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Transcript
Anomalies of Pulmonary
Arteries and Veins
NASCI 37th Annual Meeting
Pediatric/Congenital Heart Disease Boot Camp
Himesh Vyas, MD
Assistant Professor of Cardiology
University of Arkansas for Medical Sciences
Arkansas Children's Hospital,
Little Rock, AR
• No financial disclosures
• Off label use of gadolinium in children
Learning Objectives
z Recognize
common anomalies of:
z Pulmonary
arteries
z Pulmonary veins
z Describe
utility of cardiac MRI in
evaluation
Pulmonary Artery
Anomalies
Pulmonary Artery Anomalies
PA
anomalies
Anomalies of
number
Anomalies of
location
Anomalies of
size
Anomalies of Number
Unilaterally Absent PA
z
Associated with
congenital heart
disease:
z
z
Normal LPA
L
R
Absent LPA with
tetralogy of Fallot
Distal pulmonary
artery often normally
distributed
Ao LA
Absent RPA
Ductal Origin of Branch PA
CTA
RPA
from
MPA
Ductal origin of LPA
PDA
LPA
Anomalies of Location
Origin of LPA from RPA (PA sling)
MPA
Ao
RPA
Trachea
LPA
Fiore A. C. et al.; Ann Thorac Surg
2005;79:38-46
Aberrant LPA
posterior to trachea
Origin of LPA from RPA (PA sling)
z
Airway anomalies
common
z Complete
membranous rings
z Tracheomalacia
z Abnormal lung
lobulation
z Bronchus suis
CTA
Anomalies of Location
Aortic Origin of Branch PA
Branch pulmonary
artery origin from the
dorsal ascending aorta
z Right >> left
z Associations:
z
z
z
z
Tetralogy of Fallot
Patent ductus arteriosus
“Hemitruncus”
Asc. Ao
MPA
RPA
LPA
Aortic Origin of RPA
CTA
MPA
LPA
Aorta
RPA
LA
Anomalies of Size
Branch PA Stenosis/Hypoplasia
z
z
Isolated
Associated congenital
heart disease
z
z
z
Tetralogy of Fallot
Congenital rubella
Williams syndrome
z
z
RPA
Ao
LPA
Supravalvar
Branch PA
Bilateral branch PA stenosis –
Williams syndrome
Pulmonary Atresia with Ventricular
Septal Defect (PA-VSD)
z Intra-cardiac
anatomy similar to
tetralogy of Fallot
z Pulmonary blood supply variable
z PDA
z Major
aortopulmonary collaterals
PA-VSD: Goals of MRI
• Sources of pulmonary blood flow
• PA size and confluence
• Collateral communication with PAs
R
L
R
L
PDA
Confluent PAs
Collateral
Pulmonary Vein
Anomalies
Pulmonary Vein Embryology
LUNG
BUDS
RA
LA
SPLANCHNIC
VEINS
PULMONARY
XXVEINS
LA
RA
RV
LV
DEVELOPING HEART
z
z
z
Lung buds from foregut – splanchnic drainage
Left atrium connects to PV
Regression of primitive splanchnic connections
Classification of PV Anomalies
Totally anomalous pulmonary venous
connection (TAPVC)
z Partially anomalous pulmonary venous
connection (PAPVC)
z Sinus venosus defect
z Cor triatriatum
z Pulmonary vein stenosis/atresia
z
TAPVC
z Types:
LPA
Aorta
z Supracardiac
z Cardiac
RPA
z Infracardiac
z Mixed
Lower
PVs
Confluence of PV draining into
posterior right atrial wall
PAPVC
Common types:
z Left PV to innominate vein
z Right PV to SVC
z With
sinus venosus defect
z Without sinus venosus defect
z Right
PV to IVC (scimitar syndrome)
Left PV to Innominate Vein
Innominate
vein
Ascending
vertical vein
LPA
RPA
LA
Normal left
lower PV
Scimitar syndrome
z
z
z
z
Shadow on X-ray
resembles Turkish sword
(scimitar)
Hypoplasia of right lung
Pseudo-sequestration of
right lower lobe of lung
Aortopulmonary
collaterals
Mesoposed heart, hypoplastic right
lung and scimitar sign (arrows)
Scimitar Syndrome
RPA
LPA
Dextroposed heart, large
LPA, small RPA
Descending anomalous
vein (arrow)
Sinus Venosus Defect
Unroofing of right
upper PV to SVC
z Defect is dilated
orifice of right upper
pulmonary vein
SVC
z PAPVC of right PV to
SVC (blue arrow)
z
RA
LA
Cor Triatriatum
Membrane in LA
(black arrow)
z Proximal PV
“chamber”
z Stenotic connection
to LA
z
RV
RA
LV
Pulmonary LA
venous
chamber
Unilateral PV Atresia
No
perfusion to
left lung
Time resolved Gd-MRA
Atretic left PV
Post-Operative Problems
Limitations of Echocardiography
Branch pulmonary
arteries by 2D echo
Post-operative
deterioration of
acoustic windows!
Post-Operative PA Stenosis
RPA
RPA
LPA
conduit
LPA
conduit
Severe stenosis of RPA after repair of truncus
arteriosus
Post-Operative PV Stenosis/Atresia
May follow
pulmonary vein
surgery
z Progressive
z Focal or diffuse
z
LPA
RPA
Bilateral PV stenosis
Warden Procedure for Repair of
Sinus Venosus Defect
SVC to
RA
appendage
RPV to LA
baffle
Conclusion
z
Reviewed important anomalies of
z Pulmonary
arteries
z Pulmonary veins
z
MRI particularly well suited because:
z Accurate
anatomical details
z Gadolinium
MRA
z Black/bright blood imaging
z Functional
information
Acknowledgements
z ACH
cardiac MRI team:
z S.
Bruce Greenberg
z Sadaf Bhutta
z Danny Meek
z Bruce Stockmeyer
z Raj
Krishnamurthy – contributing
many of the images in this talk