European Journal of Echocardiography Advance Access originally published online on February 20, 2008
European Journal of Echocardiography 2008 9(5):694-696; doi:10.1093/ejechocard/jen015
Published on behalf of the European Society of Cardiography. All rights reserved. © The Author 2008.For permissions please email: journals.permissions@oxfordjournals.org
Late emergence of platypnea orthodeoxia: Chiari network and atrial septal hypertrophy demonstrated with transoesophageal echocardiography
R. Shakur,
A. Ryding,
J. Timperley,
H. Becher and
P. Leeson*
Department of Cardiology, John Radcliffe Hospital, Headley Way, Oxford OX3 9DU, UK
Received 5 October 2007; accepted after revision 23 December 2007; online publish-ahead-of-print 20 February 2008.
* Corresponding author. Tel: +44 1865 741166; fax: +44 1865 221111. E-mail address: paul.leeson{at}cardiov.ox.ac.uk
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Abstract
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Platypnea orthodeoxia is a rare syndrome that describes breathlessness
on standing that resolves on lying flat. We present a previously
healthy patient who developed platypnea orthodeoxia in her eighth
decade of life. Cardiovascular imaging demonstrated an atrial
septal defect, extensive Chiari network and atrial septal hypertrophy.
We propose the development of lipomatous atrial septal hypertophy
led to altered atrial compliance and a baffle to direct flow
preferentially to the left heart on standing.
Keywords: Transoesophageal echocardiography; Platypnea orthodeoxia; Chiari network; Atrial septum
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Case description
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A 74-year-old woman was referred for echocardiography with breathlessness
and saturations of 80% on room air. Respiratory investigations,
including CT pulmonary angiogram, had been normal. Transthoracic
echocardiography demonstrated normal left ventricular size and
function with no significant valve abnormalities. Right heart
was of normal size and function, with normal right ventricular
systolic pressure. An atrial septal defect was identified with
colour flow mapping. However, flow across the defect was from
left-to-right with no evidence of a significant right-to-left
shunt on agitated saline contrast injection. During the supine
examination, the patient's oxygen saturations were noted to
be 97% on room air. When the patient stood up again saturations
fell back to 80%. Agitated saline injection with the patient
standing now resulted in striking opacification of the left
ventricle consistent with a significant right-to-left shunt
(
Figure 1A and
B, see
Supplementary material online, Video 1A and B).
Platypnoea orthodeoxia was diagnosed.
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Figure 1 (A) and Video 1A: A transoesophageal bicaval 110° view with the patient lying supine demonstrates virtually no saline contrast entering the left atrium. (B) and Video (B) A transthoracic echocardiogram with the patient standing demonstrates a significant right-to-left shunt following agitated saline contrast.
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Transesophageal echocardiography demonstrated a 24-mm secundum
atrial septal defect. Of interest, there was an extensive mobile
Chiari network (
Figure 2A, see
Supplementary material online, Video 2A),
which moved dynamically with posture relative to a rigid, hypertrophied
septum. With the patient supine the geometric arrangement of
the Chiari network and septum appeared to favour preferential
flow from the inferior vena cava into the right atrium. In a
semi-supine position the Chiari network and inferior septum
shifted towards the left atrium (
Figure 2B, see
Supplementary material online, Video 2B).
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Figure 2 (A) and Video 2A: Transoesophageal echocardiography with the patient lying supine demonstrates the extensive Chiari network, hypertrophied atrial septum and atrial septal defect. The Chiari network extends across the right atrium and generates laminar flow across the septum within the right atrium (arrow). (B) and Video (B) A small change in posture to the semi-supine position alters the geometry of the septum, Chiari network and septal defect with laminar flow more likely to be directed to the left atrium (arrow).
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Platypnoea orthodeoxia describes dyspnea while ‘upright’,
relieved on resumption of a supine posture.
1,2 It was unclear
why our patient had developed this phenomenon in the eighth
decade of her life. Despite the atrial septal defect our patient
had no changes in right heart pressure that would contribute
to a right-to-left shunt. No previous imaging was available
to know whether the size of the atrial septal defect or shunt
had changed over time. However, one of the defining features
of platypnea orthodeoxia is a right-to-left shunt in the presence
of normal intracardiac pressures. Both an anatomical means for
the right-to-left shunt—in our patient the atrial septal
defect—and a functional component that alters with posture
is required.
3 Figure 2 demonstrates the dynamic relationship
of the Chiari network with the hypertrophied septum following
small changes in posture. This observation is consistent with
the proposal that platypnea orthodeoxia can occur due to postural
changes in atrial anatomy that preferentially direct vena caval
inflow towards the left atrium.
3,4 It is possible that changes
in right atrial inflow also cause regional variation in right
atrial pressure that contributes to the right-to-left shunt.
We propose the development of atrial septal hypertrophy, due
to lipomatous change, in someone with a pre-existing, extensive
Chiari network accounts for the late emergence of platypnea
orthodeoxia. The septal hypertrophy alters atrial compliance
and creates a baffle for flow to be directed to the left heart.
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Supplementary material
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Supplementary material associated with this article can be found in the online version.
Conflict of interest: none declared.
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References
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- Burchell HB, Helmholz HF Jr, Wood EH. Reflex Orthostatic dyspnea associated with pulmonary hypertension. Am J Physiol (1949) 159:563–64.
- Altman M, Robin ED. Platypnea (diffuse Zone I phenomenon)? N Engl J Med (1969) 281:1347–1348.[Web of Science][Medline]
- Cheng T. Mechanisms of platypnea-orthodeoxia: what causes water to flow uphill? Circulation (2002) 105:e47.[Medline]
- Zanchetta M, Rigatelli G, Ho SY. A mystery featuring right-to-left shunting despite normal intracardiac pressure. Chest (2005) 128:998–1002.[CrossRef][Web of Science][Medline]
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Abstract
Case description