Renal Involvement in Patients with Congenital Cyanotic Heart Disease

Abstract

Patients with congenital cyanotic heart disease may develop a glomerulopathy with proteinuria and impaired renal function. In order to investigate this problem we conducted a study on 27 patients with uncorrected cyanotic heart disease who were between 1 day and 25 years old. As a consequence of hypoxaemia haematocrit was elevated to 57%. Proteinuria was above 150 mg/day/1.73 m2 body surface in 12 patients. Only one of 9 children under 10 years of age had pathological proteinuria presenting as isolated albuminuria. Seven out of 10 patients between 11 and 20 years had an elevated proteinuria with a glomerular pattern. Creatinine clearance was normal in these patients. All four patients above 20 years of age had a considerable glomerular proteinuria with a mean excretion of 5.7 g/24 h/1.73 m2 body surface. These patients suffered additionally from chronic cardiac failure and creatinine clearance was below the normal range. There was a clear relationship between pathological proteinuria and age of the patients and thus duration of hypoxaemia. Patients with pathological proteinuria had a significant higher erythrocyte count (7.3 +/- 1.3 vs 5.6 +/- 1.4 10(12)/l p less than 0.01) and a lower mean corpuscular haemoglobin. In summary, children with persistent congenital cyanotic heart disease have substantial risk of developing a glomerulopathy if the cyanosis remains unchanged for more than ten years.

Mechanisms of development and progression of cyanotic nephropathy

“…In a previous report, more than 70% of CCHD patients over 10 years of age had significant glomerular proteinuria [11]. In the present study, we demonstrated that hematocrit levels were higher in patients with CN than in those without CN, whereas no difference was observed between these groups in terms of oxygen saturation.…”

Effect of Congenital Heart Disease on Renal Function in Childhood

“…These investigations have demonstrated that large proportions of cyanotic CHD patients have glomerular injury, as indicated by increased albuminuria and decreased GFR. Krull et al [15]studied 27 patients of age range 0–25 years who had cyanotic CHD. They found that only 1 of the patients under 10 years had pathological proteinuria, whereas all 4 patients older than 20 years exhibited this problem.…”

Failure to thrive and nephrolithiasis in a boy with congenital cyanotic heart anomaly: answers

“…Krull et al investigated 27 children with non-repaired congenital cyanotic heart disease over a range of ages, from 1-dayold infants up to adults, 25 years of age [6]. Significant proteinuria was found in 12 patients (mainly the glomerular type), but only in 1 out of 10 children younger than 10 years of age.…”

“…It has been well established that chronic cyanosis in children with congenital heart anomalies may affect glomerular structure and function, leading to glomerulopathy, manifesting with proteinuria [5][6][7]. Krull et al investigated 27 children with non-repaired congenital cyanotic heart disease over a range of ages, from 1-dayold infants up to adults, 25 years of age [6].…”