MORPHOFUNCTIONAL FEATURES OF PULMONARY ARTERY ATRESIA WITH INTACT INTERVENTRICULAR SEPTUM AND METHODS FOR VISUALIZING DEFECTS. REVIEW ARTICLE

Highlights

• Pulmonary artery atresia with intact interventricular septum is a rare congenital heart defect that depends on the ductus.

Resume

Pulmonary artery atresia with intact interventricular septum is a rare and heterogeneous congenital heart disease, belonging to the group of so-called “blue” defects. The anatomical and physiological spectrum of the defect is variable with varying degrees of tricuspid valve and right ventricular hypoplasia, from a simple membranous form with a well-developed RV to severe pancreatic hypoplasia with myocardial sinusoids and right ventricular-coronary dependent blood flow. Transthoracic echocardiography is considered the “gold standard” for diagnosis, while additional imaging techniques like computed tomography and cardiac catheterization can be used to further characterize the structural features of this anomaly. In this review, we present an overview of the morphology and physiology of right-sided heart chambers in patients with pulmonary artery atresia with an intact interventricle septum.

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