CAHAL, Center for Congenital Heart Disease Amsterdam Leiden, Leiden University Medical Center, Leiden, The Netherlands.
Department of Cardiology, Leiden Heart-Lung Center, Leiden University Medical Center, Albinusdreef 2, Leiden, 2333 ZA, The Netherlands.
ESC Heart Fail. 2022 Jun;9(3):2007-2012. doi: 10.1002/ehf2.13871. Epub 2022 Mar 30.
In congenitally corrected transposition of the great arteries, the morphological right ventricle supports the systemic circulation. This chronic exposure to pressure overload ultimately leads to systemic right ventricular (sRV) dysfunction and heart failure. Pharmacological options for the treatment of sRV failure are poorly defined and no solid recommendations are made in the most recent guidelines. Sodium-glucose cotransporter 2 (SGLT-2) inhibitors are a new class of antihyperglycaemic drugs that have been demonstrated to significantly reduce the risk of worsening heart failure and death from cardiovascular causes in patients with chronic heart failure with reduced left ventricular ejection fraction, yet no data are available in sRV patients. We report on the treatment and clinical follow-up of a patient with advanced heart failure and poor sRV function in the context of congenitally corrected transposition of the great arteries, who did not tolerate sacubitril/valsartan and had a high burden of heart-failure-related hospitalizations. Treatment with dapagliflozin was well tolerated and resulted in (small) subjective and objective functional and echocardiographic improvement and a reduction in heart-failure-related hospitalizations.
在先天性矫正性大动脉转位中，形态右心室支持体循环。这种慢性压力超负荷最终导致右心室（sRV）功能障碍和心力衰竭。sRV 衰竭的治疗药物选择定义不明确，最近的指南也没有提出明确的建议。钠-葡萄糖共转运蛋白 2（SGLT-2）抑制剂是一类新型的抗高血糖药物，已被证明可显著降低射血分数降低的慢性心力衰竭患者因心血管原因恶化心力衰竭和死亡的风险，但 sRV 患者的数据尚不可用。我们报告了一例在先天性矫正性大动脉转位背景下患有晚期心力衰竭和 sRV 功能不良的患者的治疗和临床随访情况，该患者不能耐受沙库巴曲缬沙坦，且心力衰竭相关住院负担高。达格列净治疗耐受性良好，导致（较小）主观和客观的功能和超声心动图改善，并减少心力衰竭相关住院。
