Presentation
Reduced vital capacity on respiratory function tests.
Patient Data
There is extensive narrowing of the trachea. The proximal trachea is normal in caliber. It is severely narrowed at the level of the medial ends of the clavicles at the site of the previous tracheostomy where measures <6 mm. There are multiple filling defects at this level however it is difficult to determine whether this represents sputum or mural lesions. An anterior filling defect is seen more superiorly. Extending from this severely narrowed region in the trachea continues to be narrowing throughout its length most of which measures <1 cm. It has an abnormally rounded shape and an appearance consistent with complete tracheal rings. There is a tracheal bronchus on the right to the right upper lobe.
At the are extensive, large areas of abnormally reduced lung density in the left upper and lower lobes with associated air trapping and mosaic perfusion. These are likely due to further airway abnormality, presumably also congenital. Irregular collapse is identified in the left upper lobe adjacent to the region of abnormally reduced density.
Previously identified findings of marked pulmonary artery enlargement ? Pulmonary hypertension and anomalous pulmonary drainage of the right upper and middle lobes into the SVC are unchanged.
Previous thoracotomies. Scoliosis and thoracic degenerative disease.
No ASD visible, in keeping with closure. Right atrium and right ventricle are enlarged relative to the left atrium and to a lesser extent left ventricle.
Fusiform dilation of the right pulmonary artery measures up to 2.5cm in diameter.
Moderate size right upper pulmonary vein draining the right upper and middle lobes measuring up to 1.1cm inserts into the SVC.
Right lower lobe and left sided pulmonary veins drain into the left atrium.
IVC is large but without contrast reflux. Coronary sinus is not enlarged.
No evidence of coronary artery disease. Right dominant system with PDA and small right posterolateral branch.
Left main and LAD unremarkable. No diagonal branches identified.
Circumflex and large branching OM1 unremarkable. OM2/distal circumflex artery unremarkable.
Patchy mosaic attenuation in the left lung with the lucent areas associated with reduced vessel caliber. ?air trapping. This can be correlated with expiratory HRCT as clinically appropriate.
There is a gradual narrowing of the mid to distal trachea, narrowest diameter 7.5mm. The trachea also has a round cross section, with subtle suggestion of posterior ridging, in keeping with complete cartilaginous rings. In addition there is an incidental tracheal bronchus.
Scoliosis with degenerative change.
Conclusion
RUL and RML partial anomalous pulmonary venous return with a moderately large right upper pulmonary vein draining into the SVC. Dilated right heart in keeping with left to right shunt. Fusiform right pulmonary artery dilatation/aneurysm measuring 2.5cm may be related to pulmonary hypertension.