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Giant Right Atrial Aneurysm Accompanying Intrahepatic Cholestasis

Ayse Sulu1, Osman Baspinar1, Selim Kervancıoglu2

  • 1Department of Pediatric Cardiology, Gaziantep University Medical Faculty, Gaziantep, Turkey.

Case Reports in Cardiology
|April 24, 2018
PubMed

Insights

A rare giant right atrial aneurysm was diagnosed in an infant with progressive familial intrahepatic cholestasis type 3. This case highlights a previously unreported comorbidity between these two rare conditions.

Area of Science:

  • Cardiology
  • Hepatology
  • Pediatrics

Background:

  • Right atrial aneurysms are rare congenital heart abnormalities with unknown etiology.
  • Progressive familial intrahepatic cholestasis (PFIC) type 3 is a rare inherited liver disease.
  • The co-occurrence of right atrial aneurysms and cholestasis has not been previously documented.

Observation:

  • An 11-month-old female infant presented with jaundice and a heart murmur.
  • Echocardiography revealed a giant right atrial aneurysm.
  • Liver biopsy confirmed a diagnosis of PFIC type 3.

Findings:

  • The case presents a novel comorbidity between a giant right atrial aneurysm and PFIC type 3.
  • This association suggests potential underlying genetic or developmental links between cardiac and hepatic conditions.

Implications:

  • This case expands the known clinical spectrum of right atrial aneurysms.
  • Further research is warranted to explore the potential relationship between congenital heart anomalies and inherited cholestatic liver diseases.
  • Understanding this comorbidity may improve diagnostic and management strategies for affected infants.

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