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(Chest. 2000;118:553-557.)
© 2000 American College of Chest Physicians

Platypnea-Orthodeoxia Syndrome Related to an Aortic Aneurysm Combined With an Aneurysm of the Atrial Septum*

Michèle Faller, MD; Romain Kessler, MD, PhD; Ari Chaouat, MD; May Ehrhart, MD; Hélène Petit, MD and Emmanuel Weitzenblum, MD, FCCP

* From the Service de Pneumologie (Drs. Faller, Kessler, Chaouat, Ehrhart, and Weitzenblum), Hôpital de Hautepierre; and Service de Chirurgie Cardio-Vasculaire (Dr. Petit), Strasbourg, France.

Correspondence to: Michèle Faller, MD, Service de Pneumologie, Hôpital de Hautepierre, CHU de Strasbourg, 67098 Strasbourg Cedex, France


   Abstract
TOP
 Abstract
Introduction
 Case Report
 Discussion
 References
 
We report the case of a 71-year-old man bearing a severe right-to-left shunt through a patent foramen ovale in the absence of elevated right-sided heart or pulmonary artery pressures. He presented with platypnea-orthodeoxia syndrome, but he had no pulmonary or extracardiac diseases that are known to be associated with this syndrome. Chest radiography showed a bulky aneurysm of the thoracic aorta. A peripheral contrast transesophageal echocardiography demonstrated a large right-to-left shunt through a patent foramen ovale. In addition, the atrial septum was severely deformed by an aneurysm including this patent foramen ovale. We hypothesized that the opening of the foramen ovale was the result of a mechanical deformation of the atrial septum by two contributing factors: the aneurysm of the thoracic aorta and the aneurysm of the septum itself.

Key Words: aortic aneurysm • atrial septal aneurysm • orthodeoxia • platypnea


   Introduction
TOP
 Abstract
 Introduction
Case Report
 Discussion
 References
 
Platypnea -orthodeoxia is a rare syndrome. Platypnea is defined by a dyspnea induced by the upright position and relieved by recumbency. Orthodeoxia is defined by an arterial deoxygenation increased by the upright position and improved during recumbency. Pulmonary hypertension is usually absent. The right-to-left shunting occurs without any mechanism that could explain the right-to-left shunt.1 Interatrial communications are the most common disorders associated with the platypnea-orthodeoxia syndrome. A case of platypnea-orthodeoxia related to aortic elongation associated with a large shunt through a patent foramen ovale was described.2 We report a similar case in an elderly man who presented with a platypnea-orthodeoxia syndrome and an aneurysmal thoracic aorta. Furthermore, in our patient, an atrial septal aneurysm was associated. This case report focuses on this particular abnormality of the atrial septum and its relationship with the right-to-left shunt.


   Case Report
TOP
 Abstract
 Introduction
 Case Report
Discussion
 References
 
In February 1998, a 71-year-old man was referred to our institution for severe hypoxemia, with a 6-month history of dyspnea in the upright position and during minimal exercising. He reported being in a car crash 25 years ago. Abdominal and proximal thoracic aortic aneurysms developed a few years later. He was an ex-smoker, without clinical symptoms of chronic bronchitis. He also had systemic hypertension and angina pectoris, but no history of parenchymal or vascular lung disease. On physical examination in the supine position, his BP was 140/90 mm Hg, with a steady pulse of 78 beats/min and respiratory rate of 22 breaths/min. Lung fields were clear to auscultation, and cardiac examination was normal. There was a moderate thoracic kyphoscoliosis. No peripheral edema was present. While breathing room air, the oxygen saturation measured by pulse oximetry was 86%.

The posteroanterior chest radiograph revealed a voluminous aneurysm of the thoracic aorta, which was stable for 10 years. The ECG was normal. A two-dimensional echocardiography revealed an aneurysm of the ascending thoracic aorta, with normal cardiac function. The right heart cavities were not dilated. A ventilation-perfusion scintigram was normal. A chest CT angioscan showed a 8-cm diameter saccular aneurysm of the aortic arch and an ectatic thoracic aorta. Pulmonary embolism and lung arteriovenous fistula of the lung were excluded. Doppler echocardiography of the venous system of the lower extremities was negative.

Pulmonary volumes were measured by body plethysmography, showing a mild restrictive defect with a vital capacity of 3,360 mL (80% of predicted); FEV1, 2,300 mL (78% of predicted); FEV1/vital capacity ratio, 68%; and total lung capacity, 5,740 mL (80% of predicted). The single-breath diffusing capacity of carbon monoxide was normal.

Arterial blood taken from the patient while he was in the supine position showed a moderate hypoxemia: pH, 7.40; PaCO2, 33 mm Hg; PaO2, 56 mm Hg; and saturation, 89.5%. While he was in the upright position, his hypoxemia increased: pH, 7.42; PaCO2, 31 mm Hg; PaO2, 40 mm Hg; and saturation, 76.6%.

Right heart catheterization in the supine position revealed normal right heart and pulmonary pressures: mean right atrial pressure, 0 mm Hg; mean pulmonary artery pressure, 15 mm Hg; and cardiac output, 6.68 L/min. Shunt evaluation was performed during the right heart catheterization while the patient was breathing 100% oxygen. After inhalation of 100% oxygen for 30 min in supine position, the arterial PaO2 rose to only 75 mm Hg (normal value, 500 mm Hg). The shunt represented 36% of the total cardiac output.

We suspected an intracardiac shunt, and a second two-dimensional echocardiogram with bubble study was performed but was inconclusive. Finally, peripheral contrast transesophageal echocardiography demonstrated a large right-to-left shunt through a patent foramen ovale (PFO) in an atrial septal aneurysm while the patient was in the sitting position (Fig 1 , top and bottom). This aneurysm involved the entire interatrial septum. It was well defined and measured 21 mm in length. It protruded into the right atrium. The maximal course of the apex of the atrial aneurysm was 32 mm. A persistent eustachian valve was associated. Coronary arteriography revealed significant coronary artery disease. After discussion, the patient refused the surgical options that we proposed to him. One year after the diagnosis, the patient was alive, and the arterial blood gases were unchanged.



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  		Figure 1. Top: Peripheral contrast transesophageal echocardiography demonstrating a large shunt through a PFO and an atrial septal aneurysm (OG = left atrium; OD = right atrium). Bottom: Peripheral transesophageal echocardiography demonstrating a well-defined interatrial aneurysm (arrow).

 

   Discussion
TOP
 Abstract
 Introduction
 Case Report
 Discussion
References
 
Three groups of disorders might be associated with the platypnea-orthodeoxia syndrome: intracardiac right-to-left shunts (eg, PFO), pulmonary arteriovenous shunts, and pulmonary parenchymal shunts (as in the hepatopulmonary syndrome). Interatrial communications are the most common etiology and are represented by a PFO, or rarely by a true atrial septal defect (ASD), notably ostium secondum.1 In the literature, most of the cases of shunting through interatrial communications were seen after pneumonectomy, especially after right pneumectomy.1 3 Some case reports have described right-to-left shunting across an interatrial communication, mostly across a PFO, in other circumstances than postthoracotomy. These are reported in Table 1 : without any particular condition,4 5 6 7 with pulmonary embolism,8 secondary to unilateral diaphragmatic paralysis,9 10 with a pedonculated right atrial myxoma,11 associated with eosinophilic myocardial disease,12 with pericardial effusion13 or with a decrease of right ventricular diastolic compliance,14 or related to kyphoscoliosis.15 16 In our knowledge, only six cases of interatrial shunts with an elongated ascending aorta were reported.2 15 16 17 18 In one of the symptomatic cases from the literature, the interatrial shunt was combined with an aneurysmal dilatation of the septum primum, without any other associated abnormality, such as an elongated thoracic aorta.6 Among the cases described after pneumectomy, only one case of platypnea-orthodeoxia was associated with an atrial septal aneurysm with two small perforations at the level of the fossa ovalis.19 Furthermore, in series of asymptomatic patients with uncomplicated PFO or ASDs, atrial septal aneurysms were not mentioned.20 21 22 23


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  		Table 1. Review of the Literature Describing Cases of Platypnea-Orthodeoxia With Right-to-Left Shunting, but Normal Right Heart or Pulmonary Artery Pressures*

 
The underlying pathophysiology of right-to-left shunt through a PFO or an ASD in the presence of normal pressures in the pulmonary artery may imply several mechanisms. A PFO, present in 25 to 30% of healthy subjects,24 is usually responsible of a right-to-left shunt only when pulmonary hypertension is present. In the absence of pulmonary hypertension, three main pathophysiologic mechanisms may be proposed. A first mechanism could be an extrinsic compression of the right atrium, especially in the upright position, causing an increase of pressures in the right heart. Depending on the underlying disorders, the compression may be the result of a right hydrothorax or a located pericardial effusion.13 Compression of the right atrium by a right hydrothorax could have a role after right pneumonectomy.25 26 A second mechanism could consist of a decrease in compliance of the right ventricle (eg, after right ventricle ischemia or after pneumonectomy).14 27 28 Finally, a third mechanism might consist of the development of abnormal anatomic relationships between the vena cava and the atrial septum, increasing in upright position, directing preferentially the venous blood flow from the inferior vena cava through a PFO or a small atrial defect into the left atrium.28 Another anatomic abnormality, a persistent eustachian valve, can favor interatrial right-to-left shunting with a normal atrial pressure.29 An anatomic distortion of the heart with downward displacement of the right atrium and the interatrial septum may occur. Standing upright can stretch the interatrial communication, be it a PFO or an ASD or a fenestrated atrial septal aneurysm. This third mechanism is probably responsible for the shunt seen in our own patient, in whom an aortic aneurysm may have altered the intrathoracic anatomic relationships by a horizontal straightening of the right atrial septum. In the literature, we found only six other cases of platypnea-orthodeoxia that presented abnormalities of the ascending thoracic aorta.2 15 16 17 18 In these six cases, it was supposed that the enlargement of the ascending aorta rotates the heart in a counterclockwise direction, thereby distorting the position of the atrial septum relative to caval inflow. Probably, this shift in position of the septum directs the atrial venous inflow through a patent foramen, and the shunt flow is exacerbated when the patient is in upright position. In one case, the chronic use of steroids and a thoracic spine compression fracture may have further altered the intrathoracic anatomic relationships.18

Our case is of particular interest because it was associated with an atrial septal aneurysm that was discovered by transesophageal echocardiography. Atrial septal aneurysm is a rare but well-recognized cardiac abnormality of uncertain clinical significance.30 Atrial septal aneurysm is an infrequent finding in an adult patient with a normal heart, but our patient presented an ischemic cardiopathy.31 32 The characteristics of atrial septal aneurysms include their length, the predominant side of bulging (right or left atrium), the maximal extent of protrusion, and the incidence of spontaneous oscillation of the atrial septal aneurysm. The cutoff point between a slightly redundant atrial septum and an atrial septal aneurysm is somewhat arbitrary, but usually an extent of protrusion of the aneurysm > 15 mm beyond the plane of the atrial septum into either the right or the left atrium is adopted. Atrial septal aneurysm formation can be secondary to interatrial pressure differences, but may also be a primary malformation involving the region of the fossa ovalis or the entire septum. Atrial septal aneurysm may be an isolated abnormality, but is often found in association with other structural cardiac abnormalities (eg, mitral valve prolapse, or ASDs, in particular ASD type II and PFO).33 Interatrial shunting was noted with a similar frequency in both atrial septal aneurysm involving the fossa ovalis and involving the entire septum. Transesophageal echocardiography is superior and more detailed than the transthoracic approach in the diagnosis of atrial septal aneurysm and in the analysis of associated abnormalities. One study showed that atrial septal aneurysm was missed in almost half of the patients by surface (transthoracic) echocardiography.31 The symptomatology of an interatrial aneurysm usually consists of atrial arrhythmias and pulmonary or systemic embolism.30 31 ASD appears to be a risk factor associated with cardiogenic embolism.31 In the literature, only two cases of platypnea-orthodeoxia with an interatrial septal aneurysm were described.6 19

Our patient showed a stable thoracic aneurysm. The question is why a right-to-left shunt developed with time. It is clear that it was not the atrial septal aneurysm per se, but the associated right-to-left interatrial shunting that caused platypnea-orthodeoxia. Atrial septal aneurysm may or may not be associated with an atrial defect.34 Remarkably, in most series, PFO has not been described associated with an ASD. Our patient presented with a PFO: the valve of the foramen ovale was too small to cover the whole opening of the foramen ovale, probably due both to the atrial septum aneurysm and to the traction of the atrial septum by the tortuous initial aneurysmal aorta. So in our case, a right-to-left shunt through a PFO was associated with both an atrial septal aneurysm and an initial thoracic aorta aneurysm.

Platypnea-orthodeoxia syndrome should be considered in the differential diagnosis of positional dyspnea and refractory hypoxemia. Diagnosis may be difficult unless there is a higher index of suspicion. The detection of right-to-left shunt can be performed by a 100% oxygen inhalation test, and quantification of the shunt is possible if right heart catheterization is performed simultaneously. The most sensitive modality for the noninvasive diagnosis is peripheral contrast tilt-table transesophageal echocardiography. Contrast echocardiography can detect a right-to-left shunt of < 4%. Echocardiography (transthoracic and transesophageal) also allows one to visualize the atrial and ventricular septum, and to look for defects, aneurysms, or other abnormalities.31 Thus, it should be possible to determine if PFO, a relatively frequent abnormality, is associated with another interatrial septum abnormality, such as an atrial septal aneurysm.


   Footnotes
 
Abbreviations: ASD = atrial septal defect; PFO = patent foramen ovale

Received for publication January 8, 1999. Accepted for publication February 24, 2000.


   References
TOP
 Abstract
 Introduction
 Case Report
 Discussion
 References
 

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