Introduction:
Scimitar syndrome is a rare pulmonary venolobar syndrome that is characterized by anomalous pulmonary vein (PV) drainage of the right lung, most frequently into the inferior vena cava. Associated congenital abnormalities include right lung hypoplasia and cardiac dextroposition, with or without structural heart defects. We describe a case of a patient with Scimitar syndrome and severe chronic heart failure with left bundle branch block (LBBB) who underwent cardiac resynchronization therapy with His-bundle pacing (His-CRT), resulting in significant improvements in functional capacity and left ventricular (LV) function. To the best of our knowledge, this is the first reported case of His-CRT in Scimitar syndrome. We highlight the contribution of integrated imaging modalities to guide accurate lead positioning.