Platypnea-orthodeoxia of a 69-year old woman with a patent foramen ovale

Platypnea-orthodeoxia is a rare syndrome that is often associated with interatrial shunting through a patent foramen ovale (PFO) or atrial septal defect. We describe the case of a 69-year-old woman with progressive dyspnea and hypoxia when standing, which was relieved by assuming the recumbent position. After detection of a PFO by transesophageal echocardiography the diagnosis was confirmed by transthoracic echocardiography using saline contrast injection while lying supine and standing upright. This maneuver demonstrated a large right-to-left shunt through a patent foramen ovale while the patient was in a upright position and no significant shunt while being in a recumbent position. The patient showed a rapid improvement after closure of the PFO. This case demonstrates that platypnea-orthodeoxia caused by a patent foramen ovale can be easily demonstrated by the technique of contrast transthoracic echocardiography and a simple positioning maneuver.     

Sudden Onset of Platypnea-Orthodeoxia Syndrome Caused by Traumatic Tricuspid Regurgitation With Ruptured Chordae Tendineae After Blunt Chest Trauma

An 86-year-old man was admitted our hospital because of sudden onset of dyspnea after blunt chest trauma. Because his oxygen saturation deteriorated from 92% in the supine position to 86% in the sitting position, platypnea-orthodeoxia syndrome was suspected. Transesophageal echocardiography showed severe tricuspid regurgitation (TR) caused by anterior papillary muscle rupture. Furthermore, right-to-left shunt with TR through a patent foramen ovale (PFO) was observed. The diagnosis was therefore platypnea-orthodeoxia syndrome with right-to-left shunt through PFO with shunting exacerbated by acute severe TR after blunt chest trauma. The patient underwent urgent tricuspid valve repair and PFO closure and has remained asymptomatic postoperatively.     

Rare case of pulmonary arteriovenous fistula simulating residual defect after transcatheter closure of patent foramen ovale for recurrent paradoxical embolism.

We report on a patient suffering from recurrent cerebrovascular events despite previous transcatheter closure of persistent foramen ovale (PFO) with a Helex occluder. There was evidence of persistent left-to-right atrial shunt shown by transesophageal contrast echocardiography and the patient was admitted to our institution for interventional closure of the supposed residual defect. However, the PFO was completely closed by the device and left pulmonary artery injections showed a pulmonary arteriovenous fistula in the left lower lobe. This rare malformation may well explain the recurrent paradoxical embolism. Transcatheter fistula closure with coils was performed successfully. This case underlines that the existence of an isolated pulmonary arteriovenous fistula as a right-to-left shunt in patients with cryptogenic stroke should not be overlooked, even if a PFO is present and pulmonary arteriovenous fistula is not suggested by the initial physical findings or chest X-ray.     

Anomalous origin of the circumflex artery and patent foramen ovale: a rare cause of myocardial ischaemia after percutaneous closure of the defect

A 35 year old man with a history of transient ischaemic attack and atrial septal aneurysm underwent percutaneous closure of a patent foramen ovale (PFO) with a transcatheter device. After the procedure the patient developed effort angina not present previously. Transoesophageal echocardiography confirmed the absence of residual shunt but showed an abnormal linear image running behind the aortic root. Magnetic resonance imaging detected an anomalous origin of the circumflex coronary artery from the right coronary sinus. The anomalous artery was located between the aortic root and the PFO closing device, causing coronary insufficiency. This report describes a rare complication of transcatheter PFO closure.     

Platypnea-orthodeoxia syndrome combined with multiple congenital heart anomalies

A 71-year-old woman was admitted for paralysis on the left side of her body. She developed dyspnea and hypoxemia after admission. Although pulmonary embolism was suspected, hypoxemia and dyspnea occurred repeatedly in spite of anticoagulation therapy. Transesophageal echocardiography revealed a patent foramen ovale (PFO), an atrial septal aneurysm (ASA), and a right-to-left shunt that appeared in an upright position. She was diagnosed with platypnea-orthodeoxia syndrome. Moreover, cardiac catheterization showed congenital anomalies, such as unroofed coronary sinus, partial anomalous pulmonary venous return and persistent left superior vena cava. Simple surgical closure of the ASA and PFO improved all of her symptoms.     

Closure of patent foramen ovale for paradoxical emboli: intermediate-term risk of recurrent neurological events following transcatheter device placement

OBJECTIVES: We report the largest and the longest follow-up to date of patients who underwent transcatheter patent foramen ovale (PFO) closure for paradoxical embolism. BACKGROUND: Closure of a PFO has been proposed as an alternative to anticoagulation in patients with presumed paradoxical emboli. METHODS: Data were collected for patients following PFO closure with the Clamshell, CardioSEAL or Buttoned Devices at two institutions. RESULTS: There were 63 patients (46 +/- 18 years) with a follow-up of 2.6 +/- 2.4 years. Fifty-four (86%) had effective closure of the foramen ovale (trivial or no residual shunt by echocardiography) while seven (11%) had mild and two (3%) had moderate residual shunting. There were four deaths (leukemia, pulmonary embolism, sepsis following a hip fracture and lung cancer). There were four recurrent embolic neurological events following device placement: one stroke and three transient events. The stroke occurred in a 56-year-old patient six months following device placement. A follow-up transesophageal echocardiogram showed a well seated device without residual shunting. Two of the four events were associated with suboptimal device performance (one patient had a significant residual shunt and a second patient had a "friction lesion" in the left atrial wall associated with a displaced fractured device arm). The risk of recurrent stroke or transient neurological event following device placement was 3.2% per year for all patients. CONCLUSION: Transcatheter closure of PFO is an alternative therapy for paradoxical emboli in selected patients. Improved device performance may reduce the risk of recurrent neurological events. Further studies are needed to identify patients most likely to benefit from this intervention.     

The association of different right atrium anatomical-functional characteristics correlates with the risk of paradoxical stroke: an intracardiac echocardiographic study

Background: The contribution of different right atrium anatomical-functional characteristics to the risk of paradoxical stroke has not been extensively investigated, probably in part because of the limits of standard echocardiography.
Objective: We sought to assess, using intracardiac echocardiography (ICE), the right atrium anatomical-functional characteristics and their role in the pathophysiology of paradoxical embolism in a sample of patent foramen ovale (PFO) patients undergoing transcatheter PFO closure.
Methods: Over a 36-month period, we prospectively enrolled 114 consecutive patients (mean age 38+/− 10.5 years, 67 female) referred to our center for PFO catheter-based closure. On ICE study, all sensible characteristics other than PFO and mild ASA were recorded, including prominent EV or large CN, basal shunt without Valsalva maneuver, moderate to severe ASA, and multiperforated fossa ovalis.
Results: After TEE and ICE study and measurements, a prominent EV or CN was diagnosed on ICE in 73%, a basal shunt was present in 48%, a moderate to severe ASA in 47%, and a multiperforated FO in 24% of patients. A tight correlation between number of concurrent factors and proportion of patients with curtain pattern on TC Doppler, larger right-to-left shunt, recurrent cerebral paradoxical embolism before closure, and migraine with aura was clearly evident (r ≥= 0.97). Basal shunt and concurrent ≥= 3 anatomical functional right atrium characteristics resulted as independent predictors of recurrent paradoxical embolisms.
Conclusions: Our data suggest that right atrium anatomical-functional characteristics other than PFO and mild ASA as assessed by ICE deeply affect the pathophysiology of paradoxical stroke.     

The role of the sizing balloon in selection of the patent foramen ovale closure device size

OBJECTIVES: To find a correlation between the patent foramen ovale (PFO) size measured by the sizing balloon and the appropriate closure device size. METHODS: The PFO of 57 patients was closed using a sizing balloon. A mathematical model was introduced to relate the PFO balloon waist diameter to the closure device size based on the PFO transformation from a slit-like to a circular form during balloon inflation. According to this model, PFOs smaller than 8 mm should be closed with a 25 mm device, PFOs 8 mm to 11 mm with a 35 mm device, and PFOs larger than 11 mm with an Amplatzer septal occluder. In the first group, 36 patients (63.2%) received an appropriately sized device and six patients (10.5%) received an oversized device. In the second group, 15 patients (26.3%) received an undersized device. RESULTS: A comparison of the PFO dimensions in two views showed that the PFO slit was circular when the balloon was inflated. A six-month echocardiography follow-up was obtained in 46 patients (80.7%). Five patients (13.9%) in the group with an appropriately sized device had a discrete residual shunt during Valsalva. In the second group, five patients (33.3%) had a residual shunt (P = 0.06), of which one was considered large. CONCLUSION: The sizing balloon is helpful in selecting the PFO closure device size. Consequently, the incidence of residual shunt and recurrent events may be reduced.     

Embolic Implications of Combined Risk Factors in Patients with Patent Foramen Ovale (the CARPE Criteria): Consideration for Primary Prevention Closure?

Background: Large patent foramen ovale (PFO), spontaneous right‐to‐left shunt, large atrial septal aneurysm (ASA), coagulation abnormalities, and prominent eustachian valve (EV) have all been independently suggested as risk factors for recurrent stroke. We sought to retrospectively evaluate risk of stroke and impact of transcatheter PFO closure in patients with concurrent large PFO, spontaneous right‐to‐left shunt, large ASA, coagulation abnormalities, and prominent EV. Methods: Between March 2006 and October 2008, 36 (mean age 44 ± 10.9 years, 28 females) out of 120 consecutive patients referred to our center for transcatheter PFO closure had concomitant diagnosis of (a) large PFO on transcranial Doppler (TCD) and transesophageal echocardiography (TEE), (b) spontaneous right‐to‐left shunt on TCD, (c) large ASA, (d) prominent EV, and (e) coagulation abnormalities. All patients fulfilled the standard current indications for transcatheter closure and underwent preoperative TEE and brain magnetic resonance imaging (MRI), with subsequent intracardiac echocardiographic‐guided transcatheter PFO closure. Results: Compared to the remaining PFO population in the same period, patients with all five concomitant features had more ischemic brain lesions on MRI, previous history of recurrent stroke, more frequently a history of venous thromboembolism, and more severe migraine with aura. The concomitance of all the features confers the highest risk of recurrent stroke (OR 9.9, 3.0–18 [95% CI], P < 0.001). Conclusions: Despite its small sample size and nonrandomized retrospective nature, this is the first study to suggest that patients with concurrence of all the investigated characteristics have potentially a higher risk of stroke compared to controls. We thus propose the CARP criteria as a basis for further larger, longitudinal studies to assess the potential benefits of transcatheter closure in this patient subset in the absence of clinical recurrent stroke.     

Concomitant Pulmonary and Cerebral Tumor Embolism and Intracardiac Metastasis from Bladder Cancer

An 82-year-old woman with a history of bladder cancer presented with dyspnea and loss of consciousness. Contrast-enhanced computed tomography revealed pulmonary embolism, and emergency thrombus aspiration therapy was performed, but the thrombus was not aspirated. Echocardiography showed mobile masses in the heart and a right-to-left shunt due to a patent foramen ovale (PFO). Magnetic resonance imaging showed multiple cerebral infarctions. Surgical thrombectomy and PFO closure were performed, and the patient was diagnosed with intracardiac metastasis of bladder cancer based on intraoperative histopathology. This is a rare case of concomitant pulmonary and cerebral tumor embolism and intracardiac metastasis from bladder cancer.     

A Change of Heart: Reopening of a Foramen Ovale

Platypnea-orthodeoxia is a rare syndrome characterized by dyspnea and hypoxemia that is exacerbated by assuming an upright position. The most common cause is intracardiac shunting through an atrial septal defect or patent foramen ovale (PFO). We present a 63-year-old man with dyspnea after right pneumonectomy for lung cancer, who was found to have a large PFO with right-to-left shunt in the presence of normal right-sided pressures. Percutaneous closure of the PFO led to resolution of symptoms.     

Percutaneous closure of a patent foramen ovale causing exercise hypoxemia: Case report and a review of the literature

Provoked exercise desaturation is a rare presentation of patent foramen ovale (PFO), when vigorous exercise leads to desaturation of arterial blood and subsequent dyspnea. We present a case of provoked exercise desaturation and curative percutaneous closure and review the literature. A 54-year-old male patient presented with shortness of breath during exercise in the pneumology outpatient department. During exercise spirometry, a relevant drop in arterial oxygen saturation and partial pressure of oxygen was observed and a right-left shunt suspected. In a transesophageal echocardiogram, a PFO was observed. Cardiac catheterization documented a right-left-shunt causing desaturation during exercise. Following percutaneous closure of the PFO, exercise induced desaturation was no longer detectable during exercise spirometry and there was considerable improvement in exercise capacity and subjective dyspnea. To sum up, provoked exercise desaturation is a rare but curable presentation of PFO. Percutaneous closure is a safe and effective way to treat this entity.     

TIA Caused by Contrast Echocardiography in Patient with Platypnea–Orthodeoxia

Platypnea–orthodeoxia syndrome (POS) is a rare clinical disorder characterized by dyspnea caused by the upright position and relieved at recumbent position. Few cases of POS and stroke were reported in literature, and the association between stroke and POS with evidence of patent foramen ovale (PFO) is rare. Stroke may occur in patients with cardiac shunt who undergo contrast echocardiography. We present a patient with POS who experienced transitory ischemic attack (TIA) most likely caused by injection of agitated saline microbubbles during screen for PFO. No case report of TIA/stroke during contrast echocardiography in patients with POS has previously been published.     

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

Platypnea-orthodeoxia is a rare syndrome characterised by dyspnea and hypoxemia worsened on upright posture. We report the case of a 76-year-old man treated for lung adenocarcinoma who had developed severe hypoxemia 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 eliminate pulmonary embolism. Arterial blood gas analysis in supine and upright positions demonstrated orthodeoxia. Contrast-enhanced trans-oesophageal 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 and possibly by lung carcinoma. Transcatheter closure of the atrial defect has provided excellent results, including a rapid increase in systemic saturation and improvement of symptoms without any complications.     

Transcatheter coil occlusion of residual shunt in a patient with recurrent cryptogenic stroke after PFO percutaneous closure.

A significant association has been suggested between a residual shunt and the risk of cerebral ischemic recurrences after transcatheter patent foramen ovale (PFO) closure. We report the use of a detachable coil to treat a residual shunt in a patient who had a recurrent cerebral ischemic event after transcatheter closure of a PFO.     

Management of residual shunts after initial percutaneous patent foramen ovale closure: A single center experience with immediate and long‐term follow‐up

Background: Moderate‐to‐large residual shunts following percutaneous patent foramen ovale (PFO) closure are clinically important and associated with recurrent neuroembolic events. However, their management has not been clearly established in clinical practice. We report our experience in patients of these patients with a prior history of cryptogenic stroke and/or transient ischemic attack (TIA). Methods: All patients undergoing percutaneous PFO closure were routinely screened at six‐months for residual shunts using transthoracic 2D echocardiography with antecubital administration of agitated saline contrast and color flow Doppler. Patients with evidence of moderate‐to‐large residual shunts were selected to undergo reintervention with shunt closure. Postreintervention follow‐up was performed at 24‐hr, 30 days, and every six months thereafter. Clinical predictors of the moderate‐to‐large residual shunts, and the feasibility, safety and long‐term efficacy of percutaneous residual shunt closure using a second device implant were examined. Results: Between 1995 and 2007, a total of 424 patients underwent PFO closure. Of these, 5% (21/424) had moderate‐to‐large residual shunts. Baseline characteristics among patients with moderate‐to‐large residual shunts and those with only none or small defects (n = 403) were similar. Multivariate analysis identified the 24‐hr postprocedure shunt as the only independent predictor of residual shunting at six months. Of the 21 study patients with moderate‐to‐large residual shunt (mean age, 47 ± 14), one underwent successful elective surgical repair, while the remaining 20 underwent transcatheter closure using a second device. The technique was successful in 95% (19/20), and all but one patient had complete shunt closure at six months of their percutaneous reintervention. We report no deaths, recurrent strokes or TIAs during the long‐term mean follow‐up period of 2.9 years. Conclusions: Our study suggests that in patients with moderate‐to‐large residual PFO shunts, percutaneous reintervention using a second device implant is safe and effective. © 2010 Wiley‐Liss, Inc.     

Is routine ultrasound guidance really necessary for closure of patent foramen ovale using the Amplatzer PFO occluder?

Objectives:

The aim of the study was to evaluate safety, efficacy, and long‐term clinical outcome of percutaneous closure of patent foramen ovale (PFO closure) in a low volume center using the Amplatzer PFO occluder without echocardiographic guidance.

Background:

Most centers perform PFO closure either by transesophageal echocardiography (TEE) or intracardiac echocardiography (ICE) guidance for optimal device selection. As TEE is poorly tolerated by patients in supine position and ICE is a costly alternative that increases vascular access complications, we wanted to assess the safety and efficacy of PFO closure by fluoroscopic guidance only.

Methods:

Before PFO closure, all patients had a diagnostic contrast‐TEE and morphological classification of PFO. All PFO closures were performed using the 25‐mm Amplatzer PFO occluder with fluoroscopic guidance only. Intraprocedural echocardiography was replaced by right atrial opacification using contrast angiography. Contrast TEE was done after 6 weeks, contrast TTE after 3, 6, and 12 months postprocedural.

Results:

In all 92 patients (52.4 ± 1.5 years), a 25‐mm Amplatzer PFO occluder was implanted in the correct position. Total fluoroscopic time was 8.4 ± 0.6 minutes and the application of contrast medium was 122.5 ± 5.8 mL. By contrast‐TEE, 12 patients (13 %) showed a small residual shunt (grade 1). During follow‐up (2.09 ± 0.13 years) two patients (2.1%) suffered from a recurrent event (TIA in both cases).

Conclusions:

Percutaneous closure of PFO using the 25‐mm Amplatzer PFO occluder guided by fluoroscopy only is a safe and efficacious intervention for nearly all patients. © 2009 Wiley‐Liss, Inc.     

Discrepancy in the assessment of jailed side branch lesions by visual estimation and quantitative coronary angiographic analysis: comparison with fractional flow reserve

A 59‐year‐old man suffered a transient ischemic attack (TIA) and was found to have a patent foramen ovale (PFO). Six months after closure with a 25 mm Amplatzer PFO occluder there was a significant residual shunt at transesophageal echocardiography (TEE). A coil was implanted and TEE 4 months later showed complete closure. © 2011 Wiley‐Liss, Inc.     

Implantation of a second closure device in patients with residual shunt after percutaneous closure of patent foramen ovale.

Percutaneous closure of patent foramen ovale (PFO) in patients with presumed paradoxical embolism yields complete occlusion in > 90% of patients using contemporary closure devices. Patients with a residual shunt after percutaneous PFO closure have been found at increased risk for recurrent paradoxical events. Treatment options for such patients include medical treatment using antiplatelet drugs or oral anticoagulation, surgical device removal and patch closure, and percutaneous implantation of a second closure device. We report our experience with implantation of a second closure device in 10 patients with more than a minimal residual shunt < or = 6 months after percutaneous PFO closure. Procedure and fluoroscopy times were similar for the initial and repeat intervention (32 vs. 30 min and 5 vs. 6 min, respectively; P = NS). There were no procedural complications during implantation of the second closure device. Follow-up transesophageal echocardiography 6 months after the second percutaneous intervention revealed complete PFO closure in nine (90%) patients. Therefore, implantation of a second closure device in patients with persistence of more than a residual shunt after percutaneous PFO closure appears safe and effective.     

Transcatheter closure of patent foramen ovale: Current evidence and future perspectives

Recent prospective controlled studies have demonstrated that transcatheter closure of a patent foramen ovale (PFO) reduces recurrent stroke risk in select patients, especially in patients younger than 60 years with PFO and embolic-appearing infarct and where no other mechanism of stroke was identified. Detection of PFO depends on the intensity of the Valsalva maneuver, and not all PFOs can be diagnosed using transesophageal echocardiography. Transthoracic contrast echocardiography using abdominal compression during the Valsalva maneuver is an easy method that can increase the detection sensitivity of PFO shunt. PFO with two or more of the following factors is most likely considered a “high-risk PFO” and as such, has a significantly higher probability of cryptogenic stroke: (1) a long-tunnel PFO (≥10 mm in length), (2) atrial septal aneurysm and/or hypermobile interatrial septum, (3) prominent Eustachian valve or Chiari’s network, (4) large right-to-left shunt at rest and during the Valsalva maneuver, and (5) low-angle PFO. In order to establish the benefit of catheter-based PFO closure as a safe and effective treatment in clinical practice, the degree of accuracy of PFO diagnosis and its long-term safety need to be confirmed.