- Email: [email protected]
Worsening Hypoxia Post Lung Transplant: What has Changed?
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Heart, Lung and Circulation (2016) xx, 1–3 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2015.12.097
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Worsening Hypoxia Post Lung Transplant: What has changed? [TD$FIRSNAME]Ashish H.[TD$FIRSNAME.] [TD$SURNAME]Shah[,TD$SURNAME.] MD, MD-Research, [TD$FIRSNAME]Andrew[TD$FIRSNAME.] [TD$SURNAME]Leventhal[TD$SURNAME.], MD, PhD, [TD$FIRSNAME] Eric[TD$FIRSNAME.] [TD$SURNAME]Hoclick[TD$SURNAME.], MD, [TD$FIRSNAME]Erwin[TD$FIRSNAME.] [TD$SURNAME]Oechslin[TD$SURNAME.], MD, [TD$FIRSNAME]Mark[TD$FIRSNAME.] [TD$SURNAME]Osten[TD$SURNAME.], MD * Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Center, University Health Network and University of Toronto, Toronto, Ontario, Canada Received 30 August 2015; received in revised form 15 December 2015; accepted 19 December 2015; online published-ahead-of-print xxx
Platypnoea-orthodeoxia is a rare, but under-diagnosed clinical entity, characterised by postural hypoxia and breathlessness. Underlying pathology is inter-atrial shunt or pulmonary vascular malformation, but what anatomical distortion / physiological alterations initiates right to left shunt, usually against the pressure gradient remains unknown. Keywords
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Anoxia Lung transplantation Patent foramen ovale Atrial Septal occluder device Echocardiography Transoesophageal
A 59-year-old lady underwent bilateral lung transplant for idiopathic pulmonary fibrosis. During her in-hospital stay post lung transplant, her hypoxia got worse. She was found to be persistenly hypoxic with significant high oxygen (80-100%) requirement to maintain O2 saturation >90%, while being supine in the bed. She was extensively investigated for the possibility of transplant organ rejection. Transoesophageal echocardiography demonstrated a patent foramen ovale (PFO) in the presence of aneurysmal septum and bi-directional flow, associated with bowing of the inter-atrial septum in the same direction as flow across it (Figure A & B). Similarly, right-to-left flow was confirmed with transit of significant amount of bubble into the left atrial chamber during agitated saline injection. There was a further drop of 10-15% in her oxygen saturation, with standing. With these findings, we decided to consider her for invasive assessment and closure of the atrial septal communication. At the time of the procedure the mean right and left atrial pressures were 1 and 5 mmHg respectively, similar to the one recorded during the pre-lung transplant cardiac catheterisation. Intra-cardiac echocardiography demonstrated a significant amount of inferior vena cava blood directed towards the aneurysmal atrial septum. Additionally, on transoesophageal echocardiography, the bi-directional flow across the septum was
noted. Balloon sizing demonstrated the defect to be of 18.6 mm in size, significantly larger than demonstrated on echocardiography. A 35 mm PFO closure device did not offer complete seal of the defect, whereas a 20 mm Amplatz septal occluder device offered better closure of the inter-atrial communication. Her oxygen requirement significantly reduced within minutes of device deployment. Platypnoea-orthodexia is a rare, but a serious condition characterised by arterial hypoxia and breathlessness in the upright position. Posture related shunting of blood from right to left, either at the level of atria or intra-pulmonary vasculature, is the proposed mechanism, but precisely why this happens only in a very small proportion of patients, with similar associated medical conditions remains unexplained. For shunting of blood to occur, it is necessary to have abnormal anatomical communication between atria and the existance of pressure gradient. Normally left atrial pressure is higher than that of the right atrium, so there should be no shunting of blood from right to left. Except in patients with elevated right atrial pressure (most likely due to pulmonary hypertension), there must be a precise mechanism through which blood can flow from right to left atria in the absence of adequate pressure gradient, especially when the patient stands. Patent foramen ovale/atrial septal defect are most
*Corresponding author at: Peter Munk Cardiac Centre, Toronto General Hospital, University Health Network, 200, Elizabeth Street, Toronto, ON, M5G 2C4, Canada. Tel.: +1 416 340 4615; fax: +1 416 340 4144, Email: [email protected] © 2016 Published by Elsevier Inc on behalf of Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ).
Please cite this article in press as: Shah AH, et al. Worsening Hypoxia Post Lung Transplant: What has changed?. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.12.097
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Figure 1 Images demonstrate hypermobile atrial septum (A-C) with bidirectional shunt. Right to left shunt on colour Doppler (A), whereas left to right shunt on colour Doppler (B) and right to left shunt in the form of bubble appearance in the left atrial cavity during agitated saline injection (D). Note septal curving towards direction of flow, demonstrating pressure gradient across both atria. Aneurysmal septum motion noted on M-mode (C). Defect was measured with balloon sizing (E) and closed with Amplatzer septal occluder device (F).
Figure 2 Inter-atrial septum and inferior vena cava relationship demonstrated on the CT – thorax (A), and the presence of an Amplatzer septal occluder (B). Diaphragmatic elevation was noted, pre-lung transplantation. * Identifies inter-atrial septum and the black arrow marks inferior vena cava.
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commonly associated condititons, along with aneurysmal atrial septum with/without fenestrations, loculated pericardial effusion, aortic aneurysm, pulmonary arterio-venous malformation or post pneumonectomy [1]. A transient rise, in the absence of persistently elevated right atrial pressure can divert bloodflow from right to left atria. The presence of the Eustachian valve has been demonstrated to deflect bloodflow from the inferior vena cava towards the septum, leading to shunting of blood, even in the absence of elevated pulmonary pressure. Standing up may stretch the interatrial septum with its defect; altered atrial spatial relationship to the venous drainage may result in an inappropriately large volume of blood streaming through the defect. Even though commonly found in the general population, detection of the septal defect responsible for the shunt can sometimes prove to be a diagnostic
challenge [2]. Once the problem has been identified,,transcatheter closure of the septal defect has been demonstrated to treat abnormal shunting of the blood with symptomatic improvement, and has become the treatment of choice over surgical repair [3,4]. We believe that the underlying mechanism is two-fold:
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1. Distortion in right atrium and atrial septal anatomical position, along with their relationship to the inferior vena cava may have changed postoperatively, resulting in blood streaming towards the septum; and 2. Transient altered pressure gradient between atria resulting in right-to-left shunting, although mean left atrial pressure was higher than mean right atrial pressure.
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To our knowledge this is first such case, demonstrating significant shunt from right to left atria even while the patient
Please cite this article in press as: Shah AH, et al. Worsening Hypoxia Post Lung Transplant: What has changed?. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.12.097
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was supine, and with further worsening upon standing, associated with aneurysmal inter-atrial septum and a presence of PFO, after lung transplant, that was successfully treated with transcatheter therapy.
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Funding Sources
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None
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Conflict of Interest Disclosures
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None
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References [1] Cheng TO. Platypnea-orthodeoxia syndrome: etiology, differential diagnosis, and management. Catheterization and Cardiovascular Interventions: official journal of the Society for Cardiac Angiography & Interventions 1999;47:64–6. [2] Cheng TO. Paradoxical embolism. A diagnostic challenge and its detection during life. Circulation 1976;53:564–8. [3] Waight DJ, Cao QL, Hijazi ZM. Closure of patent foramen ovale in patients with orthodeoxia-platypnea using the amplatzer devices. Catheterization and Cardiovascular Interventions: official journal of the Society for Cardiac Angiography & Interventions 2000;50:195–8. [4] Landzberg MJ, Sloss LJ, Faherty CE, Morrison BJ, Bittl JA, Bridges ND, et al. Orthodeoxia-platypnea due to intracardiac shunting–relief with transcatheter double umbrella closure. Catheterization and Cardiovascular Diagnosis 1995;36:247–50.
Please cite this article in press as: Shah AH, et al. Worsening Hypoxia Post Lung Transplant: What has changed?. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.12.097
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Heart, Lung and Circulation (2016) xx, 1–3 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2015.12.097
1 2 3
4
Q1
Q2
5 6 7 8 9
BRIEF COMMUNICATION
Worsening Hypoxia Post Lung Transplant: What has changed? [TD$FIRSNAME]Ashish H.[TD$FIRSNAME.] [TD$SURNAME]Shah[,TD$SURNAME.] MD, MD-Research, [TD$FIRSNAME]Andrew[TD$FIRSNAME.] [TD$SURNAME]Leventhal[TD$SURNAME.], MD, PhD, [TD$FIRSNAME] Eric[TD$FIRSNAME.] [TD$SURNAME]Hoclick[TD$SURNAME.], MD, [TD$FIRSNAME]Erwin[TD$FIRSNAME.] [TD$SURNAME]Oechslin[TD$SURNAME.], MD, [TD$FIRSNAME]Mark[TD$FIRSNAME.] [TD$SURNAME]Osten[TD$SURNAME.], MD * Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Center, University Health Network and University of Toronto, Toronto, Ontario, Canada Received 30 August 2015; received in revised form 15 December 2015; accepted 19 December 2015; online published-ahead-of-print xxx
Platypnoea-orthodeoxia is a rare, but under-diagnosed clinical entity, characterised by postural hypoxia and breathlessness. Underlying pathology is inter-atrial shunt or pulmonary vascular malformation, but what anatomical distortion / physiological alterations initiates right to left shunt, usually against the pressure gradient remains unknown. Keywords
10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35
Q3
Anoxia Lung transplantation Patent foramen ovale Atrial Septal occluder device Echocardiography Transoesophageal
A 59-year-old lady underwent bilateral lung transplant for idiopathic pulmonary fibrosis. During her in-hospital stay post lung transplant, her hypoxia got worse. She was found to be persistenly hypoxic with significant high oxygen (80-100%) requirement to maintain O2 saturation >90%, while being supine in the bed. She was extensively investigated for the possibility of transplant organ rejection. Transoesophageal echocardiography demonstrated a patent foramen ovale (PFO) in the presence of aneurysmal septum and bi-directional flow, associated with bowing of the inter-atrial septum in the same direction as flow across it (Figure A & B). Similarly, right-to-left flow was confirmed with transit of significant amount of bubble into the left atrial chamber during agitated saline injection. There was a further drop of 10-15% in her oxygen saturation, with standing. With these findings, we decided to consider her for invasive assessment and closure of the atrial septal communication. At the time of the procedure the mean right and left atrial pressures were 1 and 5 mmHg respectively, similar to the one recorded during the pre-lung transplant cardiac catheterisation. Intra-cardiac echocardiography demonstrated a significant amount of inferior vena cava blood directed towards the aneurysmal atrial septum. Additionally, on transoesophageal echocardiography, the bi-directional flow across the septum was
noted. Balloon sizing demonstrated the defect to be of 18.6 mm in size, significantly larger than demonstrated on echocardiography. A 35 mm PFO closure device did not offer complete seal of the defect, whereas a 20 mm Amplatz septal occluder device offered better closure of the inter-atrial communication. Her oxygen requirement significantly reduced within minutes of device deployment. Platypnoea-orthodexia is a rare, but a serious condition characterised by arterial hypoxia and breathlessness in the upright position. Posture related shunting of blood from right to left, either at the level of atria or intra-pulmonary vasculature, is the proposed mechanism, but precisely why this happens only in a very small proportion of patients, with similar associated medical conditions remains unexplained. For shunting of blood to occur, it is necessary to have abnormal anatomical communication between atria and the existance of pressure gradient. Normally left atrial pressure is higher than that of the right atrium, so there should be no shunting of blood from right to left. Except in patients with elevated right atrial pressure (most likely due to pulmonary hypertension), there must be a precise mechanism through which blood can flow from right to left atria in the absence of adequate pressure gradient, especially when the patient stands. Patent foramen ovale/atrial septal defect are most
*Corresponding author at: Peter Munk Cardiac Centre, Toronto General Hospital, University Health Network, 200, Elizabeth Street, Toronto, ON, M5G 2C4, Canada. Tel.: +1 416 340 4615; fax: +1 416 340 4144, Email: [email protected] © 2016 Published by Elsevier Inc on behalf of Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ).
Please cite this article in press as: Shah AH, et al. Worsening Hypoxia Post Lung Transplant: What has changed?. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.12.097
36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59
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A.H. Shah et al.
Figure 1 Images demonstrate hypermobile atrial septum (A-C) with bidirectional shunt. Right to left shunt on colour Doppler (A), whereas left to right shunt on colour Doppler (B) and right to left shunt in the form of bubble appearance in the left atrial cavity during agitated saline injection (D). Note septal curving towards direction of flow, demonstrating pressure gradient across both atria. Aneurysmal septum motion noted on M-mode (C). Defect was measured with balloon sizing (E) and closed with Amplatzer septal occluder device (F).
Figure 2 Inter-atrial septum and inferior vena cava relationship demonstrated on the CT – thorax (A), and the presence of an Amplatzer septal occluder (B). Diaphragmatic elevation was noted, pre-lung transplantation. * Identifies inter-atrial septum and the black arrow marks inferior vena cava.
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commonly associated condititons, along with aneurysmal atrial septum with/without fenestrations, loculated pericardial effusion, aortic aneurysm, pulmonary arterio-venous malformation or post pneumonectomy [1]. A transient rise, in the absence of persistently elevated right atrial pressure can divert bloodflow from right to left atria. The presence of the Eustachian valve has been demonstrated to deflect bloodflow from the inferior vena cava towards the septum, leading to shunting of blood, even in the absence of elevated pulmonary pressure. Standing up may stretch the interatrial septum with its defect; altered atrial spatial relationship to the venous drainage may result in an inappropriately large volume of blood streaming through the defect. Even though commonly found in the general population, detection of the septal defect responsible for the shunt can sometimes prove to be a diagnostic
challenge [2]. Once the problem has been identified,,transcatheter closure of the septal defect has been demonstrated to treat abnormal shunting of the blood with symptomatic improvement, and has become the treatment of choice over surgical repair [3,4]. We believe that the underlying mechanism is two-fold:
76
1. Distortion in right atrium and atrial septal anatomical position, along with their relationship to the inferior vena cava may have changed postoperatively, resulting in blood streaming towards the septum; and 2. Transient altered pressure gradient between atria resulting in right-to-left shunting, although mean left atrial pressure was higher than mean right atrial pressure.
82
To our knowledge this is first such case, demonstrating significant shunt from right to left atria even while the patient
Please cite this article in press as: Shah AH, et al. Worsening Hypoxia Post Lung Transplant: What has changed?. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.12.097
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Worsening Hypoxia Post Lung
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was supine, and with further worsening upon standing, associated with aneurysmal inter-atrial septum and a presence of PFO, after lung transplant, that was successfully treated with transcatheter therapy.
96
Funding Sources
97
None
98
Conflict of Interest Disclosures
99
None
93 94
References [1] Cheng TO. Platypnea-orthodeoxia syndrome: etiology, differential diagnosis, and management. Catheterization and Cardiovascular Interventions: official journal of the Society for Cardiac Angiography & Interventions 1999;47:64–6. [2] Cheng TO. Paradoxical embolism. A diagnostic challenge and its detection during life. Circulation 1976;53:564–8. [3] Waight DJ, Cao QL, Hijazi ZM. Closure of patent foramen ovale in patients with orthodeoxia-platypnea using the amplatzer devices. Catheterization and Cardiovascular Interventions: official journal of the Society for Cardiac Angiography & Interventions 2000;50:195–8. [4] Landzberg MJ, Sloss LJ, Faherty CE, Morrison BJ, Bittl JA, Bridges ND, et al. Orthodeoxia-platypnea due to intracardiac shunting–relief with transcatheter double umbrella closure. Catheterization and Cardiovascular Diagnosis 1995;36:247–50.
Please cite this article in press as: Shah AH, et al. Worsening Hypoxia Post Lung Transplant: What has changed?. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.12.097
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