Pulmonary atresia with intact ventricular septum
Range of morphology in a population-based study
Piers E. F. Daubeney, MRCP* ,*,
David J. Delany, FRCR*,
Robert H. Anderson, MD ,
George G. S. Sandor, MD ,
Zdenek Slavik, MD*,,
Barry R. Keeton, FRCP*,
Steven A. Webber, MRCP*¶ for the United Kingdom and Ireland Collaborative Study of Pulmonary Atresia with Intact Ventricular Septum
* Wessex Cardiothoracic Centre, Southampton General Hospital, Southampton, United Kingdom
Royal Brompton and Harefield Hospitals, London, United Kingdom
British Columbia Children’s Hospital, Vancouver, Canada
Institute of Child Health, University College, London, United Kingdom
¶ Children’s Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA
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Figure 1 Nomograms of tricuspid valve (TV) (A) and right ventricular (RV) inlet (B). This allows estimation of the TV and RV inlet Z scores from knowledge of the TV or RV inlet dimension and body surface area. Reproduced with kind permission from Cardiology in the Young (5).
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Figure 2 Cumulative frequency distribution of the Z-score of the tricuspid valve (TV) diameter measured by echocardiography. The Z-scores are based on the normal data of Daubeney et al. (5) (shown in Fig. 1).
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Figure 3 Spectrum of pathology in pulmonary atresia with intact ventricular septum. Right ventricular (RV) angiograms showing (A) dilated thin-walled RV and right atrium (RA) with severe tricuspid regurgitation and membranous pulmonary atresia (anteroposterior view); (B) so-called tripartite RV with membranous atresia (lateral view); (C) so-called bipartite RV with membranous atresia, with some RV-to-coronary fistulae (lateral view); (D) tiny so-called unipartite RV with muscular atresia and RV-to-coronary fistulae with retrograde filling of the aorta (lateral view).
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Figure 4 Cumulative frequency distribution of the Z-score of the right ventricular inlet length measured by echocardiography. The Z-scores are based on the normal data of Daubeney et al. (5) (shown in Fig. 1).
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Figure 5 Aortic root showing an (A) atretic right and an (B) absent left coronary artery ostium. There is also a bicuspid aortic valve.
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Figure 6 Ductal angle in pulmonary atresia with intact ventricular septum. (A) Echocardiogram showing normal angle of duct to descending aorta (DAO) (>90°) in a patient with a tripartite right ventricular (RV) and membranous atresia; (B) abnormal acute angled duct (<90°) in a patient with a so-called "unipartite" RV and muscular atresia. AO = aorta; LA = left atrium.
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