Pulmonary atresia with intact ventricular septum

Definition:

Pulmonary atresia with intact ventricular septum (PAIVS) is a severe cardiac malformation characterized by variable right ventricular development and imperforate pulmonary valve. PAIVS has a wide spectrum of clinical presentation ranging from cyanosis to shock due to closure of ductus arteriosus. The treatment follows initial stabilization with prostaglandin and establishment of antegrade flow. Because of the morphologic heterogeneity, repair or palliation strategies vary widely, ranging from single ventricle palliation to complete biventricular repair. Overall, regardless of anatomical subtype, survival is poor, reported to be as low as 50% at 5 years.

References:

[1]. Mazeni Alwi, et al. Pulmonary atresia with intact septum: the use of Conquest Pro coronary guidewire for perforation of atretic valve and subsequent interventions. Cardiol Young. 2013 Apr;23(2):197-202. [Content Brief]

[2]. R Iacobelli, et al. Role of tricuspid regurgitation in fetal echocardiographic diagnosis of pulmonary atresia with intact ventricular septum. Ultrasound Obstet Gynecol. 2008 Jul;32(1):31-5. [Content Brief]

[3]. Shweta Bakhru, et al. Transcatheter pulmonary valve perforation using chronic total occlusion wire in pulmonary atresia with intact ventricular septum. Ann Pediatr Cardiol. 2017 Jan-Apr;10(1):5-10. [Content Brief]

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