Ann Thorac Surg 2008;85:1798-1800. doi:10.1016/j.athoracsur.2007.08.011
© 2008 The Society of Thoracic Surgeons
Case Reports
Orthodeoxia-Platypnea Syndrome Presenting as Paradoxical Peripheral Embolism
Sophie Delalieux, MDa,
Kathleen De Greef, MD, PhDa,
Jeroen Hendriks, MD, PhDa,
Patrick Lauwers, MDa,
Bert Suys, MDb,
Paul Van Schil, MD, PhDa,*
a Department of Thoracic and Vascular Surgery, University Hospital of Antwerp, Edegem, Antwerp, Belgium
b Department of Pediatrics, University Hospital of Antwerp, Edegem, Antwerp, Belgium
Accepted for publication August 6, 2007.
* Address correspondence to Dr Van Schil, Department of Thoracic and Vascular Surgery, University Hospital of Antwerp, Wilrijkstraat 10, Edegem, Antwerp, B-2650, Belgium (Email: paul.van.schil{at}uza.be).
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Abstract
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A paradoxical embolus associated with orthodeoxia-platypnea syndrome and intracardiac shunting is extremely uncommon. We present a patient who was found to have a positional change in desaturation after a right pneumonectomy who suffered from gangrene of the right foot and simultaneous deep venous thrombosis of the left arm. Workup revealed a patent foramen ovale as a cause for both the right-to-left shunt and the paradoxical emboli. After percutaneous closure the orthodeoxia resolved. This case highlights the necessity of heightened awareness of this syndrome in case of severe hypoxemia after pneumonectomy and the importance of an occult patent foramen ovale.
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Introduction
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Orthodeoxia-platypnea syndrome causes posture-dependent desaturation and may occur after pneumonectomy or can be associated with cirrhosis of the liver, recurrent pulmonary embolism, and intracardiac shunting. Right-to-left interatrial shunt due to an atrial septal defect or patent foramen ovale is the most common cause of this syndrome. A patient with a deep venous thrombosis can have a paradoxical embolism to the left side due to a patent foramen ovale. We present a patient who was found to have orthodeoxia-platypnea syndrome and paradoxical embolism at the same time after a right pneumonectomy.
A 51-year-old man was referred with a histologically proven well-differentiated spinocellular carcinoma of the right upper lobe. On computed tomography of the thorax, enlarged mediastinal lymph nodes were present. Because a positive lymph node at the tracheobronchial angle (station 4R, stage IIIA–N2) was discovered during cervical mediastinoscopy, the patient underwent induction chemoradiotherapy. Re-mediastinoscopy was negative and the patient underwent a right thoracotomy with subsequent intrapericardial pneumonectomy, which was necessary because of dense hilar adhesions. Pathology of the lung revealed no residual viable tumor and all dissected intrapulmonary, hilar, and mediastinal lymph nodes were negative. Immediately after surgery, the patient was extubated and he was hemodynamically stable. However, acute dyspnea and respiratory insufficiency developed 3 days later and the patient was reintubated. One week postoperatively, a deep venous thrombosis of the left brachial vein was diagnosed. A few days later he had a trash foot with necrotic toes develop (Figs 1, 2).
At that time, a patent foramen ovale (PFO) was suspected and the diagnosis of a right-to-left interatrial shunt through a PFO was confirmed by transesophageal echocardiography with color Doppler. At postoperative day 38, a percutaneous closure of the right-to-left shunt was performed using a transcatheter device (Figs 3, 4).
The patient was able to be weaned from the ventilator 2 days later and subsequently he underwent amputation of the necrotic toes. Wound healing was normal and he was able to be discharged from the hospital 2 months after the intrapericardial pneumonectomy. The patient subsequently died 10 months postoperatively from metastatic disease.
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Fig 2. Paradoxical embolus causing necrosis of the toes (1, 3 and 5) of the right foot (ventral view).
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Comment
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Orthodeoxia (ie, posture-dependent desaturation) with platypnea (ie, flat-breathing) is an uncommon syndrome that may occur after pneumonectomy or can be associated with cirrhosis of the liver, recurrent pulmonary embolism, and intracardiac shunting. A postural change of the arterial PO
2, expressed clinically by dyspnea and cyanosis, is suggestive for this syndrome. These changes are maximal in the upright position and minimal in the supine position.
The precise cause of orthodeoxia-platypnea syndrome is unclear, but it seems to be associated with three groups of disorders: (1) intracardiac shunts, (2) anatomic pulmonary vascular shunts, and (3) pulmonary parenchymal shunts (as in hepatopulmonary syndrome). Kotoulas and colleagues [1] presented a case in which orthodeoxia-platypnea syndrome was seen without intracardiac shunt. The postural dyspnea after a right pneumonectomy was caused by hyperinflation of the left lung and a right mediastinal shifting with rotation of the heart, which decreased blood return to the right heart. Hyperinflation of the left lung changed ventilation and perfusion relationships due to redistribution of ventilation and perfusion zones, leading to shunting. This extracardiac shunt produced hypoxemia in the upright position due to the drop in the preload. The condition seemed to improve in the supine position as the right atrial diameter increased and the cardiac output almost normalized.
Right-to-left interatrial shunt due to an atrial septal defect or PFO is the most common [1]. Taking into account the high incidence of a PFO in the general population (ie, 27% in autopsy studies), this kind of complication after surgical intervention is occurring more frequently but often remains unrecognized [2]. In patients in whom hypoxemia occurs shortly after thoracic surgery, right-to-left shunt seems to be induced by hemodynamic factors. A decrease in right ventricular compliance after a reduction of the pulmonary vascular bed or by the intraoperative and perioperative fluid overload, or a combination thereof, may generate a right-to-left atrial pressure gradient (instead of a left-to-right pressure gradient). Frequently symptoms occur 3 weeks to 7 months post-pneumonectomy due to slow anatomical remodeling. It has been shown that the heart, after removal of the right lung, makes a turn along a vertical axis. Next, in the upright position, the weight of the heart in the shifted position pulls downward on the interatrial septum causing the foramen ovale to open or widen [3]. In our patient the PFO was the cause for both the orthodeoxia-platypnea syndrome and the paradoxical embolus from the left arm to the right foot. No similar case was found in the English literature.
A patent foramen ovale is no longer seen as a harmless pimple; it has been recognized as a potential conduit for paradoxical embolism of thrombus, fat, air, or desaturated blood, leading to cerebral ischemia, transient global amnesia, decompression illness in divers, refractory hypoxemia in the presence of right ventricular infarction, severe pulmonary disease, migraine with aura or orthodeoxia-platypnea syndrome as in our case [4]. After pneumonectomy the PFO clinically manifested itself as a conduit for emboli, which were scattered in the right foot.
The treatment of choice for this patient with intracardiac shunting is closure of the PFO by surgical repair or by percutaneous endovascular techniques. Because percutaneous occlusion of the PFO is safe and gives excellent results thanks to continuing improvement in available devices, this technique enables some patients in an unstable postoperative condition to avoid an open surgical closure [5–7].
It is still being discussed whether every patient referred for pneumonectomy should undergo an investigation to rule out an interatrial communication. However, should orthodeoxia-platypnea syndrome occur in the postoperative period, transcatheter closure of interatrial septum should be considered as the treatment of choice [3, 6].
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References
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- Kotoulas C, Patris K, Tsintiris K, Zoumboulides A, Lazarides K, Laoutides G. Platypnea-orthodeoxia syndrome after pneumonectomy relieved by mediastinal repositioning Ann Thorac Surg 2007;83:1524-1526.[Abstract/Free Full Text]
- Hashimoto M, Okawa Y, Baba H, Nishimura Y, Aoki M. Platypnea-orthodeoxia syndrome combined with constrictive pericarditis after coronary artery bypass surgery J Thorac Cardiovasc Surg 2006;132:1225-1263.[Free Full Text]
- Marini C, Miniati M, Ambrosino N, et al. Dyspnea and hypoxaemia after lung surgery: the role of interatrial right-to-left shunt Eur Respir J 2006;28:174-181.[Abstract/Free Full Text]
- Seiler C. How should we assess patent foramen ovale? Heart 2004;90:1245-1247.[Free Full Text]
- Guérin P, Lambert V, Godart F, et al. Transcatheter closure of patent foramen ovale in patients with platypnea-orthodeoxia: results of a multicentric French registry Cardiovasc Intervent Radiol 2005;28:164-168.[Medline]
- Yalonetsky S, Nun AB, Shwartz Y, Lorber A. Transcatheter closure of a patent foramen ovale prior to a pneumonectomy to prevent platypnea syndrome Eur J Cardiothor Surg 2006;29:622-624.[Abstract/Free Full Text]
- Ilkhanoff L, Naidu SS, Rohatgi S, Ross MJ, Silvestry FE, Herrmann HC. Transcatheter device closure of interatrial septal defects in patients with hypoxia J Interv Cardiol 2005;18:227-232.[Medline]
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Abstract
Introduction
