Dyspnea in an Adult with Atrial Septal Defect and Sjôgren’s syndrome: Who’s to blame?

ASD and more frequently the ostium secundum type is a relatively common adult congenital heart disease. Sjogren’s syndrome on the other side is the second most frequent auto immune disease, Involving the lungs in 9–20% of patients. We report the case of a 69 years old woman, complaining of a long-standing dyspnea and was diagnosed with Sjogren’s syndrom. Her CT scan and pulmonary function tests were normal. That lead to investigate for pulmonary hypertension as a potential cause for her dyspnea. A TTE was performed and estimated her systolic pulmonary pressure at 30 mmhg, and showed an OS ASD with left to right shunt. Right heart catheterization calculated her mPaP at 20 mmhg. She is scheduled for percutaneous closure of her OS ASD. There are only few cases reporting an association between ASD and Sjogren’s syndrome in polyendocrine syndrome, and even fewer without polyendocrine syndrome. Farook and Al. discussed a case of a women presenting OS ASD with SS complicated with PAH, suggesting an association between the two pathologies by inflammatory process, and Inoue et Al. presented a case of a women with polyartritis rheumatoid and Sjogren’s syndrome complicated with PAH, inducing a right to left shunt through a patent foramen oval. In our case, the ostium secundum type ASD with left to right shunt with mild pulmonary hypertension is probably the reason for her dyspnea, as there was no interstitial lung disease with normal CT and PFT.Unfortunately, there is no sufficient data suggesting an eventual association between ASD and Sjogren’s syndrome without polyendocrine syndrome. The learning point is the importance of screening for congenital heart disease in adult with unexplained dyspnea.