Large Pulmonary Arteriovenous Malformation Research Articles

Pulmonary AVM and Stroke: Case Report from Istishari Arab Hospital

Background: Pulmonary arteriovenous malformation (PAVM) is an abnormal communication between pulmonary artery and pulmonary vein which leads to multiple manifestations that range from hypoxia to neurological manifestation such as stroke due paradoxical emboli in young patients. Chest radiography and CT-enhanced studies help in diagnosis with pulmonary angiography which is considered the gold standard study for diagnosis. Management was done with embolization of the feeding artery or arteries. Case: A 31 year old female with no previous history of medical illness, presented with sudden onset left sided weakness and was diagnosed as a case of right sided ischemic stroke. After reviewing her case by multidisciplinary team and multiple imaging studies, pulmonary angiography showed that the patient had large right lower lung lobe PAVM in addition to another right middle lung lobe and left lower lung lobe smaller PAVM. After that, patient underwent embolization of with no complications. Conclusion: PAVM, although it is rare, should be considered in differential diagnosis as the cause of neurological manifestation as ischemic stroke in young patients by paradoxical emboli. Embolization of the feeding artery or arteries is the main treatment of PAVM.

UNTREATED PULMONARY ARTERIOVENOUS MALFORMATIONS IN A CARDIORESPIRATORY STORM

UNTREATED PULMONARY ARTERIOVENOUS MALFORMATIONS IN A CARDIORESPIRATORY STORM

Endovascular Embolisation of Pulmonary Arteriovenous Malformation Using Amplatzer Vascular Plugs

Pulmonary arteriovenous malformation (PAVMs) are abnormal communications between pulmonary arteries and veins. The rarity of their occurrence, coupled with the risks they pose, including brain abscess, embolic stroke, and myocardial infarction, mandates that they should not be overlooked in the differential diagnosis of patients presenting with haemoptysis, dyspnea, clubbing, cyanosis, hypoxemia, or epistaxis.We present the case of a 41-year-old local female who presented to our hospital as an outpatient with decreased oxygen saturation (SpO2) of 70%-80% for the past two years with a final diagnosis of PAVM. The initial baseline workup showed polycythemia with a hemoglobin level of 19 mg/dL and raised hematocrit. She had extensive workup in the past two years for her polycythemia including gene mutation testing and cardiac workup which all turned out normal. Her chest X-ray (CXR) showed right lung opacity which was initially considered to be infective but it did not respond to antibiotic treatment. Later on, a CT scan of the chest was performed and findings were typical of a large PAVM which had two feeding arteries. The patient was referred to a cardiothoracic surgeon who sent the patient to the interventional radiology section for endovascular management.The embolization procedure was then performed and both feeders were successfully embolised. After the procedure, the patient's SpO2 levels were restored to 95%-96%, and no post-procedure complications were noted.

English

Pulmonary arteriovenous malformation (PAVM) is one of the rare pulmonary vascular anomalies. Pulmonary arteriovenous malformation results in right to left shunt due to the abnormal communications between the pulmonary arteries and the pulmonary veins bypassing the normal capillary bed.1 This condition being rare could be easily missed, hence, it is essential for clinicians to suspect it based on the classical clinical features. This helps in early diagnosis and deciding further appropriate treatment option. Here we report the case of a patient affected by a large idiopathic pulmonary arteriovenous malformation in the right lung. Most patients with pulmonary arteriovenous malformation are asymptomatic. This is due to the chronic compensation and secondary erythrocytic response. Dyspnoea due to PAVMs are a result of right-to-left shunt. Initial diagnostic tools include chest radiography and contrast enhanced computed tomography but the gold standard is pulmonary angiography.2 Because AVM has substantial morbidity rates associated with it, all patients with PAVMs who can undergo embolization should be treated with transcatheter embolization. In rest of the patients, surgical excision should be considered. The main objective of this study was to highlight the early suspicion and diagnosis of pulmonary arteriovenous malformation as this is easily missed and leads to undue delay of treatment.

Lifesaving Successful Embolization of Aggressive Vertebral Body Hemangioma and a Large Pulmonary Arteriovenous Malformation

Vertebral hemangiomas are incidental finding and most of the time do not necessitate immediate attention unless symptomatic. Vertebral hemangiomas can be very aggressive to the extent that they can produce cord compression and paraplegia/paraparesis. Such cases need evaluation with magnetic resonance imaging to assess the cause, extent, and mass effect on the spinal cord. Transcatheter embolization and decompression surgery are well established modes of treatment as single or combined approach. Pulmonary arteriovenous malformation (AVM) is caused by the direct communication of main pulmonary artery with pulmonary vein, leading to a high-flow right-to-left shunt. This usually presents clinically as hypoxia, cyanosis, and dyspnea. The clinical signs and symptoms vary depending on the size, number, and flow of fistula as well as the associated vascular malformations. Transcatheter embolization is a treatment of choice for pulmonary AVM. However, this method may result in incomplete resolution if the malformation is large in size or supplied by multiple feeding arteries. Surgical resection may be considered in those kinds of cases. As AVM can lead to hemodynamical instability, these lesions need to be addressed first. In this case, pulmonary AVM came to notice post surgical decompression. Here, we report a case of aggressive vertebral body hemangioma and a large pulmonary AVM in the same patient, which were treated by embolization in two different settings.

HIDDEN IN PLAIN SIGHT: NEW DIAGNOSIS OF PULMONARY ARTERIOVENOUS MALFORMATION AFTER ISCHEMIC COLITIS

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Pulmonary arteriovenous malformation (PAVM) is an abnormal connection between a pulmonary artery and vein, which allows blood to bypass the filter function of the capillary beds and delivers deoxygenated blood to the left heart. This condition often remains undiagnosed until thromboembolic complications present. We report the case of new diagnosis of PAVMs in a patient presenting with ischemic colitis. CASE PRESENTATION: A 58-year-old female presented with acute onset diffuse abdominal pain and emesis. She was afebrile and normotensive with nonspecific abdominal tenderness on examination. Laboratory data was significant for mild leukocytosis and normocytic anemia. Computer Topography (CT) abdomen and pelvis showed diffuse inflammation around the cecum, consistent with ischemic colitis. She underwent partial colectomy with ileostomy placement. Postoperatively, she developed acute hypoxemia. CT angiogram showed left lower lobe pulmonary embolus and multiple PAVMs (largest in left lower lobe). She was treated with anticoagulation. However, she remained hypoxic and required oxygen at discharge. Further history revealed episodic epistaxis since childhood, three prior stillbirths, miscarriage, and multiple strokes in her sister. Hypercoagulable work-up was negative. She was readmitted multiple times for hypoxia and has undergone numerous PAVM embolization procedures. Post-embolization bubble echocardiogram showed intra- and extra-cardiac shunt both confirmed by transesophageal echocardiogram. DISCUSSION: Most PAVMs are hereditary with over 80% occurring in association with hereditary hemorrhagic telangiectasia, an autosomal dominant vascular disorder. Less commonly, PAVMs are idiopathic or secondary to trauma, infection or due to hepatopulmonary syndrome. Previously viewed as rare conditions, recent data suggest PAVMs affect 1 in 2,600. Patients with PAVMs present with hypoxemia and paradoxical embolization complications, including stroke, brain abscess, and other end-organ ischemia as seen in our patient who presented with ischemic colitis. A history of recurrent nosebleeds since childhood and family history of strokes were subtle clues to the diagnosis, but as in many other cases, the PAVMs were not identified until she presented with a paradoxical thromboembolic event. Patient has persistent hypoxemia despite embolization, and we hypothesize that obliteration of the large PAVMs led to elevated pulmonary pressures, which opened the intra-cardiac shunt in addition to new intra-pulmonary shunts. CONCLUSIONS: Pulmonary AVMs often remain undetected in patients until a thromboembolic complication occurs. Increased awareness of variable presentations of pulmonary AVMs is essential for prompt diagnosis and treatment to prevent devastating complications. Reference #1: Shovlin, C. Pulmonary arteriovenous malformations. Am J Respir Crit Care Med. 2014 Dec 1; 190 (11): 1217-1228. Reference #2: Lovering, A et al. Pulmonary pathways and mechanisms regulating transpulmonary shunting into the general circulation: An update. Injury. 2010 Nov; 41(0 2): S16-S23. Reference #3: Lee, W et al. Contrast echocardiography remains positive after treatment of pulmonary arteriovenous malformations. Chest Journal. 2003 Feb; 123 (2): 351-358. DISCLOSURES: No relevant relationships by RAMYA GORTHI, source=Web Response No relevant relationships by John Mwangi, source=Web Response No relevant relationships by Mackenzie Steck, source=Web Response

Prise en charge d’une malformation artério-veineuse pulmonaire avec hypertension pulmonaire

Pulmonary arteriovenous malformations (PAVM) are abnormal communications between arteries and pulmonary veins without interposition of capillaries. Embolization is effective both in reducing lifetime complications associated with PAVM and in improving arterial oxygenation. Embolization is however not recommended in patients with pulmonary hypertension (PH) as it abolishes low resistance pathways for pulmonary blood flow and therefore might be expected to elevate pulmonary arterial pressure (PAP). We report the case of a 70-year-old patient with a large PAVM as well as group 3 PH [mean PAP=21mmHg, pulmonary vascular resistance (PVR) at 3.4 Wood units] secondary to stage 2 chronic obstructive pulmonary disease (COPD) with emphysema. According to the measured shunt flow (26% of cardiac output), predicted post-embolization PVR was estimated at 4.6 Wood units. These values were considered compatible with embolization of the PAVM. Dyspnea and gas exchange improved after PAVM embolization, without worsening PH. Embolization of a PAVM can be considered in patients with stable PH but requires careful prior hemodynamic assessment. The estimation of the shunt flow and predicted post-embolization PVR may guide the therapeutic decision.

RENDU-OSLER-WEBER SYNDROME WITH BIG PULMONARY ARTERO-VENOUS MALFORMATION (PAVM) AND ASSOCIATED FORAMEN OVALE PATENCY (FOP)

A 55 years old woman with a personal history of brain abscesses, recurrent epistaxis and transient ischemic attacks, underwent Mininvasive Surgery with atrial septal device positioning. Due to further brain abscesses and transient ischemic attacks, subsequent diagnostic studies were carried out and a large Pulmonary Arteriovenous Malformation (PAVM) was diagnosed. The presence of 3 of the 4 Curacao criteria make the diagnosis of Rendu-Osler-Weber Syndrome.

Pulmonary arteriovenous malformations in children with hereditary hemorrhagic telangiectasia: a longitudinal study.

Pulmonary arteriovenous malformations (PAVMs) often occur in children with hereditary hemorrhagic telangiectasia (HHT). A 14-year longitudinal study of PAVMs in children with HHT was undertaken to assess the prevalence, the clinical impact, and progression of these malformations. This was a retrospective, single-center study from May 2002 to December 2016 of 129 children with HHT diagnosed using Curacao criteria and/or confirmed by genetic testing. Transthoracic contrast echocardiography (TTCE) was the primary screening modality in all patients and PAVMs were diagnosed based on Barzilai criteria. Moderately positive TTCE (Barzilai criteria ≥ 2) was confirmed with subsequent contrast chest CT. New PAVMs were diagnosed with a positive TTCE after an initial negative TTCE. Embolization of PAVMs were performed according to HHT consensus guidelines. Of 129 children with HHT, 76 (59%) were found to have PAVMs. Sixty-seven (88%) were positive for PAVMs on initial screening. Of 63 children without PAVMs on initial screening, 31 were followed for >1 year. Nine of the 31 (29%) developed new PAVMs after initial negative study. Thirty-eight (50%) of the total 76 children with PAVMs had or developed lesions large enough to be treated with embolization. Nine patients with PAVMs initially too small to be treated with embolization, developed progression of disease and ultimately were treated with embolization over time. The majority, 60% (23/38), of the children with large PAVMs had no related clinical symptoms. After embolization, 21% (8/38), of patients underwent repeat interventions. Genetic diagnosis, age, and gender were not associated with risk of having PAVM nor with need for repeat interventions. Nearly 60% of children with HHT develop PAVMs. The risk for new PAVMs to develop, small PAVMs to become large, and previously embolized PAVMs to require further intervention remains throughout childhood. Thus, children with HHT require continued follow-up until adulthood.

Successful embolization of a large pulmonary arteriovenous malformation in a symptomatic teenager from a developing cardiac program.

Pulmonary arteriovenous malformations are a rare entity in children, and can cause significant morbidity. Device embolization has become the intervention of choice over the past 2 decades as more sophisticated devices have become available. We present an adolescent with a single, large arteriovenous malformation diagnosed and treated as part of an international medical mission in Kampala, Uganda, highlighting the atypical diseases encountered in developing countries and treatment modalities that can be employed.

Screening children for pulmonary arteriovenous malformations: Evaluation of 18 years of experience.

Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant disease with multi-systemic vascular dysplasia. Early diagnosis through screening is important to prevent serious complications. How best to screen children of affected parents for pulmonary arteriovenous malformations (PAVMs) is often subject to debate. Transthoracic contrast echocardiogram (TTCE) is considered optimal in screening for PAVMs in adults. Guidelines for the screening of children are not specific, reflecting the lack of scientific evidence on the best method to use. Aims of this study are (i) to evaluate our current screening method, consisting of history, physical examination, pulse oximetry, and chest radiography and (ii) to assess whether postponing more invasive screening for PAVMs until adulthood is safe. This is a prospective observational cohort study using a patient database. Over a period of 18 years (mean follow-up 9.21 years, SD 4.72 years), 436 children from HHT families were screened consecutively. A total of 175/436 (40%) children had a diagnosis of HHT. PAVMs were detected in 39/175 (22%) children, 33/39 requiring treatment by embolotherapy. None of the screened children suffered any PAVM-associated complications with this screening method. This study shows that a conservative screening method during childhood is sufficient to detect large PAVMs and protect children with HHT for PAVM-related complications. Postponing TTCE and subsequent chest CT scanning until adulthood to detect any smaller PAVMs does not appear to be associated with major risk.

Pulmonary microvascular architecture in hereditary haemorrhagic telangiectasia

A 24-year-old Caucasian man was admitted with a known hereditary haemorrhagic telangiectasia (HHT) and heterozygous mutation of factor V Leiden following episodes of cerebral infarctions in occipital lobes, cerebellum and...

Surgical Management of Medium to Large Size Pulmonary AV Malformations- A Case Series

The communication between pulmonary artery and pulmonary vein or between a bronchial artery and the pulmonary vein is called as pulmonary arterio-venous malformation. Dyspnea on exertion, fatigue, nasal bleeding, bluish discoloration of lips and nail beds and hemoptysis are common presenting complaints. In childhood only 10% of cases are identified. Cerebral vascular accidents, brain abscess, hemothorax, life threatening hemoptysis are the complications of large pulmonary arteriovenous malformation. We present you a series of three cases that were diagnosed to have pulmonary AV malformations and presented to us with various clinical pictures. All three patients were surgically managed and discharged uneventfully.

Hemiparesis With Hemoptysis

SESSION TITLE: Student/Resident Case Report Poster - Signs and Symptoms of Chest Disease SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: A Pulmonary arterio-venous malformation(PAVM) is a direct communication between the branches of pulmonary artery and pulmonary veins and may be congenital or acquired. These lesions are anatomical right to left shunts and may cause strokes as well as high output heart failure and hypoxemia. Increased blood flow and increased venous distensibility secondary to progesterone effect in pregnancy may increase PAVM growth and its associated complications. Majority of subjects are diagnosed in the first three decades of life. CASE PRESENTATION: A 36-year-old female presented to us with complaints of “blood in her mouth”. She initially noted it two years ago during her only pregnancy. This subsided spontaneously. She subsequently had two further episodes four weeks apart prior to current presentation during menstruation. She also has history of two episodes of transient ischemic attacks with a negative neurological work up. Prior workup included a normal Gastrointestinal and Otolaryngologic evaluation. Her vitals and physical exam was entirely normal. Initial laboratory investigations revealed a normocytic anemia. Blood chemistry, coagulation parameters, and serologies were normal. A computed tomography angiography(CTA) of the chest revealed an opacity in the left lower lobe suggestive of arteriovenous malformation(AVM). A bronchoscopy was negative for endobronchial lesions or bleeding. The patient underwent embolization of the AVM feeding artery with an amplatzer plug with resolution of her symptoms. DISCUSSION: PAVM can be congenital or acquired, with the former being more common. Majority of the congenital PAVM's are associated with hereditary hemorrhagic telangiectasia (HHT)1. PAVM may be asymptomatic or present with a classic triad of dyspnea, cyanosis and clubbing. Hemoptysis can occur from intrabronchial rupture of PAVM or endobronchial telangiectasia. Pregnancy has been associated with an increased rate of PAVM growth and its associated complications2. A high index of suspicion is needed for diagnosis. Diagnosis is made by CTA, calculation of shunt fraction and contrast echocardiography. Treatment options include embolotherapy3 or lung resection if embolization fails. CONCLUSIONS: 1. PAVM are rare causes of hemoptysis, stroke and hypoxia 2. Identification is important due to association with Hereditary Hemorrhagic Telangiectasia and associated complications 3. PAVM tend to increase in size and be more symptomatic during pregnancy 4. Embolization of the feeding artery is highly effective in treatment. Reference #1: Khurshid I, Downie GH. Pulmonary arteriovenous malformation. Postgrad Med J. 2002;78(918):191-197. Reference #2: Esplin MS, Varner MW. Progression of pulmonary arteriovenous malformation during pregnancy: case report and review of the literature. Obstet Gynecol Surv. 1997;52(4):248-253. Reference #3: Lee DW, White RI, Jr., Egglin TK, et al. Embolotherapy of large pulmonary arteriovenous malformations: long-term results. Ann Thorac Surg. 1997;64(4):930-939; discussion 939-940. DISCLOSURE: The following authors have nothing to disclose: Rahul Mutneja, Aaron Glucksman, Tapas Bandyopadhyay No Product/Research Disclosure Information

Salvage pneumonectomy for pulmonary arteriovenous malformation in a 12-year-old boy with brain abscess and hemiparesis: A fatal outcome.

Large pulmonary arteriovenous malformations (PAVMs) constitute an uncommon cause of central cyanosis with septic embolism and brain abscess. This large right to left shunt can lead to chronic severe hypoxemia and significant morbidity and mortality if untreated. Conservative parenchyma-sparing lung resection was used widely as treatment of choice. However, with the advent of embolotheraphy, it is considered the preferred mode of treatment with less invasiveness. We here report a 12-year-old boy with large aneurysmal pulmonary arteriovenous fistula presented with brain abscess and hemiparesis. He underwent thoracotomy and pneumonectomy for large PAVMs, and it was complicated with bleeding and massive blood transfusion. The patient developed acute renal failure as a postoperative complication and succumbed to it. We suggest proper look out for systemic collateral and their management by embolitheraphy either alone or in combination should be tried first. We also suggest median sternotomy and intrapericardial approach for pneumonectomy in such difficult situation can be helpful.

A rare case of pulmonary arterio-venous malformation with recurrent anemia: Hereditary hemorrhagic telangiectasia.

Arteriovenous malformation (AVM) is a rare vascular anomaly of the lung, which manifests predominantly as dyspnea (due to right to left shunting) and paradoxical embolism. Hereditary Hemorrhagic Telangiectasia (HHT) being a rare genetic disorder is one of the most common causes of pulmonary arteriovenous malformation (PAVM). Here we report an interesting case of recurrent anemia in an elderly female, who was subsequently found to have multiple cutaneous and mucosal telangiectasias and a large pulmonary AVM.

Successful Treatment of a Large Pulmonary Arteriovenous Malformation by Repeated Coil Embolization.

Pulmonary arteriovenous malformations (AVMs) are caused by abnormal vascular communications between the pulmonary arteries and pulmonary veins, which lead to the blood bypassing the normal pulmonary capillary beds. Pulmonary AVMs result in right-to-left shunts, resulting in hypoxemia, cyanosis, and dyspnea. Clinical signs and symptoms vary depending on the size, number, and flow of the AVMs. Transcatheter embolization is the treatment of choice for pulmonary AVMs. However, this method can fail if the AVM is large or has multiple complex feeding arteries. Surgical resection is necessary in those kind of cases. Here, we report the case of a patient with a 6-cm pulmonary AVM with multiple feeding arteries that was successfully treated by repeated coil embolization without surgery.

Transcatheter closure of a pulmonary arteriovenous malformation in a patient with hereditary hemorrhagic telangiectasia

Pulmonary arteriovenous malformations (PAVM) are rare pulmonary vascular anomalies. Over 50% of the cases are associated with hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu Syndrome. Untreated PAVMs progressively enlarge and can cause significant right-to-left shunting. Surgical- and catheter-based approaches have been used in the management of PAVM. We report a case of a 74-year-old man who presented with dyspnea and hypoxia and was found to have a large right-sided PAVM. He underwent percutaneous closure of the PAVM with an Amplatzer device with significant improvement of his symptoms.

Large Pulmonary Arteriovenous Malformations: Long-Term Results of Embolization with AMPLATZER Vascular Plugs

PurposeTo analyze the long-term results of endovascular treatment of large pulmonary arteriovenous malformations (PAVMs) using the AMPLATZER vascular plug (AVP; AGA Medical Corp, Golden Valley, Minnesota). Materials and MethodsBetween May 2007 and April 2011, 18 patients with 24 large PAVMs, defined as PAVMs that had a feeding artery with a diameter of ≥ 8 mm, were treated with AVP I or AVP II. A single AVP device was used for each PAVM. Aneurysmal sac diameters, sac perfusion, sac shrinkage, and complete resolution before and after the intervention were analyzed. Complete histories, laboratory values, physical examinations, and multidetector computed tomography images were reviewed. The mean occlusion time for AVP I and AVP II and the mean arterial oxygen saturation (SaO2) before and after the intervention were compared. ResultsThe mean diameter of the feeding artery was 11.46 mm ± 2.18 (range, 8–13.3 mm). The mean occlusion time was 7.34 minutes ± 1.23 for AVP I and 6.25 minutes ± 1.12 for AVP II (P = .11). The mean SaO2 before and after the intervention was 63.71% ± 8.10% (range, 51%–76%) and 96.28% ± 0.49% (range, 96%–97%), respectively (P = .045). No major periprocedural complications were observed. The mean follow-up duration was 36.33 months ± 10.63 (range, 28–56 mo). During the follow-up period, there were no persistent sac perfusions, migration of the AVPs, major complications, or recanalizations. ConclusionsTreatment of large PAVMs with AVPs is an effective method for obtaining excellent long-term results. Embolization of large feeding arteries can be accomplished with AVPs without major complications.

Impact of Pulmonary Arteriovenous Malformations on Respiratory–Related Quality of Life in Patients with Hereditary Haemorrhagic Telangiectasia

Fifteen to fifty percent of patients with hereditary haemorrhagic telangiectasia have pulmonary arteriovenous malformations. The objective of this study was to measure the effect of the presence of pulmonary arteriovenous malformations and of their embolisation on respiratory-related quality of life (QoL). We prospectively recruited patients with a diagnosis of hereditary haemorrhagic telangiectasia based on the Curaçao criteria and/or the identification of a pathogenic mutation. Respiratory-related quality of life was measured using the Saint George’s Respiratory Questionnaire (SGRQ). Patients who underwent embolisation of pulmonary arteriovenous malformations completed the questionnaire before and 6–12 mo after the procedure. The 56 participants were divided into three groups: no pulmonary arteriovenous malformation (group A, n = 10), small pulmonary arteriovenous malformations not accessible to embolotherapy (group B, n = 19), and large pulmonary arteriovenous malformations accessible to embolotherapy (group C, n = 27). The SGRQ score was significantly higher in group C compared to the other groups, indicating a worse respiratory-specific QoL. There was no significant difference between groups A and B. Among the 17 patients who underwent an embolisation, the SGRQ score decreased significantly after the procedure, to a value similar to that in patients without pulmonary arteriovenous malformation. Our results indicate that the presence of large but not small pulmonary arteriovenous malformations negatively affects the respiratory-related quality of life and that embolisation of pulmonary arteriovenous malformations normalizes the respiratory-related quality of life.