congenital heart disease n.
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Congenital Heart Disease
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  1. Congenital Heart Disease

  2. Incidence and Etiology Incidence of 1% in general population. VSD is most common CHD TOF is most common cyanotic CHD TGA is most common cyanotic CHD presenting in infancy Etiology: Multifactorial inheritance 90%. Chromosomal 5% Single mutant gene 3% Environmental 2%

  3. Presentations Asymptomatic heart murmer Cyanosis Congestive heart failure Syncope Shock

  4. CHD in Chromosomal Aberrations Incidence Most common lesion Trisomy 21 50% VSD or A-V canal Trisomy 18 90+% VSD Trisomy 13 90% VSD XO Turner 35% CoA

  5. CHD in Single MutantGene Syndromes Marfan’s Aortic aneurysm Noonan’s PS, ASD Williams’ Supravalvular AS Holt-Oram ASD, VSD NF PS, CoA

  6. Teratogens and CHD Frequency Most common Alcohol 25-30% VSD Phenytoin 2-3% PS,AS,CoA,PDA Lithium 10% Ebstein Rubella 35% PPS, PDA Diabetes 3-5% Hypertrophic septum TGA, VSD, CoA (Incidence can be as high as 30-50% in poorly controlled DM) Lupus 50% 3rd degree heart block PKU 25-50% TOF, VSD, ASD

  7. Presentations Asymptomatic (heart murmur) Small VSD, ASD Cyanosis D-TGA, TOF Congestive heart failure Large L-R shunt lesions Syncope AS, PS Shock Coarc, hypoplastic left heart

  8. Birth 2w 8w 4m 1y 3-5y Adolescence VSD ASD|PDACoA ASHLHSTOFTriA/S PATGATATAPVR CHF P. HTN P. HTN CHF Often asymptomatic Shock CHF/HTN Shock CHF CHF/syncope/murmur Shock Cyanosis CHF Shock/Cyanosis Shock/Cyanosis Cyanosis HLHS=Hypoplastic left heart syndrome TriA/S=Tricuspid atresia CHF=Congestive heart failure P.HTN=Pulmonary hypertension FTT=Failure to thrive Cyanosis/CHF Shock/Cyanosis

  9. Cyanotic CHD 1. Truncus Arteriosus 2. Transposition of the Great Arteries 3. Tricuspid Atresia 4. Tetralogy of Fallot 5. Total Anomalous Pulmonary Venous Return

  10. Acyanotic CHD 1. VSD 2. ASD 3. PDA 4. Coarctation Aorta 5. Aortic Stenosis 6. Hypoplastic Left Heart

  11. Cyanotic CHD with Decreased Pulmonary Blood Flow 1. Tetralogy of Fallot 2. Tricuspid Atresia 3. Total Anomalous Pulmonary Venous Return with obstruction

  12. Cyanotic CHD with Increased Pulmonary Blood Flow 1. Transposition of the Great Arteries 2. Truncus Arteriosus 3. Total Anomalous Venous Return without obstruction

  13. Acyanotic CHD with Increased Pulmonary Blood Flow (Volume Load) 1. ASD 2. VSD 3. PDA

  14. Acyanotic CHD with Pulmonary Venous Congestion or Normal Blood Flow (Pressure Load) 1. Coarctation Aorta 2. Aortic Stenosis 3. Hypoplastic Left Heart 4. Pulmonary Stenosis

  15. Circulation before birth

  16. Circulation after birth

  17. Cyanotic CHD with Decreased Pulmonary Blood Flow 1. Tetralogy of Fallot 2. Tricuspid Atresia

  18. Tetralogy of Fallot 1. VSD 2. Pulmonary artery stenosis 3. Overriding aorta 4. Right ventricular hypertrophy

  19. Incidence of total CHD Age at presentation Clinical Auscultation Most common cyanotic CHD Usually by 6 months Cyanosis Cyanotic spells (squatting) Harsh systolic murmur Softer if worsening obstruction Tetralogy of Fallot

  20. Radiology EKG Decreased pulmonary vascularity Boot-shaped heart R-sided aortic arch RAD, RAE, RVH Tetralogy of Fallot

  21. TOF treatment 1. For cyanotic spells: Knee-chest position Morphine sulfate Vasoconstrictors Propranolol 2. Iron for anemia 3. Surgical a. Palliation Blalock-Taussig Waterston shunt Pott’s operation b. Corrective at 1-5 years of age

  22. Tetralogy of Fallot

  23. Tetralogy of Fallot

  24. Tricuspid Atresia Types 1. Normally related great arteries (69%) With small VSD and PS (most common). Intact septum with pulmonary atresia Large VSD without PS 2. D-transposition of great arteries (28%) 3. L-transposition of great arteries (4%)

  25. Incidence: Age at presentation Clinical No obstruction pulmonary blood flow Obstruction pulmonary blood flow Rare Infancy, depending on pulmonary blood flow Congestive heart failure Similar to VSD Cyanosis Variable More intense cyanosis as ductus closes Tricuspid Atresia

  26. Auscultation: Systolic murmur with single S2 Radiology: Variable Decreased pulmonary vasculature Tricuspid Atresia

  27. Treatment Tricuspid Atresia 1. PGE1 to keep ductus open 2. Balloon septostomy if no VSD 3. Surgical a. Palliation systemic-pulmonary shunt (PS) pulmonary artery banding (large VSD) b. Corrective Fontan

  28. Cyanotic CHD with Increased Pulmonary Blood Flow 1. Truncus Arteriosus 2. Transposition of the Great Arteries 3. Total Anomalous Pulmonary Venous Return

  29. Incidence Age at presentation Clinical Auscultation Pulmonary vasculature EKG Rare Neonatal Cyanosis Signs of CHF Wide pule pressure and bounding arterial pulses Harsh systolic murmur Increased BVH or RVH Truncus Arteriosus

  30. Associations Treatment Right sided aortic arch Thymic aplasia - DiGeorge Syndrome Medical Pulmonary artery bending Rastelli’s operation Truncus Arteriosus

  31. Truncus Arteriosus

  32. Transposition of the Great Vessels D-type D-transposition, complete transposition, most common form -Aorta arises from the right ventricle. -Pulmonary artery arises from the left ventricle. -PDA is the only connection between systemic and pulmonary circulations, although VSD in 40%.

  33. Transposition of the Great Vessels L-type L-transposition, also called corrected transposition -Both ventricles and great vessels are transposed

  34. Incidence Age presentation Clinical Auscultation Radiology EKG 8% of all CHD Male:female 2:1 Newborn, when ductus closes Cyanosis within 1st 48 hrs if no VSD CHF when large left to right shunts Loud single S2, no murmur Egg-on-a-string heart Increased pulmonary vasculature, depending on size shunt RVH D-TGA

  35. Treatment for D-TGA 1. Prostaglandin E 2. Surgical a. Atrial septostomy if no VSD (Rashkind, Blalock - Hanlon etc.) b. Anatomical correction (Jatene’s operation)

  36. TGA

  37. TAPVR types 1. Supracardiac emptying in the left vertical vein (most common type 80-90%) which subsequently drains into the SVC 2. Cardiac emptying into the coronary sinus or right atrium 3.Infradiaphragmatic emptying into vertical vein that descends through diaphragm into portal vein and or IVC

  38. Incidence Age at presentation Clinical findings EKG Radiology 2% Newborn Rapid cyanosis in the infra-diaphragmatic type Non-obstructive similar to ASD plus mild cyanosis RVH “Snowman” configuration Diffuse reticular opacities Looks like HMD without air bronchograms! TAPVR

  39. Associations Treatment Polysplenia Asplenia (3/4 patients also TAPVR) Surgical ligation of anomalous vein TAPVR

  40. Total anomalous venous return

  41. Acyanotic CHD with Increased Pulmonary Blood Flow (left to right shunt lesions) 1. ASD 2. VSD 3. PDA

  42. Incidence Types Age presentation Clinical Auscultation EKG Treatment 10% CHD Ostium secundum (most common) Sinus venosus defect Ostium primum (AV canal) Varies Mostly asymptomatic Slender body build Widely split and fixed S2! + SEM RAD and RVH No SBE coverage needed! Surgery for large shunts ASD

  43. ASD