Volume 216 - Issue 1
A massive pulmonary arteriovenous malformation with platypnoea–orthodeoxia: an important and reversible cause of refractory hypoxaemia
Author: Michael Toolis
Med J Aust 2022; 216 (1): 19-19. || doi: 10.5694/mja2.51356
Published online: 17 January 2022
Published online: 17 January 2022
An 82-year-old woman presented to hospital with worsening chronic dyspnoea without fever or other specific symptoms
An 82‐year‐old woman presented to hospital with worsening chronic dyspnoea without fever or other specific symptoms. Despite receiving 100% oxygen via non‐invasive ventilation, arterial oxygen saturation remained low at 70–80%. However, the oxygen saturation improved when positioned flat — a phenomenon known as platypnoea–orthodeoxia, which is associated with right‐to‐left shunts.1,2 Chest x‐ray revealed a left mid‐zone mass contiguous with the hilar vessels (Figure, A, arrow), and echocardiography excluded an intracardiac shunt. Computed tomography scan with intravenous contrast demonstrated a massive pulmonary arteriovenous malformation at the level of the pulmonary arteries (Figure, B [axial] and C [coronal], arrows). This was subsequently managed by radiological embolisation with a 20 mm type II Amplatzer plug (St Jude Medical; Figure, D), with good radiographic result and rapid improvement in arterial oxygenation.3 Pulmonary arteriovenous malformations are an important and reversible cause of hypoxaemia due to right‐to‐left shunting and can result in paradoxical embolic stroke if untreated.4
Competing interests
No relevant disclosures.
References
- Agrawal A, Palkar A, Talwar A. The multiple dimensions of platypnea–orthodeoxia syndrome: a review. Respir Med 2017; 129: 31–38.
- Henkin S, Negrotto S, Pollak PM, et al. Platypnea–orthodeoxia syndrome: diagnostic challenge and the importance of heightened clinical suspicion. Tex Heart Inst J 2015; 42: 498.
- Hart JL, Aldin Z, Braude P, et al. Embolization of pulmonary arteriovenous malformations using the Amplatzer vascular plug: successful treatment of 69 consecutive patients. Eur Radiol 2010; 20: 2663–2670.
- Shovlin CL, Condliffe R, Donaldson JW, et al. British Thoracic Society clinical statement on pulmonary arteriovenous malformations. Thorax 2017; 72: 1154–1163.
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