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

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

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

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

RDH EMERGENCY DEPARTMENT

Wherever you end up working, you will see increasing numbers of ACHD

patients presenting as emergencies

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

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

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

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

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

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

Substrate for atrial arrhythmias

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

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?

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

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

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

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

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

VT is common in repaired Tetralogy of Fallot with dilated RV

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

RV

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

Beware the abnormal resting ECG!

Beware the abnormal resting ECG!

Beware the abnormal resting ECG!

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

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

•  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?

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

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

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

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

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.

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

Coarctation of the Aorta

Problems are rare, but include: •  Acute coronary syndromes •  SAH •  Aortic dissection •  Aortic rupture •  Eroding coarctation site aneurysm

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

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

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

LMWH

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

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

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

ANY QUESTIONS

?