A teaching session I gave in 2012 to Cardiology / Acute Medicine trainees when I was working as a Staff Specialist / Professor of Cardiology in Darwin, Australia.

1. How to deal with
Acute Admissions of
Patients with Adult
Congenital Heart
Disease
Dr Colin A J Farquharson
MBChB MD FRCP FESC
Consultant Cardiologist
Royal Darwin Hospital
25 September 2012

2. Summary
• By the end of this session
– You won t be an expert in ACHD
– You will have an idea of the context of the
population
– You will have a framework by which to
manage acute presentations
– You will be aware of some of the specific
conditions which cause the most problems
– You will know where to go to ask for help

3. Why is it your problem?
• Already more adults than children with adult
congenital heart disease in the Western World
• Predicted to increase by 50% in next 10 years
• Overall incidence of 75 per 1000 live births
• Incidence of moderate / severe lesions – 8 per
1000 live births
• 90% now survive to adulthood and beyond
- approx 50 complex cases per 100 000
population

4. ACHD units in Australia
Darwin to Melbourne
3140 km
4 hours 40 min flight
Will have approx 70
adult patients in
RDH catchment area
with complex ACHD

5. RDH EMERGENCY DEPARTMENT
Wherever you end up working, you will
see increasing numbers of ACHD
patients presenting as emergencies

6. First Principles
• DON T PANIC!
• Initial treatment just the same as any
other unwell patient
• Assess ABC
• Treat arrhythmias as arrhythmias
• Treat heart failure as heart failure
• Treat chest pain as chest pain

7. Second Principles
• Repaired does not mean normal – either
structurally or physiologically
• You won t know what all the different
operations are and what their importance
is
• Common sense and caution is key
• The patient is likely to know much more
than you about their condition – so be
prepared to listen

8. Common / Serious Presentations
• Arrhythmias
• Chest pain
• Blue patients
– General acute deterioration
– Haemoptysis
– Cerebral abscess / Stroke
– Cholecystitis
• Endocarditis
• Coarctation repair site problems
• Thrombosed prosthetic valves
• Non-cardiac emergencies

9. Arrhythmias
• Commonest presenting complaint in local DGH
A&E by far
• Treat as you would any other adult patient with an
arrhythmia, just with a little more respect!
• Patients may deteriorate more quickly than you
expect
• If compromised, need to be cardioverted electrically
and usually quickly
• If not compromised – consider seeking senior
advice
• DO NOT send them home – even if the arrhythmia
has terminated

10. Supraventricular Tachycardia
• Common in any operated ACHD patient
• Related to atrial scars, atrial volume or pressure load
• Atrial flutter (typical or atypical) most common, AF, re-
entrant tachycardias less common
• Young AV node often allows very fast ventricular
response rates, leading to syncope (You may make this
problem worse by giving IV amiodarone)
• Patients with impaired systemic ventricular function
may not tolerate fast ventricular response rates well
• Patients may not tolerate loss of AV synchrony well in
some situations
• Particularly common in tetralogy of Fallot, atrial
surgery, Mustard / Senning, Fontan, Ebstein s anomaly

11. IV AMIODARONE
Atrial flutter at about 300
/ minute with 2:1 block
Ventricular response rate
150 bpm
Patient stable
Atrial flutter at about 250 / minute
with 1:1 conduction
Ventricular response rate 250 bpm
Patient unstable after IV
Amiodarone

12. Substrate for atrial arrhythmias

13. Tips & tricks when treating SVT
• DCCV if necessary
• Always put on external pacing pads when
cardioverting – many patients will be
significantly bradycardic afterwards
• Adenosine is usually worth a try
• Generally avoid flecainide acutely
• IV amiodarone rarely works acutely and
doesn t rate control very well
• IV amiodarone can slow the intrinsic flutter
rate allowing the AV node to conduct 1:1

14. MM, 24 yrs, Mustard operation for TGA,
palpitations and syncopal episode at work.
Now breathless, clammy, pulse 225/min, BP
70/40mmHg
• IV adenosine?
• IV amiodarone?
• IV beta blocker?
• Cardiovert?

15. Special case – SVT (usually atrial
flutter) in a FONTAN
• How does the blood get
round?
• Low PVR
• Good single ventricular function
• Good filling pressures
• Good systemic AV valve function
• Repetitive negative intra-thoracic
pressures from breathing
Also known as classic fontan,
modified fontan, total
cavopulmonary
connection, lateral tunnel

16. Atrial tachyarrhythmias are common in
classic Fontan s because of R. atrial
dilatation
• Tachycardia and loss of 1:1 AV synchrony leads to
reduction in ventricular filling and contractile function
• LA pressure increases, reducing transpulmonary
gradient and therefore reducing the cardiac output
• Usually very symptomatic (breathless and
hypotensive)
• Very unusual to revert spontaneously or with
pharmacological agents to SR
• Plan to cardiovert them electrically
– Immediately if compromised
– Otherwise as soon after admission as possible

18. How to cardiovert / anaesthetise in
ACHD (esp Fontan)
• IV fluids to maintain filling pressures
• Avoid delay – cardiovert ASAP
• Explain the physiology in detail to the
anaesthetist
• External pacing pads on in case need to
pace them afterwards
• Avoid prolonged positive pressure
ventilation

19. How not to cardiovert / anaesthetise
in ACHD (esp Fontan)
• Admitted at 5 pm in flutter
• Put on following morning list for DCCV
• NBM overnight, no IV fluids
• Cardioversion delayed because of an emergency
case
• At induction of anaesthesia
- Low filling pressures because of dehydration
- Vasodilatory anaesthetic agents used
- IPPV
• Subsequent avoidable circulatory collapse
• Death

20. Ventricular Tachycardia
• Particularly common in repaired Tetralogy of Fallot
(RV scar and stretch), failing systemic right
ventricles
• Treat acutely as you would any other VT
• If CVS stable try IV amiodarone
• DC cardioversion otherwise
• Overdrive pacing an be tried if VT resistant
• Can be a sign of underlying haemodynamic problem
– should be re-assessed at a specialist centre
• Some success with VT ablation in e.g. ToF
• Consider for AICD
• Try to avoid long term amiodarone if at all possible

21. VT is common in repaired Tetralogy
of Fallot with dilated RV

22. VT is common in Mustard/ Senning /
TGA patients with failing systemic
RV

23. Chest Pain
• Common in any congenital heart disease patients (?
Scar-related, ? Psychogenic)
• Most groups of patients are at no increased risk of
developing coronary artery disease (except
coarctation, arterial switch)
• Assessment as for anyone with chest pain (common
sense risk assessment)
• In Eisenmenger patients chest pain may represent RV
angina
• Take great care in patients with coarctation repairs
and chest pain - ? dissection or aneurysm

24. Beware the abnormal resting ECG!

25. Beware the abnormal resting ECG!

26. Beware the abnormal resting ECG!

27. Cyanotic patients
• (Arrhythmias)
• (Chest pain)
• Acute / sub acute general deterioration
• Haemoptysis / Intra-alveolar haemorrhage
• Paradoxical emboli leading to stroke and
cerebral abscess
• Cholecystitis
• Haematological concerns
• Gout

28. Acute / sub acute general
deterioration
• Usually driven by infection, heart failure or
arrhythmias
• Treat breathlessness, not saturations or
ABGs
• Treat any identifiable underlying cause as
normal
• NIV not contra-indicated to treat symptoms,
but take care on being guided by pO2

29. • pH 7.34
• pC02 6.88 (51.6mmHg)
• pO2 4.73 (35.5mmHg)
• HC03 27.7
• BE 0.8
• O2 SAT 59.3%
WHAT WOULD YOU DO?

30. Haemoptysis
• Common in Eisenmenger patients
• Often associated with chest infection – have
a low threshold for broad-spectrum
antibiotics
• Almost always self-limiting (although often
recurs)
• Mode of death for some patients rarely
• If severe or recurrent may be treatable by
coiling
• Beware falsely elevated INR

31. Intra-alveolar haemorrhage
• Internal haemoptysis
• Suspect in patients with small amounts of
haemoptysis who seem disproportionately
unwell
• Progressive drop in Hb (should be high
normally) and pO2
• Fluffy white shadows on CXR
• Easily identified by CT
• May require coiling to treat
• May be fatal

33. Cerebral Abscess – remember to
use IV filters in shunt patients!

34. Blue patients – haematological
concerns
• High haemoglobin and haematocrit are a physiological
response to low oxygen sats
• Injudicious venesection leads to significant deterioration
in symptoms, iron deficiency and increased risk of stroke
• Often have low platelets
• Falsely elevated INRs
• Venesection indicated for severe symptoms of
hyperviscosity only – no role in reduction of stroke risk
• Watch for iron deficiency anaemia
• Some evidence that venesection prior to surgery may
improve platelet function acutely

35. Vasodilatation in right-to-left
shunters
• Any drugs or other
measures which reduce
SVR will increase the
right-to-left shunt and
can lead to profound
cyanosis followed swiftly
by cardiac arrest
• Venesection without
isovolumic fluid
replacement at the same
time can have the same
effect.

36. Blue patients – tips and pitfalls
• Beware paradoxical emboli – use filters on all
IV lines
• Care with amiodarone, aminoglycosides,
NSAIDs
• Cerebral abscesses can present insidiously –
CT head for even minor neurological
symptoms
• If nil by mouth - give maintenance IV fluids
• O2 sats finger probes inaccurate < 85% - only
measure sats if patient is breathless
• Avoid peripheral vasodilators at all costs

37. Coarctation of the Aorta
Problems are rare, but include:
• Acute coronary syndromes
• SAH
• Aortic dissection
• Aortic rupture
• Eroding coarctation site aneurysm

38. Coarctation site aneurysm
Patients who have had
previous coarctation
repair who present
with unexplained
haemoptysis or
haematemesis
should have urgent
imaging of their thorax
– usually by CT

39. Thrombosed Metal Valves
/ Shunts
• High index of suspicion
• Risk increase during pregnancy
• More common with small old valves and narrow
shunts
• Poor compliance with anti-coagulation (or poor
advice given by doctors / nurses re INR!)
• Treatment depends on circumstance
– Re-anticoagulate with more aggressive regime
– Thrombolyse (high risk of stroke in L sided
valves)
– Surgery ? Percutaneous valve intervention

40. 22 yrs, metal MVR for endocarditis,
16/40 pregnant on unadjusted dose
LMWH

41. Non-Cardiac Emergencies
• Often general surgical, orthopaedic, gynae
• General principles of the management of
cyanotic patients (fluids, filters etc)
• Extra care with general anaesthesia –
particularly with Fontan and Eisenmenger
• Care with central venous access – central
veins are often scarred / occluded

42. General Conclusions
• Follow general principles of management of
acutely unwell patients
• Know your own limitations
• Seek help early
– Patient and family
– Hand held records (e.g. old ECGs from patient)
– Local Adult Cardiology services
– Local Paediatric Cardiology services
– Congenital cardiology team at e.g. Adelaide /
Melbourne etc

43. Remember - Alarm bells should
ring …….
• Fluttering Fontan
• Complex patients (esp Fontans or
Eisenmengers) admitted under non-
cardiological teams / needing general
anaesthesia or anticoagulant advice
• Cyanosed patients who present with
minor neurological symptoms
• Repaired coarctation with haemoptysis
or haematemesis

44. ANY
QUESTIONS
?