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ĐIỀU TRỊ TIM BẨM SINH, Đ H Y DƯỢC TP HCM

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Bài giảng dành cho sinh viên y khoa, bác sĩ đa khoa, sau đại học. ĐH Y Dược TP Hồ Chí Minh.

TREATMENT OF CONGENITAL HEART DISEASE Vu Minh Phuc M.D PhD Medical and Pharmaceutical University HCM City 1 INCIDENCE OF CHD 1.1.Common Lesions in CHD and Approximate Incidence Ventricular septal defect Atrial septal defect ASD Patent ductus arteriosus Aortic Coarctation Coarc Aortic Stenosis AS Tetralogy of Fallot TOF Pulmonary Stenosis VSD 10% PDA 10% 10% 10% PS Transposition of great arteries Pulmonary atresia PA 5% Atrioventricular septal defect AVSD Tricuspid atresia TA 3% Truncus arteriosus TA 3% Total anomalous pulmonary TAPVC venous connection Hypoplastic left heart syndrome Interrupted aortic arc IAA 1% Ebstein’s anomaly 0.5% Mitral stenosis MS rare Aortopulmonary window APW 20% 10% 10% TGA 5-8% 2-5% 2% HLHS 2% rare 1.2 Acyanotic versus Cyanotic Lesions in CHD and Approximate Incidence  ACYANOTIC VSD ASD PDA Coarc AS 10% AVSD APW IAA 1% MS rare 20% 10% 10% 10% 2-5% rare  CYANOTIC TOF PS TGA PA TA TrA TAPVC HLHS Ebstein 10% 10% 5-8% 5% 3% 3% 2% 2% 0.5% 1.3 Pathological Classification of Lesions in CHD and Approximate Incidence  ACYANOTIC Left-to-right shunts VSD 20% ASD 10% PDA 10% AVSD 2-5% APW rare  Left-sided Obstruction Coarc 10% AS 10% IAA 1% MS rare   CYANOTIC Right-to-left shunts TOF10% PS 10% PA 5% TA 3% Ebstein 0.5%  Complex mixing defects TGA 5-8% TrA 3% TAPVC 2% HLHS 2%  TREATMENT OF CHD 2.1 Medical Treatment  PDA Closure by drugs  Indication  Pulmonary and systemic blood flow are not ductus dependent  Infants < 36 weeks’ postconceptional age  Contraindication  Severe hyperbilirubinemia  Severe renal dysfunction  Evidence of necrotizing enterocolitis  Preexisting bleeding tendency 2.1 Medical Treatment  PDA Closure by drugs  Indomethacin infusion or bolus  Success rate 19% to nearly 100%  < 30 wk GA, < wk old :0.2mg/ kg/ dose q12h  doses (maximum dose 0.6 mg/ kg/ 24h)  < 48 hr old  2-7 days old 0.20mg/ kg q12h  doses  > days old 0.25mg/ kg q12h  doses  Ibuprofen 0.2 – 0.1 – 0.1 2mg/ kg q12h (Europe) infusion, PO  10 mg/ kg  mg/ kg at 24 and 48 hours from the initial dose 2.1 Medical Treatment  Manipulation of ductus arteriosus with PGE  Indication  Pulmonary blood flow is ductus dependent – Cyanosis – Right ventricular obstruction • Pulmonary atresia ( VSD) • Severe pulmonic stenosis • Tricuspid atresia  Systemic blood flow is ductus dependent – Pale and/ or gray – Left ventricular obstruction • Interrupted aortic arc • Critical coarctation of the aorta • Hypoplastic left heart (aortic or mitral atresia)  Inadequate mixing : TGA 2.1 Medical Treatment  Manipulation of ductus arteriosus with PGE  Dosage of PGE1 : 0.05-0.1 g/ kg/ infusion  Side Effects of PGE1 Hypotension, vasodilation, bradycardia  Fever, tremors, seizures  Apnea  Metabolic alkalosis  Infection  Diarrhea  Hematologic  Renal  10 VSD closure by Amplatzer occluder device 25 Vascular embolization therapy 26 2.3 Cardiac Surgery  Primary total repair  Palliate and then total repair  Palliate Total repair requires ventricles each capble of supporting the full cardiac output AV valves, or a common valve that can be devided into appropriate sized valves, and AV valve connects to each ventricle A ventricular outflow to the aorta from the left ventricle 27 2.3 Cardiac Surgery Total repair means that There are ventricles, AV valves, outflows from the heart The right ventricle pumps to the lungs and the left to the body There are no large residual septal defects 28 2.3 Cardiac Surgery Palliation Low pulmonary blood flow – BlablockTaussig shunt (BTS, mBTS) High pulmonary blood flow – PA banding Inadequate ventricular outflow to the aorta – Norwood or Damus-Kaye-Stansel (DKS ) Fontan circulation – absolute necessity for low/normal pulmonary vascular resistance 29 Classic BTS 30 Modified BTS 31 PA banding 32 Norwood Stage 33 Norwood Stage 34 Norwood Stage 35 Norwood Stage 36 Norwood Stage (HemiFontan) 37 Norwood Stage (CompleteFontan) 38 Thanks for your attention 39 ... Sildenafil 11 2.2 Interventional Cardiology  Creation of an atrial septal defect  Baloon atrial septostomy  Blade atrial septostomy  Atrial septostomy with static balloon dilation  Balloon valvuloplasty... – Right ventricular obstruction • Pulmonary atresia ( VSD) • Severe pulmonic stenosis • Tricuspid atresia  Systemic blood flow is ductus dependent – Pale and/ or gray – Left ventricular obstruction... 10% PDA 10% 10% 10% PS Transposition of great arteries Pulmonary atresia PA 5% Atrioventricular septal defect AVSD Tricuspid atresia TA 3% Truncus arteriosus TA 3% Total anomalous pulmonary TAPVC

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