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Platypnoea-orthodeoxia syndrome, an underdiagnosed cause of hypoxaemia: four cases and the possible underlying mechanisms.

Nassif M, Lu H, Konings TC, Bouma BJ, Vonk Noordegraaf A, Straver B, Blom NA, Clur SA, Backx AP, Groenink M, Boekholdt SM, Koolbergen DR, Hazekamp MG, Mulder BJ, de Winter RJ - Neth Heart J (2015)

Bottom Line: Cardiac platypnoea-orthodeoxia syndrome (POS) is a position-dependent condition of dyspnoea and hypoxaemia due to right-to-left shunting.It often remains unrecognised in clinical practice, possibly because of its complex underlying pathophysiology.We present four consecutive patients with POS and patent foramen ovale (PFO) who underwent a successful percutaneous PFO closure, describe the mechanism of their POS and provide a review of the literature.

View Article: PubMed Central - PubMed

Affiliation: Department of Cardiology, Cardiac Catheterization Laboratory, Academic Medical Center-University of Amsterdam, PO Box 22660, 1100 DD, Amsterdam, The Netherlands. r.j.dewinter@amc.uva.nl.

ABSTRACT
Cardiac platypnoea-orthodeoxia syndrome (POS) is a position-dependent condition of dyspnoea and hypoxaemia due to right-to-left shunting. It often remains unrecognised in clinical practice, possibly because of its complex underlying pathophysiology. We present four consecutive patients with POS and patent foramen ovale (PFO) who underwent a successful percutaneous PFO closure, describe the mechanism of their POS and provide a review of the literature.

No MeSH data available.


Related in: MedlinePlus

Different imaging modalities of case 1(a), 2 (b), 3(c) and 4 (d), respectively, for the purpose of diagnosing POS by PFO. a1 Chest X-ray showing the heart position against the left thoracic wall. a2 Four chamber view of a Doppler TEE with a right-to-left shunt by PFO. b1 CMR showing malposition of the right thoracic wall resulting in a heart shift to the right and presence of PFO (arrow). b2 Short axis basal view of a Bubble contrast TEE showing no resting right-to-left shunting over the PFO. c1 CT angiography showing the dilated ascending aorta (A) and aorta root (B). c2 Periprocedural angiographic image of both Amplatzer devices. d1 Chest X-ray showing a tracheal shift to the left after pneumonectomy. d2 CMR shows a right hemithorax filled with pleural effusion and a compressed right atrium (arrow). RA right atrium, LA left atrium, RV right ventricle, LV left ventricle, R right, AO aorta, PA pulmonary artery, SVC superior vena cava
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Fig1: Different imaging modalities of case 1(a), 2 (b), 3(c) and 4 (d), respectively, for the purpose of diagnosing POS by PFO. a1 Chest X-ray showing the heart position against the left thoracic wall. a2 Four chamber view of a Doppler TEE with a right-to-left shunt by PFO. b1 CMR showing malposition of the right thoracic wall resulting in a heart shift to the right and presence of PFO (arrow). b2 Short axis basal view of a Bubble contrast TEE showing no resting right-to-left shunting over the PFO. c1 CT angiography showing the dilated ascending aorta (A) and aorta root (B). c2 Periprocedural angiographic image of both Amplatzer devices. d1 Chest X-ray showing a tracheal shift to the left after pneumonectomy. d2 CMR shows a right hemithorax filled with pleural effusion and a compressed right atrium (arrow). RA right atrium, LA left atrium, RV right ventricle, LV left ventricle, R right, AO aorta, PA pulmonary artery, SVC superior vena cava

Mentions: A 46-year-old woman was referred to our hospital for percutaneous PFO closure, after having experienced symptoms of dyspnoea in the upright and left supine position for several months. She had a history of chronic obstructive pulmonary disease GOLD II and had undergone left pneumonectomy 6 months earlier, to remove an adenocystic carcinoma. In the weeks, thereafter, she developed progressive and position-dependent shortness of breath. Physical examination revealed a blood pressure of 100/65 mmHg with a resting heart rate of 122 beats/min. She had central cyanosis, with a transcutaneous oxygen saturation of 91 %, while breathing 1.5 L oxygen as measured by pulse oximetry. Her heart sounds were shifted to the left on auscultation. Chest X-ray (Fig. 1a1) and computed tomography (CT) scan of the thorax showed a midline shift of the right lung with a consequent position of the heart to the left thoracic wall. No evidence was found of cancer recurrence or other pulmonary causes such as pleural effusion or atelectasis. Echocardiographic images revealed a PFO (Fig. 1a2) and a right-to-left shunt fraction measured 20 % when breathing 100 % oxygen in an upright position. At this point the patient was severely incapacitated; she could only lie on her right side, which had resulted in severe muscular atrophy. POS was finally diagnosed 6 months after the onset of her symptoms. Cardiac catheterisation was performed, which showed a normal right atrial and pulmonary artery pressure, and pulse oximetric saturations of 90 % and 83 % in right and left supine positions, respectively. The PFO was successfully closed with a 25 mm Amplatzer PFO Occluder (St. Jude, Minneapolis, USA) after which the arterial saturation improved immediately to 97 % in the upright position. Her dyspnoea was resolved but an extensive rehabilitation course followed. She was able to gradually resume her daily routine and her exercise capacity improved significantly. No complications have been reported in 3 years of clinical follow-up. On her last transthoracic echocardiogram (TTE), the estimated systolic pulmonary artery pressure was 28 mmHg and no residual shunt was seen over the closed PFO.Fig. 1


Platypnoea-orthodeoxia syndrome, an underdiagnosed cause of hypoxaemia: four cases and the possible underlying mechanisms.

Nassif M, Lu H, Konings TC, Bouma BJ, Vonk Noordegraaf A, Straver B, Blom NA, Clur SA, Backx AP, Groenink M, Boekholdt SM, Koolbergen DR, Hazekamp MG, Mulder BJ, de Winter RJ - Neth Heart J (2015)

Different imaging modalities of case 1(a), 2 (b), 3(c) and 4 (d), respectively, for the purpose of diagnosing POS by PFO. a1 Chest X-ray showing the heart position against the left thoracic wall. a2 Four chamber view of a Doppler TEE with a right-to-left shunt by PFO. b1 CMR showing malposition of the right thoracic wall resulting in a heart shift to the right and presence of PFO (arrow). b2 Short axis basal view of a Bubble contrast TEE showing no resting right-to-left shunting over the PFO. c1 CT angiography showing the dilated ascending aorta (A) and aorta root (B). c2 Periprocedural angiographic image of both Amplatzer devices. d1 Chest X-ray showing a tracheal shift to the left after pneumonectomy. d2 CMR shows a right hemithorax filled with pleural effusion and a compressed right atrium (arrow). RA right atrium, LA left atrium, RV right ventricle, LV left ventricle, R right, AO aorta, PA pulmonary artery, SVC superior vena cava
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4608923&req=5

Fig1: Different imaging modalities of case 1(a), 2 (b), 3(c) and 4 (d), respectively, for the purpose of diagnosing POS by PFO. a1 Chest X-ray showing the heart position against the left thoracic wall. a2 Four chamber view of a Doppler TEE with a right-to-left shunt by PFO. b1 CMR showing malposition of the right thoracic wall resulting in a heart shift to the right and presence of PFO (arrow). b2 Short axis basal view of a Bubble contrast TEE showing no resting right-to-left shunting over the PFO. c1 CT angiography showing the dilated ascending aorta (A) and aorta root (B). c2 Periprocedural angiographic image of both Amplatzer devices. d1 Chest X-ray showing a tracheal shift to the left after pneumonectomy. d2 CMR shows a right hemithorax filled with pleural effusion and a compressed right atrium (arrow). RA right atrium, LA left atrium, RV right ventricle, LV left ventricle, R right, AO aorta, PA pulmonary artery, SVC superior vena cava
Mentions: A 46-year-old woman was referred to our hospital for percutaneous PFO closure, after having experienced symptoms of dyspnoea in the upright and left supine position for several months. She had a history of chronic obstructive pulmonary disease GOLD II and had undergone left pneumonectomy 6 months earlier, to remove an adenocystic carcinoma. In the weeks, thereafter, she developed progressive and position-dependent shortness of breath. Physical examination revealed a blood pressure of 100/65 mmHg with a resting heart rate of 122 beats/min. She had central cyanosis, with a transcutaneous oxygen saturation of 91 %, while breathing 1.5 L oxygen as measured by pulse oximetry. Her heart sounds were shifted to the left on auscultation. Chest X-ray (Fig. 1a1) and computed tomography (CT) scan of the thorax showed a midline shift of the right lung with a consequent position of the heart to the left thoracic wall. No evidence was found of cancer recurrence or other pulmonary causes such as pleural effusion or atelectasis. Echocardiographic images revealed a PFO (Fig. 1a2) and a right-to-left shunt fraction measured 20 % when breathing 100 % oxygen in an upright position. At this point the patient was severely incapacitated; she could only lie on her right side, which had resulted in severe muscular atrophy. POS was finally diagnosed 6 months after the onset of her symptoms. Cardiac catheterisation was performed, which showed a normal right atrial and pulmonary artery pressure, and pulse oximetric saturations of 90 % and 83 % in right and left supine positions, respectively. The PFO was successfully closed with a 25 mm Amplatzer PFO Occluder (St. Jude, Minneapolis, USA) after which the arterial saturation improved immediately to 97 % in the upright position. Her dyspnoea was resolved but an extensive rehabilitation course followed. She was able to gradually resume her daily routine and her exercise capacity improved significantly. No complications have been reported in 3 years of clinical follow-up. On her last transthoracic echocardiogram (TTE), the estimated systolic pulmonary artery pressure was 28 mmHg and no residual shunt was seen over the closed PFO.Fig. 1

Bottom Line: Cardiac platypnoea-orthodeoxia syndrome (POS) is a position-dependent condition of dyspnoea and hypoxaemia due to right-to-left shunting.It often remains unrecognised in clinical practice, possibly because of its complex underlying pathophysiology.We present four consecutive patients with POS and patent foramen ovale (PFO) who underwent a successful percutaneous PFO closure, describe the mechanism of their POS and provide a review of the literature.

View Article: PubMed Central - PubMed

Affiliation: Department of Cardiology, Cardiac Catheterization Laboratory, Academic Medical Center-University of Amsterdam, PO Box 22660, 1100 DD, Amsterdam, The Netherlands. r.j.dewinter@amc.uva.nl.

ABSTRACT
Cardiac platypnoea-orthodeoxia syndrome (POS) is a position-dependent condition of dyspnoea and hypoxaemia due to right-to-left shunting. It often remains unrecognised in clinical practice, possibly because of its complex underlying pathophysiology. We present four consecutive patients with POS and patent foramen ovale (PFO) who underwent a successful percutaneous PFO closure, describe the mechanism of their POS and provide a review of the literature.

No MeSH data available.


Related in: MedlinePlus