Combined Interatrial and Intrapulmonary Shunting in Orthodeoxia Detected by Transesophageal Echocardiography

Contrast transesophageal echocardiography was found useful in diagnosing combined interatrial and intrapulmonary right-to-left shunts in a patient presenting with orthodeoxia. This was done by separately examining the pulmonary veins and the interatrial septum during intravenous normal saline injections.     

Postural hypoxemia in the postpneumonectomy patient

A patient with platypnea and orthodeoxia after right pneumonectomy is described. Cardiac catheterization revealed arterial desaturation in the upright position associated with increased right to left intracardiac shunting through a patent foraman ovale. The absence of a pressure gradient between the left and right atrium in either the supine or sitting position indicated that the orthodeoxia resulted from an increase in streaming of blood from the inferior vena cava to the left atrium caused by mechanical distortion of the interatrial septum.     

Orthodeoxia platypnea syndrome in a patient with lipomatous hypertrophy of the interatrial septum due to long-term steroid use

We describe an unusual case of orthodeoxia platypnea syndrome exacerbated by right ventricular inflow obstruction due to iatrogenic steroid-induced adipose deposition in cardiac tissues. A 68-year-old man on long-term prednisone therapy for eosinophilic pneumonia presented with progressive dyspnea worsened by bending forward. By using pulse oximetry, he was noted to have positional hypoxemia. Transthoracic echocardiogram demonstrated normal right-sided pressures but severe right to left shunting through a patent foramen ovale. Transesophageal echocardiogram showed a large patent foramen ovale, severe lipomatous hypertrophy of the interatrial septum, and massive adipose deposition in the pericardium causing compression of the right ventricular inflow tract. The patient underwent percutaneous closure of the patent foramen ovale, which resulted in the resolution of symptoms and hypoxemia. This case is unique because long-term steroid use resulted in reverse Lutembacher physiology and clinical orthodeoxia platypnea syndrome by inducing lipomatous hypertrophy of the interatrial septum and compression of the right atrium.     

Effect of atrial septal mobility on transcatheter closure of interatrial communications

BACKGROUND: Transcatheter techniques have evolved as alternatives to surgical closure of interatrial communications due to its less invasive nature and low morbidity. The technique may be limited by the inability to deploy the device and, thus, may be complicated by significant residual shunting. Mobile or redundant septal tissue has been implicated as a possible cause of unsuccessful closure. METHODS: To determine if atrial septal hypermobility precludes successful transcatheter device closure of interatrial communications, 69 patients (mean age 46.4 +/- 17.5 years [male:female ratio 34:35]) with periprocedural transesophageal echocardiograms were reviewed. Septal excursion was defined as the maximal transit of the interatrial septum between either side of the plane connecting the superior and inferior attachment points. Unsuccessful closure was defined as subsequent cardiac surgery to close the defect, inability to deploy the device, recurrent attempts at closure, device malalignment, residual shunting greater than mild in degree on follow-up transthoracic study, and procedural complications. RESULTS: Reasons for closure were the following: embolic events (n = 48); platypnea/orthodeoxia (n = 15); atrial septal defect with right ventricular dilatation and paroxysmal atrial tachycardia (n = 2); atrial septal defect (n = 3); and prophylactic patent foramen ovale closure in a scuba diver (n = 1). Successful closure occurred in 76% of patients (n = 53). Compared with those patients with successful transcatheter closure, the mean septal excursion in patients with failed closure was 0.66 +/- 0.56 cm versus 0.76 +/- 0.47 cm (P = not significant [NS]). CONCLUSION: Our results do not support the concern that exaggerated septal mobility compromises successful device closure of interatrial communications.     

A case of orthodeoxia caused by an atrial septal aneurysm

Platypnea-orthodeoxia is an uncommon syndrome that may occur due to the postpneumonectomy state, cirrhosis of the liver, recurrent pulmonary embolism, and intracardiac shunting. We describe a patient who was found to have a positional change in desaturation after being admitted for dehydration. Workup revealed an atrial septal defect with aneurysm. Following surgical repair, the orthodeoxia resolved. Different mechanisms explain positional desaturation, such as atriovenous malformations at the lung base of cirrhotic patients. In an atrial septal defect, the increased shunting of blood across a malformed septum in an upright position may cause orthodeoxia. This case highlights the necessity of heightened awareness of this syndrome and the need for documenting orthostatic changes in cases of severe hypoxemia.     

Platypnea-Orthodeoxia Syndrome in Review: Defining a New Disease?

Platypnea-orthodeoxia syndrome is an intriguing condition that comprehends multiple pathologic phenomena. It requires a high clinical suspicion and is probably underdiagnosed. Its identification has therapeutic implications and can have a huge impact on the patient's everyday life. We made a review of the literature and analyzed 188 cases described thus far, trying to identify the underlying mechanisms and risk factors. We propose that a separate entity is created within the spectrum of platypnea-orthodeoxia syndrome that is responsible for the majority of cases, namely platypnea-orthodeoxia disease, which would be defined as the existence of an interatrial communication, with a right-to-left shunt, normal pressure in the right atrium and, of course, platypnea and orthodeoxia. Most patients with this disease were submitted to percutaneous closure of the patent foramen ovale or atrial septal defect, with favorable results, making it the first-line therapeutic approach nowadays.     

Transcatheter Closure of Right‐to‐Left Interatrial Shunts to Resolve Hypoxemia

Background. Right‐to‐left interatrial shunts through an atrial septal defect or patent foramen ovale may result from congenital and acquired conditions. Right‐to‐left shunts may occur with normal pulmonary artery pressure and resistance as in right ventricular outflow tract obstruction leading to hypertrophy and reduced diastolic function, in right ventricle ischemia, myocarditis or cardiomyopathy and in orthodeoxia–platypnea syndrome. Methods. We have tested the tolerance of trans‐catheter closure of right‐to‐left interatrial shunts in 5 selected patients in whom the morphology and hemodynamics implied that it would be well‐tolerated and improve functional capacity. Right‐to‐left shunt was demonstrated in 5 profoundly cyanotic patients by trans‐thoracic and trans‐esophageal echocardiography with intravenous injection of agitated saline. The patients were catheterized for hemo‐dynamic study and tested for tolerance of transient balloon occlusion of the defects. Results. Diastolic right ventricular dysfunction with elevated end‐diastolic pressure was the primary cause of right‐to‐left shunt. Most shunts occur via atrial septal defects. Patients' ability to tolerate temporary balloon occlusion of the defects predicted a favorable outcome following permanent device occlusion. Cyanosis resolved in all patients following closure of the defects without congestive right heart failure. A marked improvement in functional capacity was observed in 4 patients. One died of preinterventional hypoxic brain damage. Conclusion. Transcatheter closure of right‐to‐left shunts is well‐tolerated and a rewarding approach. It should be applied in selected patients following careful morphologic and hemodynamic evaluation. Tolerance of temporary occlusion of the defect is predictive of a favorable procedural outcome.     

Three‐Dimensional Echocardiographic Assessment of Patent Foramen Ovale in Platypnea‐Orthodeoxia

Platypnea‐orthodeoxia is an uncommon syndrome characterized by positional dyspnea and hypoxia when upright that improves with lying down. We present a 75‐year‐old man with platypnea‐orthodeoxia in the setting of a patent foramen ovale (PFO) and a 2.1 cm highly mobile atrial septal aneurysm with 2 cm bowing. Prior reports have established the use of three‐dimensional echocardiography to facilitate percutaneous closure of PFO and atrial septal defect, but its use in patients with platypnea‐orthodeoxia is unclear. We document three‐dimensional echocardiographic images that accurately estimated PFO defect size and confirmed placement of the occluder device.     

Platypnea-orthodeoxia syndrome in a patient with a pre-existing patent foramen ovale successfully treated with an atrial septal occluder

Platypnea orthodeoxia syndrome is associated with dyspnea and arterial oxygen desaturation accentuated by an upright posture. It can be secondary to an intracardiac shunt. We report a case of platypnea-orthodeoxia syndrome (POS) in a 58-year old male patient who had a pre-existing patent foramen ovale (PFO) and substantial pulmonary pathologies. He was successfully treated by percutaneous transcatheter closure of the PFO. Our case highlights the importance of recognition of this rare syndrome in patients who present with unexplained hypoxia for whom transcatheter closure of the interatrial shunt can be safely carried out.     

Diagnosis of endocardial cushion defect with cross-sectional and M-mode scanning echocardiography. Differentiation from secundum atrial septal defect.

Twelve cases of endocardial cushion defect were studied before and after operation with ultrasono-cardiotomography (tomography) cross-sectional echocardiography, two-dimensional echocardiography, B-scan echocardiography) and M-mode scan along a horizontal section of the heart. For comparison, 20 healthy subjects, 18 cases of mitral valvular disease, 4 cases of congestive cardiomyopathy, 1 case of partial anomalous pulmonary venous drainage, and 25 cases of atrial septal defect of secundum type were also examined with the same technique. In cases without cardiac malformation, the echo of the anterior mitral valve was usually continuous medially with that of the interatrial septum in the horizontal plane at the level of the membranous septum. This feature was clearly recorded in all cases with right heart enlargement. In ostium secundum atrial septal defect the echo of the anterior mitral valve continued into that of the interatrial septum. An echo interruption was shown, indicating the defect itself to be in the middle part of the interatrial septum. In all the cases of endocardial cushion defect which we examined discontinuity was shown between the echo of the anterior mitral valve and that of the interatrial septum. This discontinuity was interpreted as indicating the defect itself. The mitral valve ring echo was close to the basal end of that of the interventricular septum, possibly reflecting an abnormal attachment of the mitral valve. In all cases, after operation, the echo of the artificial interatrial septum was recorded, continuous with that of the anterior mitral valve. The features of the echocardiographic sweep from the anterior mitral valve to the interatrial septum were thus different in the three groups. These echocardiographic differences are thought to correspond to the anatomical differences between the normal, atrial septal defect of secundum type, and endocardial cushion defect, and are essential features differentiating them from each other.     

Platypnea-orthodeoxia in a patient with ostium primum atrial septal defect with normal right heart pressures

We describe an adult patient with an ostium primum atrial septal defect (ASD) and a patent foramen ovale (PFO) with normal right heart pressures who presented with platypnea and orthodeoxia. A dilated aortic root encroaching into the region of the ASD and PFO, along with a tricuspid regurgitant jet moving into the left atrium through the ASD found on the transesophageal echocardiogram may have been responsible for orthodeoxia. Surgical closure of these defects resulted in the disappearance of both platypnea and orthodeoxia.     

Cardiac Platypnea‐Orthodeoxia Syndrome: An Often Unrecognized Malady

Platypnea‐orthodeoxia syndrome (POS) is a rare but clinically important form of dyspnea. The syndrome is characterized by dyspnea and arterial oxygen desaturation that occurs in the upright position and improves with recumbency. In cardiac POS, an atrial septal defect or patent foramen ovale allows communication between the right‐ and left‐sided circulations. A second defect, such as a dilated aorta, prominent eustachian valve, or pneumonectomy, then contributes to right‐to‐left shunting through the interatrial connection. Diagnosis is made through pulse oximetry to confirm orthodeoxia and through transesophageal echocardiography with bubble study to visualize the shunt. Although data are limited for this rare syndrome, percutaneous closure has thus far proven safe and effective.     

Platypnoea orthodeoxia in a patient with aortic root dilatation and a patent foramen ovale.

Platypnoea orthodeoxia syndrome is a rare condition in which shortness of breath and hypoxaemia occur when upright and resolve when prone. The hypoxaemia results from increased right to left shunting of deoxygenated blood, when standing, through a patent foramen ovale (PFO), atrial septal defect (ASD) or other right to left shunt, in the absence of raised right-heart pressure. We present the case of a patient with platypnoea orthodeoxia with marked shunting through a large PFO, evaluated by transesophageal and transthoracic echo.     

Platypnea‐orthodeoxia Syndrome after Failed Percutaneous Closure of Secundum Atrial Septal Defect

We report a 51‐year‐old patient with platypnea‐orthodeoxia syndrome after percutaneous closure of a secundum atrial septal defect, an unusual complication of this modality of treatment. Echocardiography, the main diagnostic technique in the present case, showed that one of the percutaneous device's rims was fixed to the anterior wall of the inferior cava vein. Furthermore it showed that the blood flowed from the inferior cava vein, through the defect in the atrial septum, into the left atria.     

Diagnosis of atrial septal defect using magnetic resonance imaging

We studied the morphological features of defects of the interatrial septum using magnetic resonance imaging (MRI) to determine the sizes of defects and other abnormalities. MR images were obtained in 28 patients with atrial septal defect, including five cases with complicated anomalies (two with Ebstein's anomaly, one pentalogy of Fallot, and one anomalous pulmonary vein connection and azygos continuation). Images were also obtained in the control subjects including seven normal volunteers and 142 patients with various acquired heart diseases. The diagnosis of atrial septal defect was established by cardiac catheterization, angiography and two-dimensional echocardiography prior to the MRI studies, and in 14 patients, the diagnosis was confirmed by surgery. The MRI unit had a superconducting magnet and operated at 0.25 or 0.50 Tesla. A spin echo pulse sequence was used with an echo time of 40 or 60 msec. At the beginning of this study, non-gated MRI images were obtained in the 28 controls and in three patients with atrial septal defect. Nongated MRI could not image the anatomical structure of the interatrial septa of 12 of the 28 controls, or any of the three patients with atrial septal defect. Nongated MRI was, therefore, inadequate for visualizing cardiac anatomy. Gated MRI images were obtained in 141 controls and in 25 patients with atrial septal defect. Gated MRI revealed the interatrial septum, interventricular septum, atrioventricular septum, mitral valve, tricuspid valve and other intracardiac structures in most subjects. In 17 control subjects (12%), however, there was a very faint signal from the central portion of the interatrial septum. In these instances, there was a gradual fading of the signal of the interatrial septum, so that they could be distinguished from the atrial septal defect. The sudden disappearance of the signal from the interatrial septum was observed by gated MRI in all 25 patients with atrial septal defect. The sizes of the defects by MRI coincided with the findings at surgery in all 14 patients. MRI showed right atrial dilatation, right ventricular hypertrophy and dilatation, and pulmonary artery dilatation in most of the patients having atrial septal defect. Complex anomalies associated with atrial septal defect were also clearly shown by MRI, such as displacement of the tricuspid leaflets in two patients with Ebstein's anomaly, and anomalous pulmonary venous connection and persistent left superior vena cava in one patient. These results indicated that gated MRI is a valuable noninvasive method of diagnosing atrial septal defect and complicating anomalies.     

Anatomical Factors Triggering Platypnea‐Orthodeoxia in Adults

Right to left shunting through a patent foramen ovale (PFO) or atrial septal defect (ASD) can cause platypnea‐orthodeoxia even in a setting of normal pulmonary artery pressures. However, the late onset of symptoms despite the congenital origin of the anatomical defects is not well understood. We report a case series of patients presenting with dyspnea and orthodeoxia who developed right to left shunting as a result of associated anatomical changes that occur with aging such as tortuosity and elongation of the aorta. We propose that these acquired anatomical changes can favor right to left shunting in the setting of congenital abnormalities, therefore explaining the late onset of symptoms. Copyright © 2009 Wiley Periodicals, Inc.     

Right-to-left atrial shunting associated with aortic root aneurysm: A case report of a rare cause of platypnea–orthodeoxia syndrome

Platypnea–orthodeoxia is a rare syndrome characterised by dyspnoea and hypoxaemia worsened on upright posture. We report the case of a 76 year-old man treated for lung adenocarcinoma who had developed severe hypoxaemia due to right-to-left shunt through a patent foramen ovale (PFO). Diagnosis was suspected by systemic uptake of isotope during lung scintigraphy performed to exclude pulmonary embolism. Arterial blood gas analysis in supine and upright positions demonstrated orthodeoxia. Contrast-enhanced transoesophageal echocardiography revealed a slightly redundant atrial septum and large right-to-left shunt through a PFO despite normal pulmonary pressure. Chest computed tomography and echocardiography showed a 59-mm aneurysm of the thoracic aorta. The opening of the PFO seemed to be the result of mechanical deformation of the atrial septum by aortic root dilatation. Transcatheter closure of the atrial defect has provided excellent results, including a rapid increase in systemic saturation and improvement of symptoms without any complications.     

Single and two dimensional echocardiographic features of the interatrial septum in normal subjects and patients with an atrial septal defect.

The interatrial septum is one of the least studied structures in M mode echocardiography. Two dimensional echocardiography has made it possible to record simultaneous M mode and two dimensional echocardiograms. Such studies were performed in 10 normal subjects and in 9 patients with a secundum atrial septal defect. In the short axis view of the base of the heart, the interatrial septum was visualized in the two dimensional studies as a linear echo running from the posterior aortic wall to the posterior atrial wall and in the M mode records as a series of dense echoes posterior to the aorta. The great variability in echo dropout of the interatrial septum made it impossible to distinguish the normal subjects from the patients with atrial septal defect. The dense echoes of the interial septum in the M mode records gave the false impression that they were filling the left atrium. These data indicate that (1) a secundum atrial septal defect cannot be reliably differentiated from a normal septum using these echocardiographic methods, and (2) the medial location of the interatrial septum should be appreciated so that it will not be confused with a left atrial mass.     

A rare complication of pneumonectomy: diagnosis made by a literature search

This case describes platypnoea-orthodeoxia syndrome in a patient 2 months after a right pneumonectomy for adenocarcinoma of the lung. The patient complained of platypnoea (breathlessness in the upright position) and was noted to have orthodeoxia (arterial desaturation on standing) on clinical examination. This was due to anatomical changes after the pneumonectomy that resulted in direct blood flow from the inferior vena cava through a previously unrecognised atrial septal defect into the left atrium. The closure of this right to left shunt with an Amplatzer occluder produced immediate and striking symptomatic relief in the patient. The authors had no previous experience of this very rare complication of pneumonectomy. The diagnosis was made after a literature search using PubMed/Medline, underlining the direct clinical benefit provided by these databases.     

Broken-heart syndrome (Takotsubo syndrome)

We report on the rare case of partial anomalous return of four pulmonary veins in the right atrium and superior vena cava with intact interatrial septum in a five-year-old child. There were few symptoms in contrast with the left ventricular output dependent on the flow of the left upper lobe vein and from the lingula. Reduced compliance to the left led to a severe picture of pulmonary venocapillary hypertension in the immediate postoperative period, mitigated by an 8-mm interatrial septal defect. The patient progressed well after the intervention.