Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Surgical Approach to Partial and Total Anomalous Pulmonary Venous Connection Workup

  • Author: Nicola Viola, MD; Chief Editor: Stuart Berger, MD  more...
 
Updated: Jan 30, 2015
 

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 obstructed total anomalous pulmonary venous connection (TAPVC). Severe metabolic acidosis and hypoxemia are often seen.

Hematocrit levels are checked to confirm adequate oxygen-carrying capacity.

BUN and/or creatinine levels are useful in critically ill neonates presenting with obstructed pulmonary venous return.

Next

Imaging Studies

Chest radiography

In partial anomalous pulmonary venous connection (PAPVC), lung fields often demonstrate increased pulmonary vascular markings. In addition, an enlarged right-heart border from the volume loaded right heart is seen. In patients with scimitar syndrome, a diagnostic vertically-directed crescent shadow is observed to the right of the mediastinal silhouette.

In TAPVC, obstruction to pulmonary venous drainage determines the appearance of the lung fields on chest radiography. In patients without obstruction, the pulmonary vascular bed is plethoric and pulmonary artery is prominent. In patients with obstruction, severe pulmonary edema is the commonest finding. A prominence of the pulmonary artery shadow and the right atrial (RA) silhouette are often observed. In supracardiac drainage, the prominence of the upper mediastinum can create the classic snowman or figure-8 appearance.

Echocardiography

In PAPVC, 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.[14, 15]

In TAPVC, with 2-dimensional echocardiography and color-flow Doppler mapping, the anomalous venous anatomy is usually well defined. Diagnostic findings include distension of the right ventricle, the presence of a vascular confluence coupled with absent venous drainage to the left atrium on the Doppler interrogation, the presence of an accessory common vein, a dilated coronary sinus and turbulent flow in the right atrium with a right-to-left shunt. Demonstration of turbulence or flow acceleration in the pulmonary veins is also used to diagnose obstruction in the pulmonary venous circuit. In addition, right-heart pressures and other cardiac anomalies can be determined. Echocardiography has shown excellent sensitivity and specificity in fetal diagnosis[15] and as a prognostic tool and has supplanted angiography in the vast majority of cases.[16]

CT and MRI

In nonurgent cases of PAPVC or TAPVC, CT or MRI may be used to further delineate the cardiac anatomy. The ability to form 3-dimensional reconstructions with these imaging modalities is evolving rapidly.

In particular, contrast-enhanced magnetic resonance angiography (MRA) provides complete anatomical and functional assessment of the pulmonary circulation. When compared to echocardiography, MRIA has been shown to offer a better visualization of the whole length of the pulmonary veins, the communicating veins, and the accessory pathways. Additional benefits include the possibility of qualitatively and quantitatively evaluating the amount of anomalous pulmonary blood flow, the Qp:Qs (using phase-contrast MRI). The secondary effects of the shunt on pulmonary arteries as well as cardiac chambers are also detectable in the same study.[17, 18]

Previous
Next

Diagnostic Procedures

Cardiac catheterization is used infrequently for diagnosis in routine TAPVC or PAPVC 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 TAPVC.

The site of the anomalous connection is located precisely with angiography by locating the step-up in saturations along the systemic venous pathway. Catheterization is also helpful in defining the anatomy of pulmonary-vein stenosis, which may develop after TAPVC is repaired.

In older patients with PAPVC, 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.

Balloon atrial septostomy (BAS) may assist in evaluating the hemodynamic status of patient by unmasking previously undetected severe venous obstruction. The persistence of pulmonary hypertension following BAS should raise the suspicion of an obstruction to the venous drainage in an extracardiac location.

Previous
Next

Histologic Findings

TAPVC 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 TAPVC.

In patients with recurrent stenosis a diffuse fibrous proliferation of the intima is often seen, usually at the site of the surgical anastomosis, although these changes can be seen along the whole length of the vein. Occasionally, those nonspecific changes can involve the intraparenchymal portions of the venous bed, mimicking the pathological changes of the veno-occlusive disease.

Previous
 
 
Contributor Information and Disclosures
Author

Nicola Viola, MD Congenital Surgeon, Department of Cardiothoracic Surgery, Southampton University Hospital

Disclosure: Nothing to disclose.

Coauthor(s)

Christopher A Caldarone, MD Chair, Division of Cardiac Surgery, Professor of Surgery, University of Toronto; Staff Surgeon, Cardiovascular Surgery, Hospital for Sick Children, Toronto

Christopher A Caldarone, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Medical Association

Disclosure: Nothing to disclose.

Jayme S Bennetts, MBBS Fellow, Department of Cardiac and Thoracic Surgery, Flinders Medical Centre

Jayme S Bennetts, MBBS is a member of the following medical societies: Royal Australasian College of Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Mary C Mancini, MD, PhD, MMM Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in Shreveport

Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Society of Thoracic Surgeons, Phi Beta Kappa

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College 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, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

Jonah Odim, MD, PhD, MBA Section Chief of Clinical Transplantation, Transplantation Branch, Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH)

Jonah Odim, MD, PhD, MBA is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American Association for Physician Leadership, 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, 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, Society of Thoracic Surgeons, Canadian Cardiovascular Society

Disclosure: Nothing to disclose.

Acknowledgements

The authors would like to thank Dr. Shi-Joon Yoo, Head of the Department of Diagnostic Cardiac Imaging at The Hospital for Sick Children in Toronto, Canada, for the images and support.

References
  1. Moorman A, Webb S, Brown NA, Lamers W, Anderson RH. Development of the heart: (1) formation of the cardiac chambers and arterial trunks. Heart. 2003 Jul. 89(7):806-14. [Medline].

  2. Neill CA. Development of the pulmonary veins; with reference to the embryology of anomalies of pulmonary venous return. Pediatrics. 1956 Dec. 18(6):880-87. [Medline].

  3. Harris MA, Valmorida JN. Neonates with congenital heart disease, IV: total anomalous pulmonary venous return. Neonatal Netw. 1997 Dec. 16(8):63-6. [Medline].

  4. 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. 1998 Jul. 7(4):410-4. [Medline].

  5. Gaynor JW, Burch M, Dollery C, Sullivan ID, Deanfield JE, Elliott MJ. Repair of anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg. 1995 Jun. 59(6):1471-5. [Medline].

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

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

  8. Craig JM, Darling RC, Rothney WB. Total pulmonary venous drainage into the right side of the heart; report of 17 autopsied cases not associated with other major cardiovascular anomalies. Lab Invest. 1957 Jan-Feb. 6(1):44-64. [Medline].

  9. Herlong JR, Jaggers JJ, Ungerleider RM. Congenital Heart Surgery Nomenclature and Database Project: pulmonary venous anomalies. Ann Thorac Surg. 2000 Apr. 69(4 Suppl):S56-69. [Medline].

  10. Delisle G, Ando M, Calder AL, Zuberbuhler JR, Rochenmacher S, Alday LE, et al. Total anomalous pulmonary venous connection: Report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. Am Heart J. 1976 Jan. 91(1):99-122. [Medline].

  11. Rosenquist GC, Kelly JL, Chandra R, et al. Small left atrium and change in contour of the ventricular septum in total anomalous pulmonary venous connection: a morphometric analysis of 22 infant hearts. Am J Cardiol. 1985 Mar 1. 55(6):777-82. [Medline].

  12. James CL, Keeling JW, Smith NM, Byard RW. Total anomalous pulmonary venous drainage associated with fatal outcome in infancy and early childhood: an autopsy study of 52 cases. Pediatr Pathol. 1994 Jul-Aug. 14(4):665-78. [Medline].

  13. Gathman GE, Nadas AS. Total anomalous pulmonary venous connection: clinical and physiologic observations of 75 pediatric patients. Circulation. 1970 Jul. 42(1):143-54. [Medline].

  14. Valsangiacomo ER, Hornberger LK, Barrea C, Smallhorn JF, Yoo SJ. Partial and total anomalous pulmonary venous connection in the fetus: two-dimensional and Doppler echocardiographic findings. Ultrasound Obstet Gynecol. 2003 Sep. 22(3):257-63. [Medline].

  15. Patel CR, Lane JR, Spector ML, Smith PC, Crane SS. Totally anomalous pulmonary venous connection and complex congenital heart disease: prenatal echocardiographic diagnosis and prognosis. J Ultrasound Med. 2005 Sep. 24(9):1191-8. [Medline].

  16. Krabill KA, Ring WS, Foker JE, et al. Echocardiographic versus cardiac catheterization diagnosis of infants with congenital heart disease requiring cardiac surgery. Am J Cardiol. 1987 Aug 1. 60(4):351-4. [Medline].

  17. Grosse-Wortmann L, Al-Otay A, Goo HW, et al. Anatomical and functional evaluation of pulmonary veins in children by magnetic resonance imaging. J Am Coll Cardiol. 2007 Mar 6. 49(9):993-1002. [Medline].

  18. Valsangiacomo ER, Levasseur S, McCrindle BW, MacDonald C, Smallhorn JF, Yoo SJ. Contrast-enhanced MR angiography of pulmonary venous abnormalities in children. Pediatr Radiol. 2003 Feb. 33(2):92-8. [Medline].

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

  20. Raisher BD, Grant JW, Martin TC, Strauss AW, Spray TL. Complete repair of total anomalous pulmonary venous connection in infancy. J Thorac Cardiovasc Surg. 1992 Aug;. 104(2):443-8. [Medline].

  21. Bu'Lock FA, Jordan SC, Martin RP. Successful balloon dilatation of ascending vein stenosis in obstructed supracardiac total anomalous pulmonary venous connection. Pediatr Cardiol. 1994 Mar-Apr. 15(2):78-80. [Medline].

  22. Michel-Behnke I, Luedemann M, Hagel KJ, Schranz D. Serial stent implantation to relieve in-stent stenosis in obstructed total anomalous pulmonary venous return. Pediatr Cardiol. 2002 Mar-Apr. 23(2):221-3. [Medline].

  23. Ramakrishnan S, Kothari SS. Preoperative balloon dilatation of obstructed total anomalous pulmonary venous connection in a neonate. Catheter Cardiovasc Interv. 2004 Jan. 61(1):128-30. [Medline].

  24. Kyser JP, Bengur AR, Siwik ES. Preoperative palliation of newborn obstructed total anomalous pulmonary venous connection by endovascular stent placement. Catheter Cardiovasc Interv. 2006 Mar. 67(3):473-6. [Medline].

  25. Meadows J, Marshall AC, Lock JE, Scheurer M, Laussen PC, Bacha EA. A hybrid approach to stabilization and repair of obstructed total anomalous pulmonary venous connection in a critically ill newborn infant. J Thorac Cardiovasc Surg. 2006 Apr. 131(4):e1-2. [Medline].

  26. Stewart DL, Mendoza JC, Winston S, Cook LN, Sobczyk WL. Use of extracorporeal life support in total anomalous pulmonary venous drainage. J Perinatol. 1996 May-Jun. 16(3 Pt 1):186-90. [Medline].

  27. Ishino K, Alexi-Meskishvili V, Hetzer R. Preoperative extracorporeal membrane oxygenation in newborns with total anomalous pulmonary venous connection. Cardiovasc Surg. 1999 Jun;. 7(4):473-5. [Medline].

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

  29. 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. 1998 Feb. 115(2):468-70. [Medline].

  30. Cope JT, Banks D, McDaniel NL, Shockey KS, Nolan SP, Kron IL. Is vertical vein ligation necessary in repair of total anomalous pulmonary venous connection?. Ann Thorac Surg. 1997 Jul;. 64(1):23-8;. [Medline].

  31. Cheung YF, Lun KS, Chau AK, Chiu CS. Fate of the unligated vertical vein after repair of supracardiac anomalous pulmonary venous connection. J Paediatr Child Health. 2005 Jul. 41(7):361-4. [Medline].

  32. Tucker BL, Lindesmith GG, Stiles QR, Meyer BW. The superior approach for correction of the supracardiac type of total anomalous pulmonary venous return. Ann Thorac Surg. 1976 Oct. 22(4):374-7. [Medline].

  33. Hiramatsu T, Takanashi Y, Imai Y, et al. Atrial septal displacement for repair of anomalous pulmonary venous return into the right atrium. Ann Thorac Surg. 1998 Apr. 65(4):1110-4. [Medline].

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

  35. Caspi J, Pettitt TW, Fontenot EE, et al. The beneficial hemodynamic effects of selective patent vertical vein following repair of obstructed total anomalous pulmonary venous drainage in infants. Eur J Cardiothorac Surg. 2001 Oct. 20(4):830-4. [Medline].

  36. Imoto Y, Kado H, Asou T, Shiokawa Y, Tominaga R, Yasui H. Mixed type of total anomalous pulmonary venous connection. Ann Thorac Surg. 1998 Oct. 66(4):1394-7. [Medline].

  37. Honjo O, Atlin CR, Hamilton BC, Al-Radi O, Viola N, Coles JG. Primary sutureless repair for infants with mixed total anomalous pulmonary venous drainage. Ann Thorac Surg. 2010 Sep. 90(3):862-8. [Medline].

  38. Pace Napoleone C, Mariucci E, Angeli E, et al. Sinus node dysfunction after partial anomalous pulmonary venous connection repair. J Thorac Cardiovasc Surg. 2014 May. 147(5):1594-8. [Medline].

  39. 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. 1987 Aug. 76(2):298-305. [Medline].

  40. Karamlou T, Gurofsky R, Al Sukhni E, et al. Factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection. Circulation. 2007 Mar 27. 115(12):1591-8. [Medline].

  41. Hancock Friesen CL, Zurakowski D, Thiagarajan RR, et al. Total anomalous pulmonary venous connection: an analysis of current management strategies in a single institution. Ann Thorac Surg. 2005 Feb. 79(2):596-606. [Medline].

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

  43. Sadiq M, Stumper O, De Giovanni JV, et al. Management and outcome of infants and children with right atrial isomerism. Heart. 1996 Mar. 75(3):314-9. [Medline]. [Full Text].

  44. Hashmi A, Abu-Sulaiman R, McCrindle BW, Smallhorn JF, Williams WG, Freedom RM. Management and outcomes of right atrial isomerism: a 26-year experience. J Am Coll Cardiol. 1998 Apr;. 31(5):1120-6. [Medline].

  45. Gaynor JW, Collins MH, Rychik J, Gaughan JP, Spray TL. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg. 1999 Mar;. 117(3):506-13. [Medline].

  46. Heinemann MK, Hanley FL, Van Praagh S, et al. Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy. Ann Thorac Surg. 1994 Jan. 57(1):88-91. [Medline].

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

  48. 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. 2002 Nov. 74(5):1616-20. [Medline].

  49. Azakie A, Lavrsen MJ, Johnson NC, Sapru A. Early outcomes of primary sutureless repair of the pulmonary veins. Ann Thorac Surg. 2011 Aug. 92(2):666-71; discussion 671-2. [Medline].

  50. Gatzoulis MA, Freeman MA, Siu SC, Webb GD, Harris L. Atrial arrhythmia after surgical closure of atrial septal defects in adults. N Engl J Med. 1999 Mar 18. 340(11):839-46. [Medline].

  51. 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. 1998 Feb. 115(2):468-70. [Medline].

  52. Majdalany DS, Phillips SD, Dearani JA, Connolly HM, Warnes CA. Isolated partial anomalous pulmonary venous connections in adults: twenty-year experience. Congenit Heart Dis. 2010 Nov-Dec. 5(6):537-45. [Medline].

  53. Park CS, Kwak JG, Lee C, Lee CH, Lee SY, Choi EY. Partial anomalous pulmonary venous connection to the superior vena cava: the outcome after the Warden procedure. Eur J Cardiothorac Surg. 2012 Feb. 41(2):261-5. [Medline].

  54. Vida VL, Padalino MA, Boccuzzo G, Tarja E, Berggren H, Carrel T. Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study. Circulation. 2010 Sep 21. 122(12):1159-66. [Medline].

  55. Ricci M, Elliott M, Cohen GA, et al. Management of pulmonary venous obstruction after correction of TAPVC: risk factors for adverse outcome. Eur J Cardiothorac Surg. 2003 Jul;. 24(1):28-36. [Medline].

  56. Aburawi EH, Thomson J, Van Doorn C. Late anastomotic stenosis after correction of totally anomalous pulmonary venous connection. Cardiol Young. 2001 May. 11(3):320-1. [Medline].

  57. Korbmacher B, Buttgen S, Schulte HD, et al. Long-term results after repair of total anomalous pulmonary venous connection. Thorac Cardiovasc Surg. 2001 Apr. 49(2):101-6. [Medline].

  58. 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. 2005 Jan. 129(1):167-74. [Medline].

  59. Alton GY, Robertson CM, Sauve R, et al. Early childhood health, growth, and neurodevelopmental outcomes after complete repair of total anomalous pulmonary venous connection at 6 weeks or younger. J Thorac Cardiovasc Surg. 2007 Apr. 133(4):905-11. [Medline].

 
Previous
Next
 
Types of total anomalous pulmonary venous connection.
Partial anomalous pulmonary venous connection (PAPVC) of right-sided pulmonary veins to the right superior venoatrial junction. Note the enlarged confluence and superior vena cava. SVC = Superior vena cava; IVC = Inferior vena cava; RUPV = Right upper pulmonary vein; RMPV = Right middle pulmonary vein.
(A): Patient with obstructed scimitar syndrome (magnetic resonance angiography). The right upper pulmonary vein (RUPV) is diffusely narrow and presents a discrete stenosis at the confluence with the right lower pulmonary vein (red arrow). (B): The confluence at the atrial level appears unobstructed (white arrow) in the 3D reconstruction.
(A): Total anomalous pulmonary venous connection (TAPVC) of supracardiac type. All pulmonary veins drain into a long confluence and a short communicating vein is attached to the right superior vena cava (SVC). The left lower pulmonary vein appears stenosed (red arrow). (B): These findings are confirmed by the 3D reconstruction (white arrow).
Follow-up CT scan performed after repair of total anomalous pulmonary venous connection (TAPVC). The right upper pulmonary vein (RUPV) appears severely stenosed (red arrow).
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.