日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Isolated unilateral pulmonary venous atresia (PVA) is an extremely rare anomaly that typically presents with hemoptysis and pneumonia; however, the most appropriate management strategy remains controversial due to the high variability in clinical course between cases. Herein, we present three cases and discuss the diagnostic and therapeutic considerations.

Case 1: A 4-year-old girl presented with a history of recurrent hemoptysis and pneumonia since 18 months of age. Lung perfusion scintigraphy demonstrated decrease in the right pulmonary blood flow. Pulmonary arterial wedge angiography (PAWA) identified a right PVA. The patient underwent pulmonary vein reconstruction using a sutureless technique.

Case 2: A 4-year-old girl was referred due to hemoptysis. Lung perfusion scintigraphy demonstrated a decrease in left pulmonary blood flow, and the diagnosis was confirmed by PAWA. We abandoned the repair in this case due the presence of a long gap between the left pulmonary vein and left atrium.

Case 3: A 36-year-old female presented with a history of recurrent hemoptysis and pneumonia from infancy. PVA was suspected according to the results of lung perfusion scintigraphy, and the diagnosis was confirmed by PAWA. We abandoned the repair in this case as the development of pulmonary arterial obstructive disease was expected.

If lung perfusion scintigraphy demonstrates a remarkable reduction in unilateral pulmonary blood flow, PVA should be suspected and PAWA should be performed to confirm the diagnosis. Pulmonary vein reconstruction represents a treatment option in cases where the diagnosis is made in early childhood and anatomical repair is feasible. For right PVA, the sutureless technique is considered to be effective in preventing postoperative pulmonary venous stenosis.