Cyanotic heart disease paed + CASE

(marrow)

Cyanotic Congenital Heart Disease (CCHD)

Peripheral vs central cyanosis

(cardiac lesion mostly central so dont panic with newborn peripheral)

Central Cyanosis

Chronic cyanosis causes clubbing of fingers & toes (if cyanosed look for clubbing) [seperate card for clubbing]

Causes for cyanosis

respiratory diseases

heart disease

cerebral causes - hypoxia

methhaemoglobinaemia

Evaluation of cyanosis: Hyperoxia test

Cyanotic Congenital Heart DIseases (5Ts)

Tetralogy of Fallot

Transposition of great arteries TGA

Tricuspid atresia

Truncus arteriosus

Total anomalous pulmonary venous drainage

any to investigation - CXR. ECG, Echo

Tetralogy of Fallot (commonest)

Clinical features - symptoms

Majority are diagnosed in the first month or two of life with the finding of a murmur & cyanosis

Classical description of severe cyanosis, Hypercyanotic spell (pathophysiology?) , Squatting on exertion (pathophysiology?) (older?)

Clinical features - signs

Cyanosis

• Clubbing of fingers and toes

Ejection systolic murmur in the 2nd left inter-costal space (PS not VSD coz large)

Single 2nd heart sound

Investigations

left ventricle is pushed up by RVH

in cardiac lesions - comment on both cardiac contour and lung fields

Hypercyanotic spell

Severe bouts of cyanosis , hyperventilation, bradycardia ± LOC

Murmur may disappear during spells

Life-threatening if untreated

squatting is like knee chest position

Management

OTHER CLINICAL SCENARIO

CCHD presenting within first week of life

Transposition of great arteries TGA (commonest)

Tricuspid atresia

Truncus arteriosus

Total anomalous pulmonary venous drainage

TGA

(it's like two different systems)

Initial management of TGA

Goal is to improve mixing 1. Start PGE1 to prevent ductal closure 2. Open atrial septum to improve mixing at atrial level (Rashkind procedure)

Truncus arteriosus

Tricuspid atresia

Total anomalous pulmoanry venous drainage (TAPVD)

Pulmonary veins drains to SVC/IVC

Supra cardiac

Infra cardiac

...

Complications of CCHD

Polycythaemia- Polycythaemia –

– Prevent dehydration

– Phlebotomy if PVC > 65%

Transient Iscahemic Episodes (TIE)

Hemiplegia

Cerebral abscess

Eisenmenger Syndrome

Clubbing

Do slideshare

https://www.slideshare.net/draswinikumars/clubbing

Cyanotic heart disease paed + CASE

Casebook