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Platypnoea-Orthodeoxia Syndrome: Haemodynamic Complexity of Patent Foramen Ovale and Aortic Stenosis
Session:
CASOS CLÍNICOS DE INTERVENÇÃO (PERCUTÂNEA E CIRÚRGICA)
Speaker:
Rita Louro
Congress:
CPC 2025
Topic:
H. Interventional Cardiology and Cardiovascular Surgery
Theme:
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Subtheme:
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Session Type:
Sessão de Casos Clínicos
FP Number:
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Authors:
Rita Louro; Marta Figueiredo; Orlando Luquengo; Rafael Viana; António Almeida; Miguel Carias; Gustavo Sá Mendes; Manuel Trinca
Abstract
<p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"><strong>Introduction</strong></span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000">Platypnoea-orthodeoxia syndrome (POS) is a rare condition characterized by positional hypoxemia and a right-to-left interatrial shunt, commonly associated with a patent foramen ovale (PFO). This case illustrates the complex hemodynamic mechanisms issues that lead to the late appearance of POS in a patient with moderate aortic stenosis (AS). </span></span></span></p> <p style="text-align:justify"> </p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"><strong>Case Report:</strong></span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000">An 87-year-old male, previously autonomous, presented with partial respiratory insufficiency. He reported worsening dyspnea, orthopnea, and fatigue over the previous weeks. </span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000">Upon admission, physical examination revealed central cyanosis and oxygen saturation of 85% while sitting, which improved to 94% when lying down. Cardiovascular auscultation revealed a systolic murmur grade II/VI.</span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000">The initial workup excluded pulmonary embolism, infection, and ischemia. Arterial blood gases confirmed severe hypoxemia with a pO2 of 45 mmHg seated, improving to 54 mmHg supine, without hypercapnia. Transthoracic echocardiography showed moderate AS (mean gradient 18 mmHg, valve area 1.35 cm<sup>2</sup>), left ventricular hypertrophy (LVH), and preserved ejection fraction, but did not explain the positional hypoxemia. </span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000">Transesophageal echocardiography revealed a PFO with a significant right-to-left shunt exacerbated by the Valsalva maneuver, a mobile interatrial septum, and a mildly dilated pulmonary trunk (4 cm). These findings confirmed POS due to hemodynamic interactions between the right and left atria. Percutaneous closure of the PFO resulted in substantial improvement in oxygenation, though residual hypoxemia persisted in the upright position.</span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000">The late onset of POS in this patient reflects the interplay of anatomical and hemodynamic factors. Moderate AS contributed indirectly by chronically elevating LA pressure, but dynamic positional changes and mild pulmonary hypertension overcame this effect, enabling the right-to-left shunt. Early identification of POS via transoesophageal echocardiography and percutaneous PFO closure were crucial in stabilising the patient.</span></span></span></p> <p style="text-align:start"> </p> <p style="text-align:start"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000"><strong>Conclusion:</strong></span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Aptos,sans-serif"><span style="color:#000000">This case highlights the importance of recognising platypnoea-orthodeoxia syndrome in patients with unexplained positional hypoxaemia. Haemodynamic interactions between PFO, aortic stenosis, and pulmonary pressures emphasise the need for comprehensive cardiac evaluation and multidisciplinary management.</span></span></span></p>
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