Pulmonary atresia with intact ventricular septum: range of morphology in a population-based study.
OBJECTIVE
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.
Daubeney
Piers E F
PE
Wessex Cardiothoracic Centre, Southampton General Hospital, Southampton, United Kingdom. p.daubeney@rbh.thames.nhs.uk
Delany
David J
DJ
Anderson
Robert H
RH
Sandor
George G S
GG
Slavik
Zdenek
Z
Keeton
Barry R
BR
Webber
Steven A
SA
D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000473:pathology; D006345:Heart Septal Defects, Ventricular / Q000175:diagnosis / Q000473:pathology*; D006346:Heart Septum / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D007494:Ireland; D008297:Male; D011446:Prospective Studies; D018633:Pulmonary Atresia / Q000175:diagnosis / Q000473:pathology*; D011664:Pulmonary Valve / Q000473:pathology; D006113:United Kingdom