What is the management of Platypnoea orthodeoxia?

Management of Platypnea-Orthodeoxia Syndrome

The definitive management of platypnea-orthodeoxia syndrome is percutaneous closure of the intracardiac shunt (typically a patent foramen ovale), which achieves complete resolution of symptoms in 86% of patients and should be performed once the diagnosis is confirmed. 1

Diagnostic Confirmation

Before proceeding to treatment, confirm the diagnosis by demonstrating:

• Positional oxygen desaturation: Arterial oxygen saturation drops when upright and normalizes when supine 2, 3
• Intracardiac shunt identification: Transesophageal echocardiography with agitated saline contrast demonstrating right-to-left shunting across a patent foramen ovale or atrial septal defect, particularly when changing from supine to sitting position 3, 4
• Normal pulmonary pressures: Right heart catheterization showing normal right and left atrial pressures rules out pulmonary hypertension as the driving mechanism 2, 4
• Arterial blood gas analysis: Document the PaO₂/FiO₂ ratio difference between supine and upright positions 1

Definitive Treatment: Percutaneous Closure

Percutaneous transcatheter closure of the patent foramen ovale is the treatment of choice and should be performed in all confirmed cases without pulmonary hypertension. 4, 1

Expected Outcomes

• Total clinical success (SpO₂ >94% in both positions without supplemental oxygen) occurs in 86% of patients 1
• PaO₂/FiO₂ ratio improves from approximately 156 to 318 within 24 hours post-procedure 1
• Symptom resolution is immediate and durable, maintained at long-term follow-up (mean 37 months) 1
• Complete resolution of dyspnea occurs in the vast majority of successfully treated patients 3, 1

Device Selection

Use a single occluder device appropriate for the defect size (Amplatzer Cribiform occluder or similar devices have been successfully employed) 2, 1

Supportive Management Prior to Closure

While awaiting definitive closure:

• Supplemental oxygen: Administer as needed to maintain SpO₂ >90%, recognizing that oxygen requirements will be position-dependent 3
• Avoid upright positioning: Keep patients supine or semi-recumbent to minimize hypoxemia 4, 5
• Expedite closure: Do not delay definitive treatment, as symptoms are often severe (NYHA class IV dyspnea) and quality of life is significantly impaired 2

Post-Procedure Follow-Up

• Immediate assessment: Measure oxygen saturation in both supine and upright positions within 24 hours to confirm resolution 1
• Echocardiographic surveillance: Perform transthoracic echocardiography at 12-24 months to confirm absence of residual shunt 1
• Long-term monitoring: No significant residual shunts are expected if initial closure is successful 1

Critical Pitfall to Avoid

Do not attribute positional dyspnea and hypoxemia solely to pulmonary causes without ruling out intracardiac shunting. Platypnea-orthodeoxia syndrome is frequently overlooked because the dramatic positional component mimics pulmonary disease, leading to delayed diagnosis and unnecessary investigations. 3, 4 The key distinguishing feature is the immediate normalization of oxygen saturation with recumbency, which should prompt urgent echocardiographic evaluation with agitated saline contrast in both supine and sitting positions. 4, 5