Cyanotic Congenital Heart Disease

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Cyanotic Congenital Heart Disease
Dr David Coleman Consultant Paediatric
Cardiologist Our Ladys Childrens Hospital,
Crumlin Dublin
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Cyanotic Lesions

• The Ts
• ? Transposition of the great arteries (TGA)
• ? Tetralogy of Fallot ( pulmonary atresia)
• ? Tricuspid atresia
• ? Total anomalous pulmonary venous return (TAPVR)

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Other Cyanotic Lesions

• ? Critical PS
• ? Hypoplastic left heart syndrome (HLHS)

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Common Causes of Cyanosis

• ? Reduced pulmonary blood flow
• eg critical PS/pulmonary atresia
• tricuspid atresia
• ? Intracardiac mixing
• eg TAPVR
• double inlet left ventricle

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Complications of Persistent Cyanosis

• include
• ? polycythaemia
• ? relative anaemia
• ? CNS abscess
• ? thromboembolic stroke
• ? clubbing
• ? infection
• ? poor growth

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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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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

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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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Tetralogy of Fallot

• ? Exam pink or cyanosis to some degree
• finger clubbing
• loud ESM along LSE
• single loud S2
• ? ECG RAD, RVH

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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)

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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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Pulmonary Atresia

• ? Treatment shunt
• pulmonary valvuloplasty
• Fontan operation
• ? Mixed prognosis

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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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HLHS

• ? Exam ashen colour (low CO)
• cyanotic
• weak/no brachial femoral pulses
• single S2
• often no murmur
• ? ECG RVH
• ? CXR cardiomegaly
• pulmonary plethora

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HLHS

• ? Treatment
• palliative care
• or
• Norwood procedure/bidirectional Glenn
  anastomosis/Fontan procedure
• or
• ?cardiac transplant
• ? Fetal diagnosis