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.
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.
Authors: Lydia K Wright; Jessica H Knight; Amanda S Thomas; Matthew E Oster; James D St Louis; Lazaros K Kochilas Journal: Heart Date: 2019-02-02 Impact factor: 5.994
Authors: Ilias Iliopoulos; Christopher W Mastropietro; Saul Flores; Eva Cheung; Venugopal Amula; Monique Radman; David Kwiatkowski; Bao Nguyen Puente; Jason R Buckley; Kiona Y Allen; Rohit Loomba; Karan B Karki; Saurabh Chiwane; Katherine Cashen; Kurt Piggott; Yamini Kapileshwarkar; Keshava Murty Narayana Gowda; Aditya Badheka; Rahul Raman; Huaiyu Zang; John M Costello Journal: Pediatr Cardiol Date: 2022-06-25 Impact factor: 1.655
Authors: Fernando Baraona; Anne Marie Valente; Prashob Porayette; Francesca Romana Pluchinotta; Stephen P Sanders Journal: J Clin Exp Cardiolog Date: 2012-06-15
Authors: Matthew C Schwartz; Andrew C Glatz; Yoav Dori; Jonathan J Rome; Matthew J Gillespie Journal: Pediatr Cardiol Date: 2013-06-19 Impact factor: 1.655