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TSDA Congenital Curricula for the Week of October 15, 2012
Week 13 of 32
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Congenital Curricula, please forward contact information to [email protected].
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Welcome to Week 13 of the TSDA Congenital Curricula. The Congenital Curricula is based on an 32-week lesson
plan, and you can expect to receive e-mail messages every Thursday for the upcoming week, with the exception of
holiday weekends. The weekly assignments are a supplement to your program curriculum and include basic
readings for congenital fellows, faculty, and others interested in congenital heart surgery. TSDA hopes the
Congenital Curricula expands your current knowledge base and enhances your learning experience.
TOPIC: Pulmonary Atresia with Ventricular Septal Defect and MAPCAs
Outline
I. Morphology
a. Confluent Central PAs
b. Nonconfluent Central PAs
c. MAPCAs redundant
d. MAPCAs sole source of segmental pulmonary bloodflow
e. Associated lesions
i. Right aortic arch
ii. Abnormal coronary artery branching patterns
iii. Aortic insufficiency
II. Pathophysiology/Natural History
a. Right-to-left shunt at VSD
b. Left-to-right shunt with MAPCAs
c. Diagnosis/Determination of operability
i. Echocardiogram
ii. Cardiac Catheterization
1. Central PAs
2. Nakata/McGoon indices
3. Coronary artery pattern
4. Aortopulmonary collaterals
iii. CT/MRIy
III. Surgical repair
a. Indications/Timing
b. Conduct of Operation ( Including Cannulation, CPB, Myocardial Protection, Approach)
i. Palliative operations to promote central PA growth
1. Central shunt
2. RV-PA shunt
3. Transannular RVOT patch
ii. Unifocalization
iii. Unifocalization with complete repair
iv. Complete repair with RV-PA conduit
c. Associated Technical Considerations
i. Decision for VSD closure/fenestration
d. Post-operative management
e. Outcomes
IV. Controversies
a. Long-term efficacy of unifocalization
b. Single stage vs staged unifocalization and complete repair
c. Techniques of unifocalization
Talk:*
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Pulmonary Atresia with Intact Septum or VSD - John Mayer (Children’s Hospital Boston)
*If you experience technical difficulties and are sometimes unable to load TSDA’s talks, and you use Windows Vista
or Windows 7, TSDA suggests you install a patch to make the files playable on your computer. This patch has been
tested by TSDA staff and volunteers, and only takes a few minutes to install. Click here for instructions on how to
install this patch. You may also want to check with your institution’s IT administrator regarding special
considerations or requirements of your network.
Readings:
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Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals, Chapter 61, pp
1141-1150, by MacDonald MJ and Hanley FL, in The Johns Hopkins Manual of Cardiothoracic Surgery,
New York, McGraw-Hill, 2007.
Congenital Heart Surgery Nomenclature and Database Project: Pulmonary Atresia-Ventricular Septal
Defect, by Tchervenkov CI and Roy N, in The Annals of Thoracic Surgery, April 2000; 69:S97-S105.
The functional intraoperative pulmonary blood flow study is a more sensitive predictor than preoperative
anatomy for right ventricular pressure and physiologic tolerance of ventricular septal defect closure after
complete unifocalization in patients with pulmonary atresia, ventricular septal defect, and major
aortopulmonary collaterals, by Honjo O, et al., in Circulation, 2009; 120 (Suppl 11):S46-52.
Pulmonary atresia, VSD, and MAPCAs: repair without unifocalization, by Brizard CP, et al., in Seminars in
Thoracic and Cardiovasuclar Surgery Pediatric Cardiac Surgery Annual, 2009:139-44.
Clinical results of staged repair with complete unifocalization for pulmonary atresia with ventricular septal
defect and major aortopulmonary collateral arteries, by Ishibashi N, et al., in European Journal of
Cardiothoracic Surgery, 2007;32:202-8.
Surgical management of pulmonary atresia with ventricular septal defect and major aortopulmonary
collaterals: a protocol-based approach, by Malhotra SP and Hanley FL, in Seminars in Thoracic and
Cardiovasuclar Surgery Pediatric Cardiac Surgery Annual, 2009:139-44.
Determinants of outcome after surgical treatment of pulmonary atresia with ventricular septal defect and
major aortopulmonary collateral arteries, by Carotti A, et al., in Journal of Thoracic and Cardiovascular
Surgery, 2010;140:1092-1103.
Melbourne shunt promotes growth of diminutive central pulmonary arteries in patients with pulmonary
atresia, ventricular septal defect, and systemic-to-pulmonary collateral arteries, by Mumtaz MA, et al, in
The Annals of Thoracic Surgery, 2008;85:2079-83.
ADDITIONAL TSDA RESOURCES
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The TSRA Review of Cardiothoracic Surgery is available as a free PDF, e-Book, or as a print book. The
textbook, edited by the Thoracic Surgery Residents Association and authored by more than 50 residents, is
a valuable resource for CT surgery residents, faculty, and others interested in CT surgery education.
The McGraw-Hill Companies, Inc., publishers of The Johns Hopkins Manual of Cardiothoracic Surgery, has
graciously made available sections of this important textbook to TSDA Weekly Curricula subscribers. TSDA
will post three chapters for a three month period over the course of one year. Check back frequently for
new postings. The Johns Hopkins Manual of Cardiothoracic Surgery can be ordered at Amazon.com.
New congenital, cardiac, and general thoracic talk pdf files now posted on TSDA.org. Visit the New Talks
page to view new talks and download the pdf file.
Attention program directors, faculty, residents, and fellows: If you know of a congenital curriculum topic for
which you would like to create a talk, the TSDA Curriculum Committee encourages such contributions.
Instructions for creating a talk can be found on TSDA.org.
NEWS & INFORMATION
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Contact TSDA at [email protected] if you have comments or suggestions for the TSDA Congenital Curricula.
The next message will be sent on October 18 for the week of October 22.
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