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Pathophysiologic Consideration In Patients With Congenital Heart Disease SAMIA SHARAF .MD Professor Of Anaesthesia .. Ain Shams University Classification Of Congenital Heart Lesions 1) 1) 1) Obstructive lesions eg. Aortic stenosis – coarctation of aorta Increased pulmonary blood flow eg. ASD – VSD – PDA Decreased pulmonary blood flow lesions eg. Tetralogy of fallot – tricuspid atresia – pulmonary atresia Classification Of Congenital Heart Lesions Left To Right Shunt Atrial Level : ASD 5% , TAPVC Ventricular Level : VSD 33% Great Artery Level : PDA 10% Truncus Arteriosus : 1% Coronary Level : ALCAPA Right To Left Shunt TOF : 9% TGA : 1% Left Heart Obstructive Lesions Mitral Stenosis Aortic Stenosis : 8% Coarctation : 5% Hypoplastic Left Heart Syndrome Right Side Obstructive Lesions Pulmonary Stenosis / Atresia : 10% Tricuspid Stenosis Hypoplastic Right Heart Single Ventricle Others Vascular Rings Venous Anomalies Arteriovenous Fistula Clinical Presentation Of Children With CHD 1) 2) 3) 4) Cyanosis ( due to hypoxia ) Respiratory system abnormalities Cardiac failure Arrhythmias Cyanosis Pathophysiologic Effects of Hypoxia (1) Growth (2) Heart Exercise intolerance : myocardial dysfunction ventricular compliance and contractility Irreversible myocardial damage . Increased sympathetic tone down regulation of beta receptors cardiomyopathy (3) Hematology A major adaptive response to chronic hypoxia Red cell mass Polycythemia Secondary Spherocytosis Blood viscosity Risk of thromboembolic events Hemostasis : Polycythemia Coagulation abnormalities Primary fibrinolysis DIC Mechanism of coagulation abnormalities DIC Hypercoag. blood & tendency to bleed Thrombocyt openia & Low Fibrinogen & Other Factor Level Increased blood viscosity Increase intravascular strains Consumpution of platlets , fibrinogen , factor V , VIII Fibrin deposition & platlet aggreg. (4) CNS Chronic hypoxia causes impairment of neurologic development and increase risk of neurologic damage . Brain abscess : Rt. – Lt. shunt Cerebrovascular thrombosis and hemorrhage . Respiratiry System Abnormalities Anatomical abnormalities of airway Pulmonary abnormalities associated with pulmonary blood flow . or Anatomical Abnormalities Of Airway 1) 2) Short trachea eg. interrupted aortic arch large airway obstruction : ( trachea & bronchi ) Compression by enlarged aorta or pulmonary artery . Upwards displacement and increase angle of bifurcation of trachea by enlarged LA . 3) • • Small airway obstruction : Compression of lung parenchyma by enlarged heart and vessels . Pulmonary hypertension . Pulmonary Changes Associated With Pulmonary Blood Flow Patients with chronic hypoxia 1) Slight of alveolar ventilation 2) pulmonary venous PO2 is high 3) V/Q mismatch alveolar – pulmonary venous O2 gradient 4) Physiological dead space end tidal CO2 is lower than arterial PaCO2 Pulmonary Changes Associated With Pulmonary Blood Flow Obstruction of small airway Pulmonary congestion pulmonary compliance , lung water & Impaired gas exchange Progressive of pulmonary vascular resistance due to hypertrophy in muscular layer of pulmonary arteries reverse of left to right shunt Cardiac Failure Causes of limited cardiac reserve : (1) Increased cardiac workload Pressure overload : ventricular outflow tract obstruction SVR blood viscosity Volume overload : * Valvular insufficiency * Single ventricle * Left – right shunt (2) Myocardial contractility: Prolonged workload of myocardium Vascular supply to ventricles Blood hyperviscosity Chronic hypoxia Compansatory Mechanism Ventricular hypertrophy Adrenergic system changes Activation of B receptors Renal system compansation *Salt & water retention *Renin secretion Arrhythmias Types : * Congenital * Acquired Etiology : Intrinsic electrophysiology abnormalities Damage from chronic hypoxia – hemodynamic stress Surgical injury eg. F4 , Fontan operation , atrial correction of TGA Congenital Conduction System Abnormalities Congenital complete atrioventricular block Wolf – Parkinson white syndrome Supraventricular tachycardia Arrhythmias associated with Ebstien anomaly Acquired Conduction System Abnormalities Non surgical : rare Surgical by : * cardioplegia * mechanical retraction * ischemia * metabolic abnormalities Why we are conserned about the pathophysiology of CHD Provide Safe Anaesthetic Technique Decrease Anaesthetic Risk Anaesthetic Risk factors affecting anaesthetic risk in congenital heart disease Pulmonary disease Myocardial dysfunction Cyanotic heart disease Cardiovascular impairment Arrhythmias Magnitude of surgery Anaesthetic risk How To Reduce Anaesthetic Risk ?? 1 • Consultation 2 • Surgical Status 3 • How To Look To The Data Consultation Role Of Surgeon Case discussion : Pts. with CHD may not tolerate : Abdominal laparoscopic procedures ( eg. stenotic valvular lesions , single ventricle ) Absorption of CO2 ( C.O.P dependant low PVR) . One lung ventilation Prone position ( Fontan pt. ) Role Of Pediatric Cardiologist Preoperative consultation sometimes add a little benefit to anesthiologist !!!!! History data exact anatomy Previous cardiac operation Myocardial function status Murmurs Gallops Pulse in extremities Pediatric cardiologist consultation Base line O2 saturation Vital data Planned followup as needed 2-3 months interval New echo Unstable pt. Major operation Surgical Status Of The Patient Untreated Cured Corrected Palliated *Normal life expectancy *Normal CV reserve *No further medical ttt *Markedly prolonged life expectancy *Some limitation of cardiac reserve *May require further medical ttt *Prolonged life expectancy *Definitly abnormal CV physiology *Certainly require medical or surgical ttt Efficacy Of Repairs For CHD Lesions CURED PDA ASD CORRECTION VSD TFO Coarctation of aorta Pulmonary or aortic stenosis AV Canal repair PALLIATION Conduits PA banding Modified Glenn shunt