Diagnosis, management, and outcome of heart failure in congenitally corrected transposition of the great arteries (ccTGA)—a narrative review

Background and Objective: Congenitally corrected transposition of the great arteries (ccTGA) remains a rare congenital disorder with a wide range of manifestations. The management of heart failure (HF) of the systemic right ventricle (RV), arrhythmias, heart block, and acquired cardiac conditions require a complex and multi-faceted approach. The objective of this manuscript is to present the current evidence regarding diagnostic, treatment, and management strategies for HF in ccTGA, including ventricular assist device (VAD) therapy and heart transplantation. Methods: A systematic review of the literature was conducted using PubMed, covering the period between 2010 and 2024. The search terms included “heart failure”, “ccTGA”, “VAD”, “heart transplantation”, and “systemic RV failure”. Two clinical cases were included for illustrative purposes. Key Content and Findings: HF is a common occurrence in the context of ccTGA, primarily driven by progressive pressure and volume overload of the systemic RV, regurgitation of the systemic atrio-ventricular (AV) valve, and the development of arrhythmias, including complete heart block and (supra-)ventricular tachycardia. The use of HF medication is indicated for symptomatic patients, however, data on the efficacy of standardized HF medication remains limited. Timing of AV-valve replacement is essential to prevent further progression of HF. Conclusions: In ccTGA, the timing of surgery and interventional treatment approaches, the effect of pharmacological treatment in the context of HF, as well as the timing of initiation of a mechanical circulatory support, VAD and heart transplantation, are based on individualised consensus-level decisions. Optimal management remains a topic of debate due to the scarcity of outcome data. Future investigations should focus on identifying surrogate parameters for guiding treatment.