Anomalous Pulmonary Venous Return

Aka: Anomalous Pulmonary Venous Return, Partial Anomalous Pulmonary Venous Return, Partial Anomalous Pulmonary Venous Connection, Total Anomalous Pulmonary Venous Return, Total Anomalous Pulmonary Venous Connection, PAPVR, PAPVC, TAPVR, TAPVC

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II. Epidemiology

  1. Accounts for 0.7 to 1.5% of Congenital Heart Disease cases (5th most common cyanotic cause)

III. Definition

  1. Partial Anomalous Pulmonary Venous Return or Connection (PAPVR)
    1. Some, but not all of the pulmonary veins return to the right atrium or a systemic vein
    2. Results in increased right sided volumes and pressures
    3. Often accompanied by a Atrial Septal Defect (ASD)
    4. Turner Syndrome increases the risk of PAPVR
  2. Total Anomalous Pulmonary Venous Return or Connection (TAPVR, TAPVC)
    1. Cyanotic Congenital Heart Defect
    2. All four pulmonary veins return to the right atrium
      1. Must be accompanied by an Atrial Septal Defect (ASD)
    3. Pulmonary venous obstruction
      1. Presents in first week of life with severe Cyanosis, pulmonary venous congestion, and poor perfusion
    4. No pulmonary venous obstruction
      1. Presents often beyond the neonatal period with mild Cyanosis and pulmonary venous congestion

IV. Differential Diagnosis

  1. Pulmonary disease
    1. TAPVR is frequently misdiagnosed initially

V. Imaging

  1. Chest XRay
    1. Ground-glass appearance of lung fields

VI. References

  1. Fujii and Lock in Cloherty (1991) Manual Neonatal Care, Little Brown, Boston, p. 262-3
  2. Soriano in Triedman (2013) Total Anomalous Pulmonary Venous Connection, UpToDate, accessed 1/9/2015

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