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

Partial and Total Anomalous Pulmonary Venous Connection, Surgical Treatment: Workup

Author: Jayme Scott Bennetts, MD, Fellow, Department of Cardiac and Thoracic Surgery, Flinders Medical Centre
Coauthor(s): Christopher A Caldarone, MD, Associate Professor, Department of Surgery, The Hospital for Sick Children, University of Toronto
Contributor Information and Disclosures

Updated: May 1, 2009

Workup

Laboratory Studies

  • ABG values, including pO2, pCO2, pH, base excess, lactate concentration, and mixed venous oxygen saturations, permit quantitative assessment of the patient's oxygenation and systemic perfusion.
    • Acute ABG evaluation assists in the resuscitation of a neonate with pulmonary venous obstruction and total anomalous pulmonary venous drainage (TAPVD).
    • Severe metabolic acidosis and mild hypoxemia are often seen.
  • Hematocrit levels are obtained and ensure adequate oxygen-carrying capacity.
  • BUN and/or creatinine levels are useful in critically ill neonates presenting with obstructed pulmonary venous return.

Imaging Studies

  • Chest radiography
    • TAPVD: Obstruction to pulmonary venous drainage determines the appearance of the lung fields on chest radiography. In patients without obstruction, pulmonary vascularity is increased as a result of the large left-to-right shunt created by drainage of pulmonary venous return into the right heart and the resulting increase in right-sided cardiac output. In patients with obstruction, the lung fields may be extremely congested because of obstruction of the egress of blood from the pulmonary veins and the left-to-right shunt. A prominence of the pulmonary artery shadow and the right atrial (RA) silhouette are often observed. In supracardiac drainage, the prominence of the upper mediastinal silhouette can create the classic snowman or figure-eight appearance.
    • Partial anomalous pulmonary venous drainage (PAPVD): Lung fields often demonstrate increased pulmonary vascular markings in patients with atrial septal defect (ASD) and a large left-to-right shunt. In addition, an enlarged right-heart border from the volume loaded right heart is seen. In patients with scimitar syndrome, a diagnostic crescentic shadow is observed to the right of the mediastinal silhouette.
  • Echocardiography
    • TAPVD: Echocardiographic findings can help in accurately diagnosing TAPVD in most patients. With 2-dimensional echocardiography and color-flow Doppler mapping, the anomalous venous anatomy is usually well defined. Demonstration of turbulence or flow acceleration in the pulmonary veins is also used to diagnose obstruction in the pulmonary venous circuit. Echocardiography can also define any intracardiac shunts and show if these are restrictive or unrestrictive. In addition, right-heart pressures and other cardiac anomalies can be determined. Echocardiography has largely replaced angiography in the diagnosis of TAPVD.
    • PAPVD: Echocardiography is typically used to help delineate the anatomy of the pulmonary venous drainage and the atrial septum. Confirmation of the normal drainage of the remaining pulmonary veins is an important part of the echocardiographic examination.
  • CT and/or MRI
    • Although not usually required, either of these modalities may be used to further delineate the cardiac anatomy. The ability to form 3-dimensional reconstructions with these imaging modalities is evolving rapidly.
    • MRI provides the additional benefit of calculations of flow in both the systemic and pulmonary circuits, of the shunt fraction, and of the chamber volumes.

Other Tests

  • Cardiac catheterization is used infrequently for diagnosis in routine TAPVD or PAPVD because of the refinements in echocardiography. Cardiac catheterization is helpful in patients in whom echocardiographic findings are ambiguous or in patients with other complex defects. As a result of the mixing of oxygenated pulmonary venous effluent and deoxygenated systemic venous blood, oxygen saturations are almost identical in all chambers of the heart in patients with TAPVD.
  • Catheterization also is helpful in defining the anatomy of pulmonary-vein stenosis, which may develop after TAPVD is repaired.
  • In older patients with PAPVD, cardiac catheterization may be required to exclude coronary artery disease, to assess right-heart pressures, to ascertain the reversibility of any pulmonary arterial hypertension, and to calculate the shunt fraction.

Diagnostic Procedures

  • Balloon atrial septostomy (BAS) allows a intracardiac shunt to be created and may be helpful in hemodynamic stabilization.
  • BAS may assist in evaluations before to surgical repair and in patients with obstructed pulmonary venous return at the level of an absent or restrictive ASD.

Histologic Findings

  • TAPVD is associated with hypertrophy of the media of the pulmonary veins and arteries. This finding is most prominent in patients with evidence of pulmonary venous obstruction, and it is most important in the extrapulmonary and intrapulmonary veins. Intimal proliferation and fibrous thickening of the pulmonary veins, with lymphangiectasia, is a common microscopic finding in patients with TAPVD.
  • In patients with recurrent stenosis, often a diffuse fibrous proliferation of the intima is seen.

More on Partial and Total Anomalous Pulmonary Venous Connection, Surgical Treatment

Overview: Partial and Total Anomalous Pulmonary Venous Connection, Surgical Treatment
Workup: Partial and Total Anomalous Pulmonary Venous Connection, Surgical Treatment
Treatment: Partial and Total Anomalous Pulmonary Venous Connection, Surgical Treatment
Follow-up: Partial and Total Anomalous Pulmonary Venous Connection, Surgical Treatment
Multimedia: Partial and Total Anomalous Pulmonary Venous Connection, Surgical Treatment
References
Further Reading
[ CLOSE WINDOW ]

References

  1. Agematsu K, Naito Y, Aoki M, Fujiwara T. Total cavo-pulmonary connection without foreign material for asplenic heart associated with partial anomalous pulmonary venous connection. Interact Cardiovasc Thorac Surg. Apr 2008;7(2):344-6. [Medline].

  2. Talwar S, Choudhary SK, Shivaprasad MB, et al. Tetralogy of Fallot with total anomalous pulmonary venous drainage. Ann Thorac Surg. Dec 2008;86(6):1937-40. [Medline].

  3. Sagat M, Omeje IC, Nosal M, Kantorova A, Valentik P, Poruban R. Long-term results of surgical treatment of total anomalous pulmonary venous drainage in children. Bratisl Lek Listy. 2008;109(9):400-4. [Medline].

  4. Jhang WK, Chang YJ, Park CS, Oh YM, Kim YH, Yun TJ. Hybrid palliation for right atrial isomerism associated with obstructive total anomalous pulmonary venous drainage. Interact Cardiovasc Thorac Surg. Apr 2008;7(2):282-4. [Medline].

  5. Bando K, Turrentine MW, Ensing GJ. Surgical management of total anomalous pulmonary venous connection. Thirty-year trends. Circulation. Nov 1 1996;94(9 Suppl):II12-6. [Medline].

  6. Bauer M, Alexi-Meskishvilli V, Nakic Z, et al. The correction of congenital heart defects with less invasive approaches. Thorac Cardiovasc Surg. Apr 2000;48(2):67-71. [Medline].

  7. Caldarone CA, Najm HK, Kadletz M, et al. Relentless pulmonary vein stenosis after repair of total anomalous pulmonary venous drainage. Ann Thorac Surg. Nov 1998;66(5):1514-20. [Medline].

  8. Caldarone CA, Najm HK, Kadletz M, et al. Surgical management of total anomalous pulmonary venous drainage: impact of coexisting cardiac anomalies. Ann Thorac Surg. Nov 1998;66(5):1521-6. [Medline].

  9. Cope JT, Banks D, McDaniel NL, et al. Is vertical vein ligation necessary in repair of total anomalous pulmonary venous connection?. Ann Thorac Surg. Jul 1997;64(1):23-8; discussion 29. [Medline].

  10. Gaynor JW, Burch M, Dollery C, et al. Repair of anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg. Jun 1995;59(6):1471-5. [Medline].

  11. Gaynor JW, Collins MH, Rychik J, et al. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg. Mar 1999;117(3):506-13; discussion 513-4. [Medline].

  12. Gustafson RA, Warden HE, Murray GF, et al. Partial anomalous pulmonary venous connection to the right side of the heart. J Thorac Cardiovasc Surg. Nov 1989;98(5 Pt 2):861-8. [Medline].

  13. Hawkins JA, Minich LL, Tani LY, et al. Absorbable polydioxanone suture and results in total anomalous pulmonary venous connection. Ann Thorac Surg. Jul 1995;60(1):55-9. [Medline].

  14. Haworth SG. Total anomalous pulmonary venous return. Prenatal damage to pulmonary vascular bed and extrapulmonary veins. Br Heart J. Dec 1982;48(6):513-24. [Medline].

  15. Huddleston CB, Mendeloff EN. Scimitar syndrome. Adv Card Surg. 1999;11:161-78. [Medline].

  16. Imoto Y, Kado H, Asou T, et al. Mixed type of total anomalous pulmonary venous connection. Ann Thorac Surg. Oct 1998;66(4):1394-7. [Medline].

  17. Jemielity M, Perek B, Paluszkiewicz L, et al. Results of repair of partial anomalous pulmonary venous connection and sinus venosus atrial septal defect in adults. J Heart Valve Dis. Jul 1998;7(4):410-4. [Medline].

  18. Kirshbom PM, Flynn TB, Clancy RR, et al. Late neurodevelopmental outcome after repair of total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg. May 2005;129(5):1091-7. [Medline].

  19. Kirshbom PM, Myung RJ, Gaynor JW, et al. Preoperative pulmonary venous obstruction affects long-term outcome for survivors of total anomalous pulmonary venous connection repair. Ann Thorac Surg. Nov 2002;74(5):1616-20. [Medline].

  20. Najm HK, Caldarone CA, Smallhorn J, Coles JG. A sutureless technique for the relief of pulmonary vein stenosis with the use of in situ pericardium. J Thorac Cardiovasc Surg. Feb 1998;115(2):468-70. [Medline].

  21. Phillips SJ, Kongtahworn C, Zeff RH, et al. Correction of total anomalous pulmonary venous connection below the diaphragm. Ann Thorac Surg. May 1990;49(5):734-8; discussion 738-9. [Medline].

  22. Smallhorn JF, Burrows P, Wilson G, et al. Two-dimensional and pulsed Doppler echocardiography in the postoperative evaluation of total anomalous pulmonary venous connection. Circulation. Aug 1987;76(2):298-305. [Medline].

  23. Wilson WR Jr, Ilbawi MN, DeLeon SY, et al. Technical modifications for improved results in total anomalous pulmonary venous drainage. J Thorac Cardiovasc Surg. May 1992;103(5):861-70; discussion 870-1. [Medline].

  24. Yamaki S, Tsunemoto M, Shimada M, et al. Quantitative analysis of pulmonary vascular disease in total anomalous pulmonary venous connection in sixty infants. J Thorac Cardiovasc Surg. Sep 1992;104(3):728-35. [Medline].

  25. Yee ES, Turley K, Hsieh WR, Ebert PA. Infant total anomalous pulmonary venous connection: factors influencing timing of presentation and operative outcome. Circulation. Sep 1987;76(3 Pt 2):III83-7. [Medline].

  26. Yun TJ, Coles JG, Konstantinov IE, et al. Conventional and sutureless techniques for management of the pulmonary veins: Evolution of indications from postrepair pulmonary vein stenosis to primary pulmonary vein anomalies. J Thorac Cardiovasc Surg. Jan 2005;129(1):167-74. [Medline].

[ CLOSE WINDOW ]

Keywords

total anomalous pulmonary venous connection, TAPVC, partial anomalous pulmonary venous connection, PAPVC, total anomalous pulmonary venous drainage, TAPVD, partial anomalous pulmonary venous drainage, PAPVD, total anomalous pulmonary venous return, TAPVR, partial anomalous pulmonary venous return, PAPVR, scimitar syndrome, anomalous pulmonary venous drainage, sinus venosus atrial septal defect, cardiac defect, heart defect, mixed pulmonary venous drainage, pulmonary venous obstruction, cardiac surgery, atrial septal defect, ASD, right lung hypoplasia, scimitar syndrome, pulmonary hypertension, Eisenmenger syndrome, cyanosis, treatment, diagnosis

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Jayme Scott Bennetts, MD, Fellow, Department of Cardiac and Thoracic Surgery, Flinders Medical Centre
Jayme Scott Bennetts, MD is a member of the following medical societies: Royal Australasian College of Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Christopher A Caldarone, MD, Associate Professor, Department of Surgery, The Hospital for Sick Children, University of Toronto
Christopher A Caldarone, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, and American Medical Association
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.