Download Emergencies in adults with congenital heart disease: a guide for the

Adults with congenital heart
disease: a survival guide
R. Dobson
Clinical Research Fellow
Congenital Heart Disease
• CHD is by far the commonest congenital abnormality
and >85% of these patients survive to adult life
• From 2010 approximately 1 in 150 young adults will
have CHD; 1 in 300 will have lesions of moderate or
severe complexity
• Some conditions may not be diagnosed until
adulthood (e.g. ASD, aortic coarctation)
• Your local cardiologist might not know much more
than you do!
General considerations
ABCDE & stabilise patient
Seek information
Get help & plan definitive
management
ABCDE
• As per ALS but…
• Beware atrial flutter – may look like sinus
rhythm
• BP measurements may be unreliable if
previous surgical shunts – look for lateral scars
• Cyanosed patients can often tell you what
their usual SpO2 is
Seek information
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Good history
Previous clinic letters – check Portal
Old ECGs
Google image search or wikipedia for
unfamiliar terms
• For each patient try and describe how the
blood goes round
Get help
• Think what help is available locally
• Phone GJNH switchboard 0141 951 5000
– Mon-Fri 9-5pm Scottish Adult Congenital Cardiac
Service
– OOH ask for CCU at GJNH
• Will be able to access clinic letters, old ECGs and echoes
instantly and send them through
• Can speak to SpR on call for further advice
Emergencies in ACHD
1.
2.
3.
4.
5.
Supraventricular tachyarrhythmia
Blackout / collapse
Acute aortic syndromes
Endocarditis
Massive haemoptysis
1. Supraventricular tachyarrhythmia
Case history
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35yo male
“Fontan repair” for “tricuspid atresia”
Sudden onset palpitations 6h ago
Light headed on exertion
Feels OK at rest
Undistressed, looks well
On warfarin for years
ABCDE
• Talking
• RR 20 SpO2 97% lungs clear
• BP 100/60 L arm, HR 100bpm regular, warm
peripheries. No murmurs
• Apyrexial, BM 5.6, E4 V5 M6
• Abdomen soft non-tender
• Bloods OK – INR therapeutic
ECG
Previous ECG
Seek information – what is a Fontan?
What do you do now?
1.
2.
3.
4.
Do nothing?
Urgent DC cardioversion?
Start flecainide?
Start amiodarone?
What do you do now?
1.
2.
3.
4.
Do nothing?
Urgent DC cardioversion?
Start flecainide?
Start amiodarone?
What happened
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Admitted to CCU
Fasted
Started amiodarone
Plan for DCCV if amiodarone unsuccessful
Then…
• 5 hours into his amiodarone the patient became
very unwell. Repeat ECG showed ventricular rate
of over 200bpm
• Shut down, drowsy with BP 60/24
• Cardiac arrest call – single DC shock into asystole
• Prolonged CPR. Intubated and ventilated. After
30 minutes worsening acidosis with no clear
reversible cause and no sign of recovery –
resuscitation ceased
What went wrong
• Fontan is a passive circulation and utterly dependent on
central venous pressure
1. Patient fasted – dehydration, reduced systemic venous return
– Cardiac output falls...
2. Amiodarone given – negatively inotropic
– Cardiac output falls...
3. Slows to 1:1 conduction of flutter
– Cardiac output falls...
4. Positive pressure ventilation increases intrathoracic pressure.
CVP is already low no longer sufficient to drive blood to lungs
– CPR unsuccessful
How to do it
• Get advice
• Keep patient fasted but give IV fluids
(crystalloid) to maintain CVP
• Contact anaesthetist, explained potential
seriousness of situation
• Cautious GA to avoid excessive vasodilation,
minimum of PEEP
• DCCV performed successfully with single
shock
Key points
• Understand the circulation
• Patients can look deceptively well initially
• SVT poorly tolerated in single ventricle hearts
(ditto for severe ventricular impairment and
obstructive valve lesions)
• Avoid ‘anti’ arrhythmics – unpredictable effect
• Electricity is clean and quick
2. Blackout / collapse
Tetralogy of Fallot
• Complete repair (most
adult patients) involves
closure of VSD and
enlargement of RVOT
(infundibular resection or
transannular patch).
• Severe / free pulmonary
regurgitation and
progressive RV dilatation
is almost universal
• Can be tolerated well for
years
Case History
• 27 year-old female with repaired tetralogy of
Fallot
• Fit and well, no medications
• Told has a leaky heart valve which will need to
be replaced soon
• GP referral with 2 episodes T-LOC past 24
hours at rest. Sudden onset, no warning. No
witnesses.
ABCDE
• Talking, undistressed
• RR 20 SpO2 97% lungs clear
• BP 50/30 R arm. HR 80bpm regular, warm
peripheries. S1 + ESM + S2 + EDM
• Apyrexial, BM 5.6, E4 V5 M6
• Abdomen soft non-tender
Hypotension in CHD
•
Why is the BP so low?
•
How can you tell?
Blalock-Thomas-Taussig shunt
• Subclavian to ipsilateral
branch pulmonary
artery
• Increases pulmonary
blood flow
• Look for a lateral
thoracotomy scar!
12 lead ECG
Seek information
• Detailed history
• Previous ECGs and clinic letters
• She had a right BT shunt prior to corrective
surgery
• Echo / MRI – severe pulmonary regurgitation
with a severely dilated right ventricle and
referred for PVR
• Holter 2/12 ago showed NSVT
Get help
• Local cardiologist
• ACHD service
Management
• Monitored bed
• Basic bloods, echo
• Treat the haemodynamic lesion – may need
valve replacement
• May need EP studies (VStim) to risk stratify
• If symptoms infrequent could consider ILR
(Reveal)
Issues
• 20 year survival in ToF reported at 80-99%
• Sudden death occurs in 2.5% per decade of F/U
• A number of risk factors identified (QRS >180ms,
age at time of surgery, high grade ectopy on
Holter, impaired LV)
• No clear consensus on risk stratification and
device therapy
• No role for prophylactic antiarrhythmics
3. Acute aortic syndromes
Case history
• 30 year-old male
• BP checked a couple of times in the past year
– told it was “high” but no further action
• Sudden onset severe interscapular back pain
• Looks distressed and unwell
ABCDE
• Talking in sentences
• RR 24 SpO2 98% 2LPM nasal cannula, lungs
clear
• BP 200/110 R arm, HR 110bpm reg cool
peripheries. S1 + ESM + S2 + EDM
• Apyrexial E4 V5 M6 equal tone and reflexes all
4 limbs
• Abdomen soft and non tender
• What else do you want to check on
examination?
• What is the most likely diagnosis?
Initial management
• Wide bore IV access
• CT angiogram aorta
• Echo if available – check for aortic valve
involvement +/- effusion but should not delay
transfer
Get help
• Phone a cardiac surgeon!
• If type A dissection urgent cardiac surgery is
the only treatment (mortality is 1-2% per
hour)
Coarctation of the aorta
• Severe hypertension
refractory to treatment
• Premature MI, stroke,
dissection
• Associated with
bicuspid aortic valve
and ascending aortic
aneurysm
• Stenting or surgery
• Always check femorals
4. Endocarditis
Case history
• 30 year-old male
• Underwent heart surgery 4 weeks ago
• Surgeons placed a tissue valve and did
something to the blood vessel connected to
the heart as well
• This week intermittent night sweats, shivering.
Poor appetite, lethargic. Light headed on
exertion
Annals of Thoracic Surgery
ABCDE
• Airway patent
• SaO2 97% air, RR 18, lungs clear
• BP 110/70, HR 100bpm, S1 plus ESM plus pS2,
warm peripheries
• E4V5M6, BM satisfactory, no focal
neurological deficit.
• Abdomen soft and non-tender. No rashes.
Temp 37.8oC
Initial management
• Lots of blood cultures
• Imaging
• Serial 12 lead ECGs if suspect aortic valve
involvement – why?
• Antibiotics as per microbiology
Seek information
• Get clinic letters, op note
• Had a bicuspid aortic valve with severe
stenosis and moderate regurgitation in
association with dilated aortic root 55mm
• Underwent tAVR and root replacement
(Bentall)
Annals of Thoracic Surgery
Get help
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Admit CCU
Local cardiology review asap
Microbiology opinion
Phone tertiary centre – let surgeons know
Which one of these is not in the
modified Duke criteria?
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2.
3.
4.
Paravalve abscess
Glomerulonephritis
Fever >37.5°C
Pulmonary embolus
Which one of these is not in the
modified Duke criteria?
1.
2.
3.
4.
Paravalve abscess
Glomerulonephritis
Fever >37.5°C
Pulmonary embolus
Clinical course
• Transferred to tertiary centre D2 of admission
• Operated on next day after CT
• 7 hour procedure with massive blood loss and
two periods of circulatory arrest
• Global hypoxic brain injury
• Three months in hospital
Other considerations
• Right sided valve endocarditis also very
common e.g. ToF patient with preexisting
pulmonary regurgitation and poor dental
hygiene – tends to be more insidious and
better tolerated
• Always think endocarditis if CHD patient nonspecifically unwell and / bloods not
completely normal e.g. anaemia (usually Fe
deficiency), mild leucocytosis
4. Haemoptysis in cyanotic patients
Case history
• 40 year old female with large VSD and
Eisenmenger syndrome
• Coughed up about two cupfuls of fresh blood
• Recent chest infection and just finished course
of antibiotics
ABCDE
• Airway patent...for now
• SaO2 80% air, RR 18, lungs sound relatively
clear
• HR 88 regular, BP 130/86. Warm peripheries.
Clubbed++ Loud second heart sound, no
murmurs
• E4V5M6, BM OK, No neurological deficit
• Abdomen soft and non-tender
Initial management
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Always consider HDU
Anaesthetist
Wide bore IV access
FBC – beware a normal Hb
X-match
Keep fasted
Seek information
• What is Eisenmenger syndrome?
Eisenmenger physiology
• A large uncorrected shunt (e.g. VSD, PDA) with
bidirectional flow and pulmonary
hypertension
• Systemic BP = pulmonary artery BP!
• Prone to bleeding from dilated, friable
bronchial arteries, PE, pulmonary AVMs,
bronchiectasis
• A leading cause of death in these patients
www.yorksandhumberhearts.nhs.uk
Mayo clinic
Medscape
Get help & further management
• Respiratory review locally
• Control BP but avoid vasodilating
antihypertensives (increase R to L shunt)
• Carefully titrated opiates / benzodiazepines if
distressed
• CTPA
• Interventional radiology
Eisenmenger physiology
• A large uncorrected shunt (e.g. VSD, PDA) with
bidirectional flow and pulmonary
hypertension
• Systemic BP = pulmonary artery BP!
• Prone to bleeding from dilated, friable
bronchial arteries, PE, pulmonary AVMs,
bronchiectasis
• A leading cause of death in these patients
ABCDE & stabilise patient
Seek information
Get help & plan definitive
management
Questions