Case Of Pulmonary Arteriovenous Malformation Research Articles

A Rare Case of Pulmonary Arteriovenous Malformation Caused by Hereditary Hemorrhagic Telangiectasia in a Hemodialysis Patient

Hereditary hemorrhagic telangiectasia (HHT) is an uncommon autosomal dominant disorder resulting in vascular malformation, such as pulmonary arteriovenous malformation (PAVM). Here, we report a rare case of pulmonary arteriovenous malformation caused by HHT in a hemodialysis (HD) patient. A 34-year-old man receiving maintenance HD via radiocephalic arteriovenous fistula developed progressive dyspnea without definite pulmonary edema. His mother had been diagnosed with HHT. He had experienced multiple episodes of epistaxis and had been intermittently treated with blood transfusions because of severe anemia. Blood gas analysis showed hypoxia. Chest computed tomography revealed multiple dilated vessels of variable sizes, continuous with the pulmonary artery throughout both lung fields, consistent with PAVM. After treating pulmonary artery embolization at the largest PAVM, he recovered from his dyspnea symptoms and hypoxia.

A Case of Pulmonary Arteriovenous Malformation with Hereditary Hemorrhagic Telangiectasia (HHT) Diagnosed by the Chance of Medical Examination

A Case of Pulmonary Arteriovenous Malformation with Hereditary Hemorrhagic Telangiectasia (HHT) Diagnosed by the Chance of Medical Examination

A Case of Pulmonary Arteriovenous Malformation Presented with Paradoxical Brain Embolism

A Case of Pulmonary Arteriovenous Malformation Presented with Paradoxical Brain Embolism

A case of pulmonary arteriovenous malformation: role of interventional radiology in diagnosis and treatment

Pulmonary arterio-venous malformations (PAVMs) are abnormal pulmonary arteries and pulmonary veins communicating directly without interposition of a capillary bed and about 80-90% of patients with PAVMs eventually may present with hereditary hemorrhagic telangiectasia (HHT), remaining ones are sporadic cases. On the other hand, about 15-35% of HHT patients may present with PAVMs. The PAVMs have a tendency to grow and increase in size over time and various factors like puberty, pregnancy and pulmonary arterial hypertension (PAH) affect growth. This condition needs early diagnosis, aggressive management and vigilant follow up. Our article aims to review pulmonary AVMs as a rare cause of strokes in young patients. We will discuss the clinical presentation, diagnosis, complications, the therapeutic options and the follow up.

Pulmonary arteriovenous malformation: A rare cause of hemoptysis

Pulmonary arteriovenous malformation (PAVM) is a rare cause of hemoptysis and exertional dyspnea. Hereditary hemorrhagic telangiectasia is the most common cause of PAVM, but it can also occur in a variety of acquired medical conditions such as postthoracic trauma, cirrhosis of the liver, and conditions causing chronic pulmonary hypertension. We present a case of PAVM with classical cherry aneurysm of right hilar vessels on routine chest X-ray, which led to further evaluation and successful treatment of PAVM by the transseptal closure of the fistula.

An Incidental Finding of Severe Hypoxia in an Asymptomatic Patient Caused by Pulmonary Arteriovenous Malformations

SESSION TITLE: Pulmonary Vascular Disease Cases I SESSION TYPE: Affiliate Case Report Slide PRESENTED ON: Monday, October 26, 2015 at 11:00 AM - 12:00 PM INTRODUCTION: Pulmonary arteriovenous malformations (AVMs) are a rare clinical entity, especially when found in isolation with no underlying etiology. Literature review suggest that pulmonary AVMs are symptomatic in 91% of cases, with dyspnea in 82% and cyanosis in 79% of patients. Furthermore, 70% are also associated with Osler Weber Rendu Syndrome. We report a case of severe asymptomatic hypoxia, discovered incidentally on routine pre-operative evaluation, found to have isolated multiple large pulmonary AVMs. Workup failed to show any underlying etiology. CASE PRESENTATION: The patient is a 54 year old female, with a history of COPD and severe aortic valve insufficiency, diagnosed via an echocardiogram at another facility, referred to cardiovascular surgery at our hospital. During pre-surgical evaluation, she was found to be hypoxic, with oxygen saturation in the 80%s on 2L of oxygen by nasal cannula and was admitted. Upon admission, her oxygen saturation was at 85% on a non re-breather mask. She denied any symptoms. Physical examination was unremarkable. Laboratory values showed hemoglobin of 17.3g/L and hematocrit of 48.9%. Chest X-Ray revealed a mass like density in the right lower lobe (RLL). CT scan of the thorax with contrast was negative for pulmonary embolus but 4 AVMs in RLL and 2 in right upper lobe were identified. Further questioning and workup was negative for Osler-Weber-Rendu syndrome or other underlying etiology. Resting echo showed an EF of 70% and a Bubble Echocardiogram revealed extensive right to left shunting. Bubbles entered from the pulmonary venous drainage into the left atrium consistent with AVMs. She had a pulmonary angiogram and AVMs of the RLL were embolizedx2 and an amplatzer vascular occlusion plug was deployedx2. After embolization, the patient's oxygen saturation was at 95% on 6L of oxygen. A Transesophageal Echo showed moderate aortic insufficiency and she decided to forego cardiac surgery. DISCUSSION: Studies show most cases of pulmonary AVMs have an underlying etiology and are symptomatic in 91% of patients. Our case represents a patient with large asymptomatic pulmonary AVMs with no underlying etiology. CONCLUSIONS: It shows that in patients presenting with hypoxia with no clear underlying cause, pulmonary AVMs can be considered as a cause and that pulmonary angiogram and embolization of AVMS is the cornerstone of treatment of pulmonary AVMs. Reference #1: Faughnan ME, LUI YW, Wirth JA, et al. Diffuse Pulmonary arteriovenous malformations: characteristics and prognosis. Chest 2000; 117:31 DISCLOSURE: The following authors have nothing to disclose: Asad Omar No Product/Research Disclosure Information

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.

Embolizaço percutânea de fístulas arteriovenosas pulmonares em paciente com telangiectasia hemorrágica hereditária

A maioria dos casos de malformações arteriovenosas pulmonares ocorre em pacientes portadores de telangiectasia hemorrágica hereditária. É uma doença incomum, que pode se apresentar com acidentes vasculares encefálicos em pacientes jovens. Com o surgimento de dispositivos de oclusão, a embolização percutânea tornou-se o tratamento de escolha. Relatamos o caso de um paciente de 33 anos de idade, com história de múltiplos acidentes vasculares encefálicos, cianose, dispneia, epistaxes recorrentes e presença de fístulas pulmonares de alto fluxo, submetido a embolização por via percutânea. O paciente apresentou melhora clínica, encontrando-se em acompanhamento há 30 meses, sem sintomas recorrentes.

Pulmonary arteriovenous malformations presenting as difficult-to-control asthma: a case report

IntroductionAlthough pulmonary arteriovenous malformations are relatively rare disorders, they are an important part of the differential diagnosis of common pulmonary problems, such as hypoxemia, dyspnea on exertion and pulmonary nodules.Case presentationAn 11-year-old Croatian boy of Mediterranean origin with a history of asthma since childhood was admitted to our hospital for evaluation of difficult-to-control asthma during the previous six months. A chest X-ray showed a homogeneous soft tissue mass in the lingual area. Computed tomography angiography of the thorax showed two pulmonary arteriovenous malformations, one on each side of the lungs. Diagnosis of hereditary hemorrhagic telangiectasia was made clinically by Curaçao criteria. Genetic analysis revealed a mutation in the endoglin gene. The patient was treated with embolotherapy with good clinical outcome.ConclusionWe present a case of pulmonary arteriovenous malformations masquerading as refractory asthma.

Percutaneous Embolisation of Pulmonary Arteriovenous Fistulae in Patients with Hereditary Haemorrhagic Telangiectasia

Most cases of pulmonary arteriovenous malformations occur in patients with hereditary hemorrhagic telangiectasia. It is a rare disease that may present with stroke-like symptoms in young patients. With the development of occlusion devices, percutaneous embolization has become the treatment of choice. We report a case of a 33 year-old man with a history of multiple strokes, cyanosis, dyspnea, recurrent epistaxis and high flow pulmonary fistulae who was submitted to percutaneous embolization. The patient improved clinically and has had no recurring symptoms in the 30-month follow-up. Embolização Percutânea de Fístulas Arteriovenosas Pulmonares em Paciente com Telangiectasia Hemorrágica Hereditária A maioria dos casos de malformações arteriovenosas pulmonares ocorre em pacientes portadores de telangiectasia hemorrágica hereditária. É uma doença incomum, que pode se apresentar com acidentes vasculares encefálicos em pacientes jovens. Com o surgimento de dispositivos de oclusão, a embolização percutânea tornouse o tratamento de escolha. Relatamos o caso de um paciente de 33 anos de idade, com história de múltiplos acidentes vasculares encefálicos, cianose, dispneia, epistaxes recorrentes e presença de fístulas pulmonares de alto fluxo, submetido a embolização por via percutânea. O paciente apresentou melhora clínica, encontrandose em acompanhamento há 30 meses, sem sintomas recorrentes.

Right ventricular enlargement in a patient with hereditary haemorrhagic telangiectasia: a rare case of pulmonary arteriovenous malformations with concomitant atrial septal defect

We describe a rare case (32-year-old woman) with hereditary haemorrhagic telangiectasia and concomitant atrial septal defect (ASD). Transthoracic saline contrast echocardiography (TTCE) revealed several microbubbles appearing early (third cardiac cycle) and significant microbubbles appearing later (seventh cardiac cycle) in the left atrium and ventricle (LA/LV) after opacification of the right atrium in the four-chamber view …

肺炎，膿胸を合併し呼吸不全となった肺動静脈瘻に対し，肺部分切除を行った遺伝性出血性末梢血管拡張症の1例

肺炎，膿胸を合併し呼吸不全となった肺動静脈瘻に対し，肺部分切除を行った遺伝性出血性末梢血管拡張症の1例

Congenital pulmonary arteriovenous malformation: A rare cause of cyanosis in childhood

Pulmonary arteriovenous malformations (PAVMs) are caused by abnormal communications between pulmonary arteries and pulmonary veins, which are most commonly congenital in nature. Although these lesions are uncommon, they are an important part of the differential diagnosis of common pulmonary problems such as hypoxaemia, pulmonary nodules and cyanosis. PAVM is a rare disorder with an incidence of 2-3 per 1,000,000 population. It occurs twice as often in women as in men, but there is a male predominance in newborns. Around 10% cases of PAVM are identified in infancy or childhood, followed by a gradual increase in the incidence through the fifth and sixth decades. Approximately 70% of the cases of PAVM are associated with hereditary haemorrhagic telangiectasia (HHT). Conversely, about 15 to 35% of patients with HHT have PAVM. PAVMs may cause hypoxaemia and dyspnoea due to right to left shunting, but frequently remain undiagnosed. This intrapulmonary malformation is described in two patients who presented with severe cyanosis. Index words: pulmonary arteriovenous malformation; cyanosis; right to left shunting DOI: 10.4038/cmj.v55i1.1707 Ceylon Medical Journal Vol.55, No.1 March 2010 pp.23-25

Formation tumorale framboisée à l’endoscopie bronchique simulant une tumeur carcinoïde

The authors report a case of pulmonary arteriovenous malformation in a 48-year-old man. The bronchoscopy revealed a fragile lingular tumoral formation. The diagnosis was based on the thoracotomy and the histopathological findings. The endoscopic features and the different diagnostic options of pulmonary arteriovenous malformation are discussed.

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.

Quantitative Shunt Imaging in the Evaluation of Therapeutic Surgery in a Patient With Pulmonary Arteriovenous Malformation

A case of pulmonary arteriovenous malformation (PAVM) in a 32-year-old woman who underwent successful left upper lobectomy is presented. A whole-body technetium-99m-labeled macro-aggregated albumin scan was used to demonstrate intrapulmonary right-to-left shunt, and shunt fraction was calculated as 39%. The patient underwent a follow-up scan after the operation; shunt fraction was calculated to be within normal limits (5%). The whole-body technetium-99m-labeled macro-aggregated albumin scan seems to be a simple and noninvasive test in the demonstration and quantitation of the degree of the right-to-left shunt in pulmonary arteriovenous malformation. The whole-body technetium-99m-labeled macro-aggregated albumin scan may also be useful for evaluation of the hemodynamic changes after surgical treatment and follow-up of the patients with pulmonary arteriovenous malformation.

Video-assisted thoracoscopic surgery for pulmonary arteriovenous malformations: report of five cases

We experienced five cases of pulmonary arteriovenous malformations (PAVMs) that were successfully treated by video-assisted thoracoscopic surgery. Four malformations were treated by local wedge resection and one was treated by segmentectomy. Criteria for patient selection for surgery were peripheral and solitary lesions, with feeding arteries larger than 3 mm. Postoperative hospital stays were 1-7 days (median, 2 days). All patients showed unchanged or increased values of PaO(2) in arterial blood after operation. No major postoperative complication occurred in any patient, but a persistent air leak for 5 days occurred in the one patient who was treated by segmentectomy. No growth of accessory vessels or untreated malformations were seen in any patient throughout the follow-up period of 14-54 months. Thoracoscopic surgical resection for well-selected patients provides a high certainty of eliminating fistulae and was associated with lower morbidity, lower mortality, and shorter hospital stays.

Video-assisted simultaneously stapled segmentectomy for pulmonary arteriovenous malformation located in the pulmonary hilum

Pulmonary arteriovenous malformation (PAVM) consists of abnormal communications between the pulmonary arteries and veins. Because PAVM may cause neurological defects (such as stroke and brain abscess) or hemoptysis and hemothorax, embolization therapy or surgery is required. Resection using video-assisted thoracic surgery (VATS) has recently been performed for small peripheral PAVMs. For large or hilar PAVM, surgical resection with thoracotomy is required. We report herein a case of PAVM (4.0 x 3.5cm) located in the left S6 segment near the lung hilum that was resected successfully using simultaneously stapled S6 segmentectomy (simultaneous stapling of anomalous vessels of the PAVM and hillar structures of S6) with VATS.

Thoracoscopic Surgery for Pulmonary Arteriovenous Malformation

We report 6 consecutive cases of pulmonary arteriovenous malformation in the periphery of the lung, which were treated successfully by thoracoscopy. Pulmonary wedge resection was performed with a linear cutting stapler to divide the feeding artery, drainage vein, and aneurysmal lesion of the arteriovenous malformation. Operative time ranged from 30 to 95 min (mean, 50 min). Bleeding was minimal in all cases. No major surgery-related complications were observed. Postoperative hospital stay was limited to one week except for one patient who had experienced a brain infarction preoperatively, caused by migration of an embolization coil. Histological examination confirmed that the arteriovenous malformation foci were completely removed. Pulmonary wedge resection through thoracoscopy is feasible as an alternative treatment for arteriovenous malformation located in the periphery of the lung parenchyma when pulmonary embolization therapy is difficult or unsuccessful.

Pulmonary arteriovenous malformation in a patient with tuberculosis — an association?

We report a case of pulmonary arteriovenous malformation (PAVM) and associated pulmonary tuberculosis in a young girl who presented with hemoptysis. As intra-arterial coil embolization did not result in improvement of symptoms, surgical resection of the PAVM was performed, resulting in dramatic clinical improvement. Histopathology revealed a large PAVM, and in addition, caseous necrotizing granulomatous inflammation, suggestive of pulmonary tuberculosis. This case is being reported since the association of large PAVM and tuberculosis is very rare, and both could present with hemoptysis.