Platypnea–orthodeoxia syndrome in the elderly treated by percutaneous patent foramen ovale closure: A case series and literature review

Abstract

Background: Platypnea-orthodeoxia syndrome (POS) is a rare clinical phenomenon, associating normal oxygen saturation in a supine position and arterial hypoxemia in an upright position. This pathology can be secondary to an intracardiac shunt, a pulmonary vascular shunt or a ventilation-perfusion mismatch. Cardiac POS occurs in the presence of a right-to-left cardiac shunt, most commonly through a patent foramen ovale (PFO). Methods and results: From our single-center prospective database of percutaneous PFO closure we identified five patients (4 females, mean age: 77 ± 11 years) out of 224 (2.2%) patients with a PFO who presented with a POS of cardiac origin. Transthoracic and transoesophageal echocardiographic examinations revealed the underlying mechanisms of POS and the diagnosis was confirmed by right-and-left cardiac catheterization (RLC) and by measuring serial blood oxygen saturation in the pulmonary veins and left atrium in supine and upright positions. PFO was associated with atrial septal aneurysm and a persistent prominent Eustachian valve in 3 patients. All patients underwent a successful percutaneous PFO closure without any immediate or subsequent complications (mean follow-up of 24 ± 18 months). Immediately after the procedure, mean arterial oxygen saturation improved from 83% ± 3 to 93% ± 2 in an upright position and symptoms disappeared. Conclusion: POS is a rare and under-diagnosed pathology that must be actively investigated in the presence of position-dependent hypoxemia. The diagnostic work-up must exclude other causes of hypoxemia and confirm the intracardiac shunt using either contrast echocardiography or RLC. For cardiac POS, percutaneous PFO closure is a safe and effective technique that immediately relieves orthodeoxia and patient symptoms.

Platypnoea-orthodeoxia syndrome: more than just a PFO

“…As only a few case-series and case-reports have described this syndrome, it is likely under-diagnosed and probably under-reported [2][3][4]. Diagnosing POS is challenging, and our case confirms that in patients presenting with dyspnea POS is usually a "rule-out diagnosis" which is suspected only when other causes to the dyspnea have been excluded.…”

Patent foramen ovale: Unanswered questions

“…Later in 2013, Blanche et al described a case series of 5 patients with POD who underwent successful closure of PFO with subsequent improvement in oxygenation and symptom resolution [56]. Patients experienced immediate and sustained long term symptomatic relief while achieving immediate normal oxygen levels (oxygen saturation increased from 85 ± 11% to 95 ± 6%) after PFO closure [57].…”

Cardiac platypnea-orthodeoxia syndrome in a 73-year-old woman

“…6,8 Other possible diagnoses, such as pulmonary hypertension, should be excluded before closure of the defect to avoid clinical deterioration of the patient's condition after correction of right-to-left-shunt.…”

“…Contrast transthoracic echocardiography helps to detect the presence of a right-to-left shunt, whereas transesophageal echocardiography is better for assessing atrial anatomy. 6 The gold standard diagnostic test for platypnea-orthodeoxia syndrome remains cardiac catheterization with oxygen measurement directly in the left atrium. 6,7 …”

“…6 The gold standard diagnostic test for platypnea-orthodeoxia syndrome remains cardiac catheterization with oxygen measurement directly in the left atrium. 6,7 …”