Congenital pulmonary venolobar syndrome
Updates to Article Attributes
Body
was changed:
Initially described by Felson, this 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
- Can 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
- Recurent pulmonary infection/ lower lobe pneumonia and / or haemoptysis due to sequestration
- Frequently asymptomatic, picked up incidentally on plain chest radiograph or echocardiography. Investigation of choice to confirm diagnosis and evaluate right heart changes are ECG gated contrast arterial chest and cardiac contrast MRI
Associations
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 hypertrophy and failure.
Radiographic features
Plain radiography
- Enlarged right heart
- Small right hemithorax with mediastinal shift to the right
- Right sided scimitar vein
- Recurrent lower lobe consolidation
Computed tomography
- Right ventricular dilation and hypertrophy
- Anomolous pulmonary veins, eg. Scimitar, left vertical vein
- Sequestration with systemic arterial supply from upper abdominal aorta, coeliac trunk or SMA.
- small right lung with mediastinal shift to right.
- Horseshoe lung - right lung extends posterior to the heart, contiguous with the left lung.
Cardiac MRI
- Confirm anomalous pulmonary venous drainage
- Identify any further congenital heart abnormalities eg. Sinus venosus ASD
- Measure degree of left to right shunt via phase contrast flow volumes
- Quantitation of right ventricular volumes and systolic function.
- Identify 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
- In patients with a large left 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 infection.
- Conservative management with serial cMRI to monitor right heart progress.
Related articles
-<p>Initially described by Felson, this 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.</p><h5>Clinical presentation</h5><p> </p><h5>Associations</h5><p> </p><h5>Radiographic features</h5><h6>Plain radiography</h6><h6>Computed tomography</h6><h6>Cardiac MRI</h6><h5>Practical points</h5><p>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.</p><h5>Treatment</h5><h5>Related articles</h5><ul><li><a title="Scimitar syndrome" href="/articles/scimitar-syndrome">Scimitar syndrome</a></li></ul>- +<p>Initially described by Felson, this 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.</p><h5>Clinical presentation</h5><ul>
- +<li>Can 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</li>
- +<li>Recurent pulmonary infection/ lower lobe pneumonia and / or haemoptysis due to sequestration</li>
- +<li>Frequently asymptomatic, picked up incidentally on plain chest radiograph or echocardiography. Investigation of choice to confirm diagnosis and evaluate right heart changes are ECG gated contrast arterial chest and cardiac contrast MRI</li>
- +</ul><h5>Associations</h5><p>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 hypertrophy and failure.</p><h5>Radiographic features</h5><h6>Plain radiography</h6><ul>
- +<li>Enlarged right heart</li>
- +<li>Small right hemithorax with mediastinal shift to the right</li>
- +<li>Right sided scimitar vein</li>
- +<li>Recurrent lower lobe consolidation</li>
- +</ul><h6>Computed tomography</h6><ul>
- +<li>Right ventricular dilation and hypertrophy</li>
- +<li>Anomolous pulmonary veins, eg. Scimitar, left vertical vein</li>
- +<li>Sequestration with systemic arterial supply from upper abdominal aorta, coeliac trunk or SMA.</li>
- +<li>small right lung with mediastinal shift to right.</li>
- +<li>Horseshoe lung - right lung extends posterior to the heart, contiguous with the left lung.</li>
- +</ul><h6>Cardiac MRI</h6><ul>
- +<li>Confirm anomalous pulmonary venous drainage</li>
- +<li>Identify any further congenital heart abnormalities eg. Sinus venosus ASD</li>
- +<li>Measure degree of left to right shunt via phase contrast flow volumes</li>
- +<li>Quantitation of right ventricular volumes and systolic function.</li>
- +<li>Identify systemic pulmonary arterial supply with differential pulmonary perfusion</li>
- +</ul><h5>Practical points</h5><p>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.</p><h5>Treatment</h5><ul>
- +<li>In patients with a large left to right shunt, at risk of developing pulmonary hypertension and right heart failure, corrective surgery is a consideration.</li>
- +<li>Surgical resection of the sequestration in cases of recurrent haemoptysis and/ or infection.</li>
- +<li>Conservative management with serial cMRI to monitor right heart progress.</li>
- +</ul><h5>Related articles</h5><ul><li><a href="/articles/scimitar-syndrome">Scimitar syndrome</a></li></ul>
References changed:
- 1. Konen E, Raviv-Zilka L, Cohen R et al. Congenital Pulmonary Venolobar Syndrome: Spectrum of Helical CT Findings with Emphasis on Computerized Reformatting. Radiographics. 2003;23(5):1175-84. <a href="https://doi.org/10.1148/rg.235035004">doi:10.1148/rg.235035004</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/12975508">Pubmed</a>
Sections changed:
- Anatomy
- Syndromes
Systems changed:
- Chest
- Cardiac
Images Changes:
Image 1 CT (lung window) ( create )
Unable to process the form. Check for errors and try again.