Journal of Pediatric Cardiology and Cardiac Surgery

Congenital pulmonary vein stenosis (CPVS) is extremely rare, accounting for 0.03% of cases of congenital heart disease. The prognosis of CPVS is poor because the secondary pulmonary hypertension (PH) is progressive. Treatments options include surgical repair using a sutureless technique and stent implantation; however, the stenosis often recurs. Herein, a boy with a partial anomalous pulmonary venous connection was treated for CPVS using a sutureless technique at the age of 1 year old; however, the stenosis recurred, and he experienced repeated hemoptysis from the age of 2 years and 8 months. Because of the severity of his condition, surgery or a catheter intervention would have been challenging, and the bleeding points could not be identified. After detailed investigation using cardiac catheterization and three-dimensional computed tomography, a partial resection of the lower lobe of the right lung was performed, and hemoptysis was resolved. Compensatory enlargement of the remaining lobes maintained the patient’s respiratory function with no mediastinal shift. There was no evidence of hemoptysis during 5 years following the lobectomy, and he goes to school without exercise restrictions. Because lung transplantation is not a viable treatment option in Japan, lobectomy may become a therapeutic option for a pulmonary hemorrhage caused by restenosis of the pulmonary vein after CPVS repair using a sutureless technique.