Abstract

Children with cyanotic congenital heart diseases (CCHDs) have several risk factors for the development of several complications, including cyanotic nephropathy (CN). However, due to poor awareness of this problem, CN occasionally progresses to chronic kidney disease (CKD). In the long term, kidney cells subjected to chronic hypoxia undergo further damage, which increases the risk of developing end-stage kidney disease. There are no standardized therapeutic approaches for these cases, especially for children, although phlebotomy is an option. Our case demonstrates a 12-year-old boy with an inoperable, complex CCHD and persistent proteinuria with decreased kidney function. After receiving consecutive treatment with an angiotensin-converting enzyme inhibitor and angiotensin II receptor blocker, the proteinuria did not resolve, and the kidney function deteriorated. Phlebotomy was performed to treat the proteinuria, and the kidney function improved. Within the three months following the first phlebotomy procedure, the patient was less cyanotic and had improved hemodynamic stability. Proteinuria reduction and improvement of estimated glomerular filtration rate were noted; however, the proteinuria gradually worsened after three months. In conclusion, phlebotomy might delay CKD progression in children with CN due to inoperable, complex CCHD. The positive effects in our case were temporary; therefore, repeated therapeutic phlebotomies should be considered.

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