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Anesthesia for Adult Patient with Congenital Heart Disease Mohamed Saleh, MD Department of Anesthesia and Intensive Care, Ain-Shams University Case Scenario • A 17 years-old patient, was presented for emergency appendectomy. He had a history of repaired TOF at the age of 2 years. 12 years later, he started to complain of palpitations, exertional dyspnea, and was diagnosed as pulmonary regurgitation and right ventricular dysfunction. • How to manage this patient in the perioperative period? Introduction Complete surgical repair Partial or palliative surgery Journal of Cardiothoracic and Vascular Anesthesia, Vol 20, No 3 (June), 2006: pp 414-437 Non-operated Arrhythmias Hematologic dysfunction Decreased Pulmonary Blood Flow Increased Pulmonary blood Flow Hypoxemia Pulmonary HTN Hemostatic Dysfunction Renal dysfunction Neurologic disease Pressure and /or Volume over load Ventricular dysfunction Arrhythmias Pulmonary hypertension Ventricular dysfunction • Volume Overload • Pressure Overload Hematologic dysfunction • Erythrocytosis • Hyper-viscosity • Iron deficiency anemia Hemostatic Dysfunction • • • • Thrombocytopenia Platelet function abnormalities Disseminated intravascular coagulation Decreased production of coagulation factors – Impaired liver function – Vitamin K deficiency • Primary fibrinolysis Renal dysfunction • • • • • Hypercellular glomeruli Basement membrane thickening Focal interstitial fibrosis Tubular atrophy Hyalinization of afferent and efferent arterioles. Neurologic disease • Cerebral abscess • Cerebral emboli / thrombosis Common congenital heart diseases Atrial Septal Defect Anatomy Physiology Potential Issues Specific Anesthetic Management L-R shunt • Small to moderate size defects well tolerated • Atrial fibrillation (increased risk if repaired after age 40) • Risk of paradoxical emboli • Large defects lead to arrhythmias, exercise intolerance, and rarely PHT (occurs in less than 5% of patients) De-air intravenous lines Ventricular Septal Defect Anatomy Physiology Potential Issues Specific Anesthetic Management L-R shunt Unrepaired: • Large defect risk of PHT (50% by age 2) • Small to moderate size defects, risk for endocarditis, subpulmonic obstruction, subaortic obstruction, and aortic regurgitation • Right ventricular failure Repaired: • Complete heart block in some patients (rare) • Persistent PHT • Dysrhythmia Manage L-R shunt May be associated with other defects Maintain pulmonary blood flow if R-L shunt present Increased risk of postoperative pulmonary infection Manage pacemaker Coarctation of the aorta Anatomy Physiology Potential Issues Specific Anesthetic Management LV pressure overload and Hypertrophy • Blood pressure gradient between upper and lower limbs • Risk of bleeding if thoracic surgery • LV hypertrophy and LV diastolic dysfunction • Systemic hypertension • Aneurysms of ascending aorta and descending aorta • Premature coronary artery disease • Intracranial aneurysms (10%) Inaccurate blood pressure (left arm) if previous subclavian angioplasty Aortic branch collaterals Associated with bicuspid aortic valve (50-80%) Endothelial dysfunction (diffuse aortopathy) Postoperative hypertension Avoid tachycardia, Hypotension Aortic Stenosis Anatomy Physiology Potential Issues Specific Anesthetic Management LV pressure overload and Hypertrophy Unrepaired: • Pulmonary edema • PHT • Myocardial ischemia • Syncope • Post stenotic dilatation Repaired: • Aortic regurgitation • LV diastolic dysfunction Avoid tachycardia, hypotension Avoid factors that increase myocardial oxygen Consumption Tetralogy of Fallot Anatomy Physiology Potential Issues Specific Anesthetic Management Pulmonic stenosis Unrepaired: Rare, mean age of death 25 yr, R-L shunt, Cyanosis Palliated: Blalock-Taussig shunt • Chronic left ventricular volume overload • Cyanosis if shunt is too small • Pulmonary hypertension Repaired: • Sinus and AV node dysfunction • Dysrhythmia: atrial and ventricular • Ascending aortic aneurysm • Residual pulmonary regurgitation or stenosis • Residual VSD • Left ventricular dysfunction • Persistent pulmonary hypertension from previous shunts • RV failure from chronic pulmonary insufficiency Avoid tachycardia, hypovolemia, increased contractility Maintain pulmonary blood flow Maintain sytemic blood pressure Detect and manage dysrhythmias Manage pacemaker External pacing available RV hypertrophy Overriding aortic root VSD Cyanosis Transposition of Great Arteries Anatomy Physiology Potential Issues Specific Anesthetic Management PA arises from LV Unrepaired: Associated with VSD or ASD or PDA Repaired: Senning or Mustard • Atrial dysrhythmia • Sinus node dysfunction (by age 40, 50% have pacemaker) • Systemic ventricle dysfunction • Residual atrial or ventricular level shunts Repaired: Arterial switch • Myocardial ischemia (narrowed, occluded coronary arteries, endothelial dysfunction) • Ascending aortic aneurysm Maintain pulmonary blood flow Manage dysrhythmia Manage heart failure Detailed preoperative evaluation, both functional and coronary imaging study Aorta arises from RV Possible associated lesions: VSD, ASD, PDA, Pulmonary stenosis, coarctation of the aorta Abnormal coronary artery anatomy Univentricular Heart Anatomy Physiology Potential Issues Specific Anesthetic Management Double inlet atrioventricular Connections Unrepaired: Rare • Dysrhythmias • Congestive heart failure • Bidirectional shunting • Cyanosis • PHT Repaired: Blalock-Taussig shunt, Glenn shunt, or Fontan • Dysrhythmias • Heart failure • Hepatic dysfunction • Thromboemboli • Restrictive lung disease Manage dysrhythmias Maintain pulmonary blood flow Manage dysrhythmias Maintain low pulmonary vascular resistance Maintain adequate preload Replace coagulation factors Absence of one atrioventricular Connection Single welldeveloped ventricle Strategy for management of ACHD patient through non-cardiac surgery anesthetist surgeons cardiologist Adult Congenital Heart Center intensivist Classification of ACHD according to complexity 1- Define the Condition – Primary lesion – Previous palliative and corrective surgeries – Residual and sequelae 2- Assess the Surgical Risk – Predictors and risks of the proposed surgical procedure itself – Specific ACHD risk factors – Other co-morbid conditions 3- Develop a Management Plan – Understanding of anatomy and physiology – Hemodynamic goals – Perioperative management Promote Oxygen Delivery to tissues Maintain balanced Pulm. & Systemic Blood Flow Optimize Hematocrit HISTORY & PHYSICAL EXAMINATION • Assess functional status • Symptoms, signs of right sided failure • Symptoms, signs of left sided failure • Symptoms of low cardiac output • Arrhythmias • Cyanosis & its sequelae chest X – Ray Laboratory Evaluation 12 Lead ECG Preoperative investigation Cardiac Catheterization Echocardiography De-airing • All intravenous lines must be meticulously deaired. • Patent foramen ovale is present in approximately 25% of adults Indications for Prophylaxis against IEC in CHD* – Unrepaired cyanotic CHD, including palliative shunts and conduits – Completely repaired congenital heart defect with prosthetic material or device, whether placed by surgery or by catheter intervention, during the first 6 months after the procedure** – Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device (which inhibit endothelialization) – Previous IE • *Except for the conditions listed above, antibiotic prophylaxis is no longer recommended for any other form of CHD. • **Prophylaxis is reasonable because endothelialization of prosthetic material occurs within 6 months after the procedure. Premedication • Premedication with anxiolytics and hypnotics must be undertaken very cautiously. Monitoring Non-invasive • • • • ECG NIBP Pulse oximetry End tidal capnogram Invasive - Art. catheterization - CVP - PAC - TEE Choice of anesthetics • There are no evidence-based recommendations to guide the anesthetic management of patients with CHD undergoing non-cardiac surgery. Intravenous anesthetics Barbiturates Propofol Benzodiazepines Ketamine Etomidate Inhalational anesthetics Halothane Enflurane Isoflurane Sevoflurane Desflurane Regional anesthesia Spinal anesthesia Epidural anesthesia Fluid management • Minimizing the NPO interval • Preoperative hydration • Maintenance of adequate intravascular volume • Adjustment of the transfusion threshold. Positioning Lateral Anti-Trendelenberg position Trendelenburg position Prone Positive Pressure Ventilation • Control pulmonary vascular resistance • Reduces systemic venous return, in patients with single ventricle physiology Postoperative Management PACU vs. ICU Major risk factors during the postoperative period: • Bleeding. • Dysrhythmias. • Thromboembolic events. Postoperative Management • Post-operative analgesia • Fluid Management Thank you