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Partial Anomalous Pulmonary Venous Connection: Differential Diagnoses & Workup

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

Differential Diagnoses

Atrial Septal Defect, Coronary Sinus
Total Anomalous Pulmonary Venous Connection
Atrial Septal Defect, Ostium Primum
Ventricular Septal Defect, Muscular
Atrial Septal Defect, Ostium Secundum
Ventricular Septal Defect, Perimembranous
Atrial Septal Defect, Patent Foramen Ovale
Ventricular Septal Defect, Supracristal
Atrial Septal Defect, Sinus Venosus

Workup

Imaging Studies

Imaging studies in partial anomalous pulmonary venous connection (PAPVC) include the following:

  • Chest radiography
    • Cardiomegaly noted on chest radiography may be the initial reason for referral of a child with PAPVC. Other findings may include a dilated main pulmonary artery and increased pulmonary arterial vascular markings. However, chest radiography findings may be grossly normal.
    • Anomalous connection of one or more veins from the left lung into a left vertical vein that drains into the left innominate vein can create widening of the left upper mediastinal shadow. Rarely, the increased flow volume may also be sufficient to dilate the superior vena cava (SVC), widening the upper right mediastinal shadow. This can simulate the so-called "snowman" heart shape described in total anomalous pulmonary venous return to a left vertical vein.
    • In its classic appearance, scimitar syndrome causes the "scimitar sign." This sign is a linear opacity, usually (but not always) found at the base of the right lung, that widens as it courses inferiorly and ends in the inferior vena cava. It is associated with dextroposition of the heart due to right lung hypoplasia. The scimitar sign can be obscured by a significant dextropositioning of the heart.
  • Echocardiography
    • PAPVC has been diagnosed in utero. In children, echocardiography is the study of choice; however, the lesion can be easily missed if routine echocardiography does not incorporate definition of the entire pulmonary venous return. The size or the diameter of the pulmonary veins should be determined by 2-dimensional (2D) echocardiography. Spectral Doppler study of the individual pulmonary veins is also important to determine obstruction to the flow
    • In most patients, transthoracic echocardiography can be performed to diagnose PAPVC and obviate the need for cardiac catheterization. A high index of suspicion for the presence of the lesion is helpful to properly diagnose the condition.
    • In adults, contrast echocardiography can help with the diagnosis. Agitated saline in a left arm vein can reveal a negative contrast in the innominate vein at the side of the anomalous venous drainage from a left pulmonary vein.
    • Transesophageal echocardiography is also performed for better delineation of the veins, especially in the adult population.
    • Right ventricular dilation may be the first observation that indicates the presence of abnormal venous drainage. The sonographer must identify all 4 pulmonary veins and visualize their connections to the heart. The atrial septum also needs to be evaluated for defects.
  • MRI
    • MRI is rapidly becoming the procedure of choice for further investigation of PAPVC.1 Although echocardiography findings suggest the PAPVC, all the pulmonary veins may not be identified, especially in adults. With refinements in technology rapidly improving the quality of images obtained, fewer children require invasive angiography. Cardiac catheterization may be a more preferable diagnostic tool in infants with complex congenital heart conditions in whom PAPVC is one component.
    • Julsrud and Ehman (1985) reported that use of MRI in the imaging of PAPVC revealed a characteristic sign termed the "broken ring sign."2 In individuals with normal anatomy, a transverse MRI demonstrates a ringlike structure derived from mediastinal fat that surrounds the SVC. In healthy individuals, this ring is broken only at the point of entry of the azygous vein into the SVC. In some patients with partial anomalous pulmonary venous return, the ring of fat also appears to be breached or broken at the site of entry of the anomalous vein.
  • CT scanning: Contrast-enhanced CT scanning is an alternative imaging modality to detect PAPVC when transthoracic images are limited, especially in older children and adults.

Other Tests

  • Electrocardiography (ECG) findings may be normal. They may demonstrate right ventricular dilation manifested by an rSR pattern in right chest leads or right ventricular hypertrophy.
  • Right atrial dilatation may be observed with a P pulmonale on ECG.
  • Postoperative sinus node dysfunction can occur due to damage to the sinus node or its blood supply. This can manifest as significant bradycardia or junctional rhythm or sinus pauses of 3 seconds or more in duration.
  • Arrhythmias, typically supraventricular tachycardia, atrial flutter, and fibrillation, may be observed, either due to right atrial enlargement at older age or secondary to atriotomy and sutures.

Procedures

  • Cardiac catheterization is rarely necessary for precise anatomic diagnosis and hemodynamic evaluation.
  • Right heart pressures are normal in the pediatric patient.
  • Oxygen sampling may identify the location of an anomalous vein. Oxygen saturation in the right atrium that is higher than that found in the SVC strongly indicates PAPVC to the right atrium, provided that an atrial septal defect (ASD) has been ruled out.
  • Qp:Qs can be calculated.
  • Entering the anomalous vein with a catheter and injecting contrast confirms the diagnosis.
  • Selective right and left pulmonary artery angiography that reveals pulmonary venous return for each lobe from each lung provides definitive anatomic diagnosis.
  • Complications of catheterization include bleeding at the vascular entry site, infection, decreased pulses distal to an arterial entry site, and arrhythmia induction.

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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