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Persistent Pulmonary Hypertension Without Underlying Cardiac Disease as a Presentation of Pulmonary Interstitial Glycogenosis

ABSTRACT Introduction: Pulmonary interstitial glycogenosis (PIG) is an idiopathic lung condition that remains clinically underrecognized despite a growing body of literature. Case report: We present a case of PIG with pulmonary hypertension without underlying cardiac disease. This patient presented with respiratory distress and spontaneous pneumothorax at 6 months of age. Laboratory and imaging investigations demonstrated nonspecific features, but refractory pulmonary hypertension was confirmed on cardiac catheterization. Lung tissue histopathology showed glycogen positive staining of the interstitial cells, consistent with PIG. Conclusion: This unique case demonstrates that pulmonary hypertension can be seen in the setting of PIG without associated cardiac anomalies, and persists despite treatment in an otherwise self-limited condition.