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

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Presentation on theme: "Cyanotic Congenital Heart Disease Dr David Coleman Consultant Paediatric Cardiologist Our Lady’s Children’s Hospital, Crumlin Dublin."— Presentation transcript:

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: egcritical PS/pulmonary atresia tricuspid atresia ♥ Intracardiac mixing: egTAPVR 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)

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8 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

9 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’

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

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

13 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

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

17 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)

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

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


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