Postoperative pulmonary arteriovenous fistula resulting in recurrent cryptogenic stroke

We report on a 66-year-old patient with a history of two cryptogenic strokes and a patent foramen ovale (PFO) who received a transcatheter closure of his PFO in our institution, but shortly after the intervention there was still a relevant right-to-left shunt. The following work-up showed an isolated pulmonary arteriovenous fistula as the real cause for the right-to-left shunt and hence the two strokes.     

Transcatheter closure of patent foramen ovale associated with paradoxical embolism using the amplatzer PFO occluder: initial and intermediate-term results of the U.S. multicenter clinical trial.

Closure of patent foramen ovale (PFO) has been proposed as an alternative to anticoagulation in patients with presumed paradoxical emboli. We report the immediate and mid-term results of the phase 1 U.S. Multicenter Clinical Trial of patients who underwent transcatheter PFO closure for paradoxical embolism using the new Amplatzer PFO device. Fifty patients (28 male/22 female) underwent catheter closure of their PFOs at a mean age of 41 +/- 11 years. Thirty-six patients had ischemic stroke, 10 had transient ischemic attack, and 4 had peripheral embolism. Seventeen patients had atrial septal aneurysm. The implantation procedure was successful in 49/49 patients; one patient did not have a PFO. Complete closure was seen immediately after the procedure in 26/49 patients; 17 had minimal residual shunt, 4 had moderate and 2 had large residual shunts. The median fluoroscopy time was 10.5 min (2.8-43 min). There were no complications related to the device. One patient developed an arteriovenous fistula at the catheter site requiring surgical repair. At a mean follow-up interval of 16.5 +/- 7.2 months, there were no deaths or recurrent neurological or peripheral embolic events. Eight patients reported an episode of dizziness or palpitations (four of them within 18 days of the procedure). No episodes of atrial dysrhythmias were noted. Contrast bubble study at last follow-up documented complete closure in 45/48 patients; one patient had minimal, one had moderate residual shunt, and one had a large shunt. One patient was lost to follow-up. We conclude that catheter closure of PFO associated with stroke/transient ischemic attack or peripheral embolism using the new Amplatzer PFO device is a safe and effective method in preventing recurrence of such episodes. Randomized clinical trials comparing device closure versus continued medical therapy are underway.     

Hypoxemia without persistent right-to-left pressure gradient across a patent foramen ovale:A clinical challenge

Patent foramen ovale (PFO) closure for systemic hypoxemia is controversial. The first systematic, albeit retrospective, study was recently presented which showed good procedural and clinical success for PFO closure for this indication. We present a case of acute right to left intra-cardiac shunt across PFO where the shunting is not persistent. Hence making a decision on PFO closure based on the aforementioned promising trial may not have been the right decision for the patient. This case highlights that the decision on PFO closure for such indication needs to be individualized. We also review the sparse literature on PFO closure for this indication and discuss how the decision making for such indication needs to be individualized.     

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.     

A case of platypnea-orthodeoxia syndrome caused by the interaction between the presence of corrected transposition of the great arteries and patent foramen ovale

A 42-year-old was admitted to our institution for recurrent episodic dyspnea on exertion. The patient had also a history of recurrent transient ischemic attacks. Careful history taking revealed that she developed dyspnea in an upright position, whereas the symptoms were relieved in a supine position (platypnea). The patient was given the diagnosis of platypnea-orthodeoxia syndrome. Transthoracic echocardiography showed cardiac situs solitus levocardia with great artery transposition with intact interventricular septum, patent foramen ovale (PFO) and moderate right atrial dilatation. Agitated saline contrast injection demonstrated the presence of spontaneous right-to-left shunt. The patient underwent transcatheter closure of PFO. After percutaneous closure of PFO patient became asymptomatic and hypoxemia induced by orthostatic conditions did not appear again. A transthoracic echocardiography performed after two months, showed the complete closure of PFO, without residual shunt.     

Patent foramen ovale closure: a new therapy for migraine.

Migraine is a recurrent disabling disorder predominantly affecting middle-aged women. Migraine occurs with or without aura symptoms. Several studies have shown an increased prevalence of right-to-left shunts (RLSs) in migraine with aura. The overwhelming majority of these shunts were due to a patent foramen ovale (PFO). Furthermore, migraine with aura is more prevalent in clinical entities associated with a RLS, e.g. cryptogenic stroke, decompression illness in divers, or in patients with hereditary hemorrhagic teleangiectasia and pulmonary arteriovenous fistulas. Retrospective studies have consistently shown that shunt closure was associated with a significant reduction in migraine frequency. Its beneficial effect seemed to exceed the efficacy of conventional migraine therapy. Several randomized clinical trials to prospectively assess the benefit of shunt closure in migraine patients have been initiated. The only one completed, the MIST trial (Migraine Intervention with STARFLEX Technology), showed a significant reduction of migraine with aura after device implantation, compared with controls. However, the benefit of PFO closure was more modest than expected. This review recapitulates the current data regarding PFO closure and migraine with aura and summarizes in brief the current knowledge regarding migraine pathophysiology and the link to a RLS.     

Congenital coronary fistulas: the most frequent congenital coronary anomaly

Congenital arteriovenous coronary fistulae are a rare condition of a direct communication between a coronary artery and one of the cardiac chambers, the coronary sinus, the superior vena cava or the pulmonary artery. In most instances the diagnosis is made during heart catheterization for coronary or congenital heart disease. Whether congenital coronary artery fistulae should be treated by transcatheter intervention or surgery and in which patients fistula closure should be performed is controversial. This report summarizes our experience of the surgical treatment of congenital arteriovenous coronary fistulae in 14 patients at the Deutsches Herzzentrum Berlin between March 1988 and April 1997. There were seven females and seven males aged from 3 to 67 years (mean 47 years). We analyzed the symptomatic status (NYHA class) preoperatively and in the late outcome, the preoperative angiographic data and the surgical techniques. The right coronary artery was affected in six, the left in six, and both arteries in two cases. The fistulae drained into the pulmonary artery in eight cases, into the superior vena cava and into the right atrium in two cases, and into the right ventricle and into the coronary sinus once. Fistula closure was unsuccessfully attempted interventionally in two patients and surgically in one patient in another institution. Twelve of the patients exhibited additional cardiac disease requiring surgery: seven cases presented additional coronary artery disease, one mitral valve disease, one persistent ductus arteriosus, one an aneurysm of the right coronary artery, and two an atrial septal defect. We performed fistula closure either by ligating or transsecting the fistula as well as by closure of the fistula's drainage opening. Surgery and postoperative courses were uneventful in all patients. Most of the patients (93%) were in good clinical condition (NYHA I-II) after a mean follow-up period of 6.6 years (range 3-11). Fistula closure should be performed in patients who are symptomatic or who have a hemodynamic relevant shunt. In asymptomatic patients and small left-to-right shunt, fistula closure should also be performed to prevent later complications. Surgical fistula closure should be employed in patients with larger and more complex fistulae, especially if interventional therapy failed, and for patients with additional cardiac conditions that necessitate surgery.     

卵圆孔未闭结构特征对介入治疗封堵器选择的影响

目的 探讨卵圆孔未闭（PFO）结构特征对介入治疗封堵器选择的影响。方法 回顾性分析98例拟进行PFO封堵治疗患者临床资料,按经胸超声心动图(TTE)和（或）经食管超声心动图(TEE)检查结果分为简单PFO组和复杂PFO组,比较两组患者封堵器的选择及疗效。结果 PFO患者98（男41,女57）例,年龄8~74(41±14)岁。92例封堵成功,6例未成功,其中导丝无法到达左房4例,肺动静脉瘘1例,并发肺动脉高压患者1例。手术总成功率为94%。简单PFO组42例,复杂PFO组56例,简单PFO以18/25 mm PFO封堵器为主,复杂PFO以30/30 mm和25/35 mm PFO封堵器为主,两组间封堵器的选择有显著差异（P<0.01）。术后半年复查经胸超声心动图声学造影（cTTE）,完全堵闭65例（71%）,Valsalva动作后有右向左分流（RLS）27例（29%）,其中少量20例(22%),中量4例(4%),大量3例(3%)。大量RLS均发生在复杂PFO组应用30/30 mm和25/35 mmPFO封堵器的患者。复杂PFO组有1例新发阵发性心房纤颤。结论 封堵器选择与PFO特征有关,简单PFO以18/25 mm PFO封堵器为主,复杂PFO以30/30 mm和25/35 mm PFO封堵器为主。     

Use of saline contrast echo timing to distinguish intracardiac and extracardiac shunts: failure of the 3- to 5-beat rule

Patent foramen ovale (PFO) is thought to be associated with cryptogenic stroke and migraine headache. Saline contrast echocardiography (SCE) is the gold standard for identifying the presence of right-to-left shunt, whether from PFO or pulmonary arteriovenous malformation (PAVM). The timing of left heart contrast entry during SCE is used to distinguish a PFO from a PAVM, a method that is not as specific as previously thought. In this report, we describe a patient with a SCE demonstrating the early appearance of left heart bubbles during good effort Valsalva injections that is ultimately proven to be due to a PAVM. The case illustrates the limited specificity of left heart contrast timing during SCE as the sole criteria for differentiating intracardiac and extracardiac shunts.     

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.     

Diagnosis and therapy for pulmonary arteriovenous fistula in patients with presumed patent foramen ovale

An increasing number of patients are being referred for PFO closure for prevention of recurrent paradoxical embolism. Diagnosis is usually made using hand agitated echo bubble contrast with transthoracic or transesophageal echo. It is inevitable that some of these patients will have pulmonary arteriovenous fistulae (PAVF), either with or without associated PFO, which may be the sole cause of paradoxical embolism, or remain after PFO closure as a source of continued embolization. Intravenous contrast echo for the diagnosis of PFO is both insensitive and nonspecific. We describe a method to diagnose and localize PAVF during a catheterization procedure using intracardiac echo in conjunction with selective injection of bubble contrast into the pulmonary arteries. This allows for the rapid and accurate diagnosis of PAVF when a PFO is not found and for the exclusion of associated PAVF when PFO exists. © 2009 Wiley‐Liss, Inc.     

Device closure of fistula from left lower pulmonary artery to left atrium using a vascular plug: A case report

BACKGROUNDPulmonary artery-to-left atrial fistula is a variant of pulmonary arteriovenous fistula and is a developmental anomaly. Delayed presentation, cyanosis and effort intolerance are some of the important features. The diagnosis is confirmed by computed tomography or pulmonary artery angiography. Catheter-based closure is preferred to surgery.CASE SUMMARYLeft pulmonary artery-to-left atrial fistula is rare. A 40-year-old male presented with effort intolerance, central cyanosis, and recurrent seizures. He had a large and highly tortuous left pulmonary artery-to-left atrial fistula associated with a large aneurysmal sac in the course. Catheter-based closure was performed using a vascular plug.CONCLUSIONLeft pulmonary artery-to-left atrial fistula is relatively uncommon compared to right pulmonary artery-to-left atrial fistula. Percutaneous closure by either a transeptal technique or guide wire insertion into the pulmonary vein through the pulmonary artery is preferred. The need for an arteriovenous loop depends on the tortuosity of the course of the fistula and the size of the device to be implanted because a larger device needs a larger sheath, necessitating firm guide wire support to facilitate negotiation of the stiff combination of the delivery sheath and dilator.     

Massive Pulmonary Embolism and Paradoxical Migration during Surgical Embolectomy: Role of Transesophageal Echocardiogram

Patent foramen ovale (PFO) in the setting of venous thromboembolism is associated with paradoxical embolization. We describe a patient who presented with pulmonary embolism, underwent pulmonary embolectomy, and postoperatively developed paradoxical embolization to the lower extremity. A 27‐year‐old African American male presented to the hospital with shortness of breath and midsternal chest pain along with neck vein distention. A CT scan with contrast showed the presence of a saddle embolus in both pulmonary arteries. The next day, the patient developed right ventricular failure and hypotension. The patient was taken to the operating room for a pulmonary embolectomy. Postoperatively, the patient developed acute left lower extremity ischemia. The origin of the embolus was suspected to be cardiac. A transesophageal echocardiogram (TEE) revealed thrombus on the mitral valve and a PFO with right to left shunt. At this point vascular surgery for revascularization of the left lower extremity was performed. Two days later, the patient was taken for a repeat cardiac surgery and the left‐sided thrombus was removed along with a closure of the PFO. This case signifies the importance of complete TEE and a search for PFO in patients with massive pulmonary embolism especially prior to surgical embolectomy because hemodynamic disturbances of pulmonary embolism and surgical embolectomy may cause migration of the thrombus from the right side to the left side of the heart. Copyright © 2009 Wiley Periodicals, Inc.     

Percutaneous balloon catheter closure of a patent foramen ovale in a patient with pulmonary disease,profound hypoxemia,and normal right heart pressures

Right-to-left intracardiac shunting across a patent foramen ovale (PFO) has been reported in patients with pulmonary embolism, right ventricular (RV) infarction, positive pressure ventilation with positive end-expiratory pressure, heart failure with left ventricular assist devices, cardiac tamponade, and unilateral diaphragmatic paralysis. The primary driving force for these shunts is a reduction in the compliance of the pulmonary bed or right ventricle; right atrial pressure is usually elevated and pulmonary hypertension is frequently present. Significant shunting and hypoxemia are unusual in the absence of these diseases. We encountered a patient with normal pulmonary pressures, severe hypoxemia, pulmonary disease, and intracardiac shunting across a PFO in whom it was difficult to determine how great a role intracardiac shunting was playing in his hypoxemia. To assess this, we performed percutaneous balloon catheter occlusion of the PFO, using transthoracic echocardiography with contrast to confirm closure of the PFO. Therapeutic balloon occlusion has been reported in severe hypoxemia due to shunting across a PFO in a patient with RV infarction. Our case is unique, however, in two respects. First, this patient had normal right-sided cardiac pressures and normal RV function and, thus, no obvious driving force for a significant right-to-left shunt. Second, transthoracic echocardiography with contrast was used before and after balloon inflation to confirm closure of the PFO. This technique helped to answer the important clinical question of whether surgical closure of the PFO in this patient with both lung disease and intracardiac shunting would significantly improve his oxygenation.     

Percutaneous closure of an Iatrogenic fistula from the left ventricle to the coronary sinus

A patient with a history of rheumatic mitral valve disease and valve replacement in childhood presented with severe, persistent dyspnea. During an electrophyisiologic procedure, she was discovered to have a fistula from the left ventricle to the coronary sinus. She had severe pulmonary hypertension and elevated filling pressures, with a significant left‐to‐right shunt. Percutaneous closure of the fistula was performed using two vascular plugs. Subsequently the patient's hemodynamics improved and her symptoms subsided. Here, we describe the case and review the literature. © 2014 Wiley Periodicals, Inc.     

Pulmonary arteriovenous fistula discovered after percutaneous patent foramen ovale closure in a 27-year-old woman

We report the case of a 27-year-old woman with history of a transient ischemic attack who was diagnosed with a large patent foramen ovale (PFO). After percutaneous PFO closure, it was discovered that the patient had a pulmonary arteriovenous malformation (PAVM) and a diagnosis of hereditary hemorrhagic telangiectasia was made. The PAVM was later coil embolized. This case illustrates the importance of evaluating PFO closure patients for other causes of right-to-left shunting.     

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.     

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.     

Closure of patent Potts shunt with aortic endoluminal stent graft

Pulmonary hypertension secondary to residual Potts shunt is not an uncommon phenomenon. A 59-year-old male with a history of tetralogy of Fallot was noted, on a full heart study, to have persistent pulmonary hypertension, normal left ventricular function, severe aortic regurgitation, and a residual Potts shunt. A previous surgical attempt at closure of the shunt during definitive repair was unsuccessful. An aortic endoluminal stent graft was deployed to definitively close the shunt. There was a subsequent normalization of pulmonary pressures following Potts shunt closure. The patient will now proceed with surgical aortic root repair and aortic valve replacement.     

A case of multiple pulmonary arteriovenous fistulae associated with hereditary hemorrhagic telangiectasia that deteriorated during pregnancy]

A 29-year-old woman was admitted to our hospital because of persistent breathlessness on exertion after the delivery of her second child. Although at the age of 26 she had been given a diagnosis of multiple pulmonary arteriovenous fistula (PAVF), treatment was not done because of the absence of symptoms. An intrauterine growth retardation (IUGR) due to hypoxemia occurred during the second pregnancy, and then she delivered a 1,283g baby by cesarean section in the 36th gestation week. Her symptoms and hypoxemia improved after transcatheter embolization. Hereditary hemorrhagic telangiectasia was diagnosed on the basis of recurrent epistaxis since her childhood, pulmonary and hepatic vascular abnormality (PAVF and hepatic arterioportal shunt) and telangiectasis of the buccal mucosa and tongue. We should consider an early treatment of PAVF for young female because pregnancy may induce increase of arteriovenous shunt, fatal hemoptysis, infertility, miscarriage and IUGR.