CLINICAL STUDY: PEDIATRIC CARDIOLOGY
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
Manuscript received April 26, 2001;
revised manuscript received February 13, 2002,
accepted February 20, 2002.
* Reprint requests and correspondence: Dr. Piers Daubeney, Consultant Paediatric and Fetal Cardiologist, Royal Brompton Hospital, Sydney St., London SW3 6NP, United Kingdom.
p.daubeney{at}rbh.nthames.nhs.uk
OBJECTIVES: We describe the morphologic variability in pulmonary atresia with intact ventricular septum (PAIVS) within a population-based study.
BACKGROUND: An uncommon disease, PAIVS shows considerable morphologic heterogeneity. Clinical reports, based mostly on small samples of patients, may not reflect the true spectrum of pathology of this condition. We have studied the entire range of morphology in a prospective population-based study of patients over a five-year period (1991 to 1995).
METHODS: As part of the United Kingdom and Ireland Collaborative Study of PAIVS, all 18 pediatric cardiac centers were visited by a single investigator. Morphologic features of each case were determined by direct review of the echocardiograms and angiocardiograms, from surgical and autopsy reports, and by review of pathology specimens where available.
RESULTS: Among 183 live-born infants, atresia was valvar (membranous) in 74.7% and muscular in 25.3%. Muscular obliteration of the apical trabecular cavity, and in some cases its infundibulum, resulted in "bipartite" right ventricle (RV) in 33.6%, and a "unipartite" chamber in 7.7%. The remaining 58.7% had "tripartite" morphology. Coronary arterial abnormalities were identified in 45.8%, including arterial stenoses, interruptions and ectasia in 7.6%. Ebstein’s malformation coexisted in 18 patients. Median tricuspid valvar size and RV inlet Z-scores were –5.2 and –5.1, respectively.
CONCLUSIONS: This study provides unique data on the diverse pathology of PAIVS in an unselected population. This will help determine if published reports reflect the true spectrum of pathology of the condition.
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Abbreviations and Acronyms
| | CHSS | | Congenital Heart Surgeons Study | | CI | | confidence interval | | ECG | | electrocardiogram | | LAD | | left anterior descending coronary artery | | LV | | left ventricle | | PAIVS | | pulmonary atresia with intact ventricular septum | | RV | | right ventricle/ventricular | | TV | | tricuspid valve | | VSD | | ventricular septal defect |
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