Congenital pulmonary venolobar syndrome

Initially described by Felson, thisCongenital pulmonary venolobar syndrome is a condition is comprised of a rare group of cardiac and pulmonary congenital abnormalities occurring variably in combination. The abnormalities include

• anomalous pulmonary venous drainage,
  • particularly scimitar syndrome with hypogenic right lung,
• pulmonary sequestration with systemic pulmonary vascular supply and
• horseshoe lung.

Clinical presentation

• Cancan present with shortness and breath and exercise intolerance due to the degree of left to right cardiopulmonary shunt causing pulmonary hypertension with right heart failure
• Recurentrecurent pulmonary infection/ lower lobe pneumonia and / or haemoptysis due to sequestration
• Frequentlyfrequently 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 shunting, leading to pulmonary hypertension, right heart dilation, right ventricular hypertrophy and failure. Investigation

Radiographic features

investigation of choice to confirm diagnosis and evaluate right heart changes are ECG gated contrast arterial chest and cardiac contrast MRI

AssociationsPlain radiography

Anomalous pulmonary venous drainage, including scimitar syndrome, is associated with sinus venosus type atrial septal defect, which contributes to left to right shunting,  leading to pulmonary hypertension, right heart dilation, right ventricular hypertrophyNon specific and failure.may show

• Enlargedan enlarged right heart
• Smallsmall right hemithorax with mediastinal shift to the right
• Rightright sided scimitar vein
• Recurrentrecurrent lower lobe consolidation

• Rightright ventricular dilation and hypertrophy
• Anomolousanomolous pulmonary veins, eg. Scimitar, left vertical vein
• Sequestrationsequestration with systemic arterial supply from upper abdominal aorta, coeliac trunk or SMA.
• small right lung with mediastinal shift to right.
• Horseshoehorseshoe lung - right lung extends posterior to the heart, contiguous with the left lung.

Cardiac MRI

• Confirmcan confirm anomalous pulmonary venous drainage
• Identifycan identify any further congenital heart abnormalities eg. Sinus venosus ASD
• Measurecan measure the degree of left to right shunt via phase contrast flow volumes
• Quantitationquantitation of right ventricular volumes and systolic function.
• Identifyidentify systemic 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

• Inin patients with a large left to right shunt, at risk of developing pulmonary hypertension and right heart failure, corrective surgery is a consideration.
• Surgicalsurgical resection of the sequestration in cases of recurrent haemoptysis and/ or infection.
• Conservativeconservative management with serial cMRI to monitor right heart progress.

History and etymology

Initially described by Felson

Related articles

• Scimitar syndrome