Download Anaesthesia for patients with grown up congenital heart disease

Anaesthesia for patients with grown
up congenital heart disease GUCH
I Malagon FRCA, PhD, FFICM
Consultant Cardiac Anaesthesia & Intensive Care
University Hospital South Manchester, United Kingdom
Honorary Senior Lecturer University of Manchester
• 17 year old boy
• Broken wrist while playing with friends
• Needs GA, refuses manipulation under local or
light sedation.
Questions
• Referral to another hospital
• Type of congenital anomaly
• Corrected or palliated
How big is the problem
How big is the problem
• 20,000 GUCH patients estimation in the
Netherlands, only 8,000 are seen in hospital.
• UK has an estimated 800 new GUCH patients
per year
The lifetime prevalence of congenital heart disease in children and adults in Quebec, Canada,
in 2010. 95%CI indicates 95% credible interval.
Ariane J. Marelli et al. Circulation. 2014;130:749-756
Copyright © American Heart Association, Inc. All rights reserved.
The numbers and proportions of adults and children in Quebec, Canada, with all (A) and
severe (B) congenital heart disease over time in 2000, 2005, and 2010.
Ariane J. Marelli et al. Circulation. 2014;130:749-756
Copyright © American Heart Association, Inc. All rights reserved.
Distribution of the techniques used in the growing population of Fontan patients alive in
Australia and New Zealand.
Yves d’Udekem et al. Circulation. 2014;130:S32-S38
Copyright © American Heart Association, Inc. All rights reserved.
Kaplan–Meier Survival by Fontan type.
Yves d’Udekem et al. Circulation. 2014;130:S32-S38
Copyright © American Heart Association, Inc. All rights reserved.
How big is the problem
• 1970 – 2011
• More than 9000 claims
• 21 cases GUCH, 11 cardiac procedures, 10 noncardiac
procedures
• Average settlement more than 700,000 $
Congenital Heart Disease 2015;10:21-29
Anesthesiology 2013;119:762-9
Anesthesiology 2013;119:762-9
• Nationwide Inpatient Sample Data Base USA
• 2002-2009
• 10000 GUCH compared to 37,000 matched
cohort
• Mortality 4.1% compared to 3.6%
• Morbidity 21% compared to 16%
• GUCH represents 0.11% of noncardiac surgery
Anesthesiology 2013;119:762-9
Anesthesiology 2013;119:762-9
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Preoperative evaluation
Investigations
Arrythmias
Anaesthetic plan
Monitoring
Antibiotics prophylaxis
Anesthesiology 2009;111:432-40
• Preoperative evaluation
– Obtaining reliable information
– Pulmonary hypertension
– Cyanosis
– Ventricular dysfunction, conduction defects,
arrhythmias, residual shunts, regurgitant or
stenotic valves and aneurysms.
– Chronic cardiac failure
– Chronic non cardiac coexisting diseases; diabetes,
neurological, airway, renal and liver
Cyanosis
• Increase in red cell mass
– Phlebotomy not recommended if Hb less than 20 g/dl
or Hc less than 65%
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Reduced platelet count
Higher risk for thrombosis and bleeding
Renal function: reduced eGFR, proteinuria
Gallstones
Scoliosis
Liver failure and liver tumours
Pulmonary hypertension
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Eisenmenger Syndrome
Anticoagulation
Mean age of death 32.5 years
Do not do anything unless necessary
Investigations
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Exercise testing
Holter
TTE and TOE
Cardiac MRI
Cardiac catheterisation
Arrhythmias
Main cause for admission
Main cause for morbidity and mortality
SVTs are the most common
Pharmacological treatment; Amiodarone,
everything else is a disappointment
• Ablation; does not work
• Anatomical challenges for pacing and ICDs
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Anaesthetic plan
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Premedication yes/no
Gas v iv induction
Spontaneous v controlled ventilation
GA +/- regional anaesthesia
Monitoring
Postoperative care
Post operative pain relief
Antibiotic prophylaxis
Previous endocarditis
Unrepaired cyanotic CHD
Patients with palliative shunts or conduits
Presence of prosthetic material or a device, if
operation is within 6 months of insertion.
• Residual defects close to the prosthetic device
• Re-do cardiac surgery
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Cyanotic v Acianotic
Other complications
– Plastic bronchitis
– Protein losing enteropathy
– Chylothorax
– Thrombosis
– Arrhythmias
Plastic bronchitis
Plastic brochitis
• Failing Fontan circulation
• Treatment
– Antibiotics
– Steroids
– Bronchodilators
– Mucolytic agents
– Anticoagulation
– Nebulized urokinase/rtPA
Protein-losing enteropathy
• Prevalence between 4 and 24%
• Symptoms
– Peripheral oedema
– Ascitis
– Pleural/pericardial effusions
– Hypoalbuminaemia
– Elevated clearance α1-antitrypsin
Treatment
• Interventional
• Surgical
• Pharmacological
– High/Low molecular weight heparin
– Sildenafil
– Prednisone
– High doses spironolactone (4-5 mg/kg/day)
Obstetrics and GUCH
• Normal physiology in pregnancy
– 50% increase in blood volume
– 30% increase in cardiac output
– 30% increase in heart rate
– Reduction in SVR
Am Heart J 2015;169:298-304
Regional v nothing
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Single institution
1998-2004
> 4000 deliveries
151 (3.5%) if GUCH women
AA 2011;113:307-17
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20 patients over 14 years
75% had no complications
Mostly epidurals
Prognostic indicators for adversity
– Severe PR
– Pulmonary hypertension
– Impaired RV or LV function
High risk patients
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Significant aortic stenosis < 0.7 cm2
Significant coarctation
Significant mitral stenosis
Pulmonary hypertension
Marfan’s syndrome
Cyanotic heart disease
CONCLUSIONS
• The number of GUCH patients will continue to
increase.
• Ideal facilities and care pathways are yet to
materialise
• When confronted;
– Back to basics
– Make a good plan
– Discuss with other colleagues (centre if possible)