H MIZOUNI, M JRAD,E MENIF Service d’Imagerie Médicale Hôpital la Rabta. TUNIS - TUNISIE

1. VALUE OF 64 SLICE MULTIDETECTOR CT IN ANOMALOUS PULMONARY VENOUS RETURN (APVR) IN NEONATES AND INFANTS H MIZOUNI, M JRAD,E MENIF Service d’Imagerie Médicale Hôpital la Rabta. TUNIS - TUNISIE Correspondance: Dr Habiba MIZOUNI habiba_mizouni@yahoo.fr

2. INTRODUCTION • Abnormal pulmonary venous return (APVC) correspond to an abnormal connection of one or all the pulmonary veins (PV) in the circulation systemic vein. They can be total or partial. • The purpose of this presentation is to illustrate aspects CT anatomy of different varieties of APVC in a pediatric population. • All cases which were verified surgically

3. NORMAL PULMONARY VENOUS DRAINAGE Figures of normal pulmonary venous drainage

4. Partial APVR • Is defined as a left-to-right shunt where one or more pulmonary veins drain into a systemic vein or the right atrium. • Represent 70% of total APVR • All PAPVRs are left-to-right shunts, but more than 50% of the pulmonary flow drains to the right side of the heart.

5. Partial APVR • Can be associated with other congenital cardiac anomalies ( 15%) ; asinus venosus atrial septal defect (ASD) located near the SVC orifice is the more frequent one • Clinical manifestations , such us :Dyspnea, fatigue, exercise intolerance, palpitations, syncope, atrial arrhythmias, right heart failure, and pulmonary hypertension, are rare.

6. Different types of partial APVR RIGHT LEFT PARTIAL APVR INFRACARDIAC CARDIAC SUPRA CARDIAC

7. Different types of partial APVR

8. Total AVPR (TAVPR) • Is a congenital heart defect in which the pulmonary veins fail to connect to the left atrium during cardiac development and instead drain into the right atrium or one of its venous tributaries. • Accounts for approximately 1.5% of all cardiovascular anomalies • Has an incidence of 1/15,000 live births • No sex predilection has been observed • All total AVPR are associated with a septal atrium defect

9. Total APVR • TAPVR usually has no effect during fetal development due to high pulmonary vascular resistance and shunting of blood through the foramen ovale. • At birth the pulmonary vascular resistance drops and increased blood flow to the right heart and lungs results in progressive congestive heart failure and pulmonary arterial hypertension. • If not surgically corrected, TAPVR has a high mortality rate in the first year of life.

10. Total AVPR • The pulmonary venous return can be : • Supra cardiac venous return 50% • Cardiac venous return 25% • Infra cardiac venous return 20 % • Mixed venous return 5%

11. Supra Cardiac TAVPR Infra Cardiac TAVPR Cardiac TAVPR

12. Multidetector CT technique • We perform a spiral acquisition without cardiac gating using 0.6- or 0.7-mm collimation • To minimize radiation exposure of the patients, we use an 80-kV tube with high pitch (1.2–1.5) • Mean radiation dose is estimated in millisieverts (mSv) from the dose-length product after correction for body size.

13. Multidetector CT technique • Non-ionic low-osmolar contrast medium (300 mg I/ml) in a dosage of 1.5- 2 ml/kg is injected into a peripheral vein ( 22 -24 G) at a low rate (0.5–1 ml/s) to avoid streak artifacts in the SVC. • None of the patients receive anesthesia. Sedation is given if needed. • Time from injection to scanning initiation was set at 40 s to ensure homogenous contrast at the venous phase. • Experienced staff and readers were required to manage children and reading images.

14. CASES

15. CASE 1 • Infant aged 14 mouths • Dyspnea • Echocardiography: • Enlargement of right heart chambers • Sinus venosus atrial septal defect (ASD)

16. 1b 1a • Fig.1a et 1b: • Right Superior pulmonary vein ( VPS Drt) draining into superior vena cava ( VCS) at the top of the azygos vein in maximum intensity projection (MIP) • The SVC is expanded above the mouth of the azygous

17. The Right Superior pulmonary vein is draining into superior vena cava Right superior pulmonary vein • Right supra cardiac partial APVRin volume rendering (VR) technique. • This type of APVC is usually associated with a CIA-type high sinus venosus

18. Case 2 • Daughter of 3 months • Dyspnea • Echocardiography : • CIA  • Doubt on the presence of AVRP

19. Right pulmonary venous return in the inferior vena cava Right pulmonary vein

20. Hypo vascularization of the right lung • Ipsilateral pulmonary artery of small caliber • Controlateral pulmonary artery of normal caliber

21. Systemic vasculature for the right lung from: • The celiac • In the abdominal aorta

22. Hypoplastic right lung field • Anomaly of the bronchial systematization • Hypoplastic right lung reduced to one lobe • Absence of visualization of the right scissure

23. Dextroversion heart

24. Right pulmonary venous return in the inferior vena cava • Dextroversion heart • Hypo vascularization of the right lung • Hypoplastic right lung field Scimitar syndrome

25. Case 3 • Daughter of one month • Dyspnea and cyanosis • Echo: CIA and dilatation of the right cavities

26. The right pulmonary veins draining into the right atrium Partial APVC  of the total right lung draining into the right atrium

27. Dilatation of right cavities

28. Double left superior vena cava draining ina dilated coronary sinus

29. Associated skeletal malformations

30. Cardiac partial APVR • The entire right lung draining in the right atrium • Form infrequent • Form almost always associated with aCIA • Sometimes associated malformationsskeletal

31. Case 4 • 8 years-old daughter • Dyspnea • Echo: dilated right cavitiesassociated with CIA • CT scan in search of APVR

32. The left superior pulmonary vein flows through a collector into the Venous trunk left brachiocephalic • Superior vena cava dilated Venous trunk left brachiocephalic Collector The superior left pulmonary vein SVC A supra cardiac partial AVPR of the superior left lung lobe

33. Venous trunk left brachiocephalic Collector Anomaly aortic arch  associated  :right subclavian artery in retroaortic course esophageal

34. Case 5 • Infants 3 months • Cyanosis and tachypnea • Echocardiography: • Dilatation of the RV with a paradoxical septal kinetics. • Expansion of the OD and trunk of the pulmonary artery • Severe PAH • Shunt right / left through a CIA • Absence of pulmonary veins abouchant in OG. • Viewing a supra cardiac collector vein  • Doubt on a second infra cardiac collector vein

35. A left supra cardiac AVPR Volume rendering  reconstruction (VR) showing theFirst collector (  ) draining the left superior pulmonary vein is abouchnat in the venous trunkinnominate ( )

36. Infra cardiac APVR Second collector draining the entire venous return right lung ( ) and the left lower lobe ( ): vertical path ( ) is abouchant the trunk door ( )• Absence of stenosis of the collector

37. Diagnosis :Mixed supra cardiac and infra cardiac total APVR • First collector  draining the left superior pulmonary vein is abouchant in the venous trunk innominate  • Second collector draining the entire venous return right lung and the left lower lobe in the portal vein.

38. Case 6 • Daughter of three months • Dyspnea • Chest X-Ray and Echocardiography: Dilatation of right cavities

39. Venous collector ( ) opening the posterior surface of the OD and the roof of coronary sinus

40. Venous collector ( ) opening in the faceposterior OD and the roof of the coronary sinusTotal cardiac APVC

41. Conclusion • Echocardiography remains the first-line choice for diagnostic imaging in all patients with pulmonary venous anomalies. • However, when echo diagnosis is inconclusive, CT and not catheterization should be considered the next imaging modality of choice which is less invasive and more precise. • Three-dimensional reformatting provided additional assistance with surgical planning.