Congenital venolobar syndrome

Case contributed by Dr Rachael O'Rourke


Presented with febrile illness/ chest infection. Cardiac malposition on CXR. Otherwise well. Clinical examination - Normal pulses. Heart murmer indicating atrial shunt. Cardiac MRI confirmed venolobar syndrome. Later reimaged via CT.

Patient Data

Age: 7
Gender: Female


Scimitar syndrome well demonstrated on plain film.


Congenital venolobar syndrome in a 7 yo with the following features:

1. Scimitar syndrome

2. Right heart and pulmonary trunk dilation secondary to a left to right shunt

3. Right lower lobe pulmonary extralobar sequestration

4. Horseshoe lung

Case Discussion

Scimitar syndrome is a form of partial anomolous pulmonary venous drainage which can go undiagnosed for some time. The resulting left to right shunt can lead to pulmonary hypertension later in life. It is associated with sinus venosus type ASD, which results in a larger shunt.

Scimitar syndrome is also associated with pulmonary sequestration (commonly right lower lobe) and horseshoe lung. When these findings occur in the same patient, they are known as congenital venolobar syndrome. Detection of the sequestration is important prior to corrective surgery to avoid morbidity / mortality caused by intraoperative injury to the systemic vessel.

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Case information

rID: 53003
Published: 28th Apr 2017
Last edited: 9th Apr 2019
Inclusion in quiz mode: Excluded

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