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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 • • • • • • 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% • • • • • • Reduced platelet count Higher risk for thrombosis and bleeding Renal function: reduced eGFR, proteinuria Gallstones Scoliosis Liver failure and liver tumours Pulmonary hypertension • • • • Eisenmenger Syndrome Anticoagulation Mean age of death 32.5 years Do not do anything unless necessary Investigations • • • • • 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 • • • • Anaesthetic plan • • • • • • • 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 • • • • 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 • • • • Single institution 1998-2004 > 4000 deliveries 151 (3.5%) if GUCH women AA 2011;113:307-17 • • • • 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 • • • • • • 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)