2017
DOI: 10.1177/1089253217737180
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Perioperative and Anesthetic Considerations in Pulmonary Atresia With Intact Ventricular Septum

Abstract: Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare right-heart obstructive lesion with a wide anatomic and physiologic spectrum of disease, ranging from simple membranous pulmonary valve atresia with a fully developed right ventricle (RV) to a severely hypoplastic RV and ventriculocoronary (RV-coronary) fistulas. Affected neonates are dependent on prostaglandin for adequate pulmonary blood flow. Depending on the severity of disease, treatment options range from transcatheter pulmonary valve pe… Show more

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Cited by 11 publications
(3 citation statements)
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“…Newborns with PA/IVS and CPS depend on PDA to obtain su cient PBF, requiring PGE1 to maintain PDA patency. Identifying RVDCC through TTE and CT before surgery is very important and affects the next treatment plan and surgical decision [11]. Patients with wider PDA and unrestricted atrial shunting can be placed in regular wards with continuous or discontinuous low-dose PGE1 treatment depending on the condition.…”
Section: Discussionmentioning
confidence: 99%
“…Newborns with PA/IVS and CPS depend on PDA to obtain su cient PBF, requiring PGE1 to maintain PDA patency. Identifying RVDCC through TTE and CT before surgery is very important and affects the next treatment plan and surgical decision [11]. Patients with wider PDA and unrestricted atrial shunting can be placed in regular wards with continuous or discontinuous low-dose PGE1 treatment depending on the condition.…”
Section: Discussionmentioning
confidence: 99%
“…Thus, the choice of management algorithm hinges on accurate characterization of the coronary artery anatomy. Thus, PA-IVS patients frequently undergo invasive angiography with aortic root and direct RV injection [ 58 ]. In the case of PA-IVS presented, 4D FE-MRA done on day 2 of life confirmed suspicion of right coronary ostial atresia with origin of the right coronary directly from the apex of the RV ( Figure 12 ).…”
Section: Ferumoxytol-enhanced Mra (Fe-mra) and 4d Phase Contrast Mri ...mentioning
confidence: 99%
“…Chikkabyrappa and colleagues describe the perioperative physiology, imaging and management of pulmonary atresia with an intact ventricular septum (PA-IVS) with an accompanying article by Gleich and colleagues describing the perioperative anesthetic considerations. 4,5 Together, these 2 articles describe this rare, complex CHD, which has a wide anatomic and physiologic spectrum. Chikkabyrappa and colleagues show an excellent pathology specimen of PA-IVS as well as wonderful echocardiographic and angiography images.…”
mentioning
confidence: 99%