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Cyanotic Congenital Heart Disease

Cyanotic Congenital Heart Disease

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Cyanotic Congenital Heart Disease

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  1. Cyanotic Congenital Heart Disease Dr David Coleman Consultant Paediatric Cardiologist Our Lady’s Children’s Hospital, Crumlin Dublin

  2. Cyanotic Lesions The T’s: ♥Transposition of the great arteries (TGA) ♥Tetralogy of Fallot (±pulmonary atresia) ♥ Tricuspid atresia ♥ Total anomalous pulmonary venous return (TAPVR)

  3. Other Cyanotic Lesions ♥Critical PS ♥Hypoplastic left heart syndrome (HLHS)

  4. Common Causes of Cyanosis ♥ Reduced pulmonary blood flow: eg critical PS/pulmonary atresia tricuspid atresia ♥Intracardiac mixing: eg TAPVR double inlet left ventricle

  5. Complications of Persistent Cyanosis include: ♥polycythaemia ♥relative anaemia ♥CNS abscess ♥thromboembolic stroke ♥ clubbing ♥ infection ♥ poor growth

  6. TGA ♥ Parallel circulations ♥ Can mix at 3 levels: PDA PFO/ASD VSD if present ♥Life threatening cyanosis as neonate ♥Exam: single S2 (anterior aorta) often no murmur (esp if no VSD)

  7. TGA ♥ Treatment: Acute: PGE infusion to keep PDA open Balloon atrioseptostomy Surgical: Arterial Switch operation ♥Atrial switch operation (Mustard, Senning) was performed before Arterial Switch operation became available

  8. Tetralogy of Fallot ♥ Most common form of cyanotic CHD (8-10% CHD) ♥ 4 cardinal features: VSD (usually large), overriding aorta, subpulmonary stenosis, RVH ♥ Can be pink initially (‘pink tet’) and have CHF, but develop increasing cyanosis over months ♥May develop ‘cyanotic spells’

  9. Tetralogy of Fallot ♥ Exam: pink or cyanosis to some degree ±finger clubbing loud ESM along LSE single ± loud S2 ♥ ECG: RAD, RVH

  10. Tetralogy of Fallot ♥ CXR: normal heart size pulmonary oligaemia deficient MPA segment ‘boot shaped heart’ ± right aortic arch (~25%) ♥ Treatment: surgical repair 1st yr of life (occasionally shunt initially)

  11. Pulmonary Atresia ♥ Atretic pulmonary valve, hypoplastic RV, ±VSD ♥Progressive cyanosis as PDA closes ♥Exam: single S2 ± systolic murmur ♥ ECG: RAD, RVH ♥ CXR: cardiomegaly (if collaterals+) absent MPA segment

  12. Pulmonary Atresia ♥ Treatment: shunt pulmonary valvuloplasty Fontan operation ♥ Mixed prognosis

  13. HLHS ♥ Underdeveloped left heart: hypoplastic or atretic mitral valve small LV hypoplastic or atretic aortic valve small ascending aorta CoA ♥Systemic flow via PDA (right-to-left) ♥May present with cardiovascular collapse when PDA closes (hypoxia, acidosis, death)

  14. HLHS ♥Exam: ‘ashen’ colour (low CO) cyanotic weak/no brachial & femoral pulses single S2 often no murmur ♥ ECG: RVH ♥ CXR: ± cardiomegaly ± pulmonary plethora

  15. HLHS ♥Treatment: palliative care or Norwood procedure/bidirectional Glenn anastomosis/Fontan procedure or ?cardiac transplant ♥ Fetal diagnosis