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PLATYPNEA–ORTHODEOXIA
SYNDROME
Roshan Gunathilake MD
John Hunter Hospital, Newcastle
Australia
Case report
68-year-old female Progressive dyspnoea 12 weeks NYHA Class III symptoms No symptoms of heart failure No cough/ haemoptysis Nil constitutional symptoms
Background
Ex-smoker - 40 pack years, Quit 2/12 COPD - inh. Symbicort and Seretide Treated HTN x 30 years (Lercanidipine) Previous post-operative DVT 2005 Osteoporotic vertebral fractures
(bisphosphonate) Cholecystectomy
Physical Examination
BMI 29 Afebrile RR 20, spO2 94% 2L (QAS 78% RA) HR 92/ min SR BP 155/88 JVP↔ S1S2, no murmur R/ basal coarse crepts No organomegaly Mild pedal oedema, no signs of DVT
Investigations
CRP 5 Hb 118 WBC 5.2 Biochemistry normal CXR – Hyperinflated lungs, R/basal
atelectasis, Osteoporotic # Bedside spirometry: FEV1 1.4l (53%
pred) ; FVC 2.5l (75% pred); FEV1% 56% ECG RBBB
ABG
pH 7.38 PaO2 58.9 mmHg PCO2 22.5 mmHg A-a Gradient = 62
Calculate A-a Gradient
Normal
Hypoventilation
Increased
V/Q mismatch OR shunt
Oxygen
PaO2↑ PaO2 unchanged
V/Q mismatch
Shunt
Initial management plan
Therapeutic clexane CTPA next day a.m. Oxygen to maintain SpO2 >88% ICU aware ARP discussed, for full resus.
Progress
Sudden dyspnoea after mobilising to the shower, SpO2 58%
MET call activated ABG 7.49, pCo2 34 mm Hg, pO2 38 mm Hg Taken over by critical care team for
observation, repeat ABG = to base line CTPA negative for PE, no other abnormality Urgent echocardiogram – normal LVEF, no
septal defect
Supine UprightspO2 93% 74%pO2 73 41
Platypnea–orthodeoxia syndrome
“ Uncommon syndrome of dyspnoea resulting from arterial hypoxaemia, which is accentuated by the upright position and relieved by recumbency”
Platypnea–orthodeoxia syndrome
Suspect when hypoxemia is: 1. More pronounced than expected on the
basis of cardiac and pulmonary findings2. Positional
Platypnea–orthodeoxia syndrome
Intracardiac RL shuntsIsolated defectAssociated with another process
Severe V/Q mismatchPost-pneumonectomy Pulmonary embolismARDSSevere obstructive lung disease
Intrapulmonary shunting Hepato-pulmonary syndrome
Dysautonomia
TOE with agitated saline contrast
Outcome
Right heart cathterization confirmed shunt, normal pulmonary pressures
Percutaneous device closure 1. Improved symptoms and hypoxia2. Also eliminated site for a possible
paradoxical embolus Asymptomatic at 1 month follow up visit
Summary
Platypnea-orthodeoxia is a less recognized syndrome that can occur in a variety of clinical settings.
The underlying mechanism mostly involves a R L intracardiac or intrapulmonary shunt, or severe V/Q mismatch.
Definitive treatment of the underlying cause often leads to rapid improvement of symptoms.
