From repair to right heart failure: Understanding the evolving landscape in tetralogy of fallot

从修复到右心衰竭:了解法洛四联症的演变历程

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Abstract

Surgical repair and clinical management have significantly improved Tetralogy of Fallot (TOF) patient survival rates. Despite these advancements, adults with repaired TOF remain at high risk for long-term complications, particularly heart failure, which constitutes the primary cause of mortality in this population. This review synthesizes current evidence on right heart failure in repaired TOF, with emphasis on pathophysiological mechanisms, diagnostic strategies, and management approaches. The chronic hemodynamic burden of residual lesions and surgical sequalae initiates a maladaptive cascade that results in progressive right ventricular dysfunction and, ultimately, clinical manifestations of right heart failure. The discussion includes the role of multimodal imaging, electrocardiographic monitoring, and exercise testing, as well as the limited evidence for conventional pharmacological therapies in patients with repaired TOF and right heart failure. This review aims to serve as a comprehensive resource to guide clinical decision-making and to highlight key knowledge gaps that warrant further research to improve long-term outcomes in this patient group.

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