Pulmonary atresia with intact ventricular septum: from fetus to adult

Long-term results of catheter-based treatment of pulmonary atresia and intact ventricular septum

“…One ventricle repair could also be the surgical solution for cases of PAIVS associated with severe Ebstein’s anomaly 15. Different opinions exist about the treatment options of the form of PAIVS in which right ventricle has a “borderline” size 9 10 11…”

“…In patients with patent infundibulum and mild right ventricle hypoplasia, early surgical or catheter-based right ventricle decompression is advocated in order to increase the rate of long-term two-ventricle repairs 3 4 5 6 7 8. More uncertainty and different opinions exist about treatment options of that form of PAIVS with moderately severe right ventricle hypoplasia but a reasonable developed infundibulum (“intermediate form”) 9 10 11…”

Prenatal detection of congenital heart disease—results of a national screening programme

“…5,14,29,30 Hypoplastic right heart syndrome is more frequently overlooked (prenatal detection rate 66.7%), as it may present with a relatively good right ventricular cavity around 20 weeks of gestation. 31 Significant improvements were made in the prenatal detection of several (ductal dependent) outflow tract and aortic arch anomalies that require examination of the outlet views in addition to the four-chamber view. The prenatal detection of valve abnormalities in biventricular hearts and pulmonary or systemic venous return anomalies has remained relatively low.…”

327: Prenatal tricuspid valve size as a predictor of postnatal outcome in patients with severe pulmonary stenosis or pulmonary atresia with intact ventricular septum

“…The majority of both PA/IVS and SPS have no known underlying genetic/chromosomal cause or affiliation with any other major congenital malformations [1] . Depending on in utero RV growth in the face of this obstruction, SPS and PA/IVS may exhibit a spectrum of severity, requiring postnatal interventions including balloon pulmonary valvuloplasty alone, surgical biventricular repair, initial palliation with shunt and bidirectional Glenn with RV outflow tract reconstruction (so-called '1.5 ventricle repair'), or staged single-ventricle palliation.…”