title: Physiology of isolated anomalous pulmonary venous connection of a single pulmonary vein as determined by cardiac magnetic resonance imaging.
abstract: The physiology of isolated partially anomalous pulmonary venous connection of a single pulmonary vein has yet to be fully characterized. This study assessed the magnitude of the left-to-right shunt and right ventricular (RV) dilation from a single anomalous pulmonary vein using cardiac magnetic resonance imaging. Subjects with >1 anomalous pulmonary vein or associated lesions, including atrial septal defects, were excluded. In the 6 subjects identified, the median pulmonary-to-systemic flow ratio was 1.55 (range 1.3 to 1.6). The mean RV end-diastolic volume indexed to body surface area in the subjects was significantly larger than in a normal reference cohort (108 +/- 16 vs 78 +/- 18 cm(3)/m(2), p = 0.0009) and greater than the upper limit of normal in all 6 subjects. Older age did not correlate with increased magnitude of shunting (r = 0.3, p = 0.5), but increased age did correlate with RV end-diastolic volume indexed to body surface area (r = 0.96, p = 0.01). Isolated partially anomalous pulmonary venous connection with only 1 vein connecting anomalously results in a modest left-to-right shunt and mild RV dilation.
authors.affiliation: Division of Pediatric Cardiology, Columbia University College of Physicians and Surgeons, New York, New York, USA.
authors.forename: Joshua L
authors.initials: JL
authors.lastname: Dyme
authors.forename: Ashwin
authors.initials: A
authors.lastname: Prakash
authors.forename: Beth F
authors.initials: BF
authors.lastname: Printz
authors.forename: Avnit
authors.initials: A
authors.lastname: Kaur
authors.forename: Ira A
authors.initials: IA
authors.lastname: Parness
authors.forename: James C
authors.initials: JC
authors.lastname: Nielsen
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000503:physiopathology*; D006801:Humans; D018810:Magnetic Resonance Angiography / Q000379:methods*; D008297:Male; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000503:physiopathology; D013318:Stroke Volume