Copyright © 2005, The European Society of Cardiology
Platypnoea–orthodeoxia syndrome in association with an ascending aortic aneurysm
Department of Cardiology, Taunton and Somerset NHS Trust, Musgrove Park, UK
Received 28 March 2005; received in revised form 17 September 2005; accepted after revision 29 September 2005.
* Corresponding author. Tel. +44 1179 230 000. mandietownsend{at}yahoo.co.uk
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Abstract |
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Platypnoea–orthodeoxia syndrome is a uncommon condition presenting with dyspnoea and cyanosis that are characteristically worse in the upright posture and improved by lying supine.
We present the case of a patient with a thoracic aortic aneurysm and a patent foramen ovale who presented with platypnoea–orthodeoxia syndrome. Diagnosis was established using transoesophageal echocardiography. We suggest a mechanism for the development of dyspnoea and hypoxia in patients with this disorder.
Keywords: Platypnoea–orthodeoxia; Echocardiography; Cyanosis
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Case report |
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A 75-year-old female presented with a 2-month history of general malaise and recurrent collapses associated with hypotension and breathlessness. She had a past medical history of hypothyroidism, hypertension and osteoporosis. Treatment consisted of thyroxine 75µg once daily, atenolol 25mg once daily and alendronic acid 5mg once daily. On admission she was centrally cyanosed with a peripheral oxygen saturation of 85% on room air. Examination revealed an early 3/4 length diastolic murmur compatible with aortic regurgitation and a blood pressure of 158/84mmHg. The rest of the examination was unremarkable. Her resting 12 lead ECG was normal and chest X-ray demonstrated a widened mediastinum. Arterial blood gases confirmed marked hypoxaemia and hypocapnoea (PO2 5.3Kpa, PCO2 3.3Kpa). There was no improvement in the oxygen saturation with 100% inspired oxygen. A ventilation–perfusion scan showed no evidence of a lung defect. A trans-thoracic echocardiogram demonstrated a dilated ascending aorta with a root diameter of 5.5cm at the sino-tubular junction. The aortic valve was tri-foliate and moderate aortic regurgitation was present. Left ventricular ejection fraction and right heart structures were normal.
A transoesophageal echocardiogram confirmed normal left ventricular function and dimensions. The ascending aorta was large with a fusiform aneurysm measuring a maximum of 6cm in diameter. Spontaneous contrast was visualised in the aorta suggesting a low cardiac output. The atria were of normal size and a patent foramen ovale (PFO) was identified. Colour flow mapping illustrated evidence of right to left shunting through the PFO (see Fig. 1). Agitated saline was injected intravenously via the left arm and crossed the septum into the left atrium within two cardiac cycles. No contrast was visualised entering the left atrium from the four pulmonary veins, therefore excluding an intrapulmonary shunt. Moderate aortic regurgitation was confirmed and mild aortic atheroma was found.
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Cardiac catheterisation demonstrated normal pulmonary artery pressures of 26/8mmHg (mean 17mmHg). Right atrial pressure was 13/8mmHg with a mean of 8mmHg. Right ventricular pressure was 18/5mmHg with an end-diastolic pressure of 3mmHg. There was no evidence of left to right intra-cardiac shunt on measurement of oxygen saturations. An aortogram confirmed the ascending aortic aneurysm with grade III aortic regurgitation and normal left ventricular function. The coronary arteries were normal.
It was observed that her oxygen saturations improved during the transoesophageal echocardiogram whilst supine and decreased on sitting upright following the procedure. Platypnoea–orthodeoxia syndrome (POS) was suggested as a possible diagnosis. In order to investigate the mechanism of desaturation further, a second transoesophageal echocardiogram was performed with the patient on a tilt table. This demonstrated that the aortic root was abnormally displaced anteriorly, inferiorly and to the right. The PFO measured 0.7cm in supine and increased to 0.9cm in the semi-erect position. Using colour flow mapping we demonstrated the increased flow through the PFO into the left atrium in the upright posture. Peripheral agitated saline injection also confirmed a greater right–left shunt through the PFO in the upright position.
The patient was referred for cardiac surgery to close the PFO and repair the ascending aortic aneurysm. At operation a large ascending aortic aneurysm extending to the arch was found. The aortic valve was found to be incompetent and moderately degenerated. The superior limbus of the PFO was displaced anteriorly by the aneurysm. The aortic valve was replaced with a 21mm Edwards perimount prosthesis and the ascending aorta by a Gelseal graft, the PFO was closed by suturing.
Following surgery her oxygen saturations returned to normal and exercise tolerance increased.
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Discussion |
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We describe the poorly understood syndrome of platypnoea–orthodeoxia that is characterised by dyspnoea and arterial hypoxia exacerbated by upright posture.1 POS can occur in a variety of settings such as post-pneumonectomy, chronic obstructive lung disease and in some patients with pulmonary vascular shunting particularly associated with liver cirrhosis.2–4 A similar condition has been described in patients with metastatic carcinoid syndrome5 and in individuals with an atrial septal aneurysm and rarely in association with an aortic aneurysm.6–8 In such patients the right to left shunting arises even in the absence of the increased right heart pressures normally seen in cases of right to left shunting.9
The incidence of PFO occurs in up to 24% of the population,10,11 in contrast to this POS is rare and would suggest that an additional anomaly must be present to result in the syndrome. Anderson et al. have described the structure of the atrial septum.12 We propose that the anatomical distortion of the intra-atrial septum by the development of an ascending aortic aneurysm could explain the mechanism platypnoea–orthodeoxia syndrome in this patient. The aortic aneurysm in our patient resulted in anterior displacement of the superior limbus into the right atrial cavity causing the oval foramen to be held open, leading to equalisation of the atrial pressures. The shunt was greatest in the erect position due to the increased PFO aperture size. In addition there was enhanced streaming of blood flow from the inferior vena-cava across onto the base of the aorta and across the inter-atrial communication. This appears to have been due to the further anterior and inferior displacement of the aortic root due to the effects of gravity in the upright posture. This proposed mechanism is supported by the transoesophageal echocardiographic findings of an increase in the diameter of the PFO and right–left blood flow when the patient was in the erect verses the supine position.
The diagnosis of platypnoea–orthodeoxia syndrome should be considered in the differential diagnosis of patients with cyanosis and breathlessness. The most important feature is an improvement in dyspnoea and hypoxia on lying supine. Investigation of such patients should include echocardiography to look for a PFO and abnormal colour flow across the septum. The shunt may be demonstrated by peripheral venous injection of agitated saline and the anatomy further delineated using transoesophageal echocardiography. Injection of agitated saline can be used to show enhanced right to left shunting associated with the upright posture.
Platypnoea–orthodeoxia syndrome is an important diagnosis to establish, as it may be amenable to correction by either transcatheter occlusion or surgery. Cardiac ultrasound can confirm the diagnosis and should be performed when the condition is suspected. This case is of particular importance because of the demonstration of the mechanism by echocardiography.
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References |
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[Abstract/Free Full Text] - Godart F., Rey C., Prat A., Vincentelli A., Chmait A., Francart C., et al. Atrial right-to-left shunting causing severe hypoxaemia despite normal right sided pressures. Report of 11 consecutive cases corrected by percutaneous closure. Eur Heart J (2000) 21(6):483–489.
[Abstract/Free Full Text] - Seward J.B., Hayes D.L., Smith H.C., Williams D.E., Rosenow E.C., Reeder G.S., et al. Platypnoea–orthodeoxia: clinical profile, diagnostic work up, management, and report of 7 cases. Mayo Clin Proc (1984) 59:221–231.[Web of Science][Medline]
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[Abstract/Free Full Text] - Faller M., Kessler R., Chaouat M., Petit H., Weitzenblum E. Platypnoea–orthodeoxia syndrome related to an aortic aneurysm combined with an aneurysm of the atrial septum. American College of Chest Physicians (2000) 118(2):553–557.
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