eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Total Anomalous Pulmonary Venous Connection: Treatment & Medication

Author: Allen D Wilson, MD, Professor, Department of Pediatrics, Section of Pediatric Cardiology, UW Children's Hospital, University of Wisconsin at Madison
Contributor Information and Disclosures

Updated: Oct 2, 2009

Treatment

Medical Care

No catheter-corrective treatment is possible for total anomalous pulmonary venous connection (TAPVC), although atrial septostomy is used in some patients when the foramen ovale is restricted and corrective surgery is delayed for some reason. Catheter placement of a stent has been reported for pretreatment of obstructed vertical vein prior to surgery.4 If a vertical vein is left patent postoperatively and significant shunt persists it may be possible to close this vessel with an Amplatzer PDA device.5

Surgical repair is used as treatment for total anomalous pulmonary venous connection whenever it best serves the individual patient. Stabilizing the patient prior to surgery as much as possible from a cardiovascular and metabolic standpoint is important. In a newborn with obstructive total anomalous pulmonary venous connection, stabilization often involves mechanical ventilation, correction of acidosis, inotropic support, and administration of prostaglandin E1 for patency of patent ductus arteriosus and, in patients with total anomalous pulmonary venous connection type III, for patency of the ductus venous.

Nitric oxide may be useful as a pulmonary dilator postoperatively in patients experiencing episodic pulmonary hypertension that is affecting cardiac output. Reports indicate that magnesium sulfate is a useful pulmonary vasodilator in these patients. Extracorporeal membrane oxygenation (ECMO) may be life saving in some patients. If transesophageal echocardiography is used intraoperatively in infants with pulmonary vein obstruction, waiting for probe insertion until after chest is opened may be safer.6

Surgical Care

The goal of surgery is to redirect pulmonary vein flow entirely to the left atrium. In patients with a supracardiac or infracardiac connection, the common pulmonary vein is opened wide and connected side to side to the left atrium. The foramen ovale is closed, and the ascending or descending vein is usually ligated. In a cardiac connection (to right atrium or coronary sinus), the atrial septum is resected partially and a new septum is surgically created, directing pulmonary veins to the left atrium. A coronary sinus may be separately tunneled to the right atrium or left to drain with the pulmonary veins to the left atrium.

Medication

Newborns or patients in early infancy with obstructed total anomalous pulmonary venous connection (TAPVC) frequently have pulmonary edema with varying degrees of increase in pulmonary arterial and venous resistance. Pulmonary edema is probably treated best with surgical relief of the pulmonary venous obstruction, but diuretics and assisted ventilation with high fraction of inspired oxygen (FIO2) and end-expiratory pressure are often helpful preoperatively and postoperatively.

Pulmonary vasodilators

When sustained severe cyanosis or severe hypercyanotic episodes occur in patients with obstructed total anomalous pulmonary venous connection postoperatively, treatment with one or more pulmonary vasodilators may be helpful. The pulmonary vascular bed may be somewhat reactive in the postoperative period, resulting in episodic pulmonary hypertension and low cardiac output. Although this should improve over time, interim therapy with pulmonary vasodilator agents may be useful in this setting. Vasodilators that are specific for the pulmonary vasculature are rare; however, inhaled nitric oxide may be a good agent in this setting. Therefore, the following 3 vasodilators can be used to treat elevated pulmonary vascular resistance in the postoperative period. Note that this therapy is controversial in the preoperative patient with known pulmonary venous obstruction.


Nitric oxide, inhaled (INOmax)

Stimulates guanylate cyclase to form cyclic GMP, which causes relaxation of vascular smooth muscle.
Because it can be delivered by inhalation directly to alveolar units and is rapidly inactivated by hemoglobin, it is the most selective of currently available pulmonary vascular dilators (except for oxygen). Requires an inhalation delivery system (not available everywhere); approved for use in children in December 1999.

Adult

Pediatric

Initial dose 80 ppm inhaled with high FIO2; taper to 20 ppm as safer long-term dose; effect of pulmonary vasodilatation may still be observed at 5 ppm
Delivery system must measure NO concentrations in breathing gas (concentration must be constant throughout respiratory cycle) and must not generate excessive inhaled NO2

NO donor compounds (eg, nitroprusside, nitroglycerin) may increase risk of developing methemoglobinemia

Neonates with dependent right-to-left shunting of blood; methemoglobin-reductase deficiency

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Possible withdrawal problems with prolonged therapy; when weaning, may use type V phosphodiesterase inhibitor to increase cyclic GMP levels to decrease rebound pulmonary hypertension; in patients with small left atria or poorly compliant left ventricles, initial improvement in pulmonary flow can occur, then pulmonary hypertension can return with worsened clinical state; prolonged treatment can cause elevation in methemoglobin levels, which can be measured; caution in thrombocytopenia, anemia, leukopenia, or bleeding disorders


Magnesium sulfate

Reportedly useful in patients with obstructed TAPVC who have hypercyanotic episodes to decrease pulmonary vascular resistance and decrease pulmonary vascular reactivity. Mechanism of action is believed to be direct action on vascular muscle cells but may also increase formation or release of NO. MgSO4 has systemic and pulmonary vascular dilating effects, and use of a slow infusion of lower-dose MgSO4 is wise to avoid systemic hypotension.

Adult

Pediatric

20 mg/kg/h IV initially; can gradually increase to 50 mg/kg/h IV over 10-12 h; not to exceed 50 mg/kg/h IV

Concurrent use with nifedipine may cause hypotension and neuromuscular blockade; may increase neuromuscular blockade seen with aminoglycosides and potentiate neuromuscular blockade produced by tubocurarine, vecuronium, and succinylcholine; may increase CNS effects, toxicity of CNS depressants and betamethasone, and cardiotoxicity of ritodrine

Documented hypersensitivity; preexisting systemic hypotension or hypermagnesemia; heart block, myocardial damage, or severe hepatitis

Pregnancy

A - Fetal risk not revealed in controlled studies in humans

Precautions

Magnesium may alter cardiac conduction, leading to heart block in patients taking digitalis; BP, blood gases, respiratory rate, deep tendon reflex, and renal function should be monitored when administered parenterally


Alprostadil (Prostin VR)

Prostaglandin E1 (PGE1) that causes dilation of vascular smooth muscle in the ductus arteriosus, systemic arteries, and pulmonary vascular muscles. In obstructed TAPVC, PGE1 is usually used as a pulmonary vascular dilator, but its effects on the ductus arteriosus and ductus venosus can be very important (eg, in subdiaphragmatic connection, PGE1 can help dilate the ductus venosus and improve pulmonary venous flow. In other types of connection with obstruction, PGE1 can dilate pulmonary arteries and increase pulmonary flow or dilate the ductus arteriosus and systemic arteries and increase right-to-left shunting and worsen cyanosis). PGE1 is readily available and easily administered, preferably via a large vessel. Care must be taken to observe its effects in the complex circulation of TAPVC.
Each 1-mL ampule contains 500 mcg/mL.

Adult

Pediatric

Initial dose: 0.03-0.1 mcg/kg/min IV; not to exceed 0.2 mcg/kg/min IV
Usual effective maintenance dose can be lower at 0.01-0.05 mcg/kg/min IV

Limited data are available; caution with concurrent use of antiplatelet drugs or anticoagulants

Documented hypersensitivity; hyaline membrane disease or respiratory distress syndrome; systemic hypotension may be relative contraindication

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Most worrisome adverse effect is apnea, which occurs at higher doses (ie, >0.05 mcg/kg/min); adverse effects and toxicity include seizures, fever, hypotension, flushing, leukocytosis, fever, bradycardia, diarrhea, and pulmonary overcirculation; neonates may be intubated prophylactically because of potential risk of apnea (10-12%); prolonged use occasionally is necessary and may be associated with third spacing of fluid; monitor blood oxygenation and arterial pressures

More on Total Anomalous Pulmonary Venous Connection

Overview: Total Anomalous Pulmonary Venous Connection
Differential Diagnoses & Workup: Total Anomalous Pulmonary Venous Connection
Treatment & Medication: Total Anomalous Pulmonary Venous Connection
Follow-up: Total Anomalous Pulmonary Venous Connection
Multimedia: Total Anomalous Pulmonary Venous Connection
References
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References

  1. Douglas YL, Jongbloed MR, den Hartog WC, Bartelings MM, Bogers AJ, Ebels T. Pulmonary vein and atrial wall pathology in human total anomalous pulmonary venous connection. Int J Cardiol. May 29 2009;134(3):302-12. [Medline].

  2. Correa-Villasenor A, Ferencz C, Boughman JA, Neill CA. Total anomalous pulmonary venous return: familial and environmental factors. The Baltimore-Washington Infant Study Group. Teratology. Oct 1991;44(4):415-28. [Medline].

  3. Yap SH, Anania N, Alboliras ET, Lilien LD. Reversed differential cyanosis in the newborn: a clinical finding in the supracardiac total anomalous pulmonary venous connection. Pediatr Cardiol. Apr 2009;30(3):359-62. [Medline].

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

  5. Narula N, Wilson N, Kumar RS. Transcatheter closure of persistent unligated vertical vein after TAPVC surgery using the Amplatzer PDA device. Catheter Cardiovasc Interv. Jul 1 2007;70(1):117-9. [Medline].

  6. Chang YY, Chang CI, Wang MJ, et al. The safe use of intraoperative transesophageal echocardiography in the management of total anomalous pulmonary venous connection in newborns and infants: a case series. Paediatr Anaesth. Nov 2005;15(11):939-43. [Medline].

  7. Devaney EJ, Chang AC, Ohye RG, Bove EL. Management of congenital and acquired pulmonary vein stenosis. Ann Thorac Surg. Mar 2006;81(3):992-5; discussion 995-6. [Medline].

  8. Chowdhury UK, Airan B, Malhotra A, Bisoi AK, Saxena A, Kothari SS. Mixed total anomalous pulmonary venous connection: anatomic variations, surgical approach, techniques, and results. J Thorac Cardiovasc Surg. Jan 2008;135(1):106-16, 116.e1-5. [Medline].

  9. Behrendt DM, Aberdeen E, Waterson DJ, Bonham-Carter RE. Total anomalous pulmonary venous drainage in infants. I. Clinical and hemodynamic findings, methods, and results of operation in 37 cases. Circulation. Aug 1972;46(2):347-56. [Medline].

  10. Castenada AR, Jonas RA, Mayer JE, Hanley FL. Cardiac Surgery of the Neonate and Infant. Philadelphia, Pa: WB Saunders Co; 1994:165.

  11. 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. Jul 2005;41(7):361-4. [Medline].

  12. Choe YH, Lee HJ, Kim HS, Ko JK, Kim JE, Han JJ. MRI of total anomalous pulmonary venous connections. J Comput Assist Tomogr. Mar-Apr 1994;18(2):243-9. [Medline].

  13. 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. Jan-Feb 1957;6(1):44-64. [Medline].

  14. Duff DF, Nihill MR, McNamara DG. Infradiaphragmatic total anomalous pulmonary venous return. Review of clinical and pathological findings and results of operation in 28 cases. Br Heart J. Jun 1977;39(6):619-26. [Medline].

  15. Edwards JE. Pathologic and developmental considerations in anomalous pulmonary venous connection. Proc Staff Meet Mayo Clin. Aug 26 1953;28(17):441-52. [Medline].

  16. Graham TP Jr, Jarmakani JM, Canent RV Jr. Left heart volume characteristics with a right ventricular volume overload. Total anomalous pulmonary venous connection and large atrial septal defect. Circulation. Feb 1972;45(2):389-96. [Medline].

  17. 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. Feb 2005;79(2):596-606; discussion 596-606. [Medline].

  18. 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].

  19. Hong YK, Park YW, Ryu SJ, et al. Efficacy of MRI in complicated congenital heart disease with visceral heterotaxy syndrome. J Comput Assist Tomogr. Sep-Oct 2000;24(5):671-82. [Medline].

  20. Huhta JC, Gutgesell HP, Nihill MR. Cross sectional echocardiographic diagnosis of total anomalous pulmonary venous connection. Br Heart J. May 1985;53(5):525-34. [Medline].

  21. Jonas RA, Smolinsky A, Mayer JE, Castaneda AR. Obstructed pulmonary venous drainage with total anomalous pulmonary venous connection to the coronary sinus. Am J Cardiol. Feb 15 1987;59(5):431-5. [Medline].

  22. Kimball TR, Weiss RG, Meyer RA, Daniels SR, Ryckman FC, Schwartz DC. Color flow mapping to document normal pulmonary venous return in neonates with persistent pulmonary hypertension being considered for extracorporeal membrane oxygenation. J Pediatr. Mar 1989;114(3):433-7. [Medline].

  23. 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].

  24. Lin SC, Teng RJ, Wang JK. Management of severe pulmonary hypertension in an infant with obstructed total anomalous pulmonary venous return using magnesium sulfate. Int J Cardiol. Oct 11 1996;56(2):131-5. [Medline].

  25. Lucas RV, Anderson RC, Amplatz K, Adams P Jr, Edwards JE. Congenital causes of pulmonary venous obstruction. Pediatr Clin North Am. Aug 1963;10:781-836. [Medline].

  26. Maeda K, Yamaki S, Kado H, Asou T, Murakami A, Takamoto S. Hypoplasia of the small pulmonary arteries in hypoplastic left heart syndrome with restrictive atrial septal defect. Circulation. Sep 14 2004;110(11 Suppl 1):II139-46. [Medline]. [Full Text].

  27. Morin FC 3rd. Prostaglandin E1 opens the ductus venosus in the newborn lamb. Pediatr Res. Mar 1987;21(3):225-8. [Medline].

  28. Emmanouilides GC, Gutgesell HP, Riemenschneider TA, Allen HD, eds. Moss and Adams Heart Disease in Infants, Children and Adolescents: Including the Fetus and Young Adult. 5th ed. Williams & Wilkins; 1995:874-901.

  29. Neill CA. Development of the pulmonary veins; with reference to the embryology of anomalies of pulmonary venous return. Pediatrics. Dec 1956;18(6):880-7. [Medline].

  30. Norwood WI, Hougen TJ, Castaneda AR. Total anomalous pulmonary venous connection: surgical considerations. Cardiovasc Clin. 1981;11(2):353-64. [Medline].

  31. 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. Jul 2003;24(1):28-36; discussion 36. [Medline].

  32. Rosales AM, Bolivar J, Burke RP, Chang AC. Adverse hemodynamic effects observed with inhaled nitric oxide after surgical repair of total anomalous pulmonary venous return. Pediatr Cardiol. May-Jun 1999;20(3):224-6. [Medline].

  33. Sahn DJ, Allen HD, Lange LW, Goldberg SJ. Cross-sectional echocardiographic diagnosis of the sites of total anomalous pulmonary venous drainage. Circulation. Dec 1979;60(6):1317-25. [Medline].

  34. Sano S, Brawn WJ, Mee RB. Total anomalous pulmonary venous drainage. J Thorac Cardiovasc Surg. Jun 1989;97(6):886-92. [Medline].

  35. Smallhorn JF, Sutherland GR, Tommasini G, Hunter S, Anderson RH, Macartney FJ. Assessment of total anomalous pulmonary venous connection by two-dimensional echocardiography. Br Heart J. Dec 1981;46(6):613-23. [Medline].

  36. Solymar L, Sabel KG, Zetterqvist P. Total anomalous pulmonary venous connection in siblings. Report on three families. Acta Paediatr Scand. Jan 1987;76(1):124-7. [Medline].

  37. Tasaka H, Krug EL, Markwald RR. Origin of the pulmonary venous orifice in the mouse and its relation to the morphogenesis of the sinus venosus, extracardiac mesenchyme (spina vestibuli), and atrium. Anat Rec. Sep 1996;246(1):107-13. [Medline].

  38. 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. Sep 2003;22(3):257-63. [Medline].

  39. van der Velde ME, Parness IA, Colan SD, et al. Two-dimensional echocardiography in the pre- and postoperative management of totally anomalous pulmonary venous connection. J Am Coll Cardiol. Dec 1991;18(7):1746-51. [Medline].

  40. Ward KE, Mullins CE. Anomalous pulmonary venous connections. In: The Science and Practice of Pediatric Cardiology. 2nd ed. Williams & Wilkins; 1998.

  41. Ward KE, Mullins CE, Huhta JC, Nihill MR, McNamara DG, Cooley DA. Restrictive interatrial communication in total anomalous pulmonary venous connection. Am J Cardiol. May 1 1986;57(13):1131-6. [Medline].

  42. Wilson AD, Rao PS, Aeschlimann S. Normal fetal foramen flap and transatrial Doppler velocity pattern. J Am Soc Echocardiogr. Nov-Dec 1990;3(6):491-4. [Medline].

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Further Reading

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Keywords

total anomalous pulmonary venous connection, TAPVC, total anomalous pulmonary venous drainage, TAPVD, total anomalous pulmonary venous return, TAPVR, atrial septal defect, patent foramen ovale, pulmonary venous obstruction, pulmonary venous congestion, pulmonary vein obstruction, cor triatriatum, left atrial shelf, tachypnea, tachycardia, cyanosis, pulmonary hypertension, failure to thrive

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Contributor Information and Disclosures

Author

Allen D Wilson, MD, Professor, Department of Pediatrics, Section of Pediatric Cardiology, UW Children's Hospital, University of Wisconsin at Madison
Allen D Wilson, MD is a member of the following medical societies: American College of Cardiology, American Heart Association, American Society of Echocardiography, and Society of Pediatric Echocardiography
Disclosure: Nothing to disclose.

Medical Editor

Juan Carlos Alejos, MD, Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California at Los Angeles
Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and International Society for Heart and Lung Transplantation
Disclosure: Actelion Honoraria Speaking and teaching

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

John W Moore, MD, MPH, Professor of Clinical Pediatrics, Section of Pediatric Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital
John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

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.

 
 
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