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

Authors

  • Giovanni Balestrucci Department of Precision Medicine, University of Campania “L. Vanvitelli”, Italy
  • Andrè Carvalho Radiology Department, Centro Hospitalar de São João, Portugal
  • João Rebelo Cardiology Department, Centro Hospitalar de São João, Porto, Portugal; Faculdade de Medicina da Universidade do Porto, Portugal
  • Mariana Vasconcelos Cardiology Department, Centro Hospitalar de São João, Porto, Portugal; Faculdade de Medicina da Universidade do Porto, Portugal
  • Cristina Cruz Cardiology Department, Centro Hospitalar de São João, Porto, Portugal; Faculdade de Medicina da Universidade do Porto, Portugal
  • Pietro Roccatagliata Department of Precision Medicine, University of Campania “L. Vanvitelli”, Italy
  • Diego Sandro Giordano Department of Precision Medicine, University of Campania “L. Vanvitelli”, Italy
  • Alfonso Reginelli

DOI:

https://doi.org/10.3941/jrcr.5350

Abstract

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: In this case presentation of a 60-year-old male with polysplenia syndrome, are identified multiple cardiovascular abnormalities and, despite these malformations, the patient survived with minor symptoms without surgery, highlighting the need for individualized management.

Discussion: Even if polysplenia syndrome is a rare condition, not easy to manage, this case report highlights the importance of a multidisciplinary medical board in the choice of the assessment, recognizing that some patient can have a good prognosis without surgical intervention.

Balanced Steady State Free Precession (SSFP) cine image in the horizontal long-axis of the left ventricle shows dextrocardia and dextroapex. Right ventricular hypertrohpy is also seen.

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Published

2025-03-31

Issue

Vol. 19 No. 3 (2025)

Section

Cardiac Imaging

License

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