Hypo-plastic left heart syndrome

It's characterized by:

1- Under- development of the left cardiac chambers, with atresia of the mitral and aortic valves.

2- Hypoplasia of the aortic artery.

3- Patent foramen ovale between both RA and LA.

4- Flow of oxygenated blood is maintained through the patent formane ovale and the patent ductus arteriosus, which would be closed upon birth, so early diagnosis and administration Prostglandin E2 is essential.

5- As O2 is administered, dilation of the pulmonary artery occurs resulting in low pressure circuit that favors the flow of the blood towards it; therefore it would lead to further oxygenation of the blood.

6- The work right ventricle would remarkably increase, as it's the main pumping source of blood towards both the right and left ventricle and that would result in precordial hyperactivity, right ventricular enlargement (detected on X-ray- ECG, Rt axis deviation)

Also the increased flow of blood would result in accentuated 2nd heart sound and increased pulmonary end systolic pressure.

Management :

through administration of PGE2 to maintain the patency of the ductus arteriosus.

Also, skeletal muscle relaxants in order to prevent hyperventilation, and therefore increasing the pressure through the pulmonary circuit that favors transmission of blood through PDA and foramen ovale towards the systemic circulation