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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.
Abstract:
The case is described of a 4 1/2-year-old girl with a previously unreported malformation: a heart with three arterial trunks, aorta, main pulmonary artery, and an intermediate vessel, called "intermediate trunk" because it was situated between the aorta and the main pulmonary artery. Each of these three arteries had a semilunar valve at its origin, and their lumens were completely separated from each other. The pulmonary trunk arose from the right ventricle, while the aorta and the intermediate trunk arose from the left ventricle. The intermediate trunk continued as the right pulmonary artery. The pulmonary trunk continued as the left pulmonary artery. The outflow tracts of both ventricles were normal. There were therefore three arterial trunks arising from a heart with two outflow tracts. Our hypothesis on the embryopathogenesis of this case is that there was double septation of the primitive arterial trunk and the aortic sac, without involvement of the conus. In addition, there was a malalignment between the truncal and conal septa. This entity, however rare, should be considered in the differential diagnosis of absent or anomalous right pulmonary artery.