Heart diseases detected on second day of life

Valvular stenotic lesions or regurgitations
Coarctation of aorta
Cyanotic congenital heart disease

Breath holding spell

Breath holding spell has a precipiating factor which causes the child to cry incessantly and this is followed by breath holding. Breath holding does not occur abruptly. Child may become cyanosed after prolonged breath holding which may also lead on to seizures.

Williams syndrome

Williams syndrome is characterised by supravalvar aortic stenosis, elfin facies and hypercalcemia. Learning disability is often associated. Peripheral pulmonary stenosis is also an association. In supravalvar aortic stenosis, the right upper limb blood pressure can be more than that in left upper limb (anisopsphygmia) because the jet is directed towards the brachiocephalic artery. This is known as Coanda effect.

Differentiation of supravalvar aortic stenosis from valvar and subvalvular variety

Murmur of subvalvular aortic stenosis is unlikely to radiate to the carotids. Supravalvar aortic stenosis may radiate more to the right carotid. Ejection click is in favour valvular aortic stenosis. Supravalvar aortic stenosis is unlikely to be associated with aortic regurgitation, while it can occur in valvar and subvalvular variety.

Biventricular outflow obstruction in a child

Noonan syndrome can cause biventricular outflow obstruction due to hypertrophic cardiomyopathy and pulmonary stenosis.

Structural heart diseases associated with WPW syndrome

Ebstein’s anomaly, hypertrophic cardiomyopathy, mitral valve prolapse

Causes of dynamic left ventricular outflow tract (LVOT) obstruction

Hypertrophic cardiomyopathy is the most important cause of dynamic LVOT obstruction. In transposition of great arteries (TGA) with intact interventricular septum LVOT obstruction due to systolic anterior motion of mitral valve (SAM) is possible. SAM can also occur transiently following surgery for aortic stenosis and mitral valve repair and cause LVOT obstruction. Dynamic LVOT obstruction is also described as a rare association with myocardial infarction possibly due to SAM. Dobutamine can worsen the LVOT obstruction in this situation.