Transcript Anatomy and Physiology

Pulmonary
Atresia with
MAPCAs:
Anatomy and
Physiology
Angela Blankenship, RN, MS, CPNP AC/PC
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Embryology
• Development of the arterial branches
starts about day 27 of gestation
• Dual blood supply between the 27th and
50th days
• Segmental arteries involute into PAs and
branches from the descending aorta
become comparatively smaller and
develop bronchial arteries
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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
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Evaluation at birth
• Prostaglandins
• Catheterization
• Chest CT
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Clinical Presentation
• Varying degrees of cyanosis
• Murmur
• Occasional late presentation
– Some reports as late as 6 months
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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
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Pulmonary Atresia
Normal Heart
1) Atretic Pulmonary Valve
www.PedHeart.com
Pulmonary Artery Morphology
• PDA
• MAPCA- variability is dependent upon:
– Number
– Origin
– Size
– Distribution
– Connection to Main Pulmonary Arteries
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Pulmonary Artery Morphology
• PDA and MAPCAs may be present in the
same patient
• Rarely will PDA and MAPCAs coexist in
the same lung
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What is a MAPCA?
• MAPCA= Multiple Aorto-Pulmonary Collateral
• Systemic to pulmonary collaterals
• Other nomenclature:
– Bronchial Arteries
– Systemic Arteries
– Persistent Segmental Arteries
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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
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Pulmonary Atresia PA
Configuration
• Pulmonary atresia with confluent PAs
• Pulmonary atresia with diminuitive PAs
• Pulmonary atresia with absent PAs
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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
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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.
………………..……………………………………………………………………………………………………………………………………..
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.
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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
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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.
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Pulmonary Atresia with
Absent PAs
•
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•
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Atresia of the pulmonary valve
No main PA
No right or left PA
All Pulmonary blood flow is supplied via
MAPCA’s
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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
………………..……………………………………………………………………………………………………………………………………..
Physiology
• All pulmonary blood flow is derived from
systemic circulation
– Pulmonary and systemic saturations will be equal
– May have signs of under-circulation or overcirculation 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
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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
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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
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Problems Over Time
• Common areas of stenosis
– At the site of aortic insertion
– At the site of intrapulmonary anastomosis
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Problems Over Time
• Pulmonary hypertension
– Large collaterals
– No protective stenosis
– Under high pressure
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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?
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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
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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
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References
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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 2014
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Thank You
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