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

2 INTRODUCTION  Abnormal pulmonary venous return (APVC) correspond to. They can be total or partial.  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  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,.  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  ; a located near the SVC orifice is the more frequent one  Can be associated with other congenital cardiac anomalies ( 15%) ; a sinus venosus atrial septal defect (ASD) located near the SVC orifice is the more frequent one  Clinical manifestations, such us :  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 PARTIAL APVR RIGHT LEFT SUPRA CARDIAC CARDIAC INFRA CARDIAC

7 Different types of partial APVR

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

9 Total APVR  TAPVR usually has due to high pulmonary vascular resistance and shunting of blood through the foramen ovale.  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  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  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 Infra Cardiac TAVPR Cardiac TAVPR Supra Cardiac TAVPR

12 Multidetector CT technique  We perform a spiral acquisition using 0.6- or 0.7-mm collimation  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  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 ml/kg is injected into a peripheral vein ( 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 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 1a 1b

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

18 Case 2  Daughter of 3 months  Dyspnea  Echocardiography : - CIA - 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 in a 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 a CIA   Sometimes associated malformations skeletal

31 Case 4  8 years-old daughter  Dyspnea  Echo: dilated right cavities associated 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 SVC The superior left pulmonary vein 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 the First collector ( ) draining the left superior pulmonary vein is abouchnat in the venous trunk innominate ( )

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 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 face posterior OD and the roof of the coronary sinus Total 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.