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

Partial Anomalous Pulmonary Venous Connection: Follow-up

Author: Monesha Gupta, MD, MBBS, FAAP, FACC, Assistant Professor, Division of Pediatric Pediatric Cardiology, University of Texas Medical School, Children's Memorial Hermann Hospital
Coauthor(s): David J Vaughan, MBBCh, Consultant Pediatrician, Department of Pediatrics, Our Lady of Lourdes Hospital, Ireland; Jerry Zimmerman, MD, PhD, Professor, Department of Pediatrics/Anesthesia, University of Washington School of Medicine; Director, Division of Pediatric Critical Care Medicine, Children's Hospital of Seattle; Ronald G Grifka, MD, Professor of Pediatrics, Michigan State University College of Human Medicine; Chief, Cardiology Division, DeVos Children's Hospital
Contributor Information and Disclosures

Updated: Oct 7, 2008

Follow-up

Further Inpatient Care

  • Routine postoperative care of the patient who has undergone cardiac surgery for partial anomalous pulmonary venous connection (PAPVC) should be performed.
  • Pain control should be optimal to reduce the risk of atelectasis.
  • Anticipate early extubation unless contraindications are recognized; these include excessive chest tube drainage, hemodynamic instability, and oversedation.
  • Encourage early mobilization.
  • Monitor for atrial flutter, atrial fibrillation, and sinus node dysfunction.

Further Outpatient Care

  • Intermittent follow-up to assess right heart size and pressures and cardiac function and rhythm is necessary in patients who did not undergo surgical treatment. With a significant shunt, the pulmonary artery pressures can be elevated, and pulmonary vascular resistance can increase with age.
  • Postoperatively, possible obstruction of the pulmonary veins and superior vena cava (SVC) should be evaluated with echocardiography.
  • Regular electrocardiography (ECG) and 24-hour ambulatory ECG are also indicated to monitor for atrial arrhythmias.

Transfer

  • Patients should be transferred to an institution skilled in pediatric cardiology and pediatric cardiac surgery for assessment and treatment.

Deterrence/Prevention

  • PAPVC is a congenital cardiac defect with no known cause and possibly has a multifactorial origin, including a genetic component. It is also seen with Turner syndrome.

Complications

  • No significant complications develop in pediatric patients who do not undergo surgery for a sinus venosus atrial septal defect (ASD) repair with internal patch technique to include the right upper pulmonary vein in the left atrium. However, other types of repair may be associated with complications such as SVC syndrome, pulmonary venous obstruction, atrial arrhythmias, and sick sinus syndrome.
  • Arrhythmias may occur in adults with unrepaired PAPVC secondary to chronic right atrial enlargement due to volume overload.
  • Cardiac failure and, very rarely, pulmonary hypertension may develop in adults.
  • Patients with scimitar syndrome may be asymptomatic or may have symptoms related to lung pathology, including recurrent pneumonias.

Prognosis

  • Prognosis is excellent for patients with PAPVC. The perioperative mortality rate is comparable to that for ASD repair (<0.1%).
  • Prognosis becomes more guarded if the lesion is undetected for a long period and if complications, particularly pulmonary hypertension, develop.
  • The only postoperative death in the series by Gustafson et al involved a woman with pulmonary hypertension.3

Patient Education

  • Advise patient and parents regarding long-term risks if a large shunt remains unrepaired.
  • If pulmonary hypertension has developed, outline the risks of pregnancy, including death.

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose partial anomalous pulmonary venous connection (PAPVC)4
  • Late diagnosis of PAPVC
  • Risk of pulmonary hypertension
 


More on Partial Anomalous Pulmonary Venous Connection

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

  1. Lilje C, Weiss F, Weil J. Detection of partial anomalous pulmonary venous connection by magnetic resonance imaging. Pediatr Cardiol. Jul-Aug 2005;26(4):490-1. [Medline].

  2. Julsrud PR, Ehman RL. The "broken ring" sign in magnetic resonance imaging of partial anomalous pulmonary venous connection to the superior vena cava. Mayo Clin Proc. Dec 1985;60(12):874-9. [Medline].

  3. 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].

  4. Xue JR, Luo Y, Cheng P, Cao RW. [Diagnosis and treatment of partial anomalous pulmonary venous connection]. Zhonghua Yi Xue Za Zhi. Apr 15 2008;88(15):1066-8. [Medline].

  5. Coulson JD, Bullaboy CA. Concentric placement of stents to relieve an obstructed anomalous pulmonary venous connection. Cathet Cardiovasc Diagn. Oct 1997;42(2):201-4. [Medline].

  6. Danilowicz D, Kronzon I. Use of contrast echocardiography in the diagnosis of partial anomalous pulmonary venous connection. Am J Cardiol. Feb 1979;43(2):248-52. [Medline].

  7. Elami A, Rein AJ, Preminger TJ, et al. Tetralogy of Fallot, absent pulmonary valve, partial anomalous pulmonary venous return and coarctation of the aorta. Int J Cardiol. Dec 1995;52(3):203-6. [Medline].

  8. Forbess LW, O'Laughlin MP, Harrison JK. Partially anomalous pulmonary venous connection: demonstration of dual drainage allowing nonsurgical correction. Cathet Cardiovasc Diagn. Jul 1998;44(3):330-5. [Medline].

  9. Hazirolan T, Ozkan E, Haliloglu M, et al. Complex venous anomalies: magnetic resonance imaging findings in a 5-year-old boy. Surg Radiol Anat. Oct 2006;28(5):534-8. [Medline].

  10. 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].

  11. Nakahira A, Yagihara T, Kagisaki K, et al. Partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg. Sep 2006;82(3):978-82. [Medline].

  12. Powell AJ, Chung T, Landzberg MJ, Geva T. Accuracy of MRI evaluation of pulmonary blood supply in patients with complex pulmonary stenosis or atresia. Int J Card Imaging. Jun 2000;16(3):169-74. [Medline].

  13. Respondek-Liberska M, Janiak K, Moll J, et al. Prenatal diagnosis of partial anomalous pulmonary venous connection by detection of dilatation of superior vena cava in hypoplastic left heart. A case report. Fetal Diagn Ther. Sep-Oct 2002;17(5):298-301. [Medline].

  14. Ritter S, Tani LY, Shaddy RE, et al. An unusual variant of total anomalous pulmonary venous connection with varices and multiple drainage sites. Pediatr Cardiol. May-Jun 2000;21(3):289-91. [Medline].

  15. Shahriari A, Rodefeld MD, Turrentine MW, Brown JW. Caval division technique for sinus venosus atrial septal defect with partial anomalous pulmonary venous connection. Ann Thorac Surg. Jan 2006;81(1):224-9; discussion 229-30. [Medline].

  16. Valsangiacomo ER, Hornberger LK, Barrea C, et al. 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].

  17. Vanderheyden M, Goethals M, Van Hoe L. Partial anomalous pulmonary venous connection or scimitar syndrome. Heart. Jul 2003;89(7):761. [Medline]. [Full Text].

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Further Reading

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Keywords

partial anomalous pulmonary venous connection, intact atrial septum, PAPVC, isolated partial anomalous pulmonary venous connection, total anomalous pulmonary venous connection, TAPVC, sinus venosus atrial septal defect, ASD, congential heart disease, heterotaxia, scimitar syndrome, secundum ASD, secundum atrial septal defect, polysplenia, Halasz syndrome, mirror-image lung syndrome, hypogenetic lung syndrome, epibronchial right pulmonary artery syndrome, vena cava bronchovascular syndrome, congenital pulmonary venolobar syndrome, cardiac murmur, hepatomegaly, jugular venous distension, ascites, peripheral edema

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Contributor Information and Disclosures

Author

Monesha Gupta, MD, MBBS, FAAP, FACC, Assistant Professor, Division of Pediatric Pediatric Cardiology, University of Texas Medical School, Children's Memorial Hermann Hospital
Monesha Gupta, MD, MBBS, FAAP, FACC is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Society of Echocardiography, Medical Council of India, and Society of Pediatric Echocardiography
Disclosure: Nothing to disclose.

Coauthor(s)

David J Vaughan, MBBCh, Consultant Pediatrician, Department of Pediatrics, Our Lady of Lourdes Hospital, Ireland
David J Vaughan, MBBCh is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

Jerry Zimmerman, MD, PhD, Professor, Department of Pediatrics/Anesthesia, University of Washington School of Medicine; Director, Division of Pediatric Critical Care Medicine, Children's Hospital of Seattle
Jerry Zimmerman, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, Society for Pediatric Research, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

Ronald G Grifka, MD, Professor of Pediatrics, Michigan State University College of Human Medicine; Chief, Cardiology Division, DeVos Children's Hospital
Ronald G Grifka, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Medical Editor

Ira H Gessner, MD, Professor Emeritus, Pediatric Cardiology
Ira H Gessner, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, and Society for Pediatric Research
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 broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Hugh D Allen, MD, Professor, Department of Pediatrics, Division of Pediatric Cardiology and Department of Internal Medicine, Ohio State University College of Medicine
Hugh D Allen, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert 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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