing pulse, wide pulse pressure
- Displaced apex
- S3, LVF
- Long decrescendo high-pitched diastolic murmur - loudest L sternal edge 3- 4th ICS, increases with afterload (handgrip, squat)
- Austin flint murmur
- Left ventricular S2
- Signs of LV failure
Severe AR
- Avoid bradycardia
- Intra-aortic balloon pump contraindicated
Causes of acute severe AR and treatment
Indication for surgery
- Aortic dissection and endocarditis are important causes
- Common clinical and echo clues are missing
Short diastolic murmur
Non-dilated left ventricle - Can be a life-threatening emergency - will require urgent aortic valve replacement (AVR)
- Avoid bradycardia
- Intra-aortic balloon pump contraindicated - enhances diastolic resistance
Indication for Surgery
- Severe symptomatic AR
- Asymptomatic LEVF <50%
- Having CABG and other surgery
Mitral regurgitation
- All about LV dysfunction: displaced apex beat, S3, LVF
- If it’s really bad, then it’s all about RV dysfunction: loud P2, RV heave, peripheral oedema
- Small volume pulse
- Enlarged left ventricle
- Soft S1, early A2, loud S3, diastolic rumble
- High pitched holosystolic murmur radiating to axilla and sternum
- Parasternal impulse
- S4 present in severe acute MR
No S4 - AF or poorly contractile dilate LA
Causes of mitral regurgitation
Primary (degenerative): disease of the valve itself
- Mitral valve prolapse
- Myxomatous degeneration
- Chordal rupture (flail leaflet)
- Endocarditis, rheumatic heart disease, radiation
Secondary (functional): normal valve
- LV dysfunction (ischaemic/non-ischaemic), eg: MI or cardiomyopathy
- Patients with heart failure and LV dilation frequently develop mitral regurgitation
- In secondary MR, normally would not recommend surgery, would normally check if they have coronary artery disease, manage their heart failure or consider CRT but if they continue to have symptoms then surgery would be considered.
What is a complication of rheumatic heart disease?
Mitral stenosis
- Tachycardia/AF poorly tolerated by these patients –> Only time to give beta blockers in pulmonary oedema
- High thromboembolic risk with atrial fibrillation
MS = AF = ANTICOAGULATION
CHADSVasc score not relevant
Previous Exam QUESTION
- Pregnant lady in heart failure, tachycardic with mitral stenosis - answer was to give beta blocker
Severe mitral stenosis - always rheumatic. Calcific (degenerative) MS rarely become severe
Tricuspid regurgitation
- Severe TR is almost always the consequence of some other pathology (eg pulmonary hypertension). Just fixing the TR wont
fix the underlying problem.
• TV surgery only offered usually only in conjunction with LEFT-sided valve surgery because it is functional in nature and isolated TV surgery outcomes are not ideal.
• Isolated TV surgery outcomes not ideal
Beware RV dysfunction, pulmonary hypertension
What is the most common valvular lesion?
Aortic stenosis
What measurements indicate severe aortic stenosis on doppler echo?
Jet velocity: 4m/s
Mean gradient: 40mmHg
Continuity equation valve area: 1cm^2
Mean Gradient > 40
Aortic valve area < 1cm
Treatment for aortic stenosis
Symptomatic severe AS
- Surgical AVR or TAVI
TAVI is associated with higher peri-procedural stroke rates than open aortic valve replacement in the elderly with aortic stenosis
A 17-year-old male presented with episodes of low back pain.
On clinical examination he is tall and has features of Marfan syndrome. You refer him for echocardiography and he asks why it is needed.
Which of the following is the most common abnormality seen in people with Marfan syndrome?
(Please select 1 option)
Dilation of the aortic sinuses Coarctation of the aorta Bicuspid aortic valve Primum atrial septal defect Ventricular septal defect
Dilation of the aortic sinuses
For aortic stenosis, surgery vs TAVI indications + contraindications
Surgical
- Low surgical risk patient
- Mechanical vs bio-prosthetic
- Other valves/CABG
TAVI
- Intermediate to high risk
- Non-inferiority
- Home day 2 opst
TAVI Indications
- Indicated for severe AS where surgical AVR too high risk, provided that life expectancy otherwise >1 year and like to have symptomatic benefit
Contraindications
- Unsuitable anatomy
- Heart team says no
- Endocarditis
- LV thrombus
- Life expectancy <1 year
Severe AS and non cardiac surgery
- Symptomatic severe AS – AVR first unless emergency or high risk AVR (then consider valvuloplasty/TAVI)
- Asymptomatic severe AS and low/moderate risk non-cardiac surgery – no AVR, proceed with surgery
- Asymptomatic severe AS and high risk non-cardiac surgery
(eg open AAA, major thoracic) – AVR first unless patient felt to be high risk for AVR
Adults with cyanotic congenital heart disease
- Clinical features
- Associated conditions
Right-to-left cardiac shunts, such as unrepaired or palliated tetralogy of Fallot, truncus arteriosus, tricuspid atresia, and Eisenmenger syndrome, result in:
- hypoxemia, erythrocytosis and cyanosis.
- Erythrocyte mass is increased in patients with cyanosis as a compensatory response to improve oxygen transport.
- Central cyanosis and digital clubbing
- Can have hyperviscosity syndrome
Associated Conditions
- Scoilosis
- Arthropathy
- Gallstones
- Pulmonary haemorrhage
For patients with right to left shunt - require filters on IV lines to prevent paradoxical air embolism
Mitral stenosis clinical signs
- Loud S1, diastolic rumble
- Duration of murmur correlates with severity
- Possible diastolic thrill at apiex
- All the signs of pulmonary HTN
- ECG: AF is common
- CXR: left atrial enlargement almost universal
Indications for MS valve replacement
- Severe MS with symptoms
- Severe MS with pulmonary HTN
- Asymptomatic patients who go into AF often become rapidly symptomatic
The indication for PBMV (percutaneous balloon mitral valvuloplasty) is progressive exertional dyspnoea (New York Heart Association functional class II, III or IV), associated with documented evidence of moderate or severe mitral stenosis (MS) (mitral orifice area <1.5 cm)
- Options are percutaneous mitral balloon commissurotomy or surgical valve replacement
- PMC tends to be for younger patients (<60 or so), surgical MVR more common in elderly due to concomitant mitral regurgitation.
Note: balloon valvuloplasty is not suitable for MVR
In reality in Australia, most patients with MS are older with a distant history of rheumatic fever and often have concomitant MR or coronary disease so surgical MVR is more common
Mitral valve split score (wilkins)
Grade 1-4 for each of:
- Valve mobility
- Valve thickening
- Valve calcification
- Subvalvular thickening
Minimum score 4
Maximum 16
Score of <9 considered favourable for valvuloplasty but also can’t have >mild mitral regurg
What anticoagulation do you use for MS?
Warfarin!
MS or MVR = warfarin
Mitral valve regurg treatment
- Valves with severe MR can often (~90%) be repaired, where technically possible this is preferable to replacement (better longterm
haemodynamics, MVR are often mechanical so risks of longterm warfarin with higher INR targets). - Usually with an annuloplasty ring (shrinks the annulus so leaflets coapt better).
- Other options are resection of prolapse segments, oversewing of perforation etc.
Mitraclip - percutaneous approach, INFERIOR to surgical MV repair and so only intended for patients who are not surgical candidates
Pulmonary stenosis
- Almost always congenital
- Rarely carcinoid or rheumatic
- Severity/gradients same as for AS
- If severe / right heart failure, then valvuloplasty has high success rate
in children and adults - Surgical repair / replacement if severe with significant PR or valvuloplasty fails.
- Perc option
Pulmonary regurgitation
- Coexistent with PS
- Secondary to pulmonary hypertension
- Dilatation of pulmonary arteries
- Less common: Endocarditis, carcinoid, rheumatic
- Treatment is surgical repair/replacement for severe PR with symptoms/signs of RV volume overload PROVIDED there is no
uncorrected cause for pulmonary hypertension driving the PR. - Melody Valve
When do you close ASD?
Indication for closure is on haemodynamic grounds
- > 10 mm ASD
- shunt ratio >1.5
ASDs load the right heart (look for RV dilatation and dysfunction, and pulmonary hypertension).
What do you do if a patient has AF with high risk of stroke but unable to anticoagulate?
Left atrial appendage closure!
Equivalent to anti-coagulation
Increased risk of arrhythmia
Duke criteria for IE
2 major or
1 major + 3 minor or
5 minor
MAJOR
- Culture of appropriate organism from at least 2 separate cultures
- Single positive for coxiella
- Imaging showing vegetation, abscess, perforation, new dehiscence, abnormal PET/SPECT for valve >3 months since implantation
MINOR
- Predisposition - heart disease/IVDU
- Fever > 38
- Embolic phenomena
- Immunologic phenomena
- Microbiology not meeting major criteria
Valve disease and pregnancy
- SEVERE MS often becomes symptomatic, even if ok prepregnancy: Bed rest, beta blocker; consider valvuloplasty after 20 weeks.
- Severe AS – not common in the pregnant demographic. If truly asymptomatic pre pregnancy then usually ok.
Valvuloplasty if deteriorates. - Chronic AR and MR are usually ok in pregnancy provided LV function is ok.
- Cardiopulmonary bypass during pregnancy has 20-30% feotal loss rate.
What congenital disease is associated with Eisenmenger Syndrome?
PDA>VSD>ASD
Patent ductus ateriosus
Ventral septal defect
Atrial septal defect
Eisenmenger’s syndrome is defined as the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect causes pulmonary hypertension and eventual reversal of the shunt into a cyanotic right-to-left shunt.
In patients with Eisenmenger syndrome, meticulous care of intravenous lines with filters to avoid paradoxical air embolism is imperative
What is the most common complication post TAVI?
LBBB
