GUCH - A growing problem
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1. The number of adults with congenital heart disease (ACHD) is growing due to increased survival of children born with CHD. However, there is minimal guidance on managing ACHD patients in the intensive care unit (ICU). 2. A study of 372 ACHD patients admitted to the ICU found longer lengths of stay, higher resource use, and mortality risks compared to non-ACHD patients. Outcomes varied by complexity of heart condition. 3. Managing ACHD patients in the ICU requires understanding their unique cardiac anatomy and physiology to avoid potential harms from standard interventions and assessments. Special considerations are needed for procedures, monitoring, and treatment.
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GUCH - A growing problem
- 1. GUCH: a growing problem Susanna Price MD PhD Consultant Cardiologist & Intensivist Royal Brompton Hospital, NHLI, Imperial College, London, UK
- 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
- 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?
- 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
- 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?