Exploring kidney biopsy findings in congenital heart diseases: Insights beyond cyanotic nephropathy.

Jose Daniel Juarez-Villa, Iván Zepeda-Quiroz, Sebastián Toledo-Ramírez, Victor Hugo Gomez-Johnson, Francisco Pérez-Allende, Brian Ricardo Garibay-Vega, Francisco E Rodríguez Castellanos, Bernardo Moguel-González, Edgar Garcia-Cruz, Salvador Lopez-Gil
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Abstract

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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探索先天性心脏病的肾活检结果:超越紫绀型肾病的见解。
背景:先天性心脏病与慢性肾脏病之间的关系众所周知。与先天性心脏病相关的肾脏损伤机制多种多样。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症(FSGS),但这只在病例报告中得到证实,而未在观察性或临床试验中得到证实:这是一项在 "伊格纳西奥-查韦斯 "国家心脏病研究所肾脏内科进行的回顾性观察研究。研究对象包括 2000 年 1 月至 2023 年 1 月期间接受经皮肾活检的所有 16 岁以上先天性心脏病患者:结果:共发现 10 名患有先天性心脏病并接受过肾活检的患者。最常见的先天性心脏病是法洛氏四联症(2 例,占 20%)和室间隔缺损(2 例,占 20%)。在 10 个病例中,发现了 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殊治疗,推迟了肾脏替代治疗的开始时间。其余 8 例病例(80%)中,1 例为肾周型 FSGS,其余 7 例为非特异性 FSGS:结论:确定慢性肾脏病的病因有助于推迟肾脏替代治疗的时间。在我们的研究中,10 例患者中有 2 例进行了干预,推迟了肾脏替代治疗的开始时间。需要进行前瞻性研究,以确定肾活检在先天性心脏病患者中的作用。
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