Congenital pulmonary venolobar syndrome

Congenital pulmonary venolobar syndrome is a condition comprised ofcomprising a rare group of cardiac and pulmonary congenital abnormalities occurring variably in combination. The abnormalities include:

• anomalous pulmonary venous drainage
  • particularly scimitar syndrome with hypogenichypogenetic right lung
• pulmonary sequestration with systemic pulmonary vascular supply
• horseshoe lung

Clinical presentation

• can present with shortness of breath and exercise intolerance due to the degree of left-to-right cardiopulmonary shunt causing pulmonary hypertension with right heart failure
• recurentrecurrent pulmonary infection/ lower/lower lobe pneumonia and/or haemoptysis due to sequestration
• frequently asymptomatic, picked up incidentally on plain chest radiograph or echocardiography

Associations

• anomalous pulmonary venous drainage, including scimitar syndrome
• sinus venosus type atrial septal defect; which contributes to left to right-to-right shunting, leading to pulmonary hypertension, right heart dilation, and right ventricular hypertrophy and failure.

Radiographic features

Plain radiograph

Non-specific and may show:

• an enlarged right heart
• small right hemithorax with mediastinal shift to the right
• right sided scimitar vein
• recurrent lower lobe consolidation

CT

May show:

• right ventricular dilation and hypertrophy
• anomolousanomalous pulmonary veins, ege.g. Scimitarscimitar vein, left vertical vein
• sequestration with systemic arterial supply from the upper abdominal aorta, coeliacceliac trunk or SMA
• small right lung with mediastinal shift to the right
• horseshoe lung - right lung extends posteriorposteriorly to the heart, contiguous with the left lung

Cardiac MRI

• can confirm anomalous pulmonary venous drainage
• can identify any furtheradditional congenital heart abnormalities eg, e.g. Sinussinus venosus ASDatrial septal defect (ASD)
• can measure the degree of left to right-to-right shunt via phase contrast flow volumes
• perform quantitation of right ventricular volumes and systolic function
• identify systemic pulmonary-pulmonary arterial supply with differential pulmonary perfusion

Practical points

Identification of the systemic arterial supply to the lung in these patients is important in the context of planning for surgical repair of the anomalous venous drainage, in order to avoid injury and bleeding from this vessel.

Treatment and prognosis

• in patients with a large left to right-to-right shunt, at risk of developing pulmonary hypertension and right heart failure, corrective surgery is a consideration
• surgical resection of the sequestration in cases of recurrent haemoptysis and/ or/or infection
• conservative management with serial cMRIcine MRI (cMRI) to monitor right heart progress

History and etymology

The term was initially coined by Dr Benjamin Felson.

Related articles

• Scimitar syndrome