1. ………………..…………………………………………………………………………………………………………………………………….. Pulmonary Atresia with MAPCAs: Anatomy and Physiology Angela Blankenship, RN, MS, CPNP AC/PC

2. ………………..…………………………………………………………………………………………………………………………………….. Embryology Development of the arterial branches starts about day 27 of gestation Dual blood supply between the 27 th and 50 th days Segmental arteries involute into PAs and branches from the descending aorta become comparatively smaller and develop bronchial arteries

3. ………………..…………………………………………………………………………………………………………………………………….. Embryology Disruption of the forming vessels occurs early in life When the normal connection between heart and pulmonary arties is disrupted blood supply to the lungs is maintained via PDA or systemic to pulmonary collaterals

4. ………………..…………………………………………………………………………………………………………………………………….. Evaluation at birth Prostaglandins Catheterization Chest CT

5. ………………..…………………………………………………………………………………………………………………………………….. Clinical Presentation Varying degrees of cyanosis Murmur Occasional late presentation –Some reports as late as 6 months

6. ………………..…………………………………………………………………………………………………………………………………….. Anatomy of PA MAPCAs A rare and complex lesion with great morphologic variability Tetralogy of fallot-like intra-cardiac morphology Pulmonary valve atresia Highly variable pulmonary artery morphology

7. Pulmonary Atresia 1) Atretic Pulmonary Valve Normal Heart www.PedHeart.com

8. ………………..…………………………………………………………………………………………………………………………………….. Pulmonary Artery Morphology PDA MAPCA- variability is dependent upon: –Number –Origin –Size –Distribution –Connection to Main Pulmonary Arteries

9. ………………..…………………………………………………………………………………………………………………………………….. Pulmonary Artery Morphology PDA and MAPCAs may be present in the same patient Rarely will PDA and MAPCAs coexist in the same lung

10. ………………..…………………………………………………………………………………………………………………………………….. What is a MAPCA? MAPCA= Multiple Aorto-Pulmonary Collateral Systemic to pulmonary collaterals Other nomenclature: –Bronchial Arteries –Systemic Arteries –Persistent Segmental Arteries

11. ………………..…………………………………………………………………………………………………………………………………….. What is a MAPCA? Histologically similar to systemic arteries –Demonstrate reactivity –Prone to stenosis Also compared to bronchial arteries Do not grow as true PAs

14. ………………..……………………………………………………………………………………………………………………………………..

21. Pulmonary Atresia PA Configuration Pulmonary atresia with confluent PAs Pulmonary atresia with diminuitive PAs Pulmonary atresia with absent PAs

22. ………………..…………………………………………………………………………………………………………………………………….. Pulmonary Atresia with Confluent PAs Atresia of the pulmonary valve Confluence of both the left and right pulmonary arteries Blood supply to the PAs is from a PDA

23. ………………..…………………………………………………………………………………………………………………………………….. MacDonald, Malcolm J.; Hanley, Frank L.; Murphy, Daniel J. Pulmonary Atresia with Ventricular septal defect, Cardiology.Published January 1, 2010. Volume 138, Issue 3. Pages 1495-1506. © 2010.

24. ………………..…………………………………………………………………………………………………………………………………….. MacDonald, Malcolm J.; Hanley, Frank L.; Murphy, Daniel J. Pulmonary Atresia with Ventricular septal defect, Cardiology. Published January 1, 2010. Volume 138, Issue 3. Pages 1495-1506. © 2010.

25. ………………..…………………………………………………………………………………………………………………………………….. Pulmonary Atresia with Diminuitive PAs Atresia of the pulmonary valve Both left and right PAs are diminutive but still present. PAs connect to variable numbers of broncho-pulmonary segments The majority of pulmonary blood flow is supplied through MAPCA’s

26. ………………..…………………………………………………………………………………………………………………………………….. MacDonald, Malcolm J.; Hanley, Frank L.; Murphy, Daniel J. Pulmonary Atresia with Ventricular septal defect, Cardiology. Published January 1, 2010. Volume 138, Issue 3. Pages 1495-1506. © 2010.

27. ………………..…………………………………………………………………………………………………………………………………….. Pulmonary Atresia with Absent PAs Atresia of the pulmonary valve No main PA No right or left PA All Pulmonary blood flow is supplied via MAPCA’s

28. ………………..…………………………………………………………………………………………………………………………………….. MacDonald, Malcolm J.; Hanley, Frank L.; Murphy, Daniel J. Pulmonary Atresia with Ventricular septal defect, Cardiology. Published January 1, 2010. Volume 138, Issue 3. Pages 1495-1506. © 2010

29. ………………..…………………………………………………………………………………………………………………………………….. Physiology All pulmonary blood flow is derived from systemic circulation –Pulmonary and systemic saturations will be equal –May have signs of under-circulation or over- circulation of pulmonary blood flow (PBF) –May also have signs of both under-circulation and over-circulation in the same patient due to the various sources of PBF to different areas of the lung

30. ………………..…………………………………………………………………………………………………………………………………….. Physiology Saturations > 85% indicate a Qp:Qs of 2:1 Saturations in the 70’s indicate a Qp:Qs of 1 or less –High oxygen sats may indicate pulmonary overcirculation and eventual CHF –Decreasing sats in the neonatal period may indicate ductal closure

31. ………………..…………………………………………………………………………………………………………………………………….. Problems over time Stenosis –All MAPCAs are prone to stenosis –Studies show anywhere from 40-75% develop stenosis –Stenosis may be in one vessel or many –Likely to require catheter intervention

32. ………………..…………………………………………………………………………………………………………………………………….. Problems Over Time Common areas of stenosis –At the site of aortic insertion –At the site of intrapulmonary anastomosis

33. ………………..…………………………………………………………………………………………………………………………………….. Problems Over Time Pulmonary hypertension –Large collaterals –No protective stenosis –Under high pressure

34. ………………..…………………………………………………………………………………………………………………………………….. What does this mean at the bedside? You must know the anatomy of the patient’s pulmonary blood supply to understand the physiology –Will the patient be de-saturated or normally saturated? –Will the patient develop symptoms of heart failure?

35. ………………..…………………………………………………………………………………………………………………………………….. What does this mean at the bedside? The more MAPCA’s the patient has, the more variability there will be in PBF Most patients will need a surgical palliation or repair within the first days to months of life depending on the source of PBF

36. ………………..…………………………………………………………………………………………………………………………………….. Conclusion A rare lesion with great variability Delineation of pulmonary blood supply is crucial to management and planning surgical interventions Close follow up even after intervention due to risk for stenosis and pulmonary hypertension

37. ………………..…………………………………………………………………………………………………………………………………….. References MacDonald, Malcolm J.; Hanley, Frank L.; Murphy, Daniel J. Pulmonary Atresia with Ventricular septal defect, Cardiology. 2010;138- 3:1495-1506. Haworth SG, Macartney FJ. Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Br Heart J. 1980;44:14-24. Learn C, Phillips A, Chisolm J, Hill S, Cheatham J, Winch P, Galantowicz M, Holzer R. Pulmonary Atresia with Ventricular Septal Defect and Multifocal Pulmonary Blood Supple: Does an dIntensice Interventional Approach Improve the Outcome? Congenital Heart Disease 2012;7:111-121. Lofland G. An Overview of pulmonary atesia, ventricular septal defect, and multiple aorto pulmonary collateral arteries. Progress in Pediatric Cardiology 2009; 26: 65-70. Watanabe N, Mainwaring R, Reddy M, Palmon M, Hanley F. Early Complete Repair of Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals. Annals of Thoracic Surgery 2014:97:909-15. Mainwaring R, Reddy M, Peng L, Kuan C, Palmon M, Hanley, F. Hemdynamic Assessment After complete Repari of Pulmonary Atresia with Major Aortopulmonary Collaterals 2013;95:1397-402 Norgaard MA, Alphonso N, Cochrane AD, Menahem S, Brizard CP, Ukedem Y. Major aortopulmonary collateral arteries of patients with pulmonary atresia and ventricular septal defect are dilated bronchial arteries. Eur J Cardiothoracic Surg. 2006;29:653-658. WWW.PedHeart.com- accessed December 2014WWW.PedHeart.com-

38. ………………..…………………………………………………………………………………………………………………………………….. Thank You