Congenital heart disease: basic

A. Cyanotic versus acyanotic presentations
B. Most common lesions
  1. Ventricular septal defect
  2. Patent ductus arteriosus
  3. Atrial septal defect
  4. Endocardial cushion defect
  5. Anomalous pulmonary venous connections
  6. Tetralogy of Fallot
  7. Pulmonary stenosis
  8. Obstruction of the aorta (coarctation and interruption of the aorta)
  9. Transposition of the great vessels (D- vs. L- transposition)
  10. Truncus arteriosus
  11. Aortic valvular disease
  12. Vascular ring
C. Post-operative assessment of the following procedures:
  1. Atrial switch for transposition of great arteries
    a) Senning and Mustard procedures
  2. Arterial switch for transposition of great arteries
    a) Jatene switch
    b) Rastelli procedure
    c) Lecompte maneuver
  3. Single ventricle repair
    a) Norwood 1 and modifications (Sano, hybrid Norwood)
    b) Damus Kaye Stansel anastomosis
  4. Superior cavopulmonary connection
    a) Bidirectional Glenn
    b) Kawashima procedures
  5. Total cavopulmonary connection:
    a) Fenestrated Fontan procedure
    b) Modifications
    c) Atriopulmonary
    d) Lateral tunnel
    e) Extra-cardiac Fontan procedure
  6. Pulmonary blood flow augmentation as initial palliation for CHD
    a) Blalock-Taussig (classic and modified)
    b) Waterston
    c) Pott’s shunts
  7. Ross
    a) Ross-Konno procedures of the LVOT
  8. Total correction of Tetralogy of Fallot, pulmonary atresia with VSD and truncus arteriosus: including RV-PA homograft placement and unifocalization
  9. Procedures involving the aortic arch, including vascular ring repair, coarctation repair, arch augmentation, and arch advancement procedures
  10. Anomalous pulmonary vein correction
    a) TAPVR repair
    b) Warden procedure
  11. Repair of anomalous coronary artery
D. Most common indications for cardiac MRI in the setting of congenital and acquired pediatric cardiovascular disease
  1. Corrected Tetralogy of Fallot: monitor RV size, biventricular function, and degree of pulmonary regurgitation to determine timing of pulmonary valve replacement
  2. Presence and severity of RVOT and branch pulmonary artery stenosis s/p Tetralogy of Fallot repair or after RV-PA conduit placement
  3. Repaired coarctation of the aorta: to determine presence and severity of recurrent coarctation
  4. Monitor severity of aortic root and ascending aortic dilatation in conditions like connective tissue disorder, bicuspid aortic valve, s/p Ross procedure, s/p arterial switch procedure.
  5. Neonatal imaging prior to initial palliation to diagnose anomalous pulmonary venous return, source of pulmonary blood flow, aortic arch obstruction or cause of vascular-mediated airway compromise
  6. Preoperative evaluation prior to stage 2 and stage 3 Norwood as an alternative to cardiac catheterization
  7. Post-operative evaluation after atrial or arterial switch for transposition of great arteries to screen for baffle leaks, obstruction, aortic root dilation and status of branch pulmonary arteries
  8. Screen for occult left-right shunt or anomalous pulmonary venous return in the setting of unexplained RV volume overload.
  9. Myocardial disorders like myocarditis, arrhythmogenic RV cardiomyopathy, LV non-compaction and other cardiomyopathies, including Duchenne’s muscular dystrophy
  10. Primary cardiac tumors
E. Situs anomalies (asplenia and polysplenia)
                   

NASCI Curriculum

Congenital heart disease: basic

A. Cyanotic versus acyanotic presentations
  • Acyanotic lesions with increased pulmonary blood flow. (No abstract available)
    Stevenson JG.
    Pediatr Clin North Am. 1978; 25(4):743-58.
B. Most common lesions
  1. Ventricular septal defect
 
 
 
 
 
  2. Patent ductus arteriosus
 
  • Patent Ductus Arteriosus
    Douglas J. Schneider and John W. Moore
    Circulation. 2006;114:1873-1882, doi:10.1161/CIRCULATIONAHA.105.592063
 
  • Patent ductus arteriosus
    Jeffrey C Milliken, MD; Chief Editor: Stuart Berger, MD (Original by Neisch SR).
    eMedicine July 23, 2009
 
 
  3. Atrial septal defect
 

Case in Point Case

Blind Spot
Y. Reyna, MD; R Shah, MD; J. Kirsch, MD

Case in Point Case

Unroofing the diagnosis
G. Cannavale, MD; C. Higgins, MD; K. Ordovas, MD

Case in Point Case

Persistent Remnant
T. Mousa, MD; T. Kerwin, MD

 

Case in Point Case

A Circumspect Defect
A. Chaturvedi, MD; T. Dubinsky, MD; P. Cawley, MD; K. Huehnergarth, MD; J. Maki, MD, PhD

 
 
 
 
  4. Endocardial cushion defect
 
 
 
 
  5. Anomalous pulmonary venous connections
 

Case in Point Case

Persistent Remnant
T. Mousa, MD; T. Kerwin, MD

 
 
 
 
  6. Tetralogy of Fallot
 
 
 
 
 
  7. Pulmonary stenosis
 
 
 
 
  • The Pulmonary Valve (Abstract)
    Kevin P. Fitzgerald, Michael J. Lim
    Cardiology Clinics - May 2011 (Vol. 29, Issue 2, Pages 223-227, DOI: 10.1016/j.ccl.2011.01.006)
  8. Obstruction of the aorta (coarctation and interruption of the aorta)
 
 
 
 
 
 
  9. Transposition of the great vessels (D- vs. L- transposition)
 

Case in Point

Sheer Coincidence
W. Wróbel MD, PhD; M. Sosnowski MD, PhD; M. Trusz-Gluza MD, PhD

Case in Point

Sympathetic Dysfunction
Y. Akutsu, MD, PhD, FACP, T. Gokan, MD, PhD, and Y. Kobayashi, MD, PhD

Case in Point

Rare Association
A. Patrignania, A. D’Aromaa, S. Cicognaa

 

Case in Point

Transposition Inquisition
A. Zurick, III, MD; V. Menon, MD

 
 
 
 
  • Transposition of the great arteries (Abstract)
    Squarcia, Umberto; Macchi, Chiara
    Current Opinion in Pediatrics: October 2011 - Volume 23 - Issue 5 - p 518–522 doi: 10.1097/MOP.0b013e32834aa551
 
  10. Truncus arteriosus
 
 
 
 
  • Aortopulmonary Window (Abstract)
    Michael E. Barnesa, Michael E. Mitchella, b, James S. Tweddell
    Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual Volume 14, Issue 1, 2011, Pages 67-74 doi:10.1053/j.pcsu.2011.01.017 |
  11. Aortic valvular disease
 
 
 
 
 
 
 
  12. Vascular ring
 
 
 
  • Trends in vascular ring surgery
    Backer, Carl L., Mavroudis, Constantine, Rigsby, Cynthia K., Holinger, Lauren D.
    J Thorac Cardiovasc Surg 2005 129: 1339-1347 
 
C. Post-operative assessment of the following procedures:
  1. Atrial switch for transposition of great arteries
    a) Senning and Mustard procedures
   
   
   
   
   
  2. Arterial switch for transposition of great arteries
    a) Jatene switch
   
   
   
    b) Rastelli procedure
   
   
    c) Lecompte maneuver
   
   
  3. Single ventricle repair
    a) Norwood 1 and modifications (Sano, hybrid Norwood)
   
   
   
    b) Damus Kaye Stansel anastomosis
   
   
   
   
  4. Superior cavopulmonary connection
    a) Bidirectional Glenn
   
   
  • Congenital Heart Disease and Multi-modality Imaging (Abstract)
    Rajesh Puranik PhD, Vivek Muthurangu MD, David S. Celermajer PhD, Andrew M. Taylor MD
    Heart, Lung and Circulation Volume 19, Issue 3, March 2010, Pages 133-144  doi:10.1016/j.hlc.2010.01.001
   
    b) Kawashima procedures
   
   
   
  5. Total cavopulmonary connection:
    a) Fenestrated Fontan procedure
   
   
   
   
  • Fontan Operation and the Single Ventricle (Abstract)
    AboulHosn, J. A., Shavelle, D. M., Castellon, Y., Criley, J. M., Plunkett, M., Pelikan, P., Dinh, H. and Child, J. S.
    Congenital Heart Disease, 2: 2–11. doi: 10.1111/j.1747-0803.2007.00065.x 
   
  • Liver Disease in the Patient with Fontan Circulation (Abstract)
    Fred M. Wu MD, FACC, Chinweike Ukomadu MD, PhD, Robert D. Odze MD, FRCPc, Anne Marie Valente MD, FACC, John E. Mayer, Jr. MD, Michael G. Earing, MD 
    Congenital Heart Disease Volume 6, Issue 3, pages 190–201, May/June 2011  DOI: 10.1111/j.1747-0803.2011.00504.x 
    b) Modifications
   
   
   
    c) Atriopulmonary
   
   
    d) Lateral tunnel
   
   
  • Long-term results of the lateral tunnel Fontan operation
    Stamm, Christof, Friehs, Ingeborg, Mayer, John E., Jr, Zurakowski, David, Triedman, John K., Moran, Adrian M., Walsh, Edward P., Lock, James E., Jonas, Richard A., del Nido, Pedro J.
    J Thorac Cardiovasc Surg 2001 121: 28-41
   
    e) Extra-cardiac Fontan procedure
   
   
  6. Pulmonary blood flow augmentation as initial palliation for CHD
    a) Blalock-Taussig (classic and modified)
   
   
   
    b) Waterston
   
   
   
    c) Pott’s shunts
   
   
  7. Ross
    a) Ross-Konno procedures of the LVOT
   
   
  8. Total correction of Tetralogy of Fallot, pulmonary atresia with VSD and truncus arteriosus: including RV-PA homograft placement and unifocalization
 
 
 
  9. Procedures involving the aortic arch, including vascular ring repair, coarctation repair, arch augmentation, and arch advancement procedures
 
 
 
 
  10. Anomalous pulmonary vein correction
    a) TAPVR repair
   
  • Scimitar Syndrome / Clinical Perspective : A European Congenital Heart Surgeons Association (ECHSA) Multicentric Study
    Vladimiro L. Vida, Massimo A. Padalino, Giovanna Boccuzzo, Erjon Tarja, Hakan Berggren, Thierry Carrel, Sertaç Çiçek, Giancarlo Crupi, Duccio Di Carlo, Roberto Di Donato, José Fragata, Mark Hazekamp, Viktor Hraska, Bohdan Maruszewski, Dominique Metras, Marco Pozzi, Rene Pretre, Jean Rubay, Heikki Sairanen, George Sarris, Christian Schreiber, Bart Meyns, Tomas Tlaskal, Andreas Urban, Gaetano Thiene, and Giovanni Stellin
    Circulation. 2010;122:1159-1166, published online before print September 7 2010, doi:10.1161/CIRCULATIONAHA.109.926204
   
   
    b) Warden procedure
   
   
  11. Repair of anomalous coronary artery
 
 
 
D. Most common indications for cardiac MRI in the setting of congenital and acquired pediatric cardiovascular disease
  1. Corrected Tetralogy of Fallot: monitor RV size, biventricular function, and degree of pulmonary regurgitation to determine timing of pulmonary valve replacement
 
 
 
  2. Presence and severity of RVOT and branch pulmonary artery stenosis s/p Tetralogy of Fallot repair or after RV-PA conduit placement
 
 
  3. Repaired coarctation of the aorta: to determine presence and severity of recurrent coarctation
 
 
 
 
 
  4. Monitor severity of aortic root and ascending aortic dilatation in conditions like connective tissue disorder, bicuspid aortic valve, s/p Ross procedure, s/p arterial switch procedure.
 
  5. Neonatal imaging prior to initial palliation to diagnose anomalous pulmonary venous return, source of pulmonary blood flow, aortic arch obstruction or cause of vascular-mediated airway compromise
 
 
  6. Preoperative evaluation prior to stage 2 and stage 3 Norwood as an alternative to cardiac catheterization
 
 
  7. Post-operative evaluation after atrial or arterial switch for transposition of great arteries to screen for baffle leaks, obstruction, aortic root dilation and status of branch pulmonary arteries
 
 
 
  8. Screen for occult left-right shunt or anomalous pulmonary venous return in the setting of unexplained RV volume overload.
 
 
 
  9. Myocardial disorders like myocarditis, arrhythmogenic RV cardiomyopathy, LV non-compaction and other cardiomyopathies, including Duchenne’s muscular dystrophy
 

Case in Point
Rare Association
A. Patrignania, A. D’Aromaa, S. Cicognaa

 
 
  10. Primary cardiac tumors
 
 
 
E. Situs anomalies (asplenia and polysplenia)