Juarez-Villa Jose Daniel, Zepeda-Quiroz Iván, Toledo-Ramírez Sebastián, Gomez-Johnson Victor Hugo, Pérez-Allende Francisco, Garibay-Vega Brian Ricardo, Rodríguez Castellanos Francisco E, Moguel-González Bernardo, Garcia-Cruz Edgar, Lopez-Gil Salvador
Department of Nephrology, Instituto Nacional de Cardiología Ignacio Chavez, Mexico City 14080, Mexico.
Congenital Heart Disease, Instituto Nacional de Cardiología Ignacio Chavez, Mexico City 14080, Mexico.
World J Nephrol. 2024 Mar 25;13(1):88972. doi: 10.5527/wjn.v13.i1.88972.
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.
To identify baseline and clinical characteristics, as well as the findings in kidney biopsies of patients with congenital heart disease in our hospital.
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.
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.
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.
先天性心脏病与慢性肾脏病之间的关联已为人熟知。与先天性心脏病相关的肾脏损害的各种机制已被确立。肾脏疾病的病因通常被认为是继发于局灶节段性肾小球硬化（FSGS），然而，这仅在病例报告中得到证实，而未在观察性研究或临床试验中得到证实。
确定我院先天性心脏病患者的基线和临床特征以及肾活检结果。
这是一项在国家心脏病学研究所“伊格纳西奥·查韦斯”肾脏病科进行的回顾性观察性研究。纳入2000年1月至2023年1月期间所有接受经皮肾活检且年龄超过16岁的先天性心脏病患者。
发现10例先天性心脏病患者接受了肾活检。平均年龄为29.00岁±15.87岁，活检前蛋白尿为6193mg/24h±6165mg/24h。最常见的先天性心脏病是法洛四联症，有2例（20%），室间隔缺损有2例（20%）。在这10例病例中，发现1例IgA肾病和1例与免疫复合物相关的膜增生性肾小球肾炎，经组织病理学诊断后接受了特异性治疗，推迟了肾脏替代治疗的启动。在其余8例（80%）中，发现1例伴肾门周围型的FSGS，而其他7例为非特异性FSGS。
确定慢性肾脏病的病因有助于推迟肾脏替代治疗的需求。在我们研究的10例患者中，有2例进行了干预，推迟了肾脏替代治疗的启动。需要进行前瞻性研究以确定肾活检对先天性心脏病患者的有用性。
