Paediatrics: Acyanotic: congenital heart disease

Acyanotic: congenital heart disease

Definition

Failure of normal cardiac development or persistence of the foetal circulation after birth.

Incidence

·  8/1000 live births.

·  10–15% complex lesions with >1 abnormality.

·  10–15% of CHD also have a non-cardiac abnormality.

Causes

This is unknown in the majority of cases, but commonly associated with following conditions:

·  Chromosomal defects: e.g. Down, Turner syndromes.

·  Gene defects: e.g. 22q deletion, Noonan syndrome

·  Congenital infections: e.g. rubella.

·  Teratogenic drugs: e.g. phenytoin, warfarin, alcohol.

Classification

CHD can be classified into acyanotic or cyanotic types depending on whether predominant presentation is with or without central cyanosis. The latter is caused by deoxygenated blood gaining abnormal access to systemic side of the circulation via the left side of the heart or the aorta.

Acyanotic CHD

·  VSD.

·  ASD.

·  PDA.

·  Pulmonary valve stenosis.

·  Coarctation of the aorta.

·  Aortic stenosis.

·  Hypoplastic left heart syndrome.

·  Hypertrohpic obstructive cardiomyopathy.

·  Dextrocardia.

Cyanotic CHD

·  Tetralogy of Fallot.

·  Transposition of the great arteries.

·  Tricuspid atresia.

·  Total anomalous pulmonary drainage.

The diagnosis of a specific lesion is made after clinical examination; CXR, ECG, and echocardiography.