Upadhyay Shailendra, Dodeja Anudeep K, Toro-Salazar Olga, Fairchild Whitney, Han Frank
Cardiology Department, Connecticut Children's, Hartford, CT 06106, USA.
Department of Pediatrics and Pediatric Cardiology, University of Connecticut School of Medicine, Farmington, CT 06030, USA.
J Clin Med. 2025 Jun 20;14(13):4392. doi: 10.3390/jcm14134392.
As the population of adults with congenital heart disease (ACHD) continues to grow, a significant and often underrecognized complication is the development of cardiorenal syndrome (CRS)-a complex, bidirectional interaction between cardiac and renal dysfunction. While CRS has been extensively studied in acquired heart failure, its manifestations and implications in ACHD remain insufficiently understood. Emerging data suggest that renal dysfunction is highly prevalent in ACHD, with significant associations to adverse outcomes regardless of cardiac lesion type or functional status. This review explores CRS within three key physiologic categories in ACHD: patients with a systemic right ventricle, those with a subpulmonary right ventricle, and those with Fontan circulation. Each subgroup presents unique hemodynamic challenges that affect renal perfusion, filtration pressure, and systemic congestion, contributing to both acute and chronic renal impairment. The utility of renal biomarkers such as albuminuria, cystatin C, and estimated glomerular filtration rate (eGFR) is emphasized, alongside the importance of early detection and multidisciplinary management. Heart failure therapy tailored to congenital anatomy, neurohormonal modulation, and careful volume control remain the cornerstones of treatment, while transplantation strategies must consider the potential for irreversible end-organ damage. Given the profound implications of CRS on quality of life and survival, a comprehensive understanding of its pathophysiology and management in ACHD is critical to optimizing long-term outcomes in this increasingly complex patient population.
随着先天性心脏病成年患者（ACHD）数量持续增加，一种重要且常未被充分认识的并发症是心肾综合征（CRS）的发生，这是心脏和肾功能障碍之间复杂的双向相互作用。虽然CRS在获得性心力衰竭中已得到广泛研究，但其在ACHD中的表现和影响仍未得到充分了解。新出现的数据表明，肾功能障碍在ACHD中非常普遍，无论心脏病变类型或功能状态如何，都与不良结局密切相关。本综述探讨了ACHD中三个关键生理类别中的CRS：具有体循环右心室的患者、具有肺下右心室的患者以及具有Fontan循环的患者。每个亚组都呈现出独特的血流动力学挑战，这些挑战会影响肾脏灌注、滤过压和全身充血，导致急性和慢性肾功能损害。强调了尿白蛋白、胱抑素C和估算肾小球滤过率（eGFR）等肾脏生物标志物的作用，以及早期检测和多学科管理的重要性。根据先天性解剖结构定制的心力衰竭治疗、神经激素调节和谨慎的容量控制仍然是治疗的基石，而移植策略必须考虑不可逆终末器官损伤的可能性。鉴于CRS对生活质量和生存的深远影响，全面了解其在ACHD中的病理生理学和管理对于优化这一日益复杂患者群体的长期结局至关重要。
