Abnormal pulmonary venous flow patterns on fetal echocardiography and a
nutmeg lung pattern on fetal magnetic resonance imaging are seen in
patients with pulmonary venous stenosis. The association between these
findings and the degree of pulmonary venous stenosis remains unknown. We
report an extremely rare case of a fetus diagnosed with hypoplastic left
heart syndrome complicated by an absent atrial septum and supracardiac
total anomalous pulmonary venous connection with left pulmonary venous
congestion. This case suggests that compared to non-pulsatile continuous
pulmonary venous flow, the nutmeg lung pattern can only be observed with
severe pulmonary congestion and advanced pulmonary lymphangiectasia.