Cardiology Flashcards
Inspection: syndromes with associated cardiovascular disease
Down’s syndrome
Ankylosing spondylitis
Marfan’s syndrome
Turner’s syndrome
Noonan’s syndrome
Williams syndrome
Holt-Oram syndrome
Signs of cardiovascular disease in the face
Malar flush
- pulmonary hypertension
- mitral stenosis
Slate-grey rash in photo-exposed areas
- adverse effect of amiodarone
Current or previous central access scars, consider:
Endocarditis
- could be source or evidence of long term antibiotic therapy
How to present vein graft harvesting scars
This patient has ischaemic heart disease and has had surgical revascularisation with coronary artery bypass surgery
Inframammary scar could be
Mitral valvotomy
Gynaecomastia could be due to
Spironolactone
Digoxin
Auscultation: click before pulse
Mitral prosthetic valve
Auscultation: click after pulse
Aortic prosthetic valve
Auscultation: two clicks
Think of double valve replacement
- rheumatic fever
- infective endocarditis
Signs in the head for aortic regurgitation
Muller’s sign
- uvula bobbing up and down
De Musset’s sign
- head bobbing up and down
Palpable second heart sound seen in
Pulmonary hypertension
Thrill in the aortic region, consider:
Aortic stenosis
Right ventricular heave might indicate
Pulmonary hypertension
Auscultation: loud first heart sound in mitral region
Mitral stenosis
Hypertension
Auscultation: quiet first heart sound in mitral region
Mitral regurgitation
Auscultation: left lateral position emphasises
Mitral stenosis
Auscultation: systolic murmur of MR radiates to
Axilla
Auscultation: how to accentuate mitral murmurs
Ask the patient to breath out slowly
Auscultation: where to listen for 3rd and 4th heart sounds
Left sternal edge
Tricuspid area
Auscultation: how to accentuate tricuspid murmurs
Ask the patient to take a long slow breath in
Auscultation: loud second heart sound
Pulmonary hypertension
Auscultation: fixed wide-splitting of second heart sound
ASD
Auscultation: quiet second heart sound
Severe AS
Auscultation: how to elicit AR murmur
Sit forward
Take a deep breath in and out
Hold in expiration
Urine dip in endocarditis
Haematuria
Proteinuria
Glucosuria
Pulse: aortic stenosis
Slow rising
Pulse: aortic regurgitation
Collapsing
Pulse: mitral stenosis
AF
Presentation of cardiovascular examination
Which valves/what lesion
Severity/if valve replaced - functionality
Evidence of heart failure
Evidence of endocarditis
- there were no stigmata of bacterial endocarditis
Aetiology
Prognosis in AS: PC Chest pain
5 years
Prognosis in AS: PC Breathlessness
2 years
Prognosis in AS: PC Syncope
18 months
Aortic stenosis: signs of severe disease
Quiet 2nd heart sound
Signs of left or right HF
Slow rising pulse
Aortic stenosis: aetiology
Calcific degenerative
- most common
Congenital
- bicuspid valve
Rheumatic
- now very rare
Aortic stenosis: differential diagnosis
Aortic sclerosis
Pulmonary stenosis
- normal pulse
- normal aortic 2nd heart sound
- murmur louder on inspiration
Ventriculoseptal defect
- very loud murmur
- maximal at sternal edge
- thrill
HCOM
- jerky pulse
- murmur quieter if patient crouches down
- young person
- normal 2nd heart sound
Subaortic membrane
Supra-valvular obstruction
Aortic Stenosis: investigation
ECHO
- valve area and mean valve gradient for severity
Stress ECHO
- determine if impaired LV due to AS
- if so may benefit from surgery
Cardiac catheterisation
- coronary angiography necessary prior to surgery
- severely stenosed valve may lead to stroke and clinical neurological deficit
Aortic Stenosis: ECHO criteria for severe
Valve area <1cm2
Mean valve gradient >40mmHg
Aortic Stenosis: Management - asymptomatic
6-12 monthly FU
Surgery could be recommended if severe with mean gradient >40mmHg and:
- LVEF <45%
- abnormal response exercise (BP drop)
- ventricular tachycardia
- LVH >15mm
- valve area <0.6cm2
Aortic Stenosis: Management - symptomatic
Surgical
- aortic valve replacement +/- CABG
- 3-5% mortality
Percutaneous
- balloon aortic valvuloplasty
- transcutaneous aortic valve implantation (TAVI)
Aortic Stenosis: common complications
Endocarditis
Heart failure
AV block
Embolic events
Aortic Stenosis: differentiation from sclerosis
Sclerosis has
- normal pulse
- normal 2nd heart sound
- seen in elderly
Aortic Stenosis: associated conditions
Coarctation of the aorta
- check radio-femoral delay
Other valvular disease
Angiodysplasia of the colon and anaemia
- Heyde’s disease
Aortic Stenosis: indications for surgery
Symptomatic AS
- particularly urgent if syncope
Asymptomatic AS if mean gradient >40mmHg with:
- LVEF <45%
- abnormal response exercise (BP drop)
- ventricular tachycardia
- LVH >15mm
- valve area <0.6cm2
Aortic regurgitation: symptoms
Not uncommon to be asymptomatic
Breathlessness
Chest pain not uncommon
Aortic regurgitation: length of murmur with increasing severity …
Shortens as the gradient between aorta and LV diminishes
Aortic Regurgitation: common signs
Early diastolic murmur
- usually maximal at LLSE but also aortic area
Cardiac dilatation
Collapsing pulse
Wide pulse pressure
Aortic Regurgitation: uncommon signs
De Musset’s - head bobbing
Muller’s - uvula bobbing
Durozier’s - murmur over femoral arteries
Quinke’s- visible pulsating of capillaries in nail bed
Aortic Regurgitation: aetiology - acute
IE
Aortic dissection
Prosthetic valve failure
Ruptured sinus of valsalva
Acute rheumatic fever
Aortic Regurgitation: aetiology- chronic
Bicuspid aortic valve
Marfan’s syndrome
Aorto-annular ectasia
Rheumatic heart disease
Endocarditis
Seronegative arthritides
Syphilis
Osteogenesis imperfecta
Aortic Regurgitation: differential diagnosis
Pulmonary regurgitation
Aortic Regurgitation: associated conditions
Coarctation of the aorta
Marfan’s syndrome
Seronegative arthritis
Ankylosing spondylitis
- listen to lung apices
Syphilis
Aortic Regurgitation: investigation
ECHO
MRI
Coronary angiography necessary prior to surgery
Aortic Regurgitation: criteria for severe AR
Width of AR jet >65% of LVOT
RF >50%
Left ventricular end diastolic diameter >70mm
Left ventricular end systolic diameter >50mm
Pressure half time of Doppler slope of AR <200ms
Aortic Regurgitation: management
Acute and more than mild severity
- surgery
Chronic with symptoms or asymptomatic with specific features
- surgery
Chronic without symptoms
- medical management
Aortic Regurgitation: features to indicate surgery in chronic asymptomatic AR
LV dilatation
EF <50%
Significant aortic root dilatation
- >45mm in Marfan’s
- >50mm with bicuspid valve
- >55mm otherwise
Undergoing any other cardiac surgery
Aortic Regurgitation: medical management
ACEi
Diuretics
Aortic Regurgitation: complications
Endocarditis
Heart Failure
Aortic Regurgitation: signs of severe AR
Clinically dilated heart
Signs of left sided heart failure
Very wide pulse pressure
Short murmur
Mitral Stenosis: clinical features
Heart failure
AF
Hoarse voice
- compression of recurrent laryngeal nerve by enlarged LA
Malar flush
Haemoptysis
Fever
- IE in mixed MV disease
Mitral Stenosis: signs
Mid-diastolic murmur heard best in mitral area
- expiration
- with bell
Palpable P2
Loud S1 - closing snap
Loud S2
Decrescendo early diastolic murmur heard loudest in the pulmonary area from pulmonary regurgitation
Opening snap - shortly after P2
HF
Mitral Stenosis: severity
AF
Signs of pulmonary hypertension
Short gap between S2 and opening snap
Long mid diastolic murmur
Signs of right heart failure with pulmonary congestion
Mitral Stenosis: aetiology
Rheumatic fever - >90% cases
Degenerative
- severe mitral annular calcification
Non-valvular
- LA myxoma/ball-valve thrombus
- large vegetations in IE
Congenital
Drugs
- cabergoline
Mitral Stenosis: investigations
ECG
- p-mitrale
- AF
CXR
- pulmonary congestion
ECHO
- mitral valve area and gradient
- pulmonary pressure
- suitability for balloon mitral valvuloplasty
- RV function
TOE
- assess for thrombus prior to BMVP
Coronary angiography prior to surgery
Right and left heart catheterisation prior to surgery
- MVA
- PASP
- PAWP
Mitral Stenosis: indications for anticoagulation
AF
LA >55mm
Warfarin
Mitral Stenosis: indications for percutaneous balloon mitral valvuloplasty/surgery in asymptomatic patients
Moderate or severe MS
WITH
Suitable valve
AND
Pulmonary HTN
OR
new onset AF
Mitral Stenosis: indications for percutaneous balloon mitral valvuloplasty/surgery in symptomatic patients
NYHA II dyspnoea
AND
PAP >60mmHg OR mean gradient >15mmHg OR wedge pressure >25mmHg
AND
Suitable valve
Mitral Stenosis: follow up
Asymptomatic and mild
- annual
Asymptomatic, moderate to severe, and not suitable for surgery/PBMV
- annual
Symptomatic and not suitable for surgery/PBMV
- 6 monthly
Mitral Stenosis: management of moderate/severe MS with NYHA III/IV dyspnoea
PBMV
NOT surgery
Mitral Stenosis: assessment of valve suitability for PBMV
Wilkin’s Score 8 or below
CI:
- moderate or severe MR present
- LA thrombus present despite anticoagulation
Mitral Stenosis: pregnancy
Symptomatic patients with severe MS should be advised against pregnancy
Present during pregnancy as a result of increased HR and intravascular volume
Pregnancy + severe MS + severe dyspnoea
- PBMV with TOE guidance
Normal mitral valve area
4-5cm2 depending on body size
Mitral Regurgitation: clinical features
Heart failure
AF
Angina
- ischaemic MR
Symptoms of endocarditis
Mitral Regurgitation: signs
Sternotomy
- could be paravalvular MR in malfunctioning prosthetic valve
Vein harvesting scars
- ischaemic MR
Stigmata of endocarditis
Bruising from warfarin
AF
Raised JVP
Visible apex beat
Thrusting apex beat
Left parasternal heave
- RV pressure overload
Palpable P2
- pulmonary HTN
Mitral Regurgitation: auscultation
High-pitched pansystolic murmur loudest at apex
- radiates to axilla
- increased intensity with expiration
Widely split S2
S3
S4
Heart failure
S3 pathophysiology
Rapid early filling of the LV from engorged LA
S4 pathophysiology
Forceful atrial contraction against less compliant dilated LV
Mitral Prolapse: auscultation
Normal S1 and S2
Mid-systolic clicks
Soft high pitched mid-to-late systolic murmur
May develop S3 and S4 and loud P2 if severe
Mitral Regurgitation: signs of severe MR
AF
Displaced, thrusting apex beat
Pulmonary hypertension
Heart failure
Mitral Regurgitation: aetiology
Degenerative
Functional
- causes of LV dilatation
Ischaemic
Prolapse
- hereditary
- idiopathic
- Marfan’s syndrome
- connective tissue disorders
Rheumatic
Causes of mitral valve prolapse
Hereditary
- AD MMVP1 (16) and MMVP2 (11)
Idiopathic
Marfan’s syndrome
Connective tissue disorders
Mitral Regurgitation: investigations
ECG
- AF or P-mitrale
CXR
- pulmonary congestion and cardiac size
ECHO
TOE
Preoperative assessment for surgery:
- right and left heart catheterisation for pulmonary pressures and coronary anatomy
- carotid duplex (establish if there is disease requiring treatment preop)
- orthopentomogram (dental risk)
Mitral Regurgitation: indications for surgery in asymptomatic patients
EF 30-60%
Left ventricular end diastolic diameter >40mm
Mitral Regurgitation: medical management
No medical treatments are known to alter progression
Mitral Regurgitation: mild to moderate asymptomatic
Annual clinical and ECHO FU
Mitral Regurgitation: indications for surgery in symptomatic MR
Severe MR and reserved LV with:
AF
OR
Signs of pulmonary hypertension
Impaired LV:
Only recommended if medical therapy is optimal
AND
absence of serious comorbidity
AND
chordal preservation likely
Mitral Regurgitation: chronic severe asymptomatic MR FU
If EF >60% and end systolic LV dimensions >40mm
- 6 monthly with annual ECHO
Why is MV repair preferable to replacement?
Disconnection of subvalvular apparatus results in up to 20% decline in LV function
What is ischaemic MR?
Ischaemic injury to or dysfunction of the subvalvular apparatus
Ischaemic Mitral Regurgitation: management
Worse prognosis than organic MR
Lower threshold for surgery
Consider if undergoing CABG
LVEF >30% but symptomatic despite medical management
LVEF <30% and not surgical candidates
- biventricular pacing +/- ICD +/- transplantation
Are prophylactic antibiotics necessary for dental surgery in MR/MVP?
Not in uncomplicated disease
New developments in MR management
Surgical ablation of AF at time of MV surgery
Percutaneous transcatheter MV clip repair
- prolapse not fit for surgery
Assessment with 3D-TTE and TOE
Tricuspid Regurgitation: clinical features
Frequently asymptomatic
May complain of ankle swelling or breathlessness
Pulsing in the neck
Tricuspid Regurgitation: signs
Pulsation of the neck
Parasternal heave
- severe
Pansystolic murmur at LLSE
- louder on inspiration
Pulsatile hepatomegaly
Tricuspid Regurgitation: aetiology - chronic
Pulmonary hypertension
Endocarditis
Ebstein’s anomaly
Rheumatic valvular heart disease
Carcinoid syndrome
What is Ebstein’s anomaly?
Apical displacement of the tricuspid valve
- particularly the septal leaflet
Deformed TV
- at least moderately regurgitant
Tricuspid Regurgitation: aetiology - acute
IE
Trauma
Tricuspid Regurgitation: management
Treat underlying condition causing pulmonary hypertension
Diuretics
Valve replacement and repair if peripheral oedema not controlled
- last resort due to poor prognosis
Why is tricuspid endocarditis more common in IV drug use?
Particulate matter present in the materials used to ‘cut’ illegal drugs damage the TV
Then become trapped in the lungs
Damage predisposes TV to being seeded from frequent bacteraemias
Tricuspid Stenosis: clinical features
Right sided heart failure
Carcinoid syndrome
- flushing
- diarrhoea
Tricuspid Stenosis: signs
Raised JVP
Peripheral oedema
Mid-diastolic murmur
- very rarely audible
Examine liver
Tricuspid Stenosis: aetiology
Carcinoid syndrome
Rheumatic fever
Congenital
Tricuspid Stenosis: management
Diuretics
Valve replacement can be performed if uncontrolled oedema
Pulmonary Stenosis: clinical features
Often asymptomatic
Asthenia
Syncope
Right sided heart failure
Pulmonary Stenosis: signs
Ejection systolic murmur
- best heard in pulmonary area
- louder during inspiration
Soft P2
Pulmonary thrill
Palpable ejection click
Prominent a wave
RV heave
RV gallop rhythm
Pulmonary Stenosis: aetiology
Congenital in almost all cases
- maternal rubella
Rheumatic fever
Carcinoid syndrome
Pulmonary Stenosis: differential diagnosis
Aortic stenosis
Pulmonary Stenosis: associated syndrome
Tetralogy of Fallot
Pulmonary Stenosis: investigation
ECHO
Cardiac MRI
- if supravalvular abnormalities suspected
Pulmonary Stenosis: management
Mild
- no treatment
Balloon valvuloplasty in infants
Severe outflow obstruction:
- surgical reconstruction of the RV outflow
- shunts from vena cavae/RA to pulmonary artery
- percutaneous valve replacement
Astrology of mixed and multivalvular heart disease
Endocarditis
Rheumatic
Degenerative
Congenital
Aetiology of mixed mitral valve disease
Rheumatic
Causes of left sided heart failure
Ischaemic heart disease
Hypertension
Valvular heart disease
Cardiomyopathy
Myocarditis
Causes of right sided heart failure
Intrinsic lung disease
- pulmonary hypertension and cor pulmonale
Right sided cardiomyopathy
- arrhythmogenic RV dysplasia
- rare
Investigations for heart failure
ECG
CXR
ECHO
Coronary angiography - establish ischaemic heart disease
Exercise testing - prognosis
Myocardial visibility testing
- thallium scanning
- cardiac MRI
- stress ECHO
- PET
Management of heart failure
Reverse of underlying pathology if possible
Diuretics
- for congestive symptoms
- no effect on prognosis
ACEi/ARBs
- for symptoms and prognosis
B-blockers
- improve prognosis and reduce hospital admissions
Spironolactone
- improves prognosis
- eplerenone post-MI has fewer SEs
Digoxin
- improves NYHA class but no effect on prognosis
Exercise training - graded, aerobic exercise
Cardiac transplantation
- median survival 8 years
Palliation
New developments in management of heart failure
Artificial ventricular support devices
- Jarvick implantable artificial heart
EPO for anaemia
Peritoneal dialysis for congestive symptoms
Neurohumeral changes that occur in chronic CCF
Reduced CO -> reduced GFR
Reduced Na in DCT
Juxtoglomerular apparatus releases renin -> angiotensinogen -> angiotensin I -> II
Angiotensin II -> vasoconstriction and salt/water retention -> increases after load
Worsens congestive symptoms
ACEi reverses this
Reduced CO -> activated sympathetic system -> chronic high levels of adrenaline -> vasoconstriction -> increased after load -> increased myocardial demand + increased salt/water retention
B-blockers reverse this
Indications for bicentricular pacing
Class III or IV despite 1 month of optimal medical management
WITH
QRS >120ms
Improves symptoms and prognosis
Click before carotid pulse/1st heart sound
Mitral valve replacement
Click after carotid pulse/second heart sound
Aortic valve replacement
Regurgitant murmur with prosthetic heart valve
Always abnormal
Paravalvular or valvular leak
- consider endocarditis
Biological valves: auscultation
No click
Softer heart sounds
- lower timbre
May have stenosic murmur due to degeneration over time
Investigations for prosthetic valves
ECHO
- ensure valve is well seated
- detect vegetations
TOE
- if IE suspected
- further investigation of leaks
Advantages of biological valves
Lack of need for long term anticoagulation
- valve of choice for those with CI to anticoagulation
Patient preference
Who gets a biological valve?
> 70
60 with significant comorbidity
How long do prosthetic valves last?
10-15 years
Which prosthetic valve degenerates faster?
Aortic valve
- greater pressure swings
What are the advantages of a mechanical valve replacement?
More durable than prosthetic valves
Who gets a mechanical valve replacement?
<60 without CI to anticoagulation
>60 with comorbidity that would encourage anticoagulation
- AF
Which valve replacement is more like to form a thrombus?
Mitral valve
Anticoagulation targets for prosthetic valves
Aortic - 2.5
Mitral - 3.0
How to manage anticoagulation in patients with mechanical valves who require surgery
Stop anticoagulation until INR <1.5
If high risk - switch to heparin
Valve replacement and not fit for surgery
Percutaneous or transapical clips or surgery
Pulsus paradoxus definition
> 20mmHg fall in systolic pressure with inspiration
- increased filling of RV
- septum bulges into LV
- reduced size and filling of LV
Pulsus paradoxus is a sign of
Constrictive pericarditis
Constrictive pericarditis: auscultation
Pansystolic murmur
- associated with TR
Loud S3
- best heard at LLSE
Aetiology of constrictive pericarditis
Post-irradiation
Post-infectious (TB)
Post-surgical
Idiopathic
Uraemic
Autoimmune
Drug-induced
Neoplastic
Post-traumatic
Constrictive pericarditis: investigations
ECG
- AF, sinus tachycardia, p-mitrale, p-pulmonale
CXR
- pericardial calcification
- congestion, fibrosis from radiation
ECHO
- thickened pericardium, TR, septal bounce
CT/MRI
- pericardial calcification
Right and left heart catheterisation
- anatomy prior to surgical pericardectomy
Constrictive pericarditis: management
Treat underlying cause
Steroids reduce risk of CP after TB pericarditis
Diuretics
- fluid overload
Avoid BB or CCB to preserve cardiac output
- rate control AF with digoxin
Surgical pericardectomy
Duke criteria: major
Positive typical microorganisms in 2 separate blood cultures
OR
Persistently positive
Evidence of endocardial involvement
- vegetation
- intracardiac abscess
- new dehiscence of prosthetic valve
- new valvular regurgitation
Duke criteria: minor
Evidence of active infection with IE organism
OR
Positive blood cultures not reaching major criteria
Vascular Embolic phenomena
Immunologic phenomena
Fever
Predisposing heart condition or IV drug use
ECHO findings that don’t meet major criterion
Infective endocarditis common organisms
Strep viridans
Strep bovis
HACEK organism
Staphylococcus aureus
Enterococcus
HACEK organisms
Haemophilus
Actinobacillus
Cardiobacterium hominis
Eikenella corrodens
Kingella
European Society of Cardiology classification domains for IE
Disease activity
Certainty of diagnosis
Type of valvular involvement
Side of heart involved
Culture status
Population type
Infective endocarditis investigations
Blood cultures:
- 2 sets 24 hours apart or 4 sets in 1 hour
- prior to antibiotics
ECHO
TOE
Urine dip
Monitor U&Es and LFTs
ECG
- every 2 days
- lengthening PR interval indicates aortic root abscess formation
Management of infective endocarditis
IV antibiotics
- discuss with microbiology
- broad spec -> amoxicillin/flucloxacillin/gentamicin
Low threshold for surgery
Diagnosis of infective endocarditis
Duke criteria
- 2 major
- 1 major and 2 minor
- 5 minor
Complications of endocarditis
Direct tissue destruction
- acute and subacute valve failure
- extravalvular extension of infection -> aortic root abscess, septic pericarditis
Septic emboli
Renal failure
- sepsis
- immune complex glomerulonephritis
Indications for surgical intervention in infective endocarditis
Uncontrolled infection
Haemodynamic instability
Increasing heart block due to aortic root abscess
Renal failure
Indications for antibiotic prophylaxis for IE
Should not be routinely offered
Ventricular septal defect: signs
Pansystolic murmur
- best hear at LLSE
Associated thrill
Signs of RV overload
- parasternal heave
Elevation of JVP
RARE - EISENMENGER’S SYNDROME
- pulmonary hypertension develops
Ventricular Septal Defect: aetiology
Congenital
Acquired
- cardiac rupture post-MI
Ventricular Septal Defects: investigations
ECHO
Ventricular Septal Defect: complications
Large VSDs
- heart failure
- endocarditis
Ventricular Septal Defects: management
Reassurance
Large defects - surgery or catheter-based closure
Atrial Septal Defect: associations
Down’s syndrome
Holt-Oram syndrome
Paroxysmal stroke
Atrial Septal Defect: auscultation
Soft ejection systolic murmur
- loudest over the pulmonary area
Pansystolic murmur at LLSE if TR present
- secondary to RV overload
Fixed and widely split S2
- P2 delayed due to increased pulmonary flow
Loud P2 if pulmonary hypertension
Signs of RHF
Atrial Septal Defect: severity
Large ASD
- right ventricle volume overload
- pulmonary hypertension
- diastolic flow murmur
- AF
Atrial Septal Defect: types
Primum ASD
Secundum ASD
Sinus venosus ASD
Coronary sinus ASD
Atrial Septal Defect: investigations
ECG
- AF, 1st degree AV block, partial RBBB
CXR
ECHO
Right and left heart catheterisation
Cardiac MRI
- assessment of shunt size and impact on RV function
Lung biopsy
Atrial Septal Defect: management
Reassurance
Closure
- surgical or percutaneous
Atrial Septal Defect: indication for closure
Paradoxical embolism
Symptomatic
Asymptomatic with significant shunt
Significant pulmonary hypertension
Patent Ductus Arteriosis: signs
Mostly asymptomatic
Differential cyanosis and clubbing
- toes blue and clubbed, fingers pink and normal
Collapsing pulse
AF
Signs of right sided overload and pulmonary hypertension
- parasternal heave
- palpable P2
- palpable thrill
Patent Ductus Arteriosus: auscultation
Loud systolic crescendo murmur
- more lateral than pulmonary area
Loud S2
Machinery murmur
Patent Ductus Arteriosus: severity
Mild
- continuous murmur with normal pulse
- no signs of LV overload or pulmonary HTN
Moderate
- continuous murmur
- wide pulse pressure
- collapsing pulse
- volume overloaded LV
- pulmonary HTN
Severe
- eisenmenger’s syndrome
- no murmur
- differential cyanosis and clubbing
- usually present in childhood
Patent Ductus Arteriosus: aetiology
Congenital
Neonatal Rubella Syndrome
Prematurity
Birth at high altitude
Prostaglandin E1 infusion
- transposition of the great vessels
Patent Ductus Arteriosus: investigations
ECG
CXR
EXHO
- shunt fraction estimated (pulmonary to systemic flow <1.5 mild, 1.5-2.2 moderate, >2.2 severe)
Patent Ductus Arteriosus: management
Small ducts - conservative management
Infected ducts - closed once infection resolved
All other ducts should be closed unless reversal or significant irreversible pulmonary hypertension
Patent Ductus Arteriosus: closure techniques
Percutaneously deployed duct-closure device
If not feasible - surgery
Further examination of dextrocardia
Consider situs invertus
- percuss for liver edge
Consider Kartagener’s syndrome
- listen to lungs for bronchiectasis
Dextrocardia investigations
CXR
ECG
ECHO
MRI
Dextrocardia management
In isolation - nil
If associated disorders require treatment
- congenital heart disease specialists
Clinical features of Kartagener’s syndrome
Dextrocardia
Situs invertus
Bronchiectasis
Infertility in males
Pathogenesis of Kartagener’s syndrome
Autosomal recessive
Dysfunction of dynein arm of cilia
- dysmotility
Left lateral thoracotomy scar could be sign of repair of
Coarctation of the aorta
Young patients with unexplained HTN, check for
Radio-femoral delay
Diseases and complications associated with coarctation of the aorta
Bicuspid aortic valve
VSD
MV prolapse
Patent ductus arteriosus
Aortic dissection
Turner’s syndrome
Neurofibromatosis type I
Marfan’s syndrome
Subarachnoid haemorrhage
Shone’s syndrome
ECG findings for coarctation of the aorta
LVH with strain pattern
CXR findings in coarctation of the aorta
Rib notching
Aneurysmal post-stenotic aortic dilatation
Visualisation of the coarctation
- 3 shaped descending thoracic aorta
Investigations for coarctation of the aorta
ECG
CXR
ECHO
Cardiac MRI
Cardiac catheterisation if surgical repair being considered
Management of coarctation of the aorta
Primary percutaneous endovascular stenting
Surgical repair being
- resection of coarctation and end-to-end anastomosis
- longer segments treated with graft
Medical therapy
- treat HTN
Indications for surgery in coarctation of the aorta
Symptomatic patients with gradient >30mmHg across coarctation
Asymptomatic with HTN or signs of LVH
Patients requiring other surgery for cardiac indications
Exercise in coarctation of the aorta
Avoid extreme isometric exercise
- weight lifting etc
Increased risk of dissection
System for diagnosis of Marfan’s Syndrome
The Ghent System
Features of the Ghent System
Cardiovascular
Ocular
Skeletal
Respiratory
Skin and integumentary
Major criteria for diagnosis of Marfan’s syndrome using the Ghent System
Dilation of the ascending aorta
Aortic dissection
Ectopia lentis
Pectus carinatum
Pectus excavatum
Arm span/height ratio >1.05
Hyper mobility of wrist or thumb joints
Scoliosis or spondylolisthesis
Reduced elbow extension
Pes planus
Protrusio acetabulae
Lumbrosacral dural ectasia on CT or MRI
Acute presentation of Marfan’s syndrome
Aortic dissection
- mortality is 50%
Marfan’s syndrome follow up
Major objective is to avoid acute aortic dissection
Yearly ECHO to monitor aortic root
- increased if family Hx or dilatation
Mode of inheritance Marfan’s syndrome
Autosomal dominant
Variable clinical expression
Pathophysiology of Marfan’s syndrome
Autosomal dominant
Defect in fibrillin-1 gene
Chromosome 15
Marfan’s syndrome: indications for aortic root replacement
Symptomatic AR
Asymptomatic AR meeting criteria for surgery
Dilatation of the aortic root
- max diameter >45mn
Aortic growth rate of 1cm/year
What medication reduce rate of aortic dilatation?
Beta blockers
PACES patients with Fallot’s tetralogy
Shunt
OR
Signs of complications of the repair procedure
Composition of tetralogy of Fallot
Large VSD
Overriding aorta
RVOT obstruction
RVH
Blalock-Taussig shunt
Direct shunt between the subclavian and ipsilateral pulmonary arteries
- bypasses the obstructed RVOT, increasing pulmonary blood flow
Genetic counselling in tetralogy of Fallot
15% will have deletion of chromosome 22q11
Detected with FISH
Complications of complete repair in tetralogy of Fallot
Pulmonary regurgitation
Aortic regurgitation and aortic root dilatation
Rhythm disturbances
- may be amenable to ablation
- may require ICD
- manage cause of RVH
- anticoagulate AF/flutter with warfarin
Residual VSD
Treatment for neonates with tetralogy of Fallot who are not fit for surgery
Stenting of the RVOT until recovered
Signs of HCM
Infraclavicular scar with device
- dual pacemaker/ICD
Jerky pulse
Heaving apex
ESM
- LLSE
- shorter and quieter when squatting
Pansystolic murmur if MR
HF
S4
Aetiology of HCM
Hereditary
- autosomal dominant
- 11 different sarcomeric genes
- phenotype and penetrance varies
Investigations for HCM
ECG
- p-mitrale, LVH, lateral T wave inversion, deep septal Q waves, AF
Holter
- ventricular tachycardia
ECHO
Exercise tolerance test
Cardiac MRI
- useful to identify mimics (amyloid)
HCM: risk factors for sudden cardiac death
Previous arrhythmic cardiac arrest
Sustained VT
Non-sustained VT on Holter
Family history of SCD in a 1st degree relative
Unexplained syncope
Abnormal BP response to exercise
Massive LVH
LV apical aneurysm
Dilated end-stage heart failure with LVD
Management of HCM with risk of SCD
Consider ICD
Medical management of HCM
Beta-blockers
Verapamil
Disopyramide (should be combined with BB)
Diuretics for pulmonary congestion
AVOID digoxin
Warfarin for AF
Treatment for LVOT obstruction in HCM
NYHA III/IV and refractory to medical management
- percutaneous alcohol septal ablation
If not a candidate - surgical myectomy
Dual chamber pacing
- RCTs show not more affective than placebo
- can only be offered if other indication for pacing
Aortic Stenosis: indications for TAVI
High surgical risk
- log EuroSCORE >20%
Inoperable cases
Aortic Stenosis: characteristics favouring TAVI
Age >75
Previous cardiac surgery
Frailty
Chest radiation
Porcelain aorta
