Pulmonary stenosis+ VSD+overriding aorta+RVH
Cyanotic congenital heart disease (The 5 Ts. others are TGA, Truncus arteriosus, Total anomalous pulm venous return, Tricuspid atresia)
Right to left shunt leads to mixing
May not be cyanotic at birth
Difficulty feeding, polycythaemia, finger clubbing, dyspnoea, FTT, murmur
sudden, marked increase in cyanosis followed by syncope ,often precipitated by crying, feeding or exercise , may also occur on waking up.
It is due to an increase in rt to left shunting of unoxygenated blood following a decrease in systemic vascular resistance or increased pulmonary vascular resistance, favouring the "right to left to aorta shunt"
It can be relieved by squatting or placing child in knee chest position (This increases afterload thus allowing more blood to go thru pulmonary circulation rather than systemic)
Chest x ray shows boot shaped heart and decreased pulmonary vascular markings.