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Exploring kidney biopsy findings in congenital heart diseases: Insights beyond cyanotic nephropathy
Jose Daniel Juarez-Villa1, Iván Zepeda-Quiroz1, Sebastián Toledo-Ramírez1
1Department of Nephrology, Instituto Nacional de Cardiología Ignacio Chavez, Mexico City 14080, Mexico.
Insights
Kidney biopsies in patients with congenital heart disease revealed diverse kidney pathologies beyond FSGS, including IgA nephropathy and glomerulonephritis. Early diagnosis through biopsy can delay the need for kidney replacement therapy in these patients.
Area of Science:
- Nephrology
- Cardiology
- Pathology
Background:
- Congenital heart disease (CHD) is linked to chronic kidney disease (CKD).
- Kidney damage mechanisms in CHD are known, but etiology is often presumed to be focal segmental glomerulosclerosis (FSGS) without strong clinical evidence.
- This study investigates kidney biopsy findings in CHD patients.
Purpose of the Study:
- To characterize baseline and clinical features of patients with CHD undergoing kidney biopsy.
- To analyze histopathological findings from kidney biopsies in this cohort.
- To explore the diagnostic utility of kidney biopsy in managing CKD in CHD.
Main Methods:
- Retrospective observational study at the National Institute of Cardiology.
- Included patients over 16 years old with CHD who underwent percutaneous kidney biopsy (January 2000 - January 2023).
- Analysis of clinical data and kidney biopsy results.
Main Results:
- Ten CHD patients with kidney biopsies were identified (average age 29.00 ± 15.87 years).
- High pre-biopsy proteinuria (6193 ± 6165 mg/24h) was noted.
- Common CHDs included Tetralogy of Fallot and Ventricular Septal Defect. Kidney pathologies varied, with IgA nephropathy and membranoproliferative glomerulonephritis identified in two cases, and FSGS (including one perihilar variety) in the remaining eight.
Conclusions:
- Kidney biopsy findings in CHD patients are diverse, not solely FSGS.
- Histopathological diagnosis enabled specific treatments, delaying kidney replacement therapy in 20% of cases.
- Further prospective studies are recommended to confirm the value of kidney biopsy in CHD-associated CKD.
Background:
The association between congenital heart disease and chronic kidney disease is well known. Various mechanisms of kidney damage associated with congenital heart disease have been established. The etiology of kidneydisease has commonly been considered to be secondary to focal segmental glomerulosclerosis (FSGS), however, this has only been demonstrated in case reports and not in observational or clinical trials.
Aim:
To identify baseline and clinical characteristics, as well as the findings in kidney biopsies of patients with congenital heart disease in our hospital.
Methods:
This is a retrospective observational study conducted at the Nephrology Department of the National Institute of Cardiology "Ignacio Chávez". All patients over 16 years old who underwent percutaneous kidney biopsy from January 2000 to January 2023 with congenital heart disease were included in the study.
Results:
Ten patients with congenital heart disease and kidney biopsy were found. The average age was 29.00 years ± 15.87 years with pre-biopsy proteinuria of 6193 mg/24 h ± 6165 mg/24 h. The most common congenital heart disease was Fallot's tetralogy with 2 cases (20%) and ventricular septal defect with 2 (20%) cases. Among the 10 cases, one case of IgA nephropathy and one case of membranoproliferative glomerulonephritis associated with immune complexes were found, receiving specific treatment after histopathological diagnosis, delaying the initiation of kidney replacement therapy. Among remaining 8 cases (80%), one case of FSGS with perihilar variety was found, while the other 7 cases were non-specific FSGS.
Conclusion:
Determining the cause of chronic kidney disease can help in delaying the need for kidney replacement therapy. In 2 out of 10 patients in our study, interventions were performed, and initiation of kidney replacement therapy was delayed. Prospective studies are needed to determine the usefulness of kidney biopsy in patients with congenital heart disease.
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