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Anesthesia for the Patient With Congential Heart Disease For Non Cardiac Surgery Ri 許逵翔/蔡易臻/羅偉成/ CR 章宏吉/ V.S. 黃啟祥 Background Continuous improvement in medical care and surgical treatment longer the lifespan of congential Heart Disease (CHD) patient 1.2 million adults (>16 years) living with various forms of CHD in the U.S. The recommendations -How to care CHD patient?- Centers that have multi-disciplinary CHD programs and staff Every pediatric cardiology program in the center should identify a specialized, dedicated CHD program for the transition of their patients. 這是過渡期(兒童期) 須有小兒心臟專家 Patients with moderate or severe ACHD should be transferred to a dedicated ACHD program for urgent or acute care For Anesthesia -How to care about CHD patient?- Dedicated cardiac anesthesia team with expertise in the management of CHD Well consultative services and interacts at all levels Who is actually repaired? Very few types of congenital heart disease are truly fixed with no residual problems Except : PDA ligation Uncomplicated secundum ASD Uncomplicated VSD Pathophysiology of chronic CHD ventricular overload repeated myocardial ischemia altered coronary artery supply chronic hypoxemia inadequate myocardial protection Alter normal development of cardiovascular system Consequences of Congenital Heart Disease Consequences of Congenital Heart Disease CHF Hypoxemia, cyanosis Polycythemia Pulmonary vascular disease Previous surgical sequence Alter normal growth and cardiovascular development Key in approaching the CHD patient Most important consideration in approaching the patient : potential to develop or exacerbate low systemic cardiac output The other possible risk factors : contractile dysfunction the pressure- or volume-loaded ventricle pulmonary hypertension end-organ dysfunction Atrial septal defect most successful ASD repairs carry little risk of late complications However, if the defect closed late pulmonary hypertension may develop in 20~30 years Persistent atrial arrhythmias Ventricular Septal Defect If the VSD is non-complicated the defect is closed early no residual VSD of significance no associated lesions ex. outflow obstruction, subaortic membrane, heart block Function are likely to be normal However, in complicated patient LV dysfunction, pulmonary hypertension may developed Aortic Coarctation Persistent systemic hypertension independent of any residual obstruction likely due to changes in SVR in response to the presence of a coarctation Most common in older children Recurrent stenosis Most common in infant(20%), few by age 3 years(<1%) LV hypertrophy Increased risk of sudden death Atrioventricular Canal Defects These patients have large left-to-right shunts, excessive pulmonary blood flow CHF Pulmonary hypertension The most common problems after repair valvar regurgitation (especially mitral) residual VSD occasionally pulmonary hypertension TETRALOGY OF FALLOT Park: Pediatric Cardiology for Practitioners, 5th ed. Tetralogy of Fallot PA stenosis Overriding Aorta Large VSD RV hypertrophy http://www.mdconsult.com/das/patient/body/132412227- 3/0/10041/18088_en.jpg Kliegman: Nelson Textbook of Pediatrics, 18th ed. Tetralogy of Fallot • Palliative procedures Central shunt Park: Pediatric Cardiology for Practitioners, 5th ed. Tetralogy of Fallot • Total correction • Repair VSD, PA stenosis Park: Pediatric Cardiology for Practitioners, 5th ed. Tetralogy of Fallot • “Fixed but not cured” • Major problem – Abnormal RV loading (pressure & volume) – Obstruction of RV outflow tract (RVOT) reconstruction Pulmonary arteriosus Truncus Rastelli procedureatresia for TGA with pulmonary stenosis Park: Pediatric Cardiology for Practitioners, 5th ed. Tetralogy of Fallot • Major problems following TOF repair – Progressive RV dysfunction • Predictor of late morbidity and mortality • Decreased exercise tolerance • Image tools – Development of ventricular arrhythmias • Unknown of prognostic significance • Indication for ablation, antiarrhythmic agents, or an implantable cardioversion-defibrillator (ICD) – Increased risk of sudden death Tetralogy of Fallot • Factors reduced long-term survival – Older age (>4 years) at repair – Initial palliative shunting procedures • Especially central shunt – Significant residual RV hypertension • RV:LV > 0.5-0.75; RVOT obstruction, PA stnosis – Volume loading of the RV Tetralogy of Fallot • Difficult to accurately predict Positive pressure ventilation • Defining their risk factors for PVR ↑ their Intrathoracic and Pressure degree ↑of RV dysfunction PR – Positive pressure ventilation (RV afterload ↑ and RV filling ↓) to decrease cardiac output in patients with significant PR and RV dysfunction CO↓ – Dysfunctional RV to compromise systemic cardiac output (via RV decreased RV output, as well as ventricular interdependence dysfunction causing decreased LV filling and function) • Optimizing and maintaining RV function !! Tetralogy of Fallot • Peri-OP – Optimizing and maintaining RV function • Avoided factors that increase PVR – Especially in free PR and RV dilation • Maintained RV filling – But excessive volume loading may also be poorly tolerated • Avoided drugs that significantly diminish RV contractility – Keeping heart rate approximately normal – Maintaining RV oxygen delivery by maintaining blood pressure and oxygen carrying capacity • Prevent tachycardia, hypotension, acidosis, and anemia Tetralogy of Fallot • Post-OP – Invasive monitoring and postoperative care in an ICU setting – “Prophylactic” administration of inotropes to improve RV contractile TRANSPOSITION OF THE GREAT ARTERIES (TGA) Transposition of the Great Arteries http://www.mayoclinic.com/ Kliegman: Nelson Textbook of Pediatrics, 18th ed. Transposition of the Great Arteries • Atrial baffle operation • Atrial switch • Mustard or Senning operation • Physical repair TGA – Atrial Switch • Reasonable functional status and fairly normal lives into their 3rd and 4th decade • 15-20 year survival : 80-85% • Long term – Progressive deterioration of RV function – Development of TR – Signs and symptoms of right heart failure – Arrhythmias (Ventricle & atrial) (tachycardia or SSS) – Sudden death TGA – Atrial Switch • Baffle leak – Intra-atrial shunting and hypoxemia • Baffle obstruction of the systemic venous return – Superior vena cava syndrome, hepatic congestion, ascites, and peripheral edema – Protein-losing enteropathy (PLE) (alb < 3 mg/dl) • Decreased RV function, decreased cardiac index, and increased systemic venous pressure • Baffle obstruction of the pulmonary venous side – Pulmonary edema or the development of pulmonary hypertension Baffle Transposition of the Great Arteries • Arterial switch – Since 1975, Jetene http://radiology.rsnajnls.org/cgi/content- nw/full/247/3/617/F10 Park: Pediatric Cardiology for Practitioners, 5th ed. TGA – Arterial Switch • Anatomical repair • Early hospital mortality < 3% • 5-10 survivals >97-98% Arterial switch operation the preferred procedure in most centers TGA – Arterial Switch • Complication – Small incidence of supravalvar pulmonary arterial and aortic stenosis at the anastomotic sites • Can frequently be addressed by balloon dilatation. – Neoaortic valve (the anatomic pulmonary valve) regurgitation (25-30%) – Coronary ostial lesions (3-5%) • No evidence of myocardial ischemia Anesthesia and Fontan Physiology Fontan operation(1968) • Indication: patient of severe tricuspid atresia, hypoplastic left heart syndrome, etc. http://www.inova.org Physiology of Fontan patient Red: artery Transpulmonary gradient(TPG)= CVP - LVEDP ≒ 5-8 mm Hg Blue: vein Underlying diseases in patient s/p Fontan operation • Protein loss enteropathy(3~15%) • Life-long stroke rate(2~3%) • Thromboembolism(20~30%) • Arrhythmia(>60% after OP 10 years) • Venous pressure elevation • Significant stasis of RA flow • Increased pulmonary venous resistance • Myocardialdysfunction Stamm et al., J Thorac Cardiovasc Surg 2001;121:28 Pre-OP assessment for Fontan Patient • Objective factors: NYHA class, heart rate response, delayed recovery from peak exercise • Echocardiography for heart function • Possible radiofrequency ablation for arrhythmia • CVC monitor: venous filling, pulmonary artery pressure, mixed venous oxygen saturation ※May do CVC under image guidance Penny et al.. Br Heart J 1991;66:372 Gewillig et al. J Am Coll Cardiol 1990;15:1424. Peri-OP monitor for Fontan patient- 1 • Avoid large afterload and keep adequate preload • Increased bleeding risk due to collateral vessels and high venous pressure • Available pacemaker and defibrillator for arrhythmia • Possible inotropic agent use: dopamine or Milrinone (Primacor, 0.25ug/kg‧min) Dawit T. from Mayo Clinic, Pediatric Anesthesia,29 Feb 2008 Peri-OP monitor for Fontan patient- 2 • Positive ventilation: low mean airway pressures (<15-20 mm Hg), low ventilation rate with tidal volume(20ml/Kg), no PEEP use mild hyperventilation • Nebulized pulmonary vasodilator: e.x.: Prostacyclin, NO, epoprostenol continuous use to lower pulmonary venous resistance Peri-OP monitor for Fontan patient- 3 • Follow-up ABG: alkalization blood using sodium bicarbonate to minimize mean airway pressures • “Hypovolemia and increases in pulmonary vascular resistance are poorly tolerated by patients who have had a Fontan procedure. “ From Clinical Anesthesia Procedures of the Massachusetts General Hospital Post-OP care for Fontan patient • Fenoldopam use to increase mesentary and renal blood flow for maintaining end- organ function • Heparin use for prophylaxis of thromboembolism • Hydration for adequate preload Take home message • TOF – Preserve RV function • TGA – Obstruction of repair site – Arterial switch need further follow-up data • Fontan patient: Hypovolemia and increases in pulmonary vascular resistance were major points in anesthesia. • Thank you Reference • Anesthesia for the Patient With Congenital Heart Disease For Non Cardiac Surgery • Park: Pediatric Cardiology for Practitioners, 5th ed. • Kliegman: Nelson Textbook of Pediatrics, 18th ed. • http://www.mdconsult.com/das/patient/body/132412227- 3/0/10041/18088_en.jpg • http://kjronline.org/abstract/journal_figure.asp?img=05- AB.JPG&no=206&desc=desc6 • http://www.mayoclinic.com/ • http://radiology.rsnajnls.org/cgi/content-nw/full/247/3/617/F10 • Anesthesia for Cardiac Surgery, 3rd ed.
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