We describe a case of a 2-month-old female displaying clinical phenotype of HIES related disease with severe eczematous dermatitis recalcitrant to corticosteroids. Genetic panel showed a variant of unknown significance in key immune regulator of PGM3. Patient achieved nearly complete response from off-label use of dupilumab.

Swiss cheese atrial septal defect (ASD) is a structural finding of the heart used to characterize multiple or fenestrated interatrial defects found in approximately 10% of patients with ASD. 1 Presence of an aneurysm in the atrial septum is an additional malformation associated with fenestrated ASD that further increases the risk of arrhythmia and thromboembolic events. 1-2 Detailed anatomy of the interatrial septum through 2D and 3D imaging serves as diagnostic and therapeutic guidance. 3 We describe a case of Swiss Cheese ASD complicated with an atrial septal aneurysm successfully repaired with a Cribriform Amplatzer Septal Occluder (ASO) via percutaneous transcatheter approach.