Pulmonary atresia with intact ventricular septum.

The pulmonary valve leaflets are completely fused and form a membrane between right ventricle and pulmonary artery.

-Fig. 6.4
Pulmonary atresia with intact ventricular septum. All the blood tha reach right atrium flows through an atrial septal defect in left atrium then in left ventricle and aorta which maintains also pulmonary circulation through the ductus arteriosus-.

There is no egress of blood from the right ventricle, right atrial pressure increases and blood shunts via the foramen ovale into left atrium where it mixes with pulmonary veins blood and enters left ventricle. The only source of pulmonary blood occurs through the ductus arteriosus. This heart disease is ductus dependent pulmonary circulation heart disease. As the ductus will close the situation can become dramatic within hours or days. To avoid this evolution, soon after the diagnosis prostaglandin a given intravenously to maintain the ductus open and allow a relatively safe surgery.

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