Platypnea-orthodeoxia syndrome: an unusual presentation of a complex disease.

BACKGROUND: Platypnea-orthodeoxia syndrome (POS) is an uncommon disorder characterized by dyspnea (=platypnea) and desaturation (=orthodeoxia) in upright position and improvement of symptoms and blood oxygenation in supine position. Clinical presentation is heterogeneous and often confuses the clinician.

OBJECTIVES: The present case report illustrates a complicated presentation of the underlying syndrome.

METHODS: Description of a POS case in a 73-year-old female with thorough workup and tailored treatment.

RESULTS: In this case report, we describe a rather unusual presentation of POS, with posture-dependent hypoxemic-induced psychiatric symptoms. Further investigations revealed the presence of a patent foramen ovale with atrial septum aneurysm, an aortic root dilatation up to 41 mm and bilateral lower lobe lung emboli. The ventilation-perfusion mismatch aggravated the desaturation in upright position. Since the patient remained symptomatic after treatment of the functional trigger, we choose for the percutaneous closure of the anatomical defect. We used an Occlutech Figulla Flex II UNI 33/33 mm occluder resulting in a perfect closure of the defect.

CONCLUSION: Abnormal shunting in upright position may be the result of different underlying conditions, requiring a thorough workup and a tailored treatment. In case of serious co-morbid conditions, an endovascular procedure to close a patent foramen ovale, after unsuccessful treatment of precipitating conditions, should be considered.