title: Determinants of outcome in fetal pulmonary valve stenosis or atresia with intact ventricular septum.
abstract: Pulmonary valve stenosis or atresia with intact ventricular septum represents a spectrum of severity. This study aimed to identify ultrasound markers of biventricular versus non-biventricular outcome. The fetal echocardiograms of 41 fetuses diagnosed with pulmonary stenosis or atresia and right ventricular (RV)/left ventricular (LV) length ratios >0.4 from 17 to 31 weeks of gestation were reviewed. Of 27 live-born patients with intention to treat, 8 had non-biventricular outcomes and 19 had biventricular circulation. At the time of diagnosis, poor RV function, flow reversal in the arterial duct, the degree of tricuspid valve (TV) regurgitation, and inferior vena cava Doppler flow pattern did not differ between the 2 outcome groups. However, RV sinusoids, the RV/LV length ratio, the TV/mitral valve ratio, and TV inflow duration were significantly different. Cut-off values derived from receiver-operating characteristic curves yielding the best sensitivity and specificity for a non-biventricular outcome were TV/mitral valve ratio <0.7, RV/LV length ratio <0.6, TV inflow duration <31.5% of cardiac cycle length, and the presence of RV sinusoids. If 3 of these 4 criteria were fulfilled, this predicted a non-biventricular outcome with sensitivity of 100% and specificity of 75%. In conclusion, in fetuses < or =31 weeks of gestation with pulmonary stenosis or atresia and intact ventricular septum, progression to a non-biventricular outcome can be predicted by a 4-criterion scoring system. The criteria may be useful in selecting fetuses for prenatal catheter intervention to prevent progressive RV hypoplasia.
authors.affiliation: Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Kevin S
authors.initials: KS
authors.lastname: Roman
authors.forename: Jean-Claude
authors.initials: JC
authors.lastname: Fouron
authors.forename: Masaki
authors.initials: M
authors.lastname: Nii
authors.forename: Jeffrey F
authors.initials: JF
authors.lastname: Smallhorn
authors.forename: Rajiv
authors.initials: R
authors.lastname: Chaturvedi
authors.forename: Edgar T
authors.initials: ET
authors.lastname: Jaeggi
mesh_terms: D005260:Female; D005500:Follow-Up Studies; D005865:Gestational Age; D006346:Heart Septum / Q000000981:diagnostic imaging; D006801:Humans; D007231:Infant, Newborn; D011237:Predictive Value of Tests; D011247:Pregnancy; D018633:Pulmonary Atresia / Q000000981:diagnostic imaging* / Q000628:therapy*; D011666:Pulmonary Valve Stenosis / Q000151:congenital / Q000000981:diagnostic imaging* / Q000628:therapy*; D012189:Retrospective Studies; D016896:Treatment Outcome; D016216:Ultrasonography, Prenatal