Case Report: Polysplenia Syndrome, a Wide Range of Congenital Heart Malformations in an Adult

Background: Polysplenia syndrome is a rare disease where are observed two or more spleens associated with left-sided isomerism.In this condition there are many visceral and vascular congenital malformations, including cardiac anomalies. Case summary:We present a case of a 60-year-old male with polysplenia syndrome and multiple cardiovascular abnormalities, but despite these malformations, the patient survived to adulthood with minor symptoms.Discussion: This case report highlights the importance of a multimodality imaging approach in patients with complex congenital heart disease, leading to an unsuspected diagnosis at the adult age.