Multiple Pulmonary Arteriovenous Malformations Research Articles

“Unveiling the Complexity: Coexistence of Rheumatic Heart Disease and Pulmonary Arteriovenous Malformation - A Unique Case Report”

Abstract Background The coexistence of Rheumatic Heart Disease (RHD) and Pulmonary Arteriovenous Malformation (PAVM) is a rare clinical scenario that poses diagnostic and therapeutic challenges. This case report explores the clinical presentation, diagnostic journey, and multidisciplinary management of a patient presenting with both conditions. Case Summary A 47-year-old female with a history of RHD presented with symptoms of dyspnoea on exertion and cyanosis, suggestive of both cardiac and pulmonary involvement. Subsequent investigations involving imaging, echocardiography, and invasive pulmonary angiography, revealed the co-existence of RHD and multiple PAVM in patient’s left lower lobe of lung. The patient underwent a tailored treatment plan, initially involving percutaneous mitral balloon valvuloplasty for RHD, followed by a staged procedure of transcatheter PAVM closure with Amplatzer™ Vascular Plug II performed one month later. Her saturation normalized following intervention. The patient's progress was monitored closely, with adjustments made to the treatment plan based on evolving clinical scenarios. Patient remained well in short term follow up. Discussion This case highlights the complexity of managing patients having two diverse conditions RHD and PAVM coexisting together, thus emphasizing the importance of a multidisciplinary approach. The unique intersection of cardiac and pulmonary pathologies necessitates careful consideration of diagnostic nuances and tailored treatment strategies. Lessons learned from this case offer valuable insights for clinicians encountering similar scenarios and underscore the significance of individualized, patient-centred care in optimizing outcomes for those with dual pathologies.

Pulmonary AVM Embolization

Pulmonary arteriovenous malformations (PAVMs) are rare fistulous connections between pulmonary arteries and veins that, as in our case, are commonly associated with hereditary hemorrhagic telangiectasia (HHT). Embolotherapy, the mainstay of treatment for PAVMs, is a procedure in which the feeding arteries of a malformation are endovascularly occluded under fluoroscopic guidance. Effective and well-tolerated, embolotherapy has been shown to decrease right-to-left shunting following treatment and decrease risks of paradoxical embolization and lung hemorrhage and to improve pulmonary gas exchange and lung function. Patients are selected for treatment according to clinical suspicion for the presence of a PAVM and feeding artery diameter. The occlusion of PAVMs with arteries that exceed 2-3 mm in diameter is recommended. Diagnostic contrast-enhanced pulmonary angiography is performed via injection of contrast through a percutaneous catheter to characterize and confirm PAVMs suitable for embolization. Lesions are then treated by catheter-directed placement of embolic material— vascular plugs in our case—into the feeding artery, terminating blood flow to the area of the lesion. Although multiple PAVMs may be embolized during a single session, in patients with HHT, who may present with large numbers of PAVMs, treatment is limited by maximum contrast dosage, and additional sessions may be performed if PAVMs remain perfused.

Use of Polyurethane-Covered Stents for Exclusion of Pulmonary Arteriovenous Malformations.

To evaluate the safety, technical success and short-term effectiveness of polyurethane-covered stent (PK Papyrus, BioTronik, Berlin) in the treatment of pulmonary arteriovenous malformations (PAVMs) that are not amenable to embolotherapy. In this IRB-approved, retrospective study, data from patients who received polyurethane-covered stents for exclusion of PAVMs were analyzed. The study included 5 patients (all women) with a median age of 40years (range 25-60). Patients presented with hypoxemia, TIAs, and/or epistaxis; 4 were confirmed to have HHT. All had multiple PAVMs diagnosed on chest CT and underwent embolization with other devices in addition to the polyurethane-covered stent. The indication for stent placement in all cases was a short and/or tortuous feeding artery. Safety was assessed by immediate or short-term complications, e.g., migration, stent thrombosis, and fracture. Technical success was defined as the ability to accurately place the stent at the intended location. Effectiveness was defined as successful exclusion of PAVM with no perfusion across the AVM. Technical success of stent placement was 100%. AVM exclusion rate was 80% after single stent deployment; in the case of incomplete exclusion, success was achieved using an overlapping stent to completely cover a second feeding artery. During the median follow-up period of 5months (range 2-10), all stents remained patent, and AVMs were excluded without other complications. Exclusion of PAVMs with polyurethane-covered stents is technically feasible, safe, and shows short-term effectiveness for PAVMs with a short/tortuous feeding artery when traditional embolization techniques are not possible.

Positive Bubble Study But No Evidence of Interatrial Defect in a Patient with Recurrent Cryptogenic Stroke.

Pulmonary arteriovenous malformations (PAVMs) can be asymptomatic or result in a range of complications such as brain abscesses or cryptogenic emboli, which can contribute to morbidity and mortality if not diagnosed and treated in a timely manner. To date, there have been several reports of delayed diagnosis of PAVMs, which have been largely attributed to the misconception that PAVMs are too rare to be of clinical significance. Furthermore, because intracardiac shunting secondary to a patent foramen ovale (PFO) or atrial septal defect (ASD) also results in a positive saline contrast study with echocardiography, PAVM can be easily misdiagnosed as an intracardiac right-toleft shunt. However, there are unique echocardiographic features that differentiate between intracardiac shunting due to a PFO or ASD and extracardiac shunting such as in PAVM. This case details the course of a patient with recurrent cryptogenic strokes that was initially misattributed to a PFO and was only correctly diagnosed with multiple PAVMs after two failed attempts at PFO closure. This case serves as a reminder of an alternative etiology of right-to-left shunt and its presentation on imaging, which echocardiographers must be familiar with.

Multiple Pulmonary Arteriovenous Malformations in a Young Patient Presenting with Stroke

Multiple Pulmonary Arteriovenous Malformations in a Young Patient Presenting with Stroke

Anesthetic management of a patient with Osler-Weber-Rendu syndrome with multiple pulmonary arteriovenous malformations and pheochromocytoma for femoral artificial bone replacement: a case report

BackgroundOsler-Weber-Rendu syndrome is characterized by mucocutaneous telangiectasia and arteriovenous malformations in organs. Anesthesia for patients with Osler-Weber-Rendu syndrome is challenging due to complications and physiological changes.Case presentationThe case was a 49-year-old female with Osler-Weber-Rendu syndrome, multiple pulmonary arteriovenous malformations and pheochromocytoma who presented for femoral bone head fracture with metastatic adenocarcinoma. The patient was scheduled to undergo bone tumor resection and artificial bone replacement, being positioned laterally with a planned operation duration of 5 h. Anesthesia was managed with spinal and epidural anesthesia, combined with sedation by sevoflurane using a supraglottic airway (SGA) device under spontaneous breathing. Her intraoperative and postoperative courses were uneventful.ConclusionNeuraxial anesthesia combined with general anesthesia using an SGA device to maintain spontaneous ventilation in order to minimize the risk of rupture of pulmonary arteriovenous malformations could be an option.

“Rendezvous with Osler Weber Rendu” - Closure of Multiple Pulmonary Arteriovenous Malformations with Amplatzer Vascular Plug II in Hereditary Hemorrhagic Telangiectasia

“Rendezvous with Osler Weber Rendu” - Closure of Multiple Pulmonary Arteriovenous Malformations with Amplatzer Vascular Plug II in Hereditary Hemorrhagic Telangiectasia

Feasibility of Single-Shot Whole Thoracic Time-Resolved MR Angiography to Evaluate Patients with Multiple Pulmonary Arteriovenous Malformations.

ObjectiveTo evaluate the feasibility of single-shot whole thoracic time-resolved MR angiography (TR-MRA) to identify the feeding arteries of pulmonary arteriovenous malformations (PAVMs) and reperfusion of the lesion after embolization in patients with multiple PAVMs.Materials and MethodsNine patients (8 females and 1 male; age range, 23–65 years) with a total of 62 PAVMs who underwent percutaneous embolization for multiple PAVMs and were subsequently followed up using TR-MRA and CT obtained within 6 months from each other were retrospectively reviewed. All imaging analyses were performed by two independent readers blinded to clinical information. The visibility of the feeding arteries on maximum intensity projection (MIP) reconstruction and multiplanar reconstruction (MPR) TR-MRA images was evaluated by comparing them to CT as a reference. The accuracy of TR-MRA for diagnosing reperfusion of the PAVM after embolization was assessed in a subgroup with angiographic confirmation. The reliability between the readers in interpreting the TR-MRA results was analyzed using kappa (κ) statistics.ResultsFeeding arteries were visible on the original MIP images of TR-MRA in 82.3% (51/62) and 85.5% (53/62) of readers 1 and 2, respectively. Using the MPR, the rates increased to 93.5% (58/62) and 95.2% (59/62), respectively (κ = 0.760 and 0.792, respectively). Factors for invisibility were the course of feeding arteries in the anteroposterior plane, proximity to large enhancing vessels, adjacency to the chest wall, pulsation of the heart, and small feeding arteries. Thirty-seven PAVMs in five patients had angiographic confirmation of reperfusion status after embolization (32 occlusions and 5 reperfusions). TR-MRA showed 100% (5/5) sensitivity and 100% (32/32, including three cases in which the feeding arteries were not visible on TR-MRA) specificity for both readers.ConclusionSingle-shot whole thoracic TR-MRA with MPR showed good visibility of the feeding arteries of PAVMs and high accuracy in diagnosing reperfusion after embolization. Single-shot whole thoracic TR-MRA may be a feasible method for the follow-up of patients with multiple PAVMs.

Successful treatment of bilateral multiple pulmonary arteriovenous malformations in a patient with brain abscess and severe hypoxemia using a combination of transcatheter embolotherapy and surgical resection: a case report

BackgroundA pulmonary arteriovenous malformation is an abnormal dilated blood vessel that makes direct communication between a pulmonary artery and pulmonary vein and can be associated with hypoxemia or neurological complications, including brain abscess and cerebral infarction. Treatment of pulmonary arteriovenous malformation includes surgical resection and transcatheter embolotherapy, however the adaptation of therapies should be considered when a patient is in bad condition.Case presentationA 51-year-old man was admitted after developing fever, consciousness disorder, and hypoxemia. Magnetic resonance imaging of the brain showed a brain abscess. Bilateral pulmonary arteriovenous malformations were found by contrast computed tomography. Because of a family history of pulmonary arteriovenous malformation, a history of epistaxis, and the existence of oral mucosa telangiectasia, he was diagnosed with hereditary hemorrhagic telangiectasia and brain abscess caused by intrapulmonary right-to-left shunt. The brain abscess improved with antibiotic treatment; however, the administration of oxygen did not ameliorate his hypoxemia. His hypoxemia was exacerbated by positive pressure ventilation. Considering his systemic and respiratory condition, we considered surgery to involve a high degree of risk. After controlling his brain abscess and pneumonia, transcatheter embolotherapy was performed. This improved his systemic condition, enabling surgical treatment.ConclusionsThis middle-aged patient suffering from brain abscess and severe hypoxemia with multiple pulmonary arteriovenous malformations was successfully treated by a combination of transcatheter embolotherapy and surgery. The adaptation and combination of therapies, as well as the sequence of treatments, should be considered depending on the patient status and lesions.

A Rare Case of Pulmonary Arteriovenous Malformation - A Diagnostic Dilemma

Pulmonary arteriovenous malformations (PAVM) are rare anomalies of pulmonary vascular system which may be incidentally detected or can have manifestations resulting from right to left shunt. PAVM can cause various systemic implications including central nervous system complications like stroke, brain abscess due to paradoxical emboli which mostly seen in previously undiagnosed PAVMs indicating importance of early diagnosis and timely intervention in PAVM. Computed Tomography Pulmonary Angiography (CTPA) is the diagnostic method of choice in PAVM. We present here a unique case of complex multiple PAVM presented with refractory hypoxemia in a middle aged patient diagnosed in our centre initially evaluated for malignancy based on chest radiography & High Resolution Computed tomography(HRCT)thorax findings and symptoms, later on after CTPA was diagnosed to have complex PAVM which showed clinicoradiological improvement following pulmonary vascular plugging. This case shows clinical scenario, diagnostic & management methods and differential diagnosis to be discussed in similar clinicoradiological pattern and peculiarity of PAVM despite advanced age.

Pulmonary arteriovenous malformation (PAVM) embolization: prediction of angiographically-confirmed recanalization according to PAVM Diameter changes on CT

BackgroundTo assess pulmonary arteriovenous malformation (PAVM) recanalization after embolization based on PAVM diameter changes on computed tomography (CT), with pulmonary angiography used as a gold standard.MethodsA retrospective review was done of patients from 2008 to 2019 with a PAVM treated with endovascular embolization. The treatment outcome was determined by conventional angiography. Follow-up pulmonary angiography was performed when recanalization was suspected on CT, or embolization of all lesions in multiple PAVM patients could not be completed in a single session. Patients who had no preprocedural or follow-up CT were excluded. Draining vein, feeding artery, and venous sac diameter were measured on CT, and diameter reduction rates were compared with the widely-used, binary 70 % criteria.ResultsForty-one patients with 114 PAVMs were treated during the study period. Eight patients with 50 PAVMs met the inclusion criteria. Mean vein, artery, and venous sac diameter reduction rates were as follows: 59.2 ± 9.3 %, 47.5 ± 10.6 %, and 62.6 ± 13.2 %, respectively, in the occluded group and 5.4 ± 19.5 %, 11.3 ± 17.7 %, and 26.8 ± 14.2 %, respectively, in the recanalized group. The area under the receiver operating characteristic curves for PAVM recanalization for the draining vein was 1.00, showing a better result than the artery (0.97) and sac (0.99). Patients showed > 42 % draining vein diameter reduction in the occluded group and < 32 % in the recanalized group. The widely-used 70 % criteria showed low specificity for predicting recanalization (draining vein, 7.3 %; venous sac, 41.7 %) but 100 % sensitivity for both the draining vein and venous sac.ConclusionsThe widely-used 70 % binary criteria showed limited performance in predicting outcomes in this angiographically-confirmed case series. Further investigations are warranted to establish a strategy for detecting recanalization after PAVM embolization.

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

Pulmonary arteriovenous malformations: a radiological and clinical investigation of 136 patients with long-term follow-up

To assess the clinical outcome of patients with and without hereditary haemorrhagic telangiectasia (HHT) after embolisation of pulmonary arteriovenous malformations (PAVM) from a single national centre. The present register-based observational study including all patients with PAVM treated with embolisation at a reference centre for HHT and PAVM was undertaken over a 20-year period. Demographic data, HHT genotyping, clinical presentation, and outcome were registered. Patients with HHT were compared to the patients without HHT. Clinical examination, contrast-enhanced echocardiography, and computed tomography (CT) were used to assess the clinical outcome at follow-up. One hundred and thirty-six patients with 339 PAVM underwent embolisation during the study period: 22 did not have HHT; 62% had HHT1, 10% had HHT2, 4% had JP-HHT, 8% had clinical HHT without identified genetic mutations. Solitary PAVM were more common among patients without HHT than with HHT. Mean follow-up after the first embolisation was 58 months. Mean age at first embolisation was 46.5 years, and at last follow-up 51.8 years. The clinical success without shunt at follow-up was 87%. The 30-day mortality related to the embolisation was 0%. Twenty patients died during follow-up (mean age 69 years). Most patients could be treated during one session, but many will need a long follow-up with repeated clinical examinations and embolisation. The majority of patients referred for embolisation of PAVM had HHT. Multiple PAVM is associated with HHT. Patients with PAVM should be screened for HHT and patients with HHT for PAVM. Embolisation is a safe procedure with high clinical success.

Pulmonary arteriovenous malformation presenting as spontaneous haemothorax on transthoracic ultrasound

A 45-year-old man with no known chronic medical illness presented with insidious onset of exertional dyspnoea and left pleuritic chest pain. He was pale on physical examination and left chest...

Surgical Treatment of Idiopathic Multiple Pulmonary Arteriovenous Malformation Identified at the Onset of Cerebral Infarction: A Case Report

Pulmonary arteriovenous malformation (PAVM), which is asymptomatic in most cases, is often identified in patients with central nervous system disorders such as brain abscesses and/or cerebral infarctions. We have reported a patient with idiopathic multiple PAVM identified at the onset of cerebral infarction. A 69-year-old woman visited the Department of Neuropathic Internal Medicine at our hospital with chief complaints of numbness in her left hand and a feeling of weakness. The patient was given a diagnosis of subacute cerebral infarction. Multiple old lacunar infarctions were also observed in the deep white matter of the left frontal lobe. Chest computed tomography showed multiple nodular structures, mainly in the right lower lung field (S8), as well as continuous arteries and veins at the site; thus, the patient was finally diagnosed with multiple PAVM. Right lower thoracoscopic lobectomy was performed, as is typical surgical practice in such cases. The patient had a favorable postoperative course, and had no recurrence of cerebral infarction. Although the patient’s lesions were mainly restricted to S8, the fact that there were multiple lesions deemed a lobectomy as the appropriate course of treatment. This case emphasizes that attention should be paid to cases of multiple PAVM since cerebral infarction may arise from the disease.

低酸素血症を呈した遺伝性毛細血管拡張症に伴う多発性肺動静脈瘻に対して病変選択的に経カテーテル的コイル塞栓術を施行した1例

低酸素血症と家族歴より遺伝性出血性毛細血管拡張症（HHT）に合併した多発性の肺動静脈瘻（PAVM）と診断された．流入動脈径3 mm未満であったが，低酸素血症を認める多発性病変のため症状改善を目的とし右左短絡量の多い順にコイル塞栓術を施行した症例を経験した．通常流入動脈径3 mm以上の場合は重篤な中枢神経合併症のリスクが高いことから，積極的治療介入を要す．最近ではデバイスの進歩もあり，3 mm未満の病変に対する治療適応の幅も広がってきていることから症例毎の検討を要す．HHTは常優染色体遺伝する疾患であり，家族歴を有する症例においてはスクリーニング検査を施行することでPAVMの早期診断に至りうる可能性がある．特にHHT type 1は多発性PAVMの合併率が高く，慎重なフォロー，適切な治療介入による症状改善，合併症予防が大切である．

Hypoxaemia, sport and polycythaemia: a case from Imperial College London

WY : An 18-year-old footballer with no previous significant medical history sustained a tendon injury during a football match. This was repaired successfully under general anaesthetic, but the SaO2 (%...

Roles of cyclooxygenase 2 and hepatic venous flow in patients with HHT or hepatopulmonary syndrome

BackgroundHereditary hemorrhagic telangiectasia (HHT) and hepatopulmonary syndrome are disorders characterized by the development of multiple pulmonary arteriovenous malformations (PAVM). Presentation of the hypothesisCOX2 may be at the origin of a cascade of pro inflammatory events to favour angiogenesis and PAVM development. Testing the hypothesisHHT and hepatopulmonary syndrome mouse models may be used to show its effects on PAVM formation. Anti COX-2 therapy could also be tested in human individuals, particularly in patients presenting a hepatopulmonary syndrome or HHT with small PAVM. Implication of the hypothesisPAVMs are one of the main causes of morbidity in patients presenting with HHT disease, owing to the risks of rupture as well as paradoxical embolism exposing to stroke and/or cerebral abscess. Percutaneous embolization has become the treatment of choice of PAVM. Anti COX2 may prevent from PAVM development and subsequent related complications and avoid either surgery and/or percutaneous embolization and thus subsequent related complication.

La maladie de Rendu-Osler-Weber : à propos d’une nouvelle observation pédiatrique

Hereditary hemorrhagic telangiectasia, or Rendu-Osler-Weber syndrome, is an autosomal dominant multiorgan disorder. This multisystemic vascular dysplasia is determined by a mutation of one of two main genes, endoglin (ENG) or HHT1, or ACVRL1 or HHT2. These mutations induce vascular disorders that cause recurrent epistaxis and eventually multiple telangiectasia and arteriovenous visceral malformations. We report the case of a 7-year-old girl who developed severe hypoxemia due to multiple pulmonary arteriovenous malformations.

Multiple embolizations of pulmonary arteriovenous malformations during pregnancy

Abstract Pulmonary arteriovenous malformations in a pregnant patient are rare and can cause deleterious, life-threatening complications. We report a patient with multiple pulmonary arteriovenous malformations, with the subsequent diagnosis of hereditary hemorrhagic telangiectasia, requiring multiple embolizations during pregnancy. Pulmonary arteriovenous malformations can carry a high risk of morbidity in the pregnant woman; however, they can be safely treated in pregnancy.