Platypnoea–orthodeoxia syndrome (POS) is a rare disorder, manifesting as deoxygenation occurring when the patient is in the upright position. Four broad mechanisms for the condition have been described: intracardiac shunts, intrapulmonary shunts, hepatopulmonary syndrome and pulmonary ventilation–perfusion mismatch. Here, we present the first case of POS in a patient with a proven right to left intracardiac shunt occurring in the context of postural hypotension and normal right heart pressures. We highlight the need to carry out investigations in the upright position before discounting intracardiac shunting as a cause for the syndrome. Short-term improvement of the syndrome was obtained with medical management of the patient’s orthostatic hypotension and as such suggests a conservative management strategy for similar patients, which may delay the need for invasive procedures to close the anatomical defect.
- lung physiology
- rare lung diseases
- imaging/ct mri etc
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Contributors FL and FG were involved in the clinical care of the patient at the Royal Brompton Hospital and co-wrote the manuscript. RP was involved in the clinical care of the patient at Ealing Hospital and co-wrote his section of the manuscript. SDR was the patient’s lead clinician while at Ealing hospital. He selected the images and supplementary video for this case report and co-wrote his section of the manuscript. SW performed and reported the anatomical shunt study. He co-wrote his section of the manuscript. MH was involved in the clinical care of the patient at the Royal Brompton Hospital and co-wrote the manuscript. MIP was the lead clinician responsible for the patient’s care at the Royal Brompton Hospital and co-wrote the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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