A broken heart: right-to-left shunt in the setting of normal cardiac pressures

A patent foramen ovale (PFO) is a common structural cardiac variant occurring in approximately 30% of the general population. Patients are usually asymptomatic because the defect is flap-like and does not permit significant left-to-right shunting. However, pathological conditions that result in cardiac rotation or higher than normal right atrial pressures can reverse the normal left atrial to right atrial pressure gradient and cause a right-to-left shunt through a PFO. If the right-to-left shunt is persistent, systemic hypoxemia or paradoxical emboli may result. The present report describes a case of refractory hypoxemia in a critically ill patient with a PFO who had a right-to-left shunt with normal right-sided cardiac pressures.     

Percutaneous patent foramen ovale and atrial septal defect closure in adults: results and device comparison in 100 consecutive implants at a single center.

Closure of interatrial septal defects with percutaneous devices is increasingly common. However, the indications for closure and techniques for device implantation are diverse. We reviewed our first 100 consecutive implants to assess and compare the indications, results, complications, and evolution of techniques for percutaneous patent foramen ovale (PFO) and atrial septal defect (ASD) closure. The mean age of patients was 52 years and 70% were female. Paradoxical embolism was the predominant indication (94%) for PFO closure and significant left-to-right shunt was the most frequent indication (89%) for ASD closure. Implantation success was 94% with major complications in 3 patients (2.8%). Transesophageal echocardiography was utilized in the initial 27 procedures and then replaced by intracardiac echocardiography in subsequent ones, with an associated reduction in procedure and physician time. During 6 months of follow-up, 3 patients were readmitted for atrial arrhythmias (2 patients) and an MRI-negative neurologic event (1 patient). Echocardiography at 6 months in 83% of the PFO patients revealed moderate and severe positive contrast studies for right-to-left shunting in one third of patients, with differences between devices and insertion techniques. This single-center experience with percutaneous device closure of PFO and ASD in adults demonstrates excellent results with few complications.     

Five-year clinical and echocardiographic evaluation of the Das AngelWings atrial septal occluder

Background

The late outcome of patients treated with atrial septal occluder devices remains incompletely defined. The purpose of this study was to assess the late outcome (range 4-7 years postprocedure) of patients in whom the Das AngelWings septal occluder device was implanted in the atrial septum. We report the clinical and echocardiographic outcome, at an average of 5 years following the procedure, of patients treated with the Das AngelWings device used to close either a secundum atrial septal defect (ASD) or a patent foramen ovale (PFO).

Methods

Thirty-two patients underwent successful percutaneous closure of an atrial septal closure, patent foramen ovale, or fenestration in the lateral tunnel of their Fontan with the Das AngelWings device between June 1995 and March 1998 at Duke University Medical Center. Two of the 32 patients were lost to follow-up. The remaining 30 patients were divided into 3 groups based on indication for device implantation. Group 1 consisted of 14 patients with a secundum ASD and predominantly left-to-right atrial shunting. Group 2 consisted of 8 patients who had a PFO and who suffered a thromboembolic event. Group 3 (compassionate use) consisted of 10 patients with multiple comorbid medical problems with predominantly right-to-left shunting at the atrial level causing hypoxemia. Eight of the patients in group 3 were severely ill at the time of device implantation. The 2 remaining patients in group 3 underwent AngelWings implantation for closure of right-to-left shunting through a Fontan fenestration. Mean follow-up was 59 months.

Results

There was no device embolization. No patient in the ASD or PFO/stroke group had a clinical complication. By radiographic examination, 2 of 27 patients had evidence of fracture of the nitinol framework at 2-year follow-up. Residual shunting was present in 44% at 24 hours, 20% at 1 year, and 18.8% at 2 years by use of Doppler color flow imaging and/or microcavitation echocardiographic studies. Mild mitral regurgitation caused by the AngelWings device occurred in 1 patient.One patient in the compassionate use group had a subsequent neurologic event. Five of the 10 patients in the compassionate use group died of comorbid illnesses in follow-up, none directly related to device complications.

Conclusion

The late clinical outcome of secundum ASD and PFO/stroke patients in this study demonstrates that Das AngelWings closure of the atrial septum is effective and safe. These data are encouraging with respect to the expanding use of other percutaneously implanted ASD occlusion devices, although late clinical safety and efficacy data are needed for the specific devices being implanted.     

Role of intracardiac echocardiographic guidance in transcatheter closure of atrial septal defects and patent foramen ovale using the Amplatzer device

Transesophageal echocardiography (TEE) has been successfully used for guiding transcatheter device closure of secundum atrial septal defect (ASD) and patent foramen ovale (PFO). However, the use of TEE for device closure requires general anesthesia. Experience with intracardiac echocardiographic (ICE) guidance to close ASD and PFO is limited. One hundred eleven patients (76 female/35 male) with secundum ASD (82 patients) and PFO (29 patients) associated with a stroke underwent an attempt of transcatheter closure of their defects under ICE guidance using the new AcuNav catheter. The median age of patients was 40 years (range 2.5-80.7) and the median weight was 66 kg (range 12.7-128 kg). The median two-dimensional size of secundum defects as measured by ICE was 17 mm (range 3-32 mm). The median balloon stretched diameter of the ASDs was 22 mm (range 4-36 mm). Five patients had more than one defect that required placement of two devices to close the defects. The median Qp/QS ratio for patients with secundum ASD was 2.1 (range 1-18). ICE provided adequate views of the defects and surrounding structures and the various stages of device deployment. All patients had successful device placement, including the patients who received simultaneous two devices with immediate complete closure of the defects in 100 patients, whereas four and seven patients had trivial and small residual shunt, respectively. The median fluoroscopy time was 10.2 minutes (range 3.7-38.4 minutes) and the median total procedure time was 60 minutes (range 28-180 minutes). There were no complications related to the use of the AcuNav catheter. We conclude that ICE provided unique images of the atrial communications and facilitated device closure of secundum ASD and PFO in children and adults. We believe ICE should replace TEE as a guiding imaging tool for ASD and PFO device closure, thus eliminating the need for general anesthesia.     

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 closure of atrial septal defect by "buttoned" devices.

During a 21-month period ending in August 1991, 12 patients underwent transcatheter closure of atrial septal defect (ASD) with a "buttoned" device through an 8Fr sheath, using an institutional review board-approved, custom-made device protocol initially, and Food and Drug Administration-approved clinical trials subsequently. Ten children had left-to-right shunts across the ASD and 2 adults had their patent foramen ovale closed to prevent recurrent paradoxical embolism (including cerebrovascular accidents). The device dislodged in 1 of the 4 patients in whom a first-generation device was used, and retrieval of the device, and surgical closure of the ASD were performed without incident. In the remaining 11 patients (age range 7 months to 45 years, weight 3.6 to 64 kg), the device remained intact, and pulmonary-to-systemic flow ratio decreased from 2.1 +/- 0.3 to 1.04 +/- 0.06. Follow-up chest x-ray and echo-Doppler studies were available in all patients 0.5 to 18 months after the procedure. The device was intact in all patients. Small residual shunt detected by color Doppler was seen in 2 children. Right ventricular size decreased (23 +/- 6 to 17 +/- 3 mm; p less than 0.01), and paradoxical septal motion disappeared in all patients. There was no recurrence of paradoxical embolism in the 2 adults. No complications occurred during follow-up. It is concluded that transcatheter closure of ASD with the buttoned device is feasible, effective and safe, and can be accomplished with small, 8Fr sheaths (even in infants weighing 3.6 kg), follow-up results are excellent, and clinical trials on a larger number of patients are needed.     

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.     

Incidence of atrial fibrillation following transcatheter closure of atrial septal defects in adults

Transcatheter closure of secundum atrial septal defect (ASD) and patent foramen ovale (PFO) has become a routine procedure. Little is known about the effect of atrial septal device implantation on the occurrence of atrial fibrillation (AF). We evaluated the frequency of AF occurring after transcatheter PFO and ASD closure in a large population. From 1994 until 2007 a total of 1,062 patients underwent transcatheter closure of an interatrial communication. New-onset AF was defined by 12-lead electrocardiogram or Holter monitoring in patients without a history of AF at baseline. Of the 1,062 patients, 822 had a PFO and 240 had an ASD. During a median follow up of 20 months, new-onset AF was documented in 8% of patients. New-onset AF occurred in 7% of patients after PFO closure and in 12% of patients with underlying ASD. The annual incidence of new-onset AF was 2.5% and 4.1% in patients with PFO and ASD, respectively. Generally, patients with new-onset AF were older than those without AF. Device type or size did not influence the occurrence of AF. In the group of patients with PFO, residual shunt was more common in patients with AF compared with the non-AF group. In conclusion, AF is more common after PFO and ASD closure compared with the general population; although device type or size did not impact the occurrence of AF, residual shunt may influence the occurrence of AF after intervention in patients with underlying PFO.     

Non-surgical repair of atrial septal defect by "buttoned" double disk device in children. First results in 3 children

Transcatheter closure of ostium secundum atrial septal defect (ASD) with a "buttoned" double-disk device was attempted in 5 children aged 14 months to 12 years, with success in 3 cases. The diameter of the ASD was 15 to 20 mm. The occluding devices were chosen with sizes 12-15 mm greater than those of the ASDs. There were no operative complications: the ASD was practically completely occluded in 2 children and left a very small shunt in a third case. Those three patients are doing well, 8 to 9 months after the procedure. In the other two cases, the device fell into the right atrium and had to be removed surgically without any complications. The "buttoned" double-disk device seems to be relatively simple and easy to insert through an 8F catheter. Additional technical improvements should enable more complete occlusion of ASD and easier retrieval by the transvenous route when incorrectly inserted. Further experimental and clinical studies are required to assess the results and to compare them with those of the other teams.     

Buttoned Device for Atrial Septal Defect Occlusion

This article reviews the most recent results of the latest modification of the buttoned device (Custom Medical Devices, Amarillo, TX), the 4th generation. The latest advances in atrial septal defect (ASD) occlusion with further modifications of the buttoned device--inverted buttoned device, hybrid buttoned device, and centering- on-demand device (COD) (Custom Medical Devices)--that have led to the evolution of wireless and deviceless ASD occlusion are also presented.     

Percutaneous closure with Amplatzer device is a safe and efficient alternative to surgery in adults with large atrial septal defects

BACKGROUND: In adults with atrial septal defect (ASD) and large right-to-left shunt, closure of the defect is recommended. Percutaneous closure is still rarely used in this population. This study presents the results of transcatheter closure with the Amplatzer occluder in such patients. METHODS AND RESULTS: We studied 44 consecutive adult patients with a secundum ASD and 2 of the 3 following criteria: QP/QS >/=2 by oximetry, echocardiographic right ventricle overload, and ASD size >20 mm. Forty-two patients had a successful implantation. In 1 patient an unstable device was withdrawn; in another one, the device embolized in the pulmonary artery. At 6-month median follow-up, 95% had a complete closure; 2 patients with an additional defect had a small residual shunt. Major complications were the aforementioned embolization and a cerebrovascular accident in a patient with atrial fibrillation treated with aspirin. Others were minor and transitory: premature atrial beats in 3 patients, and paroxysmal atrial fibrillation and pulmonary edema in 1 patient each. CONCLUSIONS: Transcatheter closure of large ASDs with the Amplatzer device is efficient with less morbidity than surgical closure.     

Transcatheter occlusion of complex atrial septal defects.

Percutaneous device occlusion of secundum atrial septal defects (ASDs) is becoming an accepted alternative to surgical closure. This method allows us to evaluate patients with complex conditions for treatment. From a total of 70 patients with ASD evaluated for percutaneous closure, we selected for analysis 28 who had complex conditions. The mean age was 36+/-23 yr (range, 4-72). Six had heart failure, and of these six, three had atrial fibrillation. At cardiac catheterization, the pulmonary pressure was 47+/-24 mm Hg, and the QP/QS was 1.7+/-0.4; two patients had bidirectional shunt and systemic pulmonary pressure. Two patients received a buttoned device and 26 an Amplatzer septal occluder. The groups of patients with complex conditions were separated into the following groups. Group I (n = 4) underwent combined treatment of associated anomalies. Two patients had pulmonary stenosis, one had mitral stenosis, and one had an aortic root-left atrium fistula. They were treated in or during with the same procedure by combined transcatheter techniques (balloon valvuloplasty and fistula occlusion) before ASD occlusion. Group II (n = 9) had multiple defects (cribiform or two separate holes). They were treated with a single device in five instances and with two separate devices in four cases. Group III (n = 14) had large (32+/-3 mm) single defects. Nine of them underwent successful implantation using a device 33+/-3 mm in diameter; in the remaining five patients the device was removed because of instability. Group IV (n = 3) had residual defects after previous partial device occlusion. All three defects were successfully occluded with a second device. No movement or interference with the first device was observed. Group V (n = 6) had severe pulmonary hypertension (86+/-16 mm Hg). Immediately after ASD occlusion we observed significant relief in these patients (67+/-14 mm Hg; P<0.01). There were no major complications; all 23 patients with successful implants were discharged without symptoms 2-7 days later; one patient with atrial fibrillation recovered sinus rhythm. The follow-up (8+/-5 mo) Doppler echo study showed complete ASD occlusion in 22 patients and a peak pulmonary pressure of 30+/-14 mm Hg. We conclude that transcatheter occlusion of ASDs is an effective and safe treatment for patients with complex anatomic or physiopathologic conditions, as evaluated by short-term follow-up.     

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.     

Platypnea–orthodeoxia syndrome in the elderly treated by percutaneous patent foramen ovale closure: A case series and literature review

BackgroundPlatypnea–orthodeoxia syndrome (POS) is a rare clinical phenomenon, associating normal oxygen saturation in a supine position and arterial hypoxemia in an upright position. This pathology can be secondary to an intracardiac shunt, a pulmonary vascular shunt or a ventilation–perfusion mismatch. Cardiac POS occurs in the presence of a right-to-left cardiac shunt, most commonly through a patent foramen ovale (PFO).Methods and resultsFrom our single-center prospective database of percutaneous PFO closure we identified five patients (4 females, mean age: 77 ± 11 years) out of 224 (2.2%) patients with a PFO who presented with a POS of cardiac origin. Transthoracic and transoesophageal echocardiographic examinations revealed the underlying mechanisms of POS and the diagnosis was confirmed by right-and-left cardiac catheterization (RLC) and by measuring serial blood oxygen saturation in the pulmonary veins and left atrium in supine and upright positions. PFO was associated with atrial septal aneurysm and a persistent prominent Eustachian valve in 3 patients.All patients underwent a successful percutaneous PFO closure without any immediate or subsequent complications (mean follow-up of 24 ± 18 months). Immediately after the procedure, mean arterial oxygen saturation improved from 83% ± 3 to 93% ± 2 in an upright position and symptoms disappeared.ConclusionPOS is a rare and under-diagnosed pathology that must be actively investigated in the presence of position-dependent hypoxemia. The diagnostic work-up must exclude other causes of hypoxemia and confirm the intracardiac shunt using either contrast echocardiography or RLC. For cardiac POS, percutaneous PFO closure is a safe and effective technique that immediately relieves orthodeoxia and patient symptoms.     

Percutaneous transcatheter closure of atrial septal defects: initial single-centre experience and follow-up results. Initial experience with three-dimensional echocardiography

OBJECTIVE: Registry to report our single-centre experience in closing defects of the atrial septum. METHODS: Between 1996 and 2001, transcatheter device closure of significant atrial septal defects was performed in 32 adults (mean age 45.1 years, range from 15 to 76 years), using different device types. Six patients had a patent foramen ovale (PFO) and 26 patients had a secundum atrial septal defect (ASD II). Defects were selected by means of two-dimensional transoesophageal echocardiography (2D TOE). For 12 defects additional three-dimensional transoesophageal echocardiography (3D TOE) was performed. RESULTS: Mean balloon-stretched diameter was 19.3 mm (range from 11 to 24 mm). A device was successfully placed in all PFO patients (100%) without complications at follow-up. Successful immediate device placement occurred in 24 out of 26 ASD patients. Two immediate placement failures, 1 device embolisation and 1 device non-fixation, occurred and required urgent surgery. There was 1 patient with pericardial effusion after the intervention, requiring pericardiocentesis. Three late placement failures occurred: 2 device embolisations and 1 device dislocation. Clinical success after 3 to 6 months follow-up, defined as the absence of shunt or small shunt, was achieved in all 6 PFO patients and in 21 out of 23 ASD patients (1 device embolisation, requiring surgery, occurred before follow-up was completed). Two patients with significant shunt, 1 late device dislocation and 1 late device embolisation, underwent elective surgery. For patient selection, 3D TOE proved useful in the evaluation of large and/or complex defects. CONCLUSION: Transcatheter device closure of carefully selected atrial septal defects is an alternative to surgery. Although the results are promising, surgical back-up is often needed.     

纽扣式补片术治疗成人先天性心脏病113例

目的 :评价纽扣式补片术治疗成人先天性心脏病的疗效及总结其经验。方法 :采用经导管纽扣式补片治疗先天性心脏病 113例 ,其中房间隔缺损 (ASD) 45例 ,动脉导管未闭 (PDA) 45例 ,室间隔缺损 (VSD) 2 3例。结果 :ASD、PDA、VSD成功率分别为 10 0 % (4 5/45例 )、95.6 % (4 3/45例 )和 83.0 % (19/2 3例 ) ,总成功率为 94 .6 %。手术并发症主要见于 VSD患者 ,束支传导阻滞发生率为 2 6 .4 3% (6 /2 3例 )。结论 :纽扣式补片术治疗成人先天性心脏病成功率高 ,即时疗效显著 ,并发症少 ,是一种安全的介入治疗方法     

Progrediente Dyspnoe bei Karzinoidsyndrom

In patients with carcinoid syndrome, there has always to be considered cardiac impairment. We report about two patients with hepatic and bone metastases of a neuroendocrine tumor of the midgut, who suffered from progressive dyspnea. This was caused in both cases by a right-to-left atrial shunt, in case 1 based on a patent foramen ovale (PFO), in case 2 based on a secundum atrial septal defect. Symptoms were significantly reduced by percutaneous closure of PFO and ASD, respectively. Right-to-left atrial shunt was facilitated by right-sided carcinoid induced endocardial fibrosis with the consequence of severe tricuspid regurgitation, leading to an increase of right atrial pressure.     

Transcatheter closure of residual atrial septal defect following implantation of buttoned device

We report a case in which residual shunting after a buttoned device occlusion of atrial septal defect (ASD) was eliminated by transcatheter retrieval of a portion of the device, followed by implantation of a second device. This method may be helpful for those patients with residual ASDs who decline surgical device retrieval and defect closure. © 1995 Wiley-Liss, Inc.     

Right hemidiaphragmatic elevation with a right-to-left interatrial shunt through a patent foramen ovale: a case report and literature review.

A right-to-left shunt (RLS) is an uncommon complication of a patent foramen ovale (PFO) that may cause hypoxemia from venous admixture and ischemic complications from paradoxic embolization. This report presents the third described patient whose RLS through a PFO and profound hypoxemia developed in association with right hemidiaphragm dysfunction (but without a pressure gradient driving the right-to-left flow). In addition to extending the available experience with this unusual clinical event, we report on the successful closure of the PFO by a catheter-deployed double-umbrella device, after the positioning of which the patient's oxygenation normalized.     

Results of transvenous occlusion of secundum atrial septal defects with the fourth generation buttoned device: comparison with first, second and third generation devices. International Buttoned Device Trial Group

OBJECTIVES: The purpose of this study was to assess safety and effectiveness of the fourth generation buttoned device in dosing atrial septal defects (ASDs) and to test the hypothesis that introduction of double button reduces unbuttoning rate without reducing effectiveness. BACKGROUND: Because of the high unbuttoning rate (7.2%) with first, second and third generation buttoned devices, the device was modified (fourth generation) so that there were two radiopaque spring buttons 4 mm apart on the button loop attached to the occluder. METHODS: During a four-year period ending in September 1997, 423 patients, ages 1.5 to 80 years (median 16 years), underwent closure of ASD at 40 medical centers around the world. RESULTS: The ASD size varied between 5 and 30 mm (median 17 mm). The device size varied between 25 and 60 mm. Unbuttoning occurred in 4 (0.9%) of 423 patients. Effective occlusion, defined as no (n = 343) or trivial (n = 34) residual shunt on echo-Doppler studies performed within 24 h of the procedure, was demonstrated in 377 patients (90%). Thus, the unbuttoning rate (0.9 vs. 7.2%) decreased (p<0.01) while effective occlusion rate (90 vs. 92%) remained unchanged (p>0.1) with this device, compared with earlier generation devices. During follow-up from one month to five years (23+/-15 months), 21 (5%) of 417 patients required surgical (n = 12) or transcatheter (n = 9) reintervention, mostly to treat significant residual shunt. In the remaining patients there was gradual reduction and disappearance of the residual shunt. No wire integrity problems were observed. CONCLUSIONS: These data suggest that the fourth generation buttoned device is as effective as earlier generation devices, but without significant unbuttoning. Follow-up results remained good, with a reintervention-free rate of 89% at five years.