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

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

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

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

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

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)

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

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’

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

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)

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

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

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)

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

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