Platypnea-Orthodeoxia Syndrome: Two Case Reports.

Described for the first time in the middle of the last century, platypnea-orthodeoxia syndrome (POS) is an uncommon condition of positional dyspnea and hypoxemia, triggered by standing and relieved with recumbency. It is most commonly associated with right-to-left shunting through a patent foramen ovale (PFO) or atrial septal defect, however its pathophysiology is not entirely understood. As a rare syndrome, it remains underdiagnosed in many patients. We report two different cases that illustrate the challenge of this diagnosis and therapeutic approach. In the first case, a transesophageal echocardiogram (TEE) showed interatrial communication, ostium secundum type, with bidirectional shunting. Patient underwent a successful percutaneous closure of communication, with no residual shunting and clinical improvement and no positional hypoxemia. In the second case, infectious complications were the cause of hemodynamic changes producing meaningful right-to-left pressure gradients, resulting in POS. After antibiotic treatment there was a major clinical improvement and a second TEE showed bidirectional shunting with no positional variation. It was assumed resolution of POS after treatment of infectious complications with no need for immediate surgery. These two cases, with very distinctive functional and anatomic components, illustrate the challenge of understanding the exact mechanism by which POS results in clinical symptoms. A suggestive history and positional variation of oxygen saturation are very useful clues for its diagnosis in cases of unexplained hypoxemia.

Idiopathic Hypereosinophilic Syndrome: A Case Report.

Idiopathic hypereosinophilic syndrome is a rare condition characterized by persistent severe eosinophilia and organ damage without any apparent cause. A 20-year-old male patient with no significant medical history was admitted to the Emergency Department with retrosternal chest pain, fatigue and asthenia. EKG showed ST elevation I, II, III, aVF, V4-V6 and blood tests showed elevated troponin levels. An echocardiogram was performed revealing severe global left ventricular systolic dysfunction. Further evaluations included cardiac magnetic resonance imaging and endomyocardial biopsy, confirming the diagnosis of eosinophilic myocarditis. The patient was started on systemic corticosteroid therapy, resulting in clinical improvement. The patient was discharged after 12 days of hospitalization, following a recovery of biventricular function and he was told to continue oral corticosteroid therapy at home. Further investigation ruled out other causes of hypereosinophilic syndromes, therefore the diagnosis of idiopathic hypereosinophilic syndrome was assumed. Despite the attempt to reduce corticosteroid therapy, the eosinophil count became elevated again, so the dosage was increased and associated with azathioprine with subsequent favorable analytical evolution. This case highlights the challenges in diagnosing and managing idiopathic hypereosinophilic syndrome and emphasizes the importance of prompt treatment initiation to prevent complications.