Repeated phlebotomies improve and stabilise renal function in cyanotic nephropathy

Abstract

Patients over 10 years of age with cyanotic congenital heart disease (CCHD) risk developing significant glomerular proteinuria, a condition called cyanotic nephropathy. Even though the pathogenesis of glomerulopathy associated with CCHD is still unclear, a potential mechanism is hyperviscosity-induced decrease in peritubular capillary blood flow leading to an increase in glomerular capillary pressure, in turn resulting in proteinuria. Although angiotensin-converting enzyme (ACE) inhibitors have been traditionally used in the treatment of these patients with cyanotic nephropathy, they may, however, not be well tolerated. Here we present a case of an adult patient with CCHD who could not tolerate an ACE inhibitor but showed improvement and stabilisation of her renal function following treatment with repeated phlebotomies.

Phlebotomy improves kidney function in pediatric cyanotic nephropathy: A case report

“…Although not commonly used, phlebotomy is a promising option that leads to stabilization in CN patients. A study revealed that phlebotomy produced beneficial effects on kidney function by decreasing the hematocrit level, thereby reducing blood viscosity (7). This observation was in line with our findings of reduced proteinuria and hematocrit and improved eGFR in the present case.…”

“…In addition, the urine protein-tocreatinine ratio decreased by 57%. Repeated phlebotomies were scheduled at each outpatient visit, and the patient's kidney function was stabilized (7). Comparable results were observed in a 49-year-old male with operated tricuspid atresia.…”

“…Phlebotomy, a procedure commonly indicated for treating polycythemia vera (PV), has increased interest in investigating whether decreasing blood viscosity might benefit CCHD patients (5). Previously, this procedure produced favorable outcomes in adult CCHD patients developing secondary CN (6,7). However, its efficacy and safety in children have not yet been investigated.…”

Nephropathy in a Child Suffering From Tetralogy of Fallot

“…Hypoxia, polycythemia, and hyperdynamic circulation seem to be important factors deciding the rate of progression of nephropathy [7,8]. Studies have shown that phlebotomy helped to decrease proteinuria along with reducing the hematocrit [9,10]. This may be due to a reduced blood viscosity which seems to exert beneficial effects on hypoxia and glomerular permeability [11,8].…”