Associazione Nazionale Medici Cardiologi Ospedalieri



Platypnoea-orthodeoxia: a case report of a rare syndrome

Fontana Antonella Avezzano (Aq) – Ospedale Ss. Filippo E Nicola | Occhiuzzi Enrico Avezzano (Aq) – Ospedale Ss. Filippo E Nicola | Carducci Paolo L’Aquila (Aq) – Ospedale San Salvatore | Valentini Giuliano Avezzano (Aq) – Ospedale Ss. Filippo E Nicola | Balsano Clara L’Aquila (Aq) – Universit√† Degli Studi De L’Aquila

A 32-year-old non-smoker Asian female was admitted to the Emergency Department San Salvatore Hospital of L'Aquila experiencing worsening of dyspnea. Three months earlier the patient had undergone left pneumonectomy. An arterial blood gas analysis performed on air reported: pH 7.53, pCO2 23.7 mmHg, pO2 38.7 mmHg, SpO2 80%. Blood tests were within normal limits, except for a moderate increase in D-Dimer.

Considering the diagnostic hypothesis of pulmonary embolism, an Angio CT-scan of the chest was performed and showed no radiological characteristics suggesting parenchymal pathology and no evidence of thromboembolic or other vascular pathology.

From the accurate clinical evaluation of the patient, a condition of severe platypnoea was evident: supine decubitus was obliged. Together with the above position variations, a strong desaturation was observed, going from almost normal values (94-96% SpO2), to 80-82% SpO2 when she was sitting up in bed.

Based on the data above, a right-left shunt condition was assumed, and a transesophageal contrast echocardiogram was requested. The latter demonstrated patency of the foramen ovale.

The first description of a condition very similar to platypnoea-orthodeoxia syndrome following pneumonectomy dates to 1956, by Schnabel et al.

A recent pubmed database-EMBASE systematic review identified 8 studies concerning ten patients, 6 male/4 female. The mean age was 62 years. All patients underwent resection of the right lung. The time-interval between the operation and the platypnoea-orthodeoxia syndrome (POS) was from 2 days to 3 years. A PFO was present in 90%.

Considering the data, our patient shows at least two peculiarities; the first and most relevant is the young age: in fact, there is only one description of POS in a patient of younger age.

The second peculiarity is the left localization of the previous pneumonectomy, much rarer in frequency than the contralateral in the known cases. Partial or total pulmonary resection can rarely be followed by the onset of POS. This syndrome can be severely disabling, so physicians must be prepared for its prompt recognition, maintaining a high degree of suspicion.