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)

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’

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 1st 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

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)

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
?cardiac transplant
♥ Fetal diagnosis