Blood Vessels, Total Anomalous Pulmonary Venous Return [LMP28337]

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Image Details

Diagnostic Modality:

Specimen Photograph

Disease Category:

Diseases of Infancy and Childhood

Diagnosis:

Total Anomalous Pulmonary Venous Return

Species:

Human

Organ System:

Blood Vessels

Gender:

Male

Age:

11 days

Diagnosis Code:

ICD-10: Q26.2

Slide Contributor Information

Mrkonjic, M., Wilson, G.

Neonate developed jaundice and indrawing at 6 minutes of life. Echocardiography identified a Total Anomalous Pulmonary Venous Return with obstruction. Stenting of the vertical draining vein was performed without improvement. Patient deteriorated and died.

Clinical Discussion

Total Anomalous Pulmonary Venous Return (TAPVR) is a rare form (1-2%) of congenital heart disease. It is caused by the persistent communication between the pulmonary foregut plexus and umbilicovitelline system of veins. The pulmonary veins drain into a common venous confluence, which is then drained into the systemic circulation by a vertical vein. TAPVR can be subdivided into Supracardiac (drain into SVC or innominate vein), Cardiac (drain into coronary sinus or right atrium), Infracardiac (drain below diaphragm) or Mixed (a combination of the above). Definitive treatment is surgical repair, however stenting of obstructed vertical vein can be done in non-surgical candidates.

The image shows a venous confluence, located posterior to the left ventricle, that drains diminutive pulmonary veins. The heart is reflected superiorly. The vertical vein which drains the confluence into SVC is not present as it has been ligated in the dissection process.