Reported adult case of supracardiac TAPVR with additional talk about the other types

1. ‫الرحيم‬ ‫الرحمن‬ ‫هللا‬ ‫بسم‬

2.  30 year old female have an ASD detect on
echocardiography study ,CT pulmonary
angiography done to evaluate any cardiac or
vascular additional finding .

3. Pulmonary Ct angiography

6. Chest X-ray

9. Diagnosis

10. Total anomalous pulmonary venous
return /supra cardiac type

11. Definition:
 Total anomalous pulmonary venous
return (TAPVR) is a rare congenital
malformation in which all
four pulmonary veins do not connect
normally to the left atrium. Instead the
four pulmonary veins drain abnormally to
the right atrium by way of an abnormal
(anomalous) connection.

12.  Patients with obstructed TAPVR (total anomalous
pulmonary venous return) are extremely ill soon after birth.
These children are severely cyanotic. They also have
respiratory problems, with rapid breathing, grunting and
retractions of the rib cage muscles. Often such infants may
be initially thought to have pneumonia or other respiratory
diseases of the newborn, until an accurate cardiac
diagnosis is made.

13.  If obstruction to pulmonary venous return is not present,
children with total anomalous pulmonary venous
return may not have many symptoms. There may be
some rapid or difficult breathing. There is often cyanosis,
but it may be mild and difficult to detect.

14. Different type of TAPVR are seen .In
our case the anomaly is of supracardiac
type in which all the pulmonary veins
are drained to the SVC via common
trunk.

15. Findings:

16. Chest x ray reveal widening of the
mediastinum with slightly prominent
Bronchovascular marking .

18. X- ray reveal the typical snowman
apprearance

19. Selected image from the axial scan
shows a large VSD with enlarged right
atrium .

21. Looped vascular trunk formed by
united of all pulmonary veins that
drained to the SVC.

23. Reported similar case :
 24-year-old man, presenting with fever, headache,
vomiting and seizures, subsequently diagnosed with
cyanotic congenital heart disease. Evaluation revealed non-
obstructive supracardiac total anomalous pulmonary
venous connection (TAPVC) as the underlying disorder.
Surprisingly, the patient denied any past cardiac
symptoms. Presentation in adulthood is infrequent for
TAPVC, and primary manifestation with cerebral abscesses
is still more unusual.

24. vertical vein (VV), innominate vein (IV) and the superior venacava (SVC)

25. Multiple cerebral abscesses

26. Other types of TAPVR
Infracardiac Total Anomalous Pulmonary Venous
Return:
 The pulmonary veins drain to the right atrium via the hepatic (liver)
veins and inferior vena cava. In this type, the pulmonary veins join
together behind the heart and then typically drain downwards,
connecting to the liver's portal vein system. They then drain through
the vascular bed of the liver and enter the right atrium from the hepatic
veins.

28. Cardiac Total Anomalous Pulmonary Venous Return:
 The pulmonary veins come together behind the heart and then drain
to the right atrium through the coronary sinus. The coronary sinus is
the vein that normally returns blood from the heart muscle itself
back to the right atrium after its oxygen has been depleted. The
coronary sinus drains directly into the right atrium.

30. Mixed total anomalous pulmonary venous return:
when some veins drain in a supracardiac and
others in an infracardiac pattern

32. Radiopaedia was the source of most images

33. Thank you
reported by dr husam HY