Platypnea is a rare symptom of dyspnea in the upright position. It is relieved by assumption of the recumbent position and is the converse of orthopnea.
Orthodeoxia - arterial desaturation in the upright position, may also be present.
This rare syndrome may occur with COPD, following pneumonectomy, during recovery from adult respiratory distress syndrome, with recurrent pulmonary embolism, with cirrhosis of the liver, with autonomic dysfunction, or in patients with a patent foramen ovale.
Echocardiography (on a tilt table if possible) is used to look for a patent foramen ovale.
Proposed mechanisms for platypnea-orthodeoxia:
Postural increased right-to-left shunt through a patent foramen ovale.
In patients with underlying lung disease - areas with elevated alveolar pressures may exceed pulmonary arteriolar pressures. As a result, these areas are not perfused. In the upright position gravitational forces diminish pulmonary arteriolar pressure in the upper lung fields.
Loss of the normal pulmonary hypoxic vasoconstrictive response may cause shunting.
In the setting of a pericardial effusion - the effusion distorts the architecture of the right atrium when the patient is standing; in such a way as to direct the flow of blood returning from the inferior vena cava directly toward a patent foramen ovale.
Areas of lung where the pulmonary alveolar pressure exceeds the pulmonary arteriolar pressure are not perfused. This creates small amounts of pulmonary dead space in normal states. An increase in pulmonary dead space when the patient is upright may lead to platypnea.
Int J Cardiol. 2006 Jan 13;106(2):211-7.
Effects of postural change on oxygen saturation and respiration in patients after the Fontan operation: platypnea and orthodeoxia.
Suzuki H, Ohuchi H, Hiraumi Y, Yasuda K, Echigo S.
Department of Pediatrics, National Cardiovascular Center, Suita, Osaka, Japan. firstname.lastname@example.org
The aim of this study was to assess whether platypnea and orthodeoxia occur in Fontan patients. We divided 14 Fontan patients into 2 groups: 8 patients who had pulmonary arteriovenous fistulas and/or intra-atrial shunts (group A) and 6 patients who had neither pulmonary arteriovenous fistulas nor intra-atrial shunts (group B). They were compared with 9 controls (group C). Arterial oxygen saturation, minute ventilation per body weight and ventilatory equivalent for carbon dioxide were measured in the supine and then sitting positions. In group A, 1 patient had platypnea and 3 patients had orthodeoxia (changes in the saturation from the supine position to the sitting position were -4% to -7%) accompanied with slight hyperpnea, and all 4 patients had both pulmonary arteriovenous fistulas and intra-atrial shunts. Contrary, patients in group B had neither platypnea nor orthodeoxia. The saturation was significantly lower and the minute ventilation was significantly higher in the sitting position than in the supine position in group A (p < 0.05). The other groups showed no significant difference in the saturation or the minute ventilation between the 2 positions. All groups showed the ventilatory equivalent was significantly higher in the sitting position than in the supine position (p < 0.05 to 0.01). We demonstrated platypnea and orthodeoxia in Fontan patients with pulmonary arteriovenous fistulas and intra-atrial shunts. We believe platypnea and orthodeoxia should be regard as a complication in Fontan patients with pulmonary arteriovenous fistulas and/or intra-atrial shunts.
Cardiol Clin. 2005 Feb;23(1):85-9.
Patent foramen ovale and the platypnea-orthodeoxia syndrome.
Chen GP, Goldberg SL, Gill EA Jr.
Division of Cardiology, Department of Medicine, University of Washington School of Medicine, 1959 Pacific Avenue NE, Seattle, WA 98195, USA.
The platypnea-orthodeoxia syndrome is a rare but important condition caused by a variety of clinical entities. Several mechanisms have been postulated to cause platypnea and orthodeoxia.These mechanisms mainly involve intracardiac shunting, pulmonary vascular shunting, and ventilation-perfusion mismatching. Patent foramen ovale is an important type of intracardiac shunt that can produce platypnea-orthodeoxia in select patients. Concomitant pulmonary hypertension must be ruled out, but symptoms can occur without pulmonary hypertension in cases with altered intrathoracic anatomy and physiology.Diagnosis usually entails transthoracic or trans-esophageal echocardiogram, ideally with postural provocation by the tilt-table test. Treatment with surgical or percutaneous closure may result in symptomatic relief. The decision to intervene is based mainly on the severity of symptoms and potential morbidities associated with the defect.
J Am Soc Echocardiogr. 2001 Oct;14(10):1039-41.
Patent foramen ovale presenting as platypnea-orthodeoxia: diagnosis by transesophageal echocardiography.
Roxas-Timonera M, Larracas C, Gersony D, Di Tullio M, Keller A, Homma S.
Department of Medicine, Division of Cardiology, Columbia University, New York, NY, USA.
Platypnea-orthodeoxia is a rare syndrome that is often associated with interatrial shunting through a patent foramen ovale or atrial septal defect. We describe the case of a 65-year-old woman with progressive dyspnea and hypoxia when standing and walking, which was relieved by assuming the recumbent position. The diagnosis was confirmed by tilt-table transesophageal echocardiography demonstrating a large right-to-left shunt through a patent foramen ovale while the patient was in a semiupright position and no significant shunt while in a recumbent position. This case demonstrates that platypnea-orthodeoxia caused by a patent foramen ovale can be clearly demonstrated by the technique of contrast transesophageal echocardiography performed on a tilt table.
Catheter Cardiovasc Interv. 2001 Sep;54(1):77-82.
Comment in: Catheter Cardiovasc Interv. 2002 Apr;55(4):529-30.
Platypnea-orthodeoxia: management by transcatheter buttoned device implantation.
Rao PS, Palacios IF, Bach RG, Bitar SR, Sideris EB.
Saint Louis University School of Medicine, St. Louis, Missouri 63104, USA. email@example.com
Dyspnea and arterial desaturation on upright position in elderly subjects is described as platypnea-orthodeoxia syndrome (POS) and in some patients it is due to right-to-left shunt across the atrial septal defect (ASD)/patent foramen ovale (PFO). Surgical closure of ASD/PFO has been the only available treatment option. Buttoned device has been used for occlusion of ostium secundum ASD, PFO associated with presumed paradoxical embolism and cerebrovascular accidents and ASD/PFO in association with other congenital heart defects causing right-to-left shunt. The objective of this article is to describe the use of buttoned device in effectively occluding ASD/PFO to relieve hypoxemia of POS. During a 4-year period ending January 2000, 10 patients, ages 71 +/- 9 (range 60-83) years with POS underwent buttoned device closure of their ASD/PFO. Echocardiographic and balloon-stretched atrial defect sizes were 8 +/- 3 mm and 12 +/- 3 mm, respectively. The ASD/PFO were occluded with devices ranging in size from 25 to 40 mm delivered via 9 French, long, blue Cook sheaths; eight had an additional 25- or 35-mm occluder placed on the right atrial side. The oxygen saturation increased (P < 0.001) from 76 +/- 7% (range 69-86%) to 95 +/- 2% (range 92-98%). No complications were encountered. Relief of symptoms was seen in all patients. Follow-up of 1-36 months (median 12 months) revealed persistent improvement of symptoms. Buttoned device occlusion of ASD/PFO to relieve hypoxemia of POS is feasible, safe, and effective and is an excellent alternative to surgery. Cathet Cardiovasc Intervent 2001;54:77-82. Copyright 2001 Wiley-Liss, Inc.
Can Respir J. 2001 Mar-Apr;8(2):105-7.
Orthodeoxia and platypnea secondary to a patent foramen ovale despite normal right-sided cardiac pressures.
Varkul M, Robinson T, Ng E, Hyland R.
Department of Medicine, University of Toronto, Ontario, Canada.
Numerous cases of orthodeoxia and platypnea have been reported. Some have been found to be due to intracardiac shunts despite normal intracardiac pressures. In the case presented, a 79-year-old woman was noted to have orthodeoxia and platypnea secondary to a patent foramen ovale (PFO) despite normal intracardiac pressures. She recovered after closure of the PFO. The authors postulate that recent vertebral fractures and subsequent kyphosis, as well as a dilated thoracic aorta, altered intrathoracic relationships and may have led to a stream of venous return being directed across the PFO in the upright position, despite normal intracardiac pressures.
Chest. 2000 Aug;118(2):553-7
Platypnea-orthodeoxia syndrome related to an aortic aneurysm combined with an aneurysm of the atrial septum.
Faller M, Kessler R, Chaouat A, Ehrhart M, Petit H, Weitzenblum E.
Service de Pneumologie, Hopital de Hautepierre, Strasbourg, France.
We report the case of a 71-year-old man bearing a severe right-to-left shunt through a patent foramen ovale in the absence of elevated right-sided heart or pulmonary artery pressures. He presented with platypnea-orthodeoxia syndrome, but he had no pulmonary or extracardiac diseases that are known to be associated with this syndrome. Chest radiography showed a bulky aneurysm of the thoracic aorta. A peripheral contrast transesophageal echocardiography demonstrated a large right-to-left shunt through a patent foramen ovale. In addition, the atrial septum was severely deformed by an aneurysm including this patent foramen ovale. We hypothesized that the opening of the foramen ovale was the result of a mechanical deformation of the atrial septum by two contributing factors: the aneurysm of the thoracic aorta and the aneurysm of the septum itself.
Chest. 1997 Dec;112(6):1682-4.
Comment in: Chest. 1997 Dec;112(6):1449-51.
Platypnea-orthodeoxia related to aortic elongation.
Popp G, Melek H, Garnett AR Jr.
Department of Internal Medicine, Eastern Virginia Medical School, Norfolk, USA.
An 80-year-old woman presented with progressive shortness of breath. There was no history of pulmonary or cardiac disease. Results of a physical examination were normal. She had significant oxygen desaturation while she was in an upright position. Admission to the hospital for workup followed, and evaluation included tilt-table transesophageal echocardiogram and cardiac catheterization. A massive right-to-left shunt through a patent foramen ovale was detected, and surgical intervention resulted in dramatic improvement of symptoms. In this patient, it seems that the syndrome of platypnea-orthodeoxia was related to aortic elongation, allowing significant right-to-left shunt.
J Am Soc Echocardiogr. 1993 Nov-Dec;6(6):624-7. Related Articles, Links
Diagnostic value of transesophageal echocardiography in platypnea.
Herregods MC, Timmermans C, Frans E, Decramer M, Daenen W, De Geest H.
Department of Cardiology, Pneumology, and Cardiac Surgery, University Hospital Gasthuisberg, Leuven, Belgium.
Platypnea is a rare syndrome of orthostatic dyspnea frequently caused by an interatrial right-to-left shunt. The diagnosis is difficult. Assessment of arterial blood gases reveals orthostatic desaturation. In the past, definite diagnosis necessitated catheterization in the supine and upright position. Now transesophageal echocardiography on a tilt table combined with a peripheral venous contrast study provides correct diagnosis in a safe and easy way.
Acta Cardiol. 1993;48(6):583-90.
Comment in: Acta Cardiol. 1994;49(3):217. Acta Cardiol. 1995;50(1):103-4.
Platypnea-orthodeoxia syndrome: a report of two cases.
Timmermans C, Frans E, Herregods C, Decramer M, Daenen W, De Geest H.
Department of Cardiology, University Hospital Gasthuisberg, Leuven, Belgium.
Two cases of orthostatic dyspnea and arterial desoxygenation, as a postpneumonectomy complication, are reported. In one patient, echocardiography and cardiac catheterization revealed the presence of an atrial septal aneurysm, which has never been described in association with the platypnea-orthodeoxia syndrome. The other case illustrates that, despite actual technical possibilities, clinical suspicion remains a prerequisite for proper diagnosis of the entity.
J Card Surg. 1991 Mar;6(1):62-7.
Platypnea-orthodeoxia: an unusual indication for surgical closure of a patent foramen ovale.
Murray KD, Kalanges LK, Weiland JE, Binkley PF, Howanitz EP, Galbraith TA, Myerowitz PD.
Division of Thoracic and Cardiovascular Surgery, Ohio State University Hospitals and Clinics, Columbus.
Platypnea-orthodeoxia is an infrequently reported clinical form of position dependent dyspnea and oxygen desaturation. There are several diverse etiologies for this syndrome. This case report of a patient with platypnea-orthodeoxia is the first known presentation of a case secondary to a patent foramen ovale and idiopathic hemidiaphragm paralysis. A major problem with this disease is the clinical recognition of its existence. Full recovery is possible with proper assessment of clinical signs and symptoms, appropriate noninvasive diagnostic tests, and corrective surgery in selective cases.
Platypnea-orthodeoxia and progressive autonomic failure.
Fox JL, Brown E, Harrison JK, Williams J, Terry PB.
Department of Medicine, Johns Hopkins Hospital, Baltimore, Maryland.
Platypnea-orthodeoxia has been described in patients with pulmonary shunts, intracardiac shunts, and severe lung disease. We report a case of platypnea-orthodeoxia in a patient with minimal obstructive lung disease and progressive autonomic failure. We have shown our patient's symptoms to be the result of an orthostatic increase in ventilation-perfusion mismatching and to be corrected with fluid challenge.
Mayo Clin Proc. 1984 Apr;59(4):221-31.
Platypnea-orthodeoxia: clinical profile, diagnostic workup, management, and report of seven cases.
Seward JB, Hayes DL, Smith HC, Williams DE, Rosenow EC 3rd, Reeder GS, Piehler JM, Tajik AJ.
Platypnea-orthodeoxia is a rare and poorly understood syndrome of orthostatic accentuation of a right-to-left shunt, usually across a patent foramen ovale. The syndrome is most commonly recognized in patients with a history of a major pulmonary disorder such as pneumonectomy, recurrent pulmonary emboli, or chronic lung disease. Pulmonary artery pressures are typically normal. The physiologic mechanism is unknown. We recommend that initial assessment consist of measurement of blood gases with the patient in the supine and upright positions. Orthostatic desaturation should prompt further investigation. A definitive diagnosis can most easily be obtained by tilt-table two-dimensional echocardiography with peripheral venous contrast medium. The shunt can be localized at the atrial level and directly visualized and semiquantitated. The decision about surgical closure of the patent foramen ovale is based on the degree of clinical disability. Because a significant shunt is manifest only in the upright position, astute clinical suspicion is of paramount importance for proper diagnosis. Increased awareness of this syndrome and ease of echocardiographic diagnosis will facilitate recognition of this potentially treatable cause of orthostatic hypoxia.
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