Treatment Of Pulmonary Arteriovenous Malformations Research Articles

Efficacy of Venous Sac Embolization for Pulmonary Arteriovenous Malformations: Comparison with Feeding Artery Embolization

PurposeTo examine the efficacy of venous sac embolization (VSE) in comparison with transcatheter feeding artery embolization (FAE) for treatment of pulmonary arteriovenous malformations (PAVMs). Materials and MethodsFrom 1989–2009, 21 patients underwent embolization of 37 PAVMs. Safety and long-term efficacy of VSE were evaluated retrospectively and compared with FAE. ResultsFAE was performed in 22 (18 simple and 4 complex type) PAVMs, and VSE was performed in 15 (14 simple and 1 complex type) PAVMs. There were significant differences between FAE and VSE in treated periods, PAVM location, coil type used, number of coil combinations and coils per PAVM, coil position, and reperfusion; there were no significant differences in most PAVM characteristics, follow-up durations (58 mo±54 vs 42 mo±42; P= .32), and minor complications (pleurisy [2 vs 2]). Reperfusion occurred in 11 (50%) of 22 PAVMs in the FAE group and no PAVMs in the VSE group (P<.01). Of 22 PAVMs in the FAE group, 17 (77%) were treated with 0.035-inch coils alone; of 15 PAVMs in the VSE group, 14 (93%) were treated with 0.018-inch interlocking detachable coils (IDCs), 0.018-inch pushable fibered coils, or IDCs and pushable fibered coils combined (P<.01). The number of coils used was 8±4 in the VSE group and 4±4 in the FAE group (P= .002). ConclusionsThe high reperfusion rate in the FAE group was mainly due to the use of large 0.035-inch coils alone. Although more coils are needed, VSE can be used to treat PAVMs with a venous sac safely and achieve long-term efficacy.

Distal Cross-sectional Occlusion Is the “Key” to Treating Pulmonary Arteriovenous Malformations

Pulmonary arteriovenous malformations (PAVMs) represent a significant source of morbidity, especially in patients with the autosomal-dominant disease hereditary hemorrhagic telangiectasia (HHT), which is found in 56%–97% of patients with PAVMs (1–6). In the HHT population, reported incidences of PAVM range from 23% to 61%, and it is more common in patients with HHT1 (ie, endoglin mutation) than HHT2 (ie, activin receptor–like kinase 1 mutation) (7–12). Clinical manifestations eventually occur in more than 70% of patients, typically by the fourth through sixth decades, although exertional dyspnea and fatigue from hypoxemia can be gradual and even unrecognized by the patient. More serious consequences relate to the risks of paradoxic embolization and rupture (1,3–6,13– 16). Stroke or transient ischemic attack is seen in 11%– 55% of cases, seizures in 5%–15%, brain abscess in 5%–25%, and hemoptysis or hemothorax in as many as 18%. Pregnancy appears to present a greater risk for these complications (17). Finally, the incidence of migraine headaches is higher in patients with PAVM. During the past few decades, embolization has been shown to be highly effective for the treatment of PAVM, with low morbidity rates and minimal inconvenience to the patient. It is generally now performed as an outpatient procedure, so it has largely replaced operative options (1,3–5,15,18–24). The prevalent technique has been embolization of the feeding artery by using a variety of mechanical agents. Detachable balloons were commonly used in the past, when these were available, whereas ‘‘pushable’’ coils have been used all along, detachable coils later, and AMPLATZER Vascular Plugs (AVPs; St. Jude Medical, St. Paul, Minnesota) more recently. The

Long-Term Follow-Up of Treatment of Pulmonary Arteriovenous Malformations with AMPLATZER Vascular Plug and AMPLATZER Vascular Plug II Devices

Purpose To assess the feasibility, complications, and long-term success of embolization of pulmonary arteriovenous malformations (PAVMs) with the AMPLATZER Vascular Plug and AMPLATZER Vascular Plug II. Materials and Methods The study included 15 consecutive patients (19 embolization episodes) who had embolization of PAVMs between April 2004 and April 2009 with an AMPLATZER Vascular Plug or AMPLATZER Vascular Plug II. There were 4 men and 11 women, with a mean age of 56 years (range 24–74 years). A prospective database of all cases of PAVM embolization is kept in the department. Patient history, detailed procedural records, and clinical and radiological follow-up were reviewed. Results Among the 19 PAVMs, an AMPLATZER Vascular Plug was deployed in 11, and an AMPLATZER Vascular Plug II was deployed in 8. The technical success of the procedure was 100% for PAVM occlusion; 30-day mortality in the group was zero. Successful radiologic follow-up with the AMPLATZER Vascular Plug was a mean of 28 months (range 0–60 months) and with the AMPLATZER Vascular Plug II was a mean of 18 months (range 12–36 months). There was one recanalization of an AMPLATZER Vascular Plug 36 months after embolization giving an annual event rate of 0.03 recanalizations per AMPLATZER Vascular Plug or AMPLATZER Vascular Plug II per year. There were no major complications. Clinically, there was one (1 of 18 cases [5%]) immediate complication of chest pain that resolved in 24 hours with simple analgesia. There were no early or late clinical complications. Conclusions The treatment of PAVM with either an AMPLATZER Vascular Plug or an AMPLATZER Vascular Plug II is safe and effective and associated with a low reintervention rate. Further follow-up is ongoing to ensure continued occlusion of treated PAVMs.

Successful treatment of pulmonary arteriovenous malformation and infantile hepatic hemangioendothelioma with alpha-interferon

Successful treatment of pulmonary arteriovenous malformation and infantile hepatic hemangioendothelioma with alpha-interferon

Embolization of pulmonary arteriovenous malformations using the Amplatzer vascular plug: successful treatment of 69 consecutive patients

The technique of embolization of pulmonary arteriovenous malformations (PAVMs) with the Amplatzer vascular plug (AVP) has been reported, but no large series has evaluated the effectiveness of this relatively new embolic device. The purpose of this study is to assess the role of AVPs in the treatment of PAVMs. Sixty-nine consecutive patients underwent embolization of pulmonary arteriovenous malformations between September 2006 and December 2008. Clinical, procedural, and physiological data were reviewed retrospectively. Of 161 PAVMs, 120 (75%) were successfully embolized with Amplatzer vascular plugs alone. Complete and rapid occlusion of feeding vessels was easily achieved at the site of arteriovenous communication without complication. Particularly small or tortuous feeding arteries supplying 27 complex and 14 simple PAVMs were occluded with coils. There have been no documented instances of recanalization on follow-up. Amplatzer vascular plugs allow the rapid and safe distal occlusion of the majority of PAVMs.

Pulmonary Arteriovenous Malformation in the Korean Population: Clinical Manifestations and the Long-Term Results of Transarterial Embolotherapy

Purpose: This study was designed to evaluate the general features and the long-term results of transarterial embolotherapy (TAE) for the treatment of pulmonary arteriovenous malformations (PAVMs). Materials and Methods: Thirteen (n=13) patients who underwent TAE for PAVMs were identified. These patients were comprised of 4 men and 9 women, aged between 19 and 67 years (mean age, 44 years). The authors evaluated the general features of PAVMs, including, type, location, size, and symptoms. In addition, results following TAE were analyzed to evaluate its efficacy, and to investigate arterial oxygen pressure changes. Results: In total, 13 patients presented with 21 angiographically confirmed PAVMs. Nine (69.2%) patients had single PAVM. Of the 21 PAVMs, 19 were simple, 16 were located in lower lobes, and 17 were located in the subpleural region. Furthermore, 2 of the 13 patients experienced coughing, 2 experienced dyspnea, and 1 experienced massive hemoptysis. All PAVMs were subjected to superselective TAE using a detachable balloon or coils. Six PAVMs (28.6%) in three patients (23.1%) were subsequently recanalized as determined by contrast-enhanced CT scan. All 3 patients were identified during long-term follow up (mean: 61.9 months). One of the 3 patients experienced an acute stroke due to thrombi migration in the middle cerebral artery following PAVM recanalization. Conclusion: TAE with coils is effective for the treatment of PAVMs, and it should be noted that patients who undergo embolotherapy require careful surveillance, due to the need for recanalization during follow-up.

Management of a Patient with a Clot-filled Pulmonary Arteriovenous Malformation

Transcatheter coil embolization is an established treatment for pulmonary arteriovenous malformations (PAVMs). Encountering a patient with a symptomatic PAVM partially occluded with a prominent clot is rare. Successful and safe coil embolization of such a PAVM is described in this case report.

Simultaneous Treatment of Pulmonary AV Malformation with Rheumatic Heart Disease

Only 13 cases of pulmonary arteriovenous malformation (PAVM) combined with rheumatic heart disease have been reported, and only 3 have been treated simultaneously by surgery. Transcatheter embolotherapy, the first-line treatment of PAVM, avoids major surgery, general anesthesia, and the loss of pulmonary parenchyma, but the subsequent PAVM reperfusion can occur through recanalization of the embolized arteries. We report a case of PAVM with rheumatic heart disease, in which both diseases were simultaneously treated by surgery.

Management of pulmonary arteriovenous malformations in pulmonary hypertensive patients: a pressure to embolise?: Table. 1—

Pulmonary arteriovenous malformations (PAVMs) carry significant risks [1, 2]. Pulmonary arterial blood passing through these right-to-left shunts cannot be oxygenated, leading to hypoxaemia, with arterial oxygen tension and arterial oxygen saturation ( S a,O2) inversely related to the size of the shunt fraction. The fragile wall of the PAVM sac may rupture, leading to haemoptysis or haemothorax [3]. In addition, the absence of a filtering capillary bed allows particulate matter to reach the systemic circulation where it impacts in other capillary beds, including those of the cerebral circulation, resulting in embolic cerebrovascular accidents and cerebral abscesses. In a recent series of 219 patients that corrected for ascertainment bias, the rates for ischaemic stroke and cerebral abscess were 11.3% and 9%, respectively, with risks unrelated to PAVM severity or symptoms [4]. Treatment of PAVMs by embolisation is an effective means of reducing lifetime risks of paradoxical embolic stroke and cerebral abscess [4], improving oxygenation [1, 5, 6], and treating PAVM-induced haemoptysis [3, 7]. Embolisation usually carries minimal risk in expert hands and, as a result, is generally offered to patients with PAVMs of a size amenable to embolisation, irrespective of the presence of respiratory symptoms. PAVMs themselves have a lower vascular resistance than that of the surrounding normal lung, due to the absence of a microvascular network of capillary vessels. However, most individuals with PAVMs have underlying hereditary …

Redirection of the Hepatic Venous Flow for the Treatment of Pulmonary Arteriovenous Malformations after Fontan Operation

A 45-month-old boy who had complex cardiac anomalies with interrupted inferior vena cava and polysplenia underwent extracardiac Fontan operation. He redeveloped deep cyanosis postoperatively. Cardiac catheterization showed a preferential flow of the hepatic venous blood to the right lung and arteriovenous malformations (PAVMs) in the left lung. He underwent revision of the conduit to attain balanced hepatic venous drainage with subsequent regression of the PAVMs and disappearance of cyanosis. Our experience shows that deficiency of hepatic venous flow played a crucial role in the development of PAVMs and that redistribution of the flow retained potential to ameliorate the pathological condition.

Redirection of Hepatic Drainage for Treatment of Pulmonary Arteriovenous Malformations Following the Fontan Procedure

The development of unilateral pulmonary arteriovenous malformations in patients after total cavopulmonary connection with an extracardiac conduit has been reported. Unequal distribution of hepatic venous flow to the lung is theorized to be the causative factor. We report the surgical management of pulmonary arteriovenous malformations in a patient with heterotaxy syndrome, single ventricle, and interrupted inferior vena cava. The patient had previously undergone a total cavopulmonary connection with an extracardiac conduit draining hepatic venous flow to the right branch pulmonary artery. In the subsequent operation, we redirected the extracardiac conduit to the innominate vein. This operation provided the affected lung with hepatic venous blood without exposing the patient to the morbidity associated with cardiopulmonary bypass.

Surgical Treatment of Pulmonary Arteriovenous Malformation: Report of Two Cases and Review of the Literature

Pulmonary arteriovenous malformations (PAVMs) are rare anomalies. The degree of right-to-left shunting, which can lead to cyanosis and paradoxical embolism causing neurological complications, determines the prognosis. We report two cases of PAVM and review the literature. A 45-year-old woman with clinical signs and symptoms of PAVM was examined using several different scanning techniques, which showed a large PAVM in the lower lobe of her right lung. A lobectomy was performed, which revealed a 5 cm diameter PAVM with one feeding artery and multiple veins. Intravenous angiography of a 7-year-old girl with symptoms of fatigue and acro-cyanosis confirmed the presence of a large PAVM in her right lower lobe. The PAVM had two major arteries arising from the aorta, which were ligated during a lobectomy. Both patients recovered well following surgery. Although PAVMs are rare, their neurological and haemodynamic consequences may be fatal. Interventional treatment techniques, including surgery, are usually curative.

Treatment of Pulmonary Arteriovenous Malformation Using Platinum Coils: Case Report

Pulmonary arteriovenous malformation (PAVM) is an abnormal direct communication between the pulmonary arteries and veins without any capillary network. The patients may be completely asymptomatic or may they develop serious complications including hemoptysis and brain abscess. We present here a case of incidentally found PAVM in a 33-year-old male who underwent embolization using platinum coils. Coil embolization is a safe, highly effective procedure that should be considered more often for the treatment of PAVM.

Segmental arterial ligation under video-assisted thoracoscopy combined with transcatheter embolotherapy for multiple bilateral pulmonary arteriovenous malformations

Multiple bilateral pulmonary arterio-venous malformations (PAVM) were diagnosed in a 15-year-old girl with erythrocytosis. Although transcatheter embolotherapy is generally accepted as a treatment of PAVM, the largest lesion in the right lower lobe was fed by a 13-mm diameter feeding artery without downstream narrowing, which was unsafe for the embolization of coils or balloons. Instead, we surgically ligated the largest feeding artery under video-assisted thoracoscopy, without losing any lung parenchyma. The other PAVM were successfully treated by embolization of metallic coils. The arterial partial pressure of oxygen (O(2)) increased from 37 to 64mmHg on room air, and from 46 to 410mmHg on 100% O(2).

Reperfusion of Complex Pulmonary Arteriovenous Malformations After Embolization: Report of Three Cases

The purpose of this report is to discuss the different mechanisms of reperfusion of pulmonary arteriovenous malformations (PAVMs) after embolization. Transcatheter embolotherapy is currently the first-line treatment of PAVMs to prevent neurologic complications or pulmonary hemorrhage. Initial good results can be expected but we report three cases of reperfusion of complex large PAVMs after coil embolization. After adequate embolization, reperfusion of PAVMs may occur by several mechanisms including recanalization of embolized arteries, recruitment of normal arterial branches, growth or enlargement and development of a systemic arterial supply.

Contrast Echocardiography Remains Positive After Treatment of Pulmonary Arteriovenous Malformations

Pulmonary arteriovenous malformations (PAVMs) in patients with hereditary hemorrhagic telangiectasia (HHT) can cause hemorrhage, stroke, and cerebral abscess. Therapy consists of transcatheter embolotherapy (TCET) to occlude the PAVMs. Contrast transthoracic echocardiography (TTE) can be used to screen for PAVMs, but little is known about the performance of contrast TTE after TCET has been performed. Our objective was to determine the effect of the successful performance of TCET on the performance of contrast TTE, specifically, in what proportion of patients the findings of contrast TTE normalized or remained positive after the performance of TCET. Retrospective chart review. HHT clinic at university teaching hospital. Patients who have undergone TCET for the treatment of PAVMs. Patients were screened for PAVMs with a chest radiograph (CXR), oxygen shunt test (OST), and contrast TTE. Pulmonary angiography was recommended for patients with any positive findings on a screening test. PAVMs > or = 3 mm were occluded by TCET. Contrast TTE, OST, and CXR were performed approximately 1 month later. The results of contrast TTE before and after patients underwent TCET were compared. Thirty-nine patients underwent contrast TTE prior to undergoing TCET, and 29 patients underwent contrast TTE both prior to and after undergoing TCET. In all patients, TTE findings were positive prior to TCET. All PAVMs with feeding vessels > or = 3 mm were successfully occluded based on completion angiography. After TCET, 48% of patients had no detectable residual PAVMs, and the remainder had small (ie, < 3 mm) residual PAVMs. Of the 29 patients, 90% had positive contrast TTE findings after undergoing TCET. In the subset of patients who had no residual PAVMs on the completion angiography, 80% had positive contrast TTE findings after undergoing TCET. In most patients, contrast TTE findings remain positive after they undergo TCET, even in patients without residual PAVMs seen on angiography. This may reflect residual PAVMs that are too small to visualize using angiography. These findings have important implications for the follow-up and management of HHT patients.

Percutaneous transluminal embolization with coils-treatment of pulmonary arteriovenous malformations.

The clinical values of coils embolization in the treatment of pulmonary arteriovenous malformations (PAVM) and related complications were investigated. Eleven patients with PAVMs verified by pulmonary arterial angiography were treated by transcatheter coils embolization. Chest X-ray (11 cases), computer tomography (7 cases) and/or magnetic resonance imaging (2 cases) were performed before embolization. Blood-gas analysis was done in 5 cases before and after embolization. The follow-up materials of 8 patients were collected to evaluate the effect of embolization with coils. The clinical manifestations included cerebral embolus, hemoptysis and decreased oxygenation in 9 patients and the remaining 2 had no symptoms. 9/11 cases were found by chest X-ray and 8 were diagnosed definitely. 7/7, 2/2 cases were diagnosed by CT or MR and diagnosis was made in all cases. Embolization was performed in 29 vessels. Partial pressure of oxygen in arterial blood of 5 cases changed significantly before and after embolization. Slight complications occurred in 6 patients, such as low fever, chest pain, pleurisy. The follow-up results showed that 7 cases were cured effectively. No primary and secondary device migration, and no medical paradoxical embolization occurred. It was concluded that coils embolization is a well-established method for treating PAVMs. It is a minimally invasive lung preserving treatment with high efficiency and less complication.

Transcatheter Embolotherapy of Maternal Pulmonary Arteriovenous Malformations During Pregnancy

To determine if transcatheter embolotherapy is safe and effective for the treatment of pulmonary arteriovenous malformations during pregnancy. Prospective study. Specialized hereditary hemorrhagic telangiectasia centers at Yale University School of Medicine and St. Michael's Hospital, University of Toronto. Seven pregnant women (age range, 24 to 34 years; gestational age range, 16 to 36 weeks) undergoing transcatheter embolotherapy. Transcatheter embolotherapy in all patients. Thirteen pulmonary arteriovenous malformations in seven patients were embolized with detachable silicone balloons and/or stainless steel coils without incident. The estimated fetal radiation dose ranged from < 50 to 220 mrad. No complications of pulmonary arteriovenous malformations occurred in any of the patients after transcatheter embolotherapy. The mothers went on to deliver healthy babies in all cases. Transcatheter embolotherapy of maternal pulmonary arteriovenous malformations performed by an experienced radiologist appears to be safe and effective after 16 weeks of gestational age.

Pulmonary arteriovenous malformations: cerebral ischemia and neurologic manifestations.

There is an increasingly recognized association between pulmonary arteriovenous malformations (PAVM) and cerebral ischemia, frequently attributed to paradoxical embolization. PAVM occur in 20 to 30% of the hereditary hemorrhagic telangiectasia (HHT) population. To evaluate the risk determinants for cerebral ischemia and neurologic manifestations in patients with PAVM. A retrospective cross-sectional study was performed on consecutive patients admitted between 1988 and 1992 for treatment of PAVM. The number of PAVM, feeding artery (FA) diameters, and aneurysmal sizes were determined by pulmonary angiography. Patients were categorized as having single or multiple PAVM with an FA diameter of > or = 3 mm. History, examination, and cerebral imaging studies were used to determine the prevalence of neurologic manifestations. Patients were defined as having cerebral paradoxical embolization if there was radiologic evidence of cortical infarction. There were 75 cases: 26 single PAVM and 49 multiple PAVM. Cortical infarction was present in 14% of patients with single PAVM. Patients with multiple PAVM had a greater prevalence of any infarction (OR 3.2; 95% CI, 1.2 to 9.44, p = 0.030), cortical infarctions (OR 2.3; 95% CI, 0.58 to 9.2, p = 0.230), subcortical infarctions (OR 2.1; 95% CI, 0.58 to 7.95, p = 0.249), abscesses (OR 2.3; 95% CI, 0.46 to 11.94; p = 0.295), and seizures (OR 6.4, 95% CI 0.77 to 53.2, p = 0.054). Patients with multiple PAVM had markedly greater odds of having any clinical or radiologic evidence of cerebral ischemic involvement (OR 4.5; 95% CI, 1.47 to 14; p = 0.008). There is a strong association between single PAVM and various neurologic manifestations. The prevalence is greater for patients with multiple PAVM, suggesting increased predisposition for paradoxical embolization with a greater number of malformations.

Fatal Outcome after Embolotherapy for Hepatic Arteriovenous Malformations of the Liver in Two Patients with Hereditary Hemorrhagic Telangiectasia

JVIR 2000; 11:855–858 PATIENTS with hepatic arteriovenous malformation (AVM) associated with hereditary hemorrhagic telangiectasia (HHT) present with diverse clinical manifestations ranging from cirrhosis to cholestasis and multiple biliary strictures (1–4). Left-to-right shunting may also lead to abdominal pain, weight loss, high output heart failure, pulmonary hypertension, and encephalopathy (5– 12). Therapeutic options reported for symptomatic AVMs include hepatic artery embolization, ligation, or liver transplantation (12–15). Hepatic artery embolization is assumed to be low risk and of benefit based on favorable results seen in the treatment of hepatomas, liver metastases, and hemangiomas (13,16,17). Although embolization is effective in the treatment of pulmonary AVMs in patients with HHT (18), successful implementation of this approach for the more complex HHT liver lesions remains unproven. Several previous reports (including ours) support embolization of HHT-related hepatic AVMs (8–10,12–15,19). However, the utility, morbidity, and mortality of the procedure has not been critically evaluated. We report the deaths of two patients with HHT after hepatic AVM embolization to treat their high flow-related symptoms.