A heart with three arterial trunks (tritruncal heart). Report of a case

Insights

A rare congenital heart defect features three separate arterial trunks: the aorta, main pulmonary artery, and a novel "intermediate trunk" supplying the right pulmonary artery. This finding is crucial for diagnosing absent or anomalous right pulmonary artery.

Area of Science:

  • Cardiology
  • Developmental Biology
  • Pediatric Medicine

Background:

  • Congenital heart diseases represent a spectrum of structural abnormalities present at birth.
  • Understanding the embryological origins of cardiac malformations is key to diagnosis and treatment.

Observation:

  • A previously unreported congenital heart malformation in a 4 1/2-year-old girl.
  • The heart presented with three distinct arterial trunks: aorta, main pulmonary artery, and an "intermediate trunk" originating from the left ventricle.
  • Each artery possessed a semilunar valve at its origin, with completely separated lumens.

Findings:

  • The aorta and intermediate trunk arose from the left ventricle; the pulmonary trunk arose from the right ventricle.
  • The intermediate trunk supplied the right pulmonary artery, and the main pulmonary trunk supplied the left pulmonary artery.
  • Both ventricular outflow tracts were normal, resulting in three arterial trunks from two outflow tracts.

Implications:

  • The embryopathogenesis is hypothesized to involve double septation of the primitive arterial trunk and aortic sac, with truncal-conal septal malalignment.
  • This rare entity must be considered in the differential diagnosis of absent or anomalous right pulmonary artery.
  • Highlights the importance of detailed anatomical and embryological understanding in congenital heart disease.

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