Partial anomalous pulmonary venous return

Case contributed by RMH Report Writing

Presentation

Routine CXR.

Patient Data

Age: 20-year-old
Gender: Female

Chest radiographs

Modality: X-ray

The heart size and shape is within normal limits.

The aorta is left-sided and normal.

The right hemithorax shows changes of anomalous pulmonary venous drainage with a scimitar sign and pulmonary venous drainage  probably  into the IVC, below the right hemidiaphragm.

The appearances are those of  partial anomalous pulmonary venous drainage.

There are changes of hypoplasia of the right lung when compared to the left.

Case Discussion

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital cardiovascular condition in which some of the pulmonary veins, but not all, drain into the systemic circulation rather than into the left atrium.

Associations

  • in ~40% of patients with right-sided PAPVR, an atrial septal defect is seen 
  • more rarely it is seen with ostium primum defect, a subtype of atrioventricular defects

Chest radiographic features are particular to each subtype of PAPVR. The abnormal vein is rarely identified, except in cases of Scimitar syndrome. Pulmonary venous congestion can be seen if the venous drainage is obstructed.

Cardiomegaly can also be seen if significant abnormal intracardiac venous drainage occurs.

Complications: Patients with large shunts may present with symptoms of dyspnea, chest pain and palpitations, signs like tachycardia and murmur can be encountered. Cases of secondary pulmonary arterial hypertension have been reported. 

 

Scimitar syndrome is characterised by a hypoplastic lung that is drained by an anomalous vein into the systemic venous system. It is a type of partial anomalous pulmonary venous return and is one of the several findings in congenital pulmonary venolobar syndrome.

It is essentially a combination of pulmonary hypoplasia and partial anomalous pulmonary venous return (PAPVR). It almost exclusively occurs on the right side.

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Case Information

rID: 45007
Case created: 11th May 2016
Last edited: 24th Mar 2017
Systems: Cardiac, Chest
Tag: rmh
Inclusion in quiz mode: Included

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