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 cm3/m2, 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.
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