Abstract
Shone's complex (SC), a rare congenital cardiovascular malformation featuring multilevel left-sided obstructive lesions, presents particular diagnostic challenges in adult populations. The complex interplay between congenital anatomical defects and acquired cardiovascular adaptations in adult SC patients requires multidisciplinary management. Clinicians must maintain a high suspicion for congenital substrates when evaluating young adults with unexplained cardiopulmonary deterioration, particularly when accompanied by refractory hypertension, severe pulmonary hypertension (PH), and right ventricular dysfunction.