Platypnea orthodeoxia syndrome in a patient with pulmonary arterial hypertension

Sinanis, Theodoros; Krüger, Ulrich; Schöls, Wolfgang

doi:10.15761/ccrr.1000374

Cited by 2 publications

(2 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…With intrapulmonary shunts, the shunting mechanism increases pulmonary vascular resistance, subsequently increasing right atrial pressure, leading to a reversal of the left-to-right pressure gradient [4,5]. Additionally, pulmonary arteriovenous malformations, often seen in the setting of cirrhosis due to an imbalance in the ratio of pulmonary vasoconstrictors and vasodilators, may result in shunt physiology and the failure to oxygenate venous blood adequately [6]. An upright position may result in further hypoxemia and manifest as POS because the location of the defects is predominantly in the lung bases, and the upright position causes a gravitationally mediated increase in pulmonary blood flow through these defects [7].…”

Section: Discussionmentioning

confidence: 99%

Patent Foramen Ovale and Ascending Aortic Dilatation Causing Platypnea-Orthodeoxia Syndrome

Ahmed¹,

Rupal²,

Walker³

et al. 2022

Cureus

View full text Add to dashboard Cite

Platypnea-orthodeoxia syndrome (POS) is an underdiagnosed clinical syndrome characterized by dyspnea (platypnea) and hypoxemia (orthodeoxia) in the upright position that resolves when recumbent. POS is often due to an underlying right-to-left shunt. Four broad mechanisms for the shunt have been described: intracardiac shunts, intrapulmonary shunts, hepatopulmonary syndrome, and pulmonary ventilationperfusion mismatch.A 68-year-old male with a past medical history of chronic obstructive pulmonary disease (COPD), obstructive sleep apnea, ascending aortic dilation (3.9 cm), myelofibrosis, and status post stem cell transplant complicated by graft versus host disease was found hypoxemic (oxygen saturation: 82%) on routine visit prompting hospitalization. Hypoxemia initially responded to 40% FiO2 but subsequently progressed to refractory hypoxemia on 100% FiO2. A chest computed tomography (CT) scan showed evidence of multiple segmental pulmonary emboli with patent central pulmonary arteries. Hypoxemia out of proportion to pulmonary embolism clot burden and examination findings consistent with orthodeoxia prompted further investigations. Nuclear medicine scan showed radiotracer activity in both brain and kidneys consistent with a small right-to-left shunt (5.9%). Transesophageal echocardiography (TEE) revealed a patent foramen ovale (PFO) with a right-to-left shunt across the atrial septum, with a maximum opening of 3.5 mm and tunnel length of 25 mm. Right heart catheterization (RHC) is consistent with the right-to-left shunt and normal right heart pressures. The degree of the shunt was not significant enough to explain the degree of hypoxemia, but all the diagnostic studies were performed in a supine position, possibly underestimating the degree of the shunt. PFO closure with transcatheter 30-mm Gore device (GORE® CARDIOFORM, Arizona, USA) decreased supplemental oxygen requirement from 75% high-flow nasal cannula (NC) to room air (RA) immediately after the procedure. The patient was subsequently discharged home on a baseline oxygen requirement of 2 L NC at nighttime. POS should be suspected when a patient develops severe hypoxemia after changing from a recumbent position to a sitting or standing position. The identification and correction of the shunting or mismatch often allow complete resolution of POS. Transthoracic echocardiography with agitated saline, TEE, and RHC are the diagnosis modalities of choice. Left heart cardiac catheterization remains the gold standard, which would demonstrate a mismatch in oxygen saturation between the pulmonary vein and the aorta. Our patient's PFO was successfully closed by a percutaneous transcatheter closure device leading to the complete resolution of hypoxemia immediately.

show abstract

Section: Discussionmentioning

confidence: 99%

Patent Foramen Ovale and Ascending Aortic Dilatation Causing Platypnea-Orthodeoxia Syndrome

Ahmed¹,

Rupal²,

Walker³

et al. 2022

Cureus

View full text Add to dashboard Cite

show abstract

“…In third place, the right atrial pressure (RAP) must be considered. Elevated RAP is the result of pulmonary hypertension (by left heart or pulmonary diseases), pneumonectomy, and less frequently, pericardial disorders [13][14][15][16] . Patients with POS and normal RAP generally have accompanying anatomical modifications in the ascending aorta or spine.…”

Section: Intracardiac Shuntmentioning

confidence: 99%

Mecanismos del síndrome de platipnea-ortodesoxia

Salas-Pacheco¹

2021

ACM

View full text Add to dashboard Cite

Platypnea orthodeoxia syndrome (POS) is a clinical entity described in the middle of the last century. It is characterized by dyspnea and hypoxemia triggered by standing and relieved with recumbency. The diagnosis is predominately clinical. The degree of hypoxemia is variable; however, the diagnostic criteria include the decrease in arterial oxygen pressure more than 4 mmHg or oxygen saturation more than 5%. Even though many diseases cause this syndrome, there are only two responsible mechanisms, intracardiac, and intrapulmonary shunts. The coexistence of diverse structural and physiological abnormalities joined to gravitational forces that induce blood shunt after standing is crucial in each mechanism. The intracardiac mechanism is characterized by right to left blood shunt through atrial septal communications and, the right atrium pressure could be normal or increased. In addition, some patients have one or more coexistent aortic, spinal, or intracardiac alterations. The intrapulmonary mechanism is less frequent and is caused by parenchymal or vascular pathologies. Transthoracic echocardiogram is the first diagnostic modality; however, understanding the pathophysiology is the key for a rational diagnostic approach and subsequent diagnostic studies. Treatment is possible and effective in the majority of intracardiac mechanisms and some intrapulmonary. This review focuses on the pathophysiologic mechanisms of POS and their diagnostic workup.

show abstract

Platypnea orthodeoxia syndrome in a patient with pulmonary arterial hypertension

Cited by 2 publications

References 9 publications

Patent Foramen Ovale and Ascending Aortic Dilatation Causing Platypnea-Orthodeoxia Syndrome

Patent Foramen Ovale and Ascending Aortic Dilatation Causing Platypnea-Orthodeoxia Syndrome

Mecanismos del síndrome de platipnea-ortodesoxia

Contact Info

Product

Resources

About