Pulmonary Atresia Intact Ventricular Septum

Pulmonary atresia with intact ventricular septum (PAIVS) is a complex congenital heart defect characterized by pulmonary valvular atresia. As such, the colour box presented below does not show any passage of blood flow through the valve. The disease is PGE dependent - ductal dependent - to allow adequate pulmonary circulation. Patients may have various presentations, with myocardial sinusoids complicating the course of their management. More on PAIVS here.

Subcostal view showing a right to left inter-atrial shunt in PAIVS.

Apical 4 chamber view demonstrating a hypoplastic right ventricle (very muscular), in the context of PAIVS.

Supra-sternal view showing a left to right tortuous duct in a patient with PAIVS.

PAIVS presentation prepared by Laila Wazneh (NNP) and Dr Shiran S Moore (NHCR fellow) at Montreal Children's Hospital


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