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TOF, VSD in children

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TOF, VSD in children. MBBS lecture

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TOF, VSD in children

  1. 1. Congenital Heart Disease Dr. Muhammad Sajjad Sabir MBBS, DCH, MCPS, FCPS Assistant Professor of Paediatrics
  2. 2. VSD ASD PDA Tetralogy of Fallot(TOF) Transposition of great arties(TGA) Ebstein anomaly Hypoplastic left heart syndrome Total anomalous pulmonary venous return (TAPVR) Aortic Stenosis Pulm. Stenosis Coarctation of aorta
  3. 3. Tetralogy of Fallot
  4. 4. • Tetralogy of Fallot (TOF) is common cyanotic congenital heart disorders (CHD) • Tetralogy of Fallot results in an inadequate flow of blood to lungs for oxygenation (right-to-left shunt) • Patients with Tetralogy of Fallot initially present with cyanosis
  5. 5. ToF Four anatomic malformations: A- PulmonaryValve Stenosis B- Over riding of aorta C-Ventricular Septal Defect D- RightVentricular Hypertrophy
  6. 6. Clinical Presentation Birth weight is low Clinical presentation is directly related to the degree of pulmonary stenosis Severe stenosis results in immediate cyanosis following birth Mild stenosis will not present until later Growth is retarded – insufficient oxygen and nutrients Development and puberty may be delayed
  7. 7. Poor feeding Cyanosis Cyanosis during feeding Anoxic spells Dyspnea on exertion Tachypnea Exercise intolerance Fussiness and agitation Clinical Presentation
  8. 8. Clinical Presentation • “Tet spells” at 2-3 yrs • Paroxysmal attacks of dyspnea • Anoxic spells • Predominantly after waking up • child becomes distressed and inconsolable, without apparent reason “Tet Spell”
  9. 9. • Child cries→ Dyspnea (hyperpnea not tachypnea)→ progressively deeper Cyanosis → Loss of consciousness → Convulsion (may experience syncope) • During the spell  diminished/absent murmur • Frequency once a few days to many attack everyday “Tet Spell”
  10. 10. • Sitting posture – squatting • Compensatory mechanism • Squatting ↑ses peripheral (systemic) vascular resistance • Which diminishes right-to-left shunt • Increases pulmonary blood flow • Increases oxygenation of blood “Tet Spell”
  11. 11. Physical Examination • Clubbing + Cyanosis (Variable) • Squatting position • bulging left hemithorax • Prominent “a” waves JVP • Normal heart size • Mild parasternal impulse • Systolic trill (30%)
  12. 12. Investigations • CBC - hematocrit↑ • ECG -RVH, RAD • Echocardiogram A- PulmonaryValve Stenosis B- Over riding of aorta C-V S D D- RightVentricular Hypertrophy
  13. 13. CXR •boot-shaped heart secondary to uplifting of the cardiac apex from RVH • Decreased pulmonary vascularity • Right atrial enlargement • Right-sided aortic arch (20-25% of patients)
  14. 14. Course and Complication • Each anoxic spell is potentially fatal • Polycytemia • Cerebral thrombosis • Cerebral abcess • Seizures • Hypoxic damage • Infective Endocarditis & vegetations • Postoperative strokes
  15. 15. MANAGEMENT
  16. 16. Management of anoxic spell 1)Calm the baby 2) increase SVR 3)Knee chest position 4)Humidified O2 5)Morphine 0.1 -0.2 mg/Kg Subcutaneous 6)Correct acidosis – Sodium Bicarb IV 6)Inj Phenyelphrine 7)Propranolol 0.1mg/kg/IV during spells 0.5 to 1.0 mg/kg/ 4-6hourly orally 7)Vasopressors 8)Correct anemia 9) PHELABOTOMY if polycythemia
  17. 17. Blalock Taussig Shunt • Classic BT shunt 1945… • Lt Subclavian artery – Lt Pulmonary artery anastomosis
  18. 18. Modified Blalock Taussig Shunt Rt Subclavian artery – Rt Pulmonary artery anastomosis
  19. 19. Potts Shunt Side - side anastomosis of Lt Pulmonary Artery to Descending Aorta
  20. 20. Waterston shunt Side-side anastomosis of Rt Pulmonary Artery to Ascending Aorta
  21. 21. Palliative Procedures
  22. 22. Surgical Intervention Complete intracardiac repair • Repair theVSD with a patch  Transcatheter patches  Open repair • Repair of Pulm stenosis  either PA removed or  removing the excessive muscle tissue of PA
  23. 23. Complications: •Infective bacterial endocarditis •pulmonic regurgitation •Arrhythmias •RBBB •Left anterior hemiblock
  25. 25. VENTRICULAR SEPTAL DEFECTVENTRICULAR SEPTAL DEFECT • most common ACHD • 2nd most common CHD(32%) • SYNONYMS * Roger’s disease * Interventricular septal defect
  26. 26. PATHOPHYSIOLOGYPATHOPHYSIOLOGY • Primarily depends on ----size ofVSD ----status of pulm. vascular bed (rather than location) • Small communication (less than 0.5cm`)VSD is restrictive & rt.ventricular pressure is normal – does not cause significant hemodynamic derangement (Qp:Qs =1.75:1.0) • Moderately restrictive VSD with a moderate shunt(Qp:Qs =1.5-2.5:1.0) &poses hemodynamic burden on LV • Large nonrestrictive VSDs(more than 1.0cm`) Rt & Lt ventricular pressure are equalised (Qp:Qs is >2:1)
  27. 27. PATHOPHYSIOLOGYPATHOPHYSIOLOGY • LargeVSDs at birth ,PVR may remain higher than normal and Lt to Rt shunt may intially limited – involution of media of small pulm.arterioles,PVR decreases—large Lt to Rt shunt ensues • In some infants largeVSDs ,pulm. arteriolar thickness never decreases –pulm. obstructive disease develops .when Qp:Qs=1:1 shunt becomes bidirectional,signs of heart failure abate &pt. becomes cyanotic. (Eisenmenger syndrome)
  28. 28. ANATOMICAL CLASSIFICATION MEMBRANOUS SEPTUM paramembranous/perimembranous defect (or infracristal, subaortic, conoventricular) MUSCULAR SEPTUM inlet, trabecular, central, apical, marginal or swiss-cheese OUTLET SEPTUM deficient supracristal,subpulmonary,infundibular or conoseptal SEPTAL DEFICIFNCY AVseptal defect (AVcanal)
  29. 29. CLINICAL FEATURESCLINICAL FEATURES • Race : no particular racial predilection • Sex :no particular sex preference • Age :infantsinfants– difficult in postnatal period,although ccf during first 6mths is frequent,X-ray&ECG are normal. childrenchildren—after first year variable clinical picture emerges. smallVSD – asymptomatic largeVSD – symptomatic
  30. 30. Common Symptoms of largeVSD • Palpitation • Breathing dificulty • Dyspnoea on exertion • Feeding difficulties • Poor growth • Frequent chest infections
  31. 31. PHYSICAL FINDINGSPHYSICAL FINDINGS • Pulse pressure - wide • hyperkinetic Precordium with systolic thrill LSB • S1&S2 are masked by a PSM at Lt. sternal border • Max. intensity of the murmur is best heard at 3rd ,4th & 5th Lt intercostal space • Lt. 2nd space –widely split & accentuated P2 • Maladie de RogerMaladie de Roger – smallVSD presenting in older children as a loud PSM w/o other significant hemodynamic changes
  32. 32. INVESTIGATIONSINVESTIGATIONS • ECHOCARDIOGRAPHYECHOCARDIOGRAPHY two-dimensional & doppler colour flow • CHEST RADIOGRAPHYCHEST RADIOGRAPHY - normal - biventricular hypertrophy - pulmonary plethora • ANGIOGRAPHY (cardiac catheterization and angiography)
  33. 33. ECG Small restrictive VSDs Normal ECG Medium-sized VSDs Lt atrial overload- Broad, notched P wave Signs of LV volume overload — deep Q and tall R waves with tall T waves in leads V5 and V6 Atrial fibrillation Large VSDs Rt ventr hypertrophy - right-axis deviation Biventricular hypertrophy - P waves notched or peaked
  34. 34. COMPLICATIONSCOMPLICATIONS • Congestive cardiac failure • Infective endocarditis on Rt. ventricular side • Aortic insufficiency • Complete heart block • Delayed growth & development (FTT) in infancy • Damage to electrical conduction system during surgery (causing arrythmias) • Pulmonary hypertension
  35. 35. INTERVENTIONINTERVENTION 3 MAJOR TYPES • SMALLSMALL ((surface area < 0.5surface area < 0.5 cm2 or <1/3or <1/3rdrd of Aortic root size)of Aortic root size) - hemodynamically insignificant - b/w 80-85% of allVSDs - all close spontaneously * 50% by 2yrs * 90% by 6yrs * 10% during school yrs - muscular close sooner than membranous
  36. 36. • MODERATE VSDsMODERATE VSDs * surface areasurface area 0.5-1cm2 or <1/2 of<1/2 of Aortic root sizeAortic root size * least common group of children(3- 5%) * w/o evidence of ccf/ pulm.htn can be followed until spontaneous closure occurs.
  37. 37. • LARGE VSDs WITH NORMAL PVRLARGE VSDs WITH NORMAL PVR * surface areasurface area >1 cm2 or ≥ of Aortic root≥ of Aortic root sizesize * usually requires surgery otherwise… develop CCF & FTT by age of 3-6mths. Conservative treatment - Treat CCF & prevent development of pulm. vascular disease - prevention & treatment of infective endocarditis
  38. 38. INDICATIONS for SURGERYINDICATIONS for SURGERY • VSDs at any age where clinical symptoms and FTT cannot be controlled medically. • Infants b/w 6-12mths of age with large defects ass. with PH ,even if symptoms are controlled by medication. • Pt.s older than 24mths of age with Qp:Qs is greater than 2:1. • Pt.s with supracristalVSD of any size, because of high risk of development of AI. CONTRAINDICATIONCONTRAINDICATION –severe pulmonary vascular disease.
  39. 39. Surgical correction has to be done before irreversible damage to pulmonary vasculature occurs
  40. 40. Operative procedureOperative procedure
  41. 41. Patch closure by RV approach
  42. 42. Percutaneous Device Closure • MuscularVSDs can typically be closed percutaneously
  43. 43. Much more to comeMuch more to come Are we all still awake?