EDUCATION IN HEART
Congenital heart disease
Pulmonary atresia with intact ventricular septum: from fetus to adult
Brompton Paediatric and Fetal Cardiology, Royal Brompton Hospital, London, UK
Correspondence to:
Dr Elliot A Shinebourne, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK; e.shinebourne@rbht.nhs.uk
| The first 150 words of the full text of this article appear below. |
Fascination with pulmonary atresia and intact ventricular septum (PAIVS) reflects its diversity and evolving management strategies. We will discuss PAIVS with situs solitus and concordant atrioventricular and ventriculoarterial connections.
Usually the cavity of the right ventricle (RV) is small (hypoplastic), the RV myocardium is thickened, and the RV pressure is at supra systemic levels. In a minority with severe tricuspid regurgitation the RV is dilated and thin walled and postnatally the RV pressure is low. Also contributing to the morphological heterogeneity are the size and structure of the tricuspid valve (TV), whether RV outflow tract obstruction (RVOTO) is muscular or valvar, and the presence or absence of coronary artery abnormalities. Postnatal survival initially is dependent on persistence of the arterial duct. Management strategies are influenced by the nature of the RVOTO and whether the RV is considered suitable for eventual inclusion in the circulation. Should there be
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