Hypoxemia-orthodeoxia related to patent foramen ovale without pulmonary hypertension

Toffart, Anne Claire; Bouvaist, Hélène; Feral, Virginie; Blin, Dominique; Pison, Christophe

doi:10.1016/j.hrtlng.2007.09.006

Cited by 42 publications

(28 citation statements)

References 12 publications

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“…Even if an interatrial communication (PFO or ASD) is necessary for cardiac POS development, and ASA or prominent Eustachian valve act as contributing factors, the main cause for right-to-left shunt development is right chamber anatomy modification in later life [16]. Several mechanical conditions -mainly right diaphragmatic paralysis and ascension [17,18], kyphoscoliosis [19], restrictive lung disease, previous pneumonectomy [4,5,20], pleural effusion, and ectatic/aneurysmal ascending aorta [21,22] -may lead to atrial chamber or septum deformity, thus changing the anatomic relationship between the atrial septum and the inferior vena cava and therefore facilitating desaturated blood flow redirection through the PFO. In our case series, the septum anatomical modifications were due to aortic dilatation, right diaphragmatic paralysis and ascension, restrictive lung disease and kyphoscoliosis.…”

Section: Discussionmentioning

confidence: 99%

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

Blanche

Noble

Roffi

et al. 2013

European Journal of Internal Medicine

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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.

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Section: Discussionmentioning

confidence: 99%

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

Blanche

Noble

Roffi

et al. 2013

European Journal of Internal Medicine

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“…Pneumectomy and lung surgery were among the earliest conditions associated with POS [52], and both types of mechanisms can play a part. Marini et al [53] described 36 cases of such conditions, most of which occurred more than 1 month after surgery.…”

Section: Introductionmentioning

confidence: 99%

Platypnea-Orthodeoxia Syndrome in Review: Defining a New Disease?

Rodrigues¹,

Palma²,

Sousa-Pereira³

2012

Cardiology

127

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Platypnea-orthodeoxia syndrome is an intriguing condition that comprehends multiple pathologic phenomena. It requires a high clinical suspicion and is probably underdiagnosed. Its identification has therapeutic implications and can have a huge impact on the patient’s everyday life. We made a review of the literature and analyzed 188 cases described thus far, trying to identify the underlying mechanisms and risk factors. We propose that a separate entity is created within the spectrum of platypnea-orthodeoxia syndrome that is responsible for the majority of cases, namely platypnea-orthodeoxia disease, which would be defined as the existence of an interatrial communication, with a right-to-left shunt, normal pressure in the right atrium and, of course, platypnea and orthodeoxia. Most patients with this disease were submitted to percutaneous closure of the patent foramen ovale or atrial septal defect, with favorable results, making it the first-line therapeutic approach nowadays.

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“…9 Symptoms are exacerbated by an upright position because increased septal stretching causes preferential flow from the inferior vena cava through the shunt and a secondary reduction of pulmonary blood flow. 3,11 The prevalence of PFO or ASD as a cause of physiologically significant POS is unknown. The PFO defect itself occurs in perhaps 20% to 34% 12 of people, and ASDs are less common than that.…”

Section: Discussionmentioning

confidence: 99%

Platypnea-Orthodeoxia Syndrome: Diagnostic Challenge and the Importance of Heightened Clinical Suspicion

Henkin

Negrotto

Pollak³

et al. 2015

Texas Heart Institute Journal

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P latypnea-orthodeoxia syndrome (POS) is an uncommon condition of positional dyspnea (platypnea) and hypoxemia (orthodeoxia). The symptoms occur when the patient is upright and resolve quickly with recumbency. These findings are the opposite of those typically seen in cases of advanced heart failure and can pose a diagnostic dilemma.Even though POS was first described in the late 1940s, 1 the pathophysiologic triggers of orthodeoxia and platypnea are still not completely understood. It appears that both a functional component and an anatomic component are required.2 The syndrome has been associated with cardiac, pulmonary, abdominal, and vascular disease (functional component) in conjunction with a shunt (anatomic component). 2 The most frequently described cause is right-to-left interatrial shunting from a patent foramen ovale (PFO) or an atrial septal defect (ASD) in the presence of pulmonary hypertension.3 Positional variation in the degree of shunting in these situations remains incompletely understood. It is believed that the upright position changes or stretches the conformation of the interatrial communication, increasing the proportion of blood flow from the inferior vena cava through the defect and into the left atrium. 3 We report our experience in treating a patient who presented with POS in the absence of substantial pulmonary hypertension. Case ReportIn September 2013, an 83-year-old woman with a history of paroxysmal atrial fibrillation was admitted to the hospital after several weeks of progressive positional dyspnea that worsened with standing and improved with lying supine. On admission, the patient's pulse oximetry on room air was 96% while supine and 81% while standing. Physical examination was noteworthy for marked kyphoscoliosis. Computed tomographic evaluation for lung disease revealed a tortuous thoracic aorta with otherwise normal lung parenchyma. The patient's arterial blood gases on room air showed a significant drop in arterial oxygen tension upon postural change (from lying down to standing), confirming our suspicion of POS (Table I).A transthoracic echocardiogram revealed normal pulmonary artery pressure, with an atrial bidirectional shunt. A transesophageal echocardiogram revealed an ASD of moderate size (area, 2.2 cm 2 ) (Fig. 1), together with bidirectional shunting while the patient was supine and sedated.

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Hypoxemia-orthodeoxia related to patent foramen ovale without pulmonary hypertension

Cited by 42 publications

References 12 publications

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

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

Platypnea-Orthodeoxia Syndrome in Review: Defining a New Disease?

Platypnea-Orthodeoxia Syndrome: Diagnostic Challenge and the Importance of Heightened Clinical Suspicion

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