Left pulmonary vein stenosis associated with transposition of the great arteries

Two patients with unilateral left pulmonary vein stenosis associated with dextrotransposition of the great arteries and intact ventricular septum are described. Excessive preferential pulmonary artery blood flow to the unaffected side is diagnostic. Awareness and recognition of this rare association is important and the obstruction can be managed with excision of the diaphragm and venoatrioplasty when needed during a Senning or Mustard repair.