Book contents
- Frontmatter
- Contents
- List of contributors
- Preface
- Acknowledgment
- Section 1 Head and neck
- Section 2 Thoracic imaging
- Section 3 Cardiac imaging
- Case 23 Tetralogy of Fallot with pulmonary atresia
- Case 24 Left pulmonary artery sling
- Case 25 Vascular ring
- Case 26 Scimitar syndrome
- Case 27 Portosystemic shunt and portopulmonary syndrome
- Case 28 Aortic coarctation and interrupted aortic arch
- Case 29 Ebstein’s anomaly
- Case 30 Transposition of the great arteries
- Case 31 Total anomalous pulmonary venous return
- Case 32 Aberrant left coronary artery arising from the pulmonary artery
- Section 4 Vascular and interventional
- Section 5 Gastrointestinal imaging
- Section 6 Urinary imaging
- Section 7 Endocrine - reproductive imaging
- Section 8 Fetal imaging
- Section 9 Musculoskeletal imaging
- Index
Case 29 - Ebstein’s anomaly
from Section 3 - Cardiac imaging
Published online by Cambridge University Press: 05 June 2014
- Frontmatter
- Contents
- List of contributors
- Preface
- Acknowledgment
- Section 1 Head and neck
- Section 2 Thoracic imaging
- Section 3 Cardiac imaging
- Case 23 Tetralogy of Fallot with pulmonary atresia
- Case 24 Left pulmonary artery sling
- Case 25 Vascular ring
- Case 26 Scimitar syndrome
- Case 27 Portosystemic shunt and portopulmonary syndrome
- Case 28 Aortic coarctation and interrupted aortic arch
- Case 29 Ebstein’s anomaly
- Case 30 Transposition of the great arteries
- Case 31 Total anomalous pulmonary venous return
- Case 32 Aberrant left coronary artery arising from the pulmonary artery
- Section 4 Vascular and interventional
- Section 5 Gastrointestinal imaging
- Section 6 Urinary imaging
- Section 7 Endocrine - reproductive imaging
- Section 8 Fetal imaging
- Section 9 Musculoskeletal imaging
- Index
Summary
Imaging description
A term infant with trisomy 21 developed cyanosis within the first week of life. A chest radiograph demonstrated a markedly enlarged cardiac silhouette seen in association with normal to diminished pulmonary flow (Fig. 29.1). This “wall-to-wall” or “box-shaped heart” configuration in a newborn is highly suggestive of right atrial enlargement, most often due to Ebstein’s anomaly. Cardiac MRI in a different four-month-old infant shows the typical appearance of Ebstein’s anomaly including right atrial dilatation (Fig. 29.2a) with apical displacement of the septal and posterior leaflets of the tricuspid valve leading to atrialization of the inflow portion of the right ventricle (Fig. 29.2b, c). MRI also demonstrates marked tricuspid regurgitation (TR) across the dysplastic tricuspid valve (Fig. 29.2d). A secundum atrial septal defect (ASD) is present, a common association.
Importance
Ebstein’s anomaly results from embryologic dysgenesis of the tricuspid valve, characterized by incomplete delamination or undermining of the septal and posterior leaflets from the right ventricle. A strong association has been reported between oral lithium therapy during pregnancy and this type of valvular dysgenesis. As a result, affected valve leaflets are apically displaced with atrialization of the inflow portion of the right ventricle. The anterior leaflet may assume a sail-like configuration, and widening of the tricuspid annulus leads to marked tricuspid regurgitation.
- Type
- Chapter
- Information
- Pearls and Pitfalls in Pediatric ImagingVariants and Other Difficult Diagnoses, pp. 122 - 126Publisher: Cambridge University PressPrint publication year: 2014