Introduction: Pulmonary artery thrombosis presents a rare and formidable challenge, especially in individuals with untreated complex congenital heart diseases and concurrent valvular abnormalities.
Presentation Of Case: This case details a distinctive instance of pulmonary artery thrombosis in a patient with untreated congenital atrial septal defect, severe pulmonary hypertension, pulmonary aneurysm, valvular heart disease, and triple-positive antiphospholipid antibodies. Collaborative decision-making by a heart team guided the diagnostic and therapeutic processes, resulting in surgical interventions encompassing pulmonary thrombectomy, mitral and tricuspid regurgitation repair, and atrial septal defect closure.