GUCH - A growing problem by Dr Susanna Price.

1. GUCH: a growing problem
Susanna Price MD PhD
Consultant Cardiologist & Intensivist
Royal Brompton Hospital, NHLI, Imperial College, London, UK

2. • Nil
Disclosures

3. Outline

4. • 80-85% patients born with CHD survive to adulthood
• 106 patients >20 years with ACHD in USA
• Predicted increase 1,600 per annum in UK (moderate-severe)
• Many at complex end of spectrum:
• Lifelong follow-up
• Repeat surgical and electrophysiological interventions
• Increasing surgical burden (complexity)
Warnes CA, JACC 2001, Wren J, Heart 2001, Somerville J, Heart 2002
ACHD/GUCH

6. GUCH – a global challenge

7. Srinathan SK et al., Heart 2005
Changing patient landscape

8. Changing surgical landscape

9. Evidence-based medicine?

10. • Guidelines recognise special requirements
• Staffing
• Transition clinics
• Specialised services
• Training and education
• No comments regarding critical care
• No recognition of acute management
• No guidelines regarding emergency management
• No ACHD in ICU training
• No ICU training in ACHD
• No guidance regarding impact of expansion on ICU
Guidelines

13. • More patients
• More complex
• Changing interventions
• No guidance/minimal guidance regarding ICU management
A growing problem?

14. Gatzoulis, Cardiothoracic Intensive Care, 2010
Are GUCH patients really different?

15. The ICU literature?

16. • Total number admissions: 5315
• Potential ACHD admissions: 372
• 17 excluded - (Marfan’s, HCM, MVP, 1st time AVR, arrhythmia admissions to level 3 facility)
• Duration ITU admission: 3.1+ 6.8 days (non-ACHD 5.7+0.1)
• Emergency admissions: 24 (6.4%)
• ICU readmission: 6 (1.6%)
• ICU mortality: 14 (3.7%)
Results

17. 4 32
20
100
1215
44
13
4
3
10
4
2
11
8
12
64 PS
Coarctation
AVSD
AV disease
ccTGA
PS/PA+VSD
TOF
Fontan
Eisenmenger
PAPVD
VSD
LA isomerism
Truncus
miscellaneouos
Ebsteins
TGA
ASD
Primary cardiac diagnosis

18. Elective Emergency
Corrective surgery 279 2
Palliative surgery 26 1
Non-cardiac surgery 2 1
Arrhythmia 0 11
Sepsis 0 4
Post-op complication 0 6
Other 0 10
Pulmonary oedema
Hepatic failure
Haemoptysis
PP cardiomyopathy
Indications for ICU admission

19. MedicalACHD
APACHEII
TotalACHD
SAPSII
MODS
ODIN
LODS
SurgicalACHD
EuroSCORE
Ontario
Parsonnet
0
10
20
30
40 ns
ns
ns
*
%predictedmortality
• Severity of disease:
• Simple: 27% (0%)
• Moderately complex: 46% (0-
12%)
• Complex: 27% (0-36%)
• Total mortality: 4.1%
• Medical mortality: 37%
Mortality

20. 0-4 5-9 10-14 14-19 >20
0
5
10
15
20
Predicted
Observed
Parsonnet score
Mortality(%)
0-1 2-3 4-7 8-11 >11
0
10
20
30
40
Predicted
Observed
euroSCORE
Mortality(%)
0-2 3-5 6-8 9-11 >11
0
10
20
30
40
Predicted
Observed
Ontario score
Mortality(%)
Graphs comparing observed outcomes of surgery in ACHD patients shown as a function of pre-operative risk assessment
using the Parsonnet, Logistic EuroSCORE and Ontario systems.
Scores are divided into low/fair/moderate/high/extremely high-risk categories. * denotes p<0.05.
Scoring systems in GUCH

21. • Demographics
complexity (p=0.005) emergency
(p=0.0022) gender, age, sex, BMI
• Investigations
thyroid function (p=0.0048)
bilirubin (p=0.0021)
creatinine (p=0.0032) albumin,
urea, PT, PCV, Hb
• Cardiovascular
arrhythmia
endocarditis, CAD previous
surgery ventricular function,
pulmonary artery pressure
• Other conditions
severe pulmonary disease other
chronic conditions abnormal
neurology syndromes
Mortality predictors

22. • Demographics
complexity (p=0.0047)
emergency (p=0.0012)
gender, age, sex, BMI
• Investigations
thyroid function
(p=0.0014) bilirubin
(p=0.0006)
creatinine
albumin, urea, PT PCV, Hb
• Cardiovascular
arrhythmia
(p=0.0046)
endocarditis, CAD
previous surgery,
ventricular function,
pulmonary artery
pressure
• Other conditions
severe pulmonary
disease, other chronic
conditions, abnormal
neurology, syndrome
Morbidity predictors

23. Procedure/
diagnosis
Repeat
surgery (%)
CPB time
(mins)
X-clamp time
(mins)
Theatre time
(mins)
Aortic valve repair/replacement 100 *153.5+8.0 *103.0+5.0 *393.0+2.2
Aortic valve reapir/replacement + other surgery 73 *124.6+9.9 *89.3+6.1 *317.4+8.0
Resection sub-aortic stenosis 33 *53.5+4.0 †33.1+3.8 249.0+0.5
Coarctation repair 58 108.4+11.6 63.7+9.8 *363.0+3.4
Atrial septal defect repair 5 †46.3+2.2 †28.7+1.9 *188.4+0.5
Ventricular septal defect repair + other surgery 43 77.2+8.4 †4.4+6.3 *322.8+0.74
Atrio-ventricular septal defect repair 55 91.1+12.1 58.6+7.5 *282.0+2.4
Tetralogy 10repair 57 113.8+12.2 72.3+10.4 *316.8+2.5
Tetralogy redo surgery 100 110.8+9.0 68.8+4.1 *441.0+1.1
Ebstein’s anomaly repair 50 70.4+11.4 †27.7+6.4 *380.4+4.3
Pulmonary atresia + ventricular septal defect 100 *138.7+17.3 *76.4+11.0 *448.2+1.35
Previous Fontan 100 *192.8+24.8 61.0+4.8 *660.0+8.1
Transposition of great arteries 66 128.6+20.1 79.0+11.7 *507.0+7.3
Double discordance 89 *160.9+27.0 *103.2+15.0 *377.4+3.6
Non-ACHD 11 102+0.7 62+0.4 246.0+0.1

24. ICU-related cardiovascular interventions

25. ICU-related respiratory interventions

26. • Mean TISS-28: 49.8+0.7 (non-ACHD 48.3+0.1, ns)
• simple 42.3+1.0
• moderately complex 49.5+1.0
• complex 59.8+1.4, p<0.001
• Cost per admission ($ USD)
• simple $5,391 + 130
• moderate $13,218 + 261
• complex $30,074 + 689, p<0.001
Unit cost implications

27. • More patients
• More complex
• Changing interventions
• No guidance/minimal guidance regarding ICU management
• Scoring systems don’t help
• Resource-intensive – interventions and financial
A growing problem?

28. Cardiothoracic Intensive Care, 2010
GUCH ICU: the clinician’s challenge

30. 1. Know the cardiopulmonary anatomy
2. Know the normal physiology of your patient
3. Understand how supportive/interventional therapies might affect the
circulation
4. Anticipate the particular potential pitfalls related to ICU
monitoring/interventions
Basic principles for approaching the critically ill GUCH patient

31. Blood
pressure
Circulating
volume
Cardiac
output
Pulse
oximetry
Pacing ECG INR
Principles: assessment & monitoring

32. Parameter Previous intervention/pathology Comment
Blood pressure Previous classical/modified BT shunt Will under-read. Place catheter/cuff on contralateral
arm
Previous bilateral shunts Lower body pressure measurements more accurate
Previous Coarctation/residual Coarctation, previous
femoral bypass/multiple cardiac catheterisations
Lower limb pressures under-represent central
pressure
Radial line cannulation/surgical cutdown (esp
neonatal)
Ulnar dominant/absent radial artery. Cuff accurate,
avoid ulnar cannulation
Circulating volume Cyanotic ACHD Tolerate hypovolaemia poorly
Univentricular heart Tolerate hypovolaemia poorly, but may have
significantly impaired ventricular function/AV valve
regurgitation
Fontan/TCPC “CVP” often misleading as represents pulmonary
artery pressures
Pulmonary vein stenosis Basal crepitations not indicative of systemic
ventricular failure
Pulse oximetry Compromised arterial supply / systemic hypotension Digital oximeters may be unreliable, use central
oximetry (ear lobe sensors/reflectance oximeters)
Cyanotic ACHD Oximetry may be inaccurate (calibrated to be
accurate at SpO2 >80%)
Cardiac output Tricuspid/pulmonary atresia/Fontan/TCPC PA catheter placement not possible
Intra/extra-cardiac shunts PA catheter unreliable
Chronic low CO state Oesophageal Doppler unreliable (small aorta)
Pacing Multiple previous access, cutdowns etc Expert in access required
Fontan, TCPC, tricuspid/pulmonary atresia Standard trans-venous pacing is not possible. In an
emergency transcutaneous pacing may be required.
ECG Massive atrial enlargement and univentricular
circulation
Atrial tachycardia may be disguised as sinus
tachycardia. High index of suspicion, comparison with
previous ECGS, CSM/adenosine/pacemaker
interrogation may be useful
INR Cyanotic patients If haematocrit >60, need citrate adjusted samples for
accurate measurement
Principles: assessment & monitoring

33. Principles: intervention
PULMONARY CARDIOVASCULAR GASTROINTESTINAL

34. Intended intervention/diagnosis Comment
Associated anatomical defects  Asplenia/polysplenia
 GI/renal malformation
Cyanotic congenital heart disease Associated renal impairment is common
Enteral feeding Severe right heart failure may necessitate low feeding rates
Tolerance to fluid loading/restriction • Univentricular circulations may require relatively full
intravascular volume (beware though AV valve regurgitation)
• Acute right heart dilatation may occur (e.g. Ebstein’s anomaly)
Liver function Abnormal liver function tests common post-operatively, and are
associated with increased mortality
Thyroid function Commonly abnormal in GUCH
Haemoglobin Cyanotic congenital heart disease associated with erythrocytocis
and may require a more liberal transfusion policy
GASTROINTESTINAL/
METABOLIC
Intended intervention/diagnosis Comment
Absent or abnormal connections  Expected (e.g Fontan/TCPC)
 Unexpected (e.g. persistent left superior vena cava)
Multiple previous cannulations/interventions Challenging vascular access
Potential/actual right-left shunting Air filters required on all lines
Cardiac output measurement • Intracardiac shunt may complicate
• Absent pulmonary artery/right-sided connection
• Small aorta may invalidate oesophageal Doppler measurements
Transvenous pacing Consider access routes to right heart as may be absent (e.g
Fontan/TCPC)
Vasoactive drugs Differential effects on systemic and pulmonary vasculature:
 Unpredictable
 May affect cardiac output and saturations
CARDIOVASCULAR
Intended intervention/diagnosis Comment
Intubation Carniofacial abnormalities in associated syndromes may
complicate the process
Tracheostomy  Presence of collateral blood vessels
 Abnormal neck and/or airway anatomy
Associated congenital pulmonary disease  Hypoplastic lung
 Severe congenital V/Q mismatch
Lung reperfusion injury post-operatively ALI/ARDS-like picture, which may be unilateral/bilateral
Pulmonary hypertension May not need treating per se
In presence of inadequate cardiac output may require pulmonary
vasodilators (inhaled/nebulised/intravenous/oral)
Previous cardiac surgery Possibility of phrenic nerve palsy
Difficulty with ventilatory weaning Associated congenital musculoskeletal deformities not uncommon
PULMONARY

35. Intended intervention/diagnosis Comment
Associated anatomical defects  Asplenia/polysplenia
 GI/renal malformation
Cyanotic congenital heart disease Associated renal impairment is common
Enteral feeding Severe right heart failure may necessitate low feeding rates
Tolerance to fluid loading/restriction • Univentricular circulations may require relatively full
intravascular volume (beware though AV valve regurgitation)
• Acute right heart dilatation may occur (e.g. Ebstein’s anomaly)
Liver function Abnormal liver function tests common post-operatively, and are
associated with increased mortality
Thyroid function Commonly abnormal in GUCH
Haemoglobin Cyanotic congenital heart disease associated with erythrocytocis
and may require a more liberal transfusion policy
GASTROINTESTINAL/
METABOLIC
Intended intervention/diagnosis Comment
Absent or abnormal connections  Expected (e.g Fontan/TCPC)
 Unexpected (e.g. persistent left superior vena cava)
Multiple previous cannulations/interventions Challenging vascular access
Potential/actual right-left shunting Air filters required on all lines
Cardiac output measurement • Intracardiac shunt may complicate
• Absent pulmonary artery/right-sided connection
• Small aorta may invalidate oesophageal Doppler measurements
Transvenous pacing Consider access routes to right heart as may be absent (e.g
Fontan/TCPC)
Vasoactive drugs Differential effects on systemic and pulmonary vasculature:
 Unpredictable
 May affect cardiac output and saturations
CARDIOVASCULAR

36. Intended intervention/diagnosis Comment
Associated anatomical defects  Asplenia/polysplenia
 GI/renal malformation
Cyanotic congenital heart disease Associated renal impairment is common
Enteral feeding Severe right heart failure may necessitate low feeding rates
Tolerance to fluid loading/restriction • Univentricular circulations may require relatively full
intravascular volume (beware though AV valve regurgitation)
• Acute right heart dilatation may occur (e.g. Ebstein’s anomaly)
Liver function Abnormal liver function tests common post-operatively, and are
associated with increased mortality
Thyroid function Commonly abnormal in GUCH
Haemoglobin Cyanotic congenital heart disease associated with erythrocytocis
and may require a more liberal transfusion policy
GASTROINTESTINAL/
METABOLIC

37. Diagnosis Potential pitfall
Cyanotic Under-recognition of diuretic requirements
Severe hyperkalaemia post-contrast
Failing sub-pulmonary ventricle Under-diagnosis of pulmonary hypertension
Univentricular heart Unpredictable response to all interventions
Balance of pulmonary vs systemic flow difficult
Interpretation of hypoxaemia difficult
Coarctation GI ischaemia associated with enteral feeding
ASD Over-treatment of PHT
Inadequate HR
AVSD LVOTO
TOF Reperfusion pulmonary oedema
Under-recognition of RV dysfunction
Under-recognition of LV dysfunction
Ebstein’s Difficult to measure CO
Arrhythmias difficult to diagnose and treat
Shunts Difficult to measure BP accurately
Anaesthetic hypotension: reduce pulmonary flow
Pulmonary hypertension Avoid venesection
Avoid hypocapnia
Fontan Arrhythmias difficult to diagnose and treat
CO difficult to measure
Balance benefits vs harm from IPPV
Fenestrated: hypoxia difficult to assess
Anaesthesia may cause haemodynamic collapse
Fluid loading may cause severe MR
Additional pitfalls

38. • As in acquired cardiac disease
• CAD considered rare anecdotally
• Ageing population
• Arterial switch
• Four specific scenarios to consider:
• Left sided (systemic) failure
• Right sided (sub-pulmonary) failure
• Univentricular heart
• Systemic right ventricle
Even ‘heart failure’ is different

39. • Many pitfalls with respect to cardiac intensive care
• Differs from paediatric intensive care
• Implications for:
• Funding
• Theatre scheduling
• ICU bed occupancy
• Availability of investigations requiring specialist expertise
• Training
• Staffing
• Supervision of junior staff
• If familiar with GUCH, easy to under-estimate the challenge for non-congenital
ICU teams
• Interesting and outcomes are good
GUCH intensive care

40. • More patients
• More complex
• Changing interventions
• No guidance/minimal guidance regarding ICU management
• Scoring systems don’t help
• Resource-intensive – interventions and financial
• Critical care of the GUCH patient?
• Challenging
• Very different
• Evidence-free zone
• Little guidance
• Few centres for training
A growing problem?

41. A growing problem?