Valvular dilated cardiomyopathy (DCM) represents a clinically significant subtype of heart failure where progressive left ventricular dysfunction arises secondary to chronic volume or pressure overload due to valvular lesions. Unlike idiopathic DCM, valvular DCM offers potential for reversal if the primary mechanical cause is addressed timely. This chapter provides a comprehensive overview of contemporary diagnostic modalities and evolving management strategies for valvular DCM, with focus on heart transplantation for end-stage disease. Advanced diagnostic technologies have revolutionized valvular DCM assessment. Artificial intelligence integration into cardiac imaging enables automated chamber quantification and real-time segmentation with expert-level accuracy, particularly excelling in mitral regurgitation assessment with AUCs exceeding 0.916. The biomarker landscape has expanded beyond traditional B-type natriuretic peptides to include soluble ST2, galectin-3, high-sensitivity troponins, and circulating microRNAs, providing deeper insights into disease mechanisms including inflammation, fibrosis, and metabolic dysfunction. Precision imaging approaches, including strain imaging and three-dimensional echocardiography powered by AI, offer personalized diagnostic protocols tailored to individual patient characteristics. Surgical decision-making has evolved significantly, with the concept of “reverse remodeling potential” emerging as the key determinant of surgical success. Evidence from randomized controlled trials challenges traditional repair dogma, demonstrating that complex neochordal reconstruction techniques and careful assessment of leaflet tethering are crucial for optimal outcomes. When conservative management fails, heart transplantation remains the definitive treatment for end-stage valvular DCM, offering excellent long-term survival rates.