Background:
A 57 year old male, had been suffering for more than 10 years with vague general symptoms of malaise, fatigue, abdominal discomfort and pruritus together with shortness of breath, chest tightness and some occasional palpitations on exertion. A CT scan revealed a multi-systemic disease process involving most of the anterior mediastinal lymph nodes together with parenchymal infiltrations of the liver, the kidneys and pleura. An anterior mediastinotomy and pleural biopsy were performed to clarify the diagnosis. A trans-oesophageal echo (TOE) was performed to exclude any myocardial or valve involvement of this systemic process. The histology report was highly suspicious of IgG4 related disease. The TOE showed severe left superior pulmonary vein stenosis by infiltration of the right superior pulmonary vein, causing a jet back to the left atrium.

Material and Methods:
After undergoing several investigations including gastroscopy, barium swallow, MRI neck, and several biopsies there were no significant findings. Histology revealed reactive or non-specific inflammatory changes with no evidence of malignancy.

Result:
Open pleural biopsy via mediastinotomy was performed during which a transesophageal echocardiogram was also performed. This revealed severe superior pulmonary stenosis.

Conclusion:
This is a rare finding of IgG4-Related disease, where there is infiltration of the pulmonary vein causing left superior pulmonary vein stenosis, which was discovered during intra-operative TOE. Clinicians ought to be aware that when symptoms of shortness of breath and palpitations are reported by a patient with rare pathologies, like IgG4-RD, a TOE investigation could be an additional aid to diagnosis.