eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiothoracic Surgery

Pulmonary Atresia With Ventricular Septal Defect: Follow-up

Author: Edwin Rodriguez-Cruz, MD, Assistant Professor, Department of Pediatrics, San Juan Bautista Medical School and Medical Center; Consulting Interventional/Clinical Pediatric Cardiologist, Department of Pediatrics, Hospital El Maestro and San Juan Bautista Medical Center; Consulting Interventional/Clinical Pediatric Cardiologist, Department of Cardiology, Cardiovascular Center of Puerto Rico and the Caribbean and Veterans Affairs Hospital and Medical Center of Puerto Rico
Coauthor(s): Sanjeev Aggarwal, MD, MBBS, Staff Physician, Department of Pediatrics, Children's Hospital of Michigan; Ralph E Delius, MD, Associate Professor, Department of Surgery, Wayne State University
Contributor Information and Disclosures

Updated: Mar 10, 2009

Outcome and Prognosis

Patients may require repeated surgeries for a complete repair of pulmonary atresia with ventricular septal defect (PA-VSD). Educate family members regarding congenital heart disease and how to perform cardiopulmonary resuscitation (CPR). Genetic counseling for future pregnancies is necessary.

 


More on Pulmonary Atresia With Ventricular Septal Defect

Overview: Pulmonary Atresia With Ventricular Septal Defect
Workup: Pulmonary Atresia With Ventricular Septal Defect
Treatment: Pulmonary Atresia With Ventricular Septal Defect
Follow-up: Pulmonary Atresia With Ventricular Septal Defect
Multimedia: Pulmonary Atresia With Ventricular Septal Defect
References

References

  1. Collison SP, Dagar KS, Kaushal SK, Radhakrishanan S, Shrivastava S, Iyer KS. Coronary artery fistulas in pulmonary atresia and ventricular septal defect. Asian Cardiovasc Thorac Ann. Jan 2008;16(1):29-32. [Medline].

  2. Durongpisitkul K, Saiviroonporn P, Soongswang J, Laohaprasitiporn D, Chanthong P, Nana A. Pre-operative evaluation with magnetic resonance imaging in tetralogy of fallot and pulmonary atresia with ventricular septal defect. J Med Assoc Thai. Mar 2008;91(3):350-5. [Medline].

  3. Parker RI. Blood transfusions in patients with 22q11.2 deletion syndrome: assessment of risk requires identification of the at-risk patient. Pediatr Crit Care Med. Sep 2007;8(5):502-3. [Medline].

  4. Beauchesne LM, Warnes CA, Connolly HM, et al. Prevalence and clinical manifestations of 22q11.2 microdeletion in adults with selected conotruncal anomalies. J Am Coll Cardiol. Feb 15 2005;45(4):595-8. [Medline].

  5. Bharati S, Paul MH, Idriss FS, et al. The surgical anatomy of pulmonary atresia with ventricular septal defect: pseudotruncus. J Thorac Cardiovasc Surg. May 1975;69(5):713-21. [Medline].

  6. Black MD, Shukla V, Freedom RM. Direct neonatal ventriculo-arterial connections (REV): early results and future implications. Ann Thorac Surg. Apr 1999;67(4):1137-41. [Medline].

  7. Calvani M, Aiuti F, Marconi M, Franchi F, Talamo D. [Thymus dysplasia with circulating T-lymphocytes but not responsive to mitogen. Post-transfusional fatal graft-versus-host disease]. Minerva Pediatr. Nov 15 1979;31(21):1555-64. [Medline].

  8. d'Udekem Y, Alphonso N, Norgaard MA, et al. Pulmonary atresia with ventricular septal defects and major aortopulmonary collateral arteries: unifocalization brings no long-term benefits. J Thorac Cardiovasc Surg. Dec 2005;130(6):1496-502. [Medline].

  9. de Groot NM, Schalij MJ. Foetal echocardiography: tool to predict the future of patients with congenital heart defects?. Eur Heart J. Jun 2008;29(11):1344-5. [Medline].

  10. Dodds GA 3rd, Warnes CA, Danielson GK. Aortic valve replacement after repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot. J Thorac Cardiovasc Surg. Apr 1997;113(4):736-41. [Medline].

  11. El-Said HG, Clapp S, Fagan TE, et al. Stenting of stenosed aortopulmonary collaterals and shunts for palliation of pulmonary atresia/ventricular septal defect. Catheter Cardiovasc Interv. Apr 2000;49(4):430-6. [Medline].

  12. Faletra F, Giardina A, Petroni R, Carraro C, Pasotti E, Pedrazzini G, et al. Evaluation of pulmonary atresia with 64-slice multidetector computed tomography (MDCT). Echocardiography. Oct 2007;24(9):998-9. [Medline].

  13. Fiane AE, Lindberg HL, Seem E, Geiran OR. Homografts for right ventricular outflow tract reconstruction in congenital heart disease. Scand Cardiovasc J. 1997;31(6):351-6. [Medline].

  14. Gill CC, Moodie DS, McGoon DC. Staged surgical management of pulmonary atresia with diminutive pulmonary arteries. J Thorac Cardiovasc Surg. Mar 1977;73(3):436-42. [Medline].

  15. Hausdorf G, Schneider M, Schulze-Neick I, Lange PE. [Pulmonary valve atresia with ventricular septum defect: interventional recanalization of the right ventricular outflow tract]. Z Kardiol. Sep 1992;81(9):496-9. [Medline].

  16. Horer J, Friebe J, Schreiber C, et al. Correction of tetralogy of Fallot and of pulmonary atresia with ventricular septal defect in adults. Ann Thorac Surg. Dec 2005;80(6):2285-91. [Medline].

  17. Huhta JC, Piehler JM, Tajik AJ, et al. Two dimensional echocardiographic detection and measurement of the right pulmonary artery in pulmonary atresia-ventricular septal defect: angiographic and surgical correlation. Am J Cardiol. Apr 1 1982;49(5):1235-40. [Medline].

  18. Jedele KB, Michels VV, Puga FJ, Feldt RH. Velo-cardio-facial syndrome associated with ventricular septal defect, pulmonary atresia, and hypoplastic pulmonary arteries. Pediatrics. May 1992;89(5 Pt 1):915-9. [Medline].

  19. Julsrud PR. Magnetic resonance imaging of the pulmonary arteries and veins. Semin Ultrasound CT MR. Jun 1990;11(3):184-205. [Medline].

  20. Lim ZS, Vettukattill JJ, Salmon AP, Veldtman GR. Sildenafil therapy in complex pulmonary atresia with pulmonary arterial hypertension. Int J Cardiol. Nov 12 2007;[Medline].

  21. Mair DD, Julsrud PR. Diagnostic evaluation of pulmonary atresia and ventricular septal defect: Cardiac catheterization and angiography. Prog Pediatr Cardiol. 1992;1(1):23-36.

  22. Mohammadi S, Belli E, Martinovic I, et al. Surgery for right ventricle to pulmonary artery conduit obstruction: risk factors for further reoperation. Eur J Cardiothorac Surg. Aug 2005;28(2):217-22. [Medline].

  23. Najm HK, Jha NK, Godman M, Al Mutairi M, Rezk AI, Momenah T. Pulmonary atresia, VSD in association with coronary-pulmonary artery fistula. Asian Cardiovasc Thorac Ann. Aug 2007;15(4):335-8. [Medline].

  24. Puga FJ. Surgical treatment of pulmonary atresia and ventricular septal defect. Prog Pediatr Cardiol. 1992;1(1):37-49.

  25. Rabinovitch M, Herrera-deLeon V, Castaneda AR, Reid L. Growth and development of the pulmonary vascular bed in patients with tetralogy of Fallot with or without pulmonary atresia. Circulation. Dec 1981;64(6):1234-49. [Medline].

  26. Reddy VM, McElhinney DB, Amin Z, et al. Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients. Circulation. Apr 18 2000;101(15):1826-32. [Medline].

  27. Reichenspurner H, Netz H, Uberfuhr P, et al. Heart-lung transplantation in a patient with pulmonary atresia and ventricular septal defect. Ann Thorac Surg. Jan 1994;57(1):210-2. [Medline].

  28. Schulze-Neick I, Ho SY, Bush A, et al. Severe airflow limitation after the unifocalization procedure: clinical and morphological correlates. Circulation. Nov 7 2000;102(19 Suppl 3):III142-7. [Medline].

  29. Tchervenkov CI, Roy N. Congenital Heart Surgery Nomenclature and Database Project: pulmonary atresia--ventricular septal defect. Ann Thorac Surg. Apr 2000;69(4 Suppl):S97-105. [Medline].

  30. Tchervenkov CI, Salasidis G, Cecere R, et al. One-stage midline unifocalization and complete repair in infancy versus multiple-stage unifocalization followed by repair for complex heart disease with major aortopulmonary collaterals. J Thorac Cardiovasc Surg. Nov 1997;114(5):727-35; discussion 735-7. [Medline].

  31. Tireli E, Ugurlucan M, Banach M. eComment: The anastomosis between aorta and extension conduit of the pulmonary artery. Interact Cardiovasc Thorac Surg. Apr 2008;7(2):194. [Medline].

Further Reading

Keywords

pulmonary atresia with ventricular septal defect, PA-VSD, ventricular septal defect, VSD, tetralogy of Fallot with pulmonary atresia, TOF, pseudotruncus, truncus arterious type 4, velocardiofacial syndrome, DiGeorge syndrome, patent ductus arteriosus, atrial septal defect, ASD, patent foramen ovale, PFO, right-sided aortic arch, tricuspid atresia, tricuspid stenosis, complete atrioventricular canal, corrected transposition of the great arteries, left superior vena cava, anomalies of the coronary sinus, dextrocardia, asplenia, polysplenia syndrome, double-outlet right ventricle, pulmonary stenosis

Contributor Information and Disclosures

Author

Edwin Rodriguez-Cruz, MD, Assistant Professor, Department of Pediatrics, San Juan Bautista Medical School and Medical Center; Consulting Interventional/Clinical Pediatric Cardiologist, Department of Pediatrics, Hospital El Maestro and San Juan Bautista Medical Center; Consulting Interventional/Clinical Pediatric Cardiologist, Department of Cardiology, Cardiovascular Center of Puerto Rico and the Caribbean and Veterans Affairs Hospital and Medical Center of Puerto Rico
Edwin Rodriguez-Cruz, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Heart Association, American Medical Association, American Society of Echocardiography, Puerto Rico Medical Association, Society of Cardiac Angiography and Interventions, and Society of Pediatric Echocardiography
Disclosure: Nothing to disclose.

Coauthor(s)

Sanjeev Aggarwal, MD, MBBS, Staff Physician, Department of Pediatrics, Children's Hospital of Michigan
Sanjeev Aggarwal, MD, MBBS is a member of the following medical societies: American Academy of Pediatrics and American Medical Association
Disclosure: Nothing to disclose.

Ralph E Delius, MD, Associate Professor, Department of Surgery, Wayne State University
Ralph E Delius, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Thoracic Surgery, American College of Chest Physicians, American College of Surgeons, American Heart Association, American Medical Association, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

Medical Editor

Jonah Odim, MD, PhD, MBA, Senior Medical Officer, Transplantation Immunology Branch, Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases, National Institutes of Health
Jonah Odim, MD, PhD, MBA is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American College of Physician Executives, American College of Surgeons, American Heart Association, American Society for Artificial Internal Organs, American Society of Transplant Surgeons, Association for Academic Surgery, Association for Surgical Education, Canadian Cardiovascular Society, International Society for Heart and Lung Transplantation, National Medical Association, New York Academy of Sciences, Royal College of Physicians and Surgeons of Canada, Society of Critical Care Medicine, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Mary C Mancini, MD, PhD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Phi Beta Kappa, Society of Thoracic Surgeons, and Southern Surgical Association
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.