Pulmonary Arteriovenous Fistula Research Articles

Omitted Pulmonary Arteriovenous Fistula Complicated With Brain Abscess

A 52-year-old female, diagnosed with a bilateral lower lobe pulmonary arteriovenous fistula (PAVF) at the age of 14 due to dyspnea, underwent a lobectomy of the right lower lung with a severe lesion, and symptom relief without treatment of the minor lesion in the other lung. She had active chest tightness since 42 years old and headaches 10 days before hospitalized, was diagnosed with PAVF and brain abscess, and cured by a successful PAVF embolization and a surgical resection of brain abscess. In the same patient, both methods of treatment for PAVF were all satisfactory, and each has advantages and indications. J Med Cases. 2017;8(5):149-151 doi: https://doi.org/10.14740/jmc2798w

Gestational Trophoblastic Disease: Clinical and Imaging Features.

Gestational trophoblastic disease (GTD) is a spectrum of both benign and malignant gestational tumors, including hydatidiform mole (complete and partial), invasive mole, choriocarcinoma, placental site trophoblastic tumor, and epithelioid trophoblastic tumor. The latter four entities are referred to as gestational trophoblastic neoplasia (GTN). These conditions are aggressive with a propensity to widely metastasize. GTN can result in significant morbidity and mortality if left untreated. Early diagnosis of GTD is essential for prompt and successful management while preserving fertility. Initial diagnosis of GTD is based on a multifactorial approach consisting of clinical features, serial quantitative human chorionic gonadotropin (β-hCG) titers, and imaging findings. Ultrasonography (US) is the modality of choice for initial diagnosis of complete hydatidiform mole and can provide an invaluable means of local surveillance after treatment. The performance of US in diagnosing all molar pregnancies is surprisingly poor, predominantly due to the difficulty in differentiating partial hydatidiform mole from nonmolar abortion and retained products of conception. While GTN after a molar pregnancy is usually diagnosed with serial β-hCG titers, imaging plays an important role in evaluation of local extent of disease and systemic surveillance. Imaging also plays a crucial role in detection and management of complications, such as uterine and pulmonary arteriovenous fistulas. Familiarity with the pathogenesis, classification, imaging features, and treatment of these tumors can aid in radiologic diagnosis and guide appropriate management. ©RSNA, 2017.

Multiple Pulmonary Arteriovenous Fistula in a Mother and His Son: A Case Report

Multiple Pulmonary Arteriovenous Fistulas (PAVFs) are rare vascular abnormalities in the general population, with a report on 15000 autopsies revealing only 3 cases of multiple PAVFs. However, these abnormal communications between pulmonary arteries and veins are commonly seen in patients with Hereditary Hemorrhagic Telangiectasia (HHT). Several conditions such as cirrhosis, chest trauma, metastatic carcinoma, schistosomiasis, and systemic amyloidosis are accountable for acquired form PAVF. We describe 2 patients (mother and son) with multiple PAVFs and no apparent signs or symptoms suggestive of HHT. A pulmonary artery injection wes performed for Case 1 (mother) and showed bilateral PAVFs with aneurysmal dilation. Given the diagnosis of PAVFs in the mother, we considered the same pathology for the child’s symptoms. Therefore, the boy also underwent pulmonary angiography, which revealed multiple bilateral PAVFs. Both patients underwent interventional angiography and the PAVFs were occulted with stainless coil. In conclusion, the presence of multiple PAVFs should be considered in the differential diagnosis of patients with unexplained exertional dyspnea and cyanosis, even in the absence of diagnostic features suggestive of HHT.

Omitted Pulmonary Arteriovenous Fistula Complicated With Brain Abscess

A 52-year-old female, diagnosed with a bilateral lower lobe pulmonary arteriovenous fistula (PAVF) at the age of 14 due to dyspnea, underwent a lobectomy of the right lower lung with a severe lesion, and symptom relief without treatment of the minor lesion in the other lung. She had active chest tightness since 42 years old and headaches 10 days before hospitalized, was diagnosed with PAVF and brain abscess, and cured by a successful PAVF embolization and a surgical resection of brain abscess. In the same patient, both methods of treatment for PAVF were all satisfactory, and each has advantages and indications. J Med Cases. 2017;8(5):149-151 doi: https://doi.org/10.14740/jmc2798w

Clinical Characteristics and Independent Factors Related to Long-Term Outcomes in Patients with Left Isomerism.

Background and ObjectivesWe studied the results of patient management for left isomerism (LI) and sought to determine factors that may influence survival and prognosis.Subjects and MethodsWe reviewed the medical records of 76 patients who were compatible with LI criteria between 1982 and 2014.ResultsOf the total study population, 29 patients (38.1%) had functional univentricular heart disease, 43 patients (56.5%) had cardiac anomalies suitable for biventricular hearts, and four patients (5.2%) had normal heart structure. Extracardiac anomalies were noted in 38.1% of the study population, including biliary atresia in 7.8% of all patients. Of the 25 patients who underwent Kawashima procedures, 24.0% developed pulmonary arteriovenous fistulas (PAVFs). During the median follow-up period of 11.4 years (range: 1 day to 32 years), 14 patients died. The 10-year, 20-year, and 30-year survival rates were 87%, 84%, and 76%, respectively. Preoperative dysrhythmia and uncorrected atrioventricular valve regurgitation were significantly associated with late death. There was no significant difference in the number of surgical procedures and in survival expectancy between patients in the functional single-ventricle group and in the biventricular group. However, late mortality was higher in functional single-ventricle patients after 18 years of age.ConclusionPatients with LI need to be carefully followed, not only for late cardiovascular problems such as dysrhythmia, valve regurgitation, and the development of PAVFs, but also for noncardiac systemic manifestations.

気管支ブロッカー留置の工夫により施行し得た肺動静脈瘻に対する小児完全胸腔鏡下肺葉切除術

幼児以下の小児は分離肺換気が困難であることから，鏡視下手術は広く普及していない．今回我々は肺動静脈瘻を有する4歳児平均相当の体格をした7歳女児に対して，完全胸腔鏡下肺葉切除術を安全に施行し得た．分離肺換気に際して，気管挿管前に気管支ブロッカーを留置する麻酔管理を行った．気管支ブロッカーがチューブ外にあるため，気管チューブが細くても気管支鏡操作と気管支ブロッカーとが干渉せず，良好な位置を保持し得た．気管支ブロッカー留置の工夫が，良好な分離肺換気とworking spaceの確保を可能とし，小児における安全な完全胸腔鏡下肺葉切除術の実施に有用と考えられた．

Brain Abscesses Associated with Asymptomatic Pulmonary Arteriovenous Fistulas

Brain abscess commonly occurs secondary to an adjacent infection (mostly in the middle ear or paranasal sinuses) or due to hematogenous spread from a distant infection or trauma. Pulmonary arteriovenous fistulas (AVFs) are abnormal direct communications between the pulmonary artery and vein. We present two cases of brain abscess associated with asymptomatic pulmonary AVF. A 65-year-old woman was admitted with a headache and cognitive impairment that aggravated 10 days prior. An magnetic resonance (MR) imaging revealed a brain abscess with severe edema in the right frontal lobe. We performed a craniotomy and abscess removal. Bacteriological culture proved negative. Her chest computed tomography (CT) showed multiple AVFs. Therapeutic embolization of multiple pulmonary AVFs was performed and antibiotics were administered for 8 weeks. A 45-year-old woman presented with a 7-day history of progressive left hemiparesis. She had no remarkable past medical history or family history. On admission, blood examination showed a white blood cell count of 6290 cells/uL and a high sensitive C-reactive protein of 2.62 mg/L. CT and MR imaging with MR spectroscopy revealed an enhancing lesion involving the right motor and sensory cortex with marked perilesional edema that suggested a brain abscess. A chest CT revealed a pulmonary AVF in the right upper lung. The pulmonary AVF was obliterated with embolization. There needs to consider pulmonary AVF as an etiology of cerebral abscess when routine investigations fail to detect a source.

Congenital pulmonary arteriovenous fistula

Congenital pulmonary arteriovenous fistula is a clinical rare abnormality of pulmonary vessels.The main reason is pulmonary capillary network hypoplasia.The clinical disease progression is occult.Exertional dyspnea, cyanosis and acropachy are the classical phenotypes.The key diagnosis methods include angiogram and multi-slice spiral CT angiography.The research progress of this disease was reviewed in this paper. Key words: Congenital pulmonary arteriovenous fistula; Diagnosis; Treatment

Pulmonary Arteriovenous Fistula: Clinical and Histologic Spectrum of Four Cases.

Pulmonary arteriovenous fistula (PAVF) is abnormally dilated vessels that provide a right-to-left shunt between pulmonary artery and pulmonary vein and is clinically divided into simple and complex type. Here, we report four cases of surgically resected sporadic PAVFs presenting various clinical and histologic spectrums. Cases 1 (a 57-old-female) and 2 (a 54-old-female) presented as incidentally identified single aneurysmal fistulas and the lesions were surgically removed without complication. On the other hand, case 3 (an 11-old-male) showed diffuse dilated vascular sacs involving both lungs and caused severe hemodynamic and pulmonary dysfunction. Embolization and surgical resection of the main lesion failed to relieve the symptoms. Case 4 (a 36-old-male) had a localized multiloculated cyst clinically mimicking congenital cystic adenomatoid malformation. Microscopically, the lesion consisted of dilated thick vessels, consistent with the diagnosis of fistulous arteriovenous malformation/hemangioma.

Abstract WP216: Stroke Recurrence in Patients With Cryptogenic Stroke

Background & Purpose: In cryptogenic stroke or transient ischemic attack (TIA) patients, aortic arch atheroma or patent foramen ovale (PFO) evaluated by using transesophageal echocardiography (TEE) is well known as possible embolic source. These findings were sometimes associated with stroke recurrence. We investigated predictive factors for stroke recurrence in patients with cryptogenic stroke. Methods: From February 2008 to December 2013, among 1832 consecutive stroke or TIA patients, 315 patients (male, 57.8%; age, 73.3±10.9) who were admitted within 7 days after the symptom onset without both atrial fibrillation and significant stenosis (50% or more in diameter) in the cerebral arteries underwent TEE for evaluating embolic sources; embolism was the probable etiological mechanism for their event. Patients with malignancy, cerebral arterial dissection, or infarcts in the perforating artery territory were excluded. We observed stroke recurrence during one year and investigated predictive factors for stroke recurrence. Results: In TEE, spontaneous echo contrast in the left atrium, aortic arch atheroma of 4.0 mm or more in thickness (mean, 4.65±2.06 mm), PFO, and pulmonary arteriovenous fistula were observed in 24 (7.6%), 223 (70.3%), 26 (8.2%), and 20 (6.3%) patients, respectively. Stroke recurrence was observed in 39 (12.4%) patients. Aortic arch atheroma of 4.0 mm or more, male, and prior hemorrhagic stroke were more frequent, and the initial diastolic blood pressure (DBP) and HbA1c were higher in patients with than without stroke recurrence. On the multivariate analysis using those factors and age, aortic arch atheroma (OR 3.73, 95%CI 1.46–11.7) and the initial DBP (OR 1.03, 95%CI 1.00–1.05) were positive predictors for a stroke recurrence. With regard to the operating characteristic curve analysis, the most accurate cut off values for a stroke recurrence were 4.1mm (AUC 0.59) in aortic arch atheroma thickness and 91mmHg in DBP (AUC 0.58). Conclusions: In acute cryptogenic stroke, aortic arch atheroma was observed frequently. Aortic arch atheroma and DBP were significantly associated with one-year stroke recurrence. TEE was required for evaluating aortic arch atheroma in patients with cryptogenic stroke

血胸で発見され緊急帝王切開後に胸腔鏡手術を行った肺動静脈瘻の1例

血胸で発見され緊急帝王切開後に胸腔鏡手術を行った肺動静脈瘻の1例

Brain Abscess in a Patient with Rendu-Osler-Weber Syndrome: Value of Proton Magnetic Resonance Spectroscopy

The diagnosis of intracranial focal lesions based on imaging studies is a challenge. In brain abscess, proton magnetic resonance spectroscopy (1H-MRS) is useful to define etiology, differentiating anaerobic from aerobic pathogens and inclusive sterile abscess based on the behavior of certain metabolites. We report a patient with Rendu-Osler-Weber syndrome, pulmonary arteriovenous fistulas, and brain abscesses characterized by brain magnetic resonance imaging (MRI), with a spectroscopy pattern compatible with anaerobic infection.

Pulmonary arteriovenous fistula mimicking as acyanotic heart disease with shunt reversal

Pulmonary arteriovenous malformations (PAVMs) or pulmonary arterio venous (PAV) fistula are relatively rare pulmonary vascular disorders. Although most of the patients are asymptomatic, PAVMs can cause dyspnea, clubbing, and central cyanosis and is an important differential diagnosis of common cardiopulmonary problems, such as hypoxemia and congenital heart diseases with reversal of shunt. There is a strong association between PAVM and hereditary hemorrhagic telangiectasia. Chest radiography, contrast echocardiography, and contrast enhanced computed tomography are important initial diagnostic tools, but pulmonary angiography is the gold standard. Therapeutic options include angiographic coil embolization or balloon occlusion and surgical excision.

An unusual case of right-to-left shunt: Pulmonary artery to left atrium fistula

We present a rare variant of pulmonary arteriovenous (AV) fistula (malformation), right pulmonary artery (RPA) to left atrium (LA) fistula, with its clinical and imaging findings with multidetector computed tomography (MDCT) pulmonary angiography and dynamic 3.0 T magnetic resonance imaging (MRI) pulmonary angiography in a 2-year-old female child who presented with central cyanosis at the age of 18 months.

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.

Half a Century’s Experience With the Superior Cavopulmonary (Classic Glenn) Shunt

Five decades after its introduction, the Glenn shunt remains an integral step for patients undergoing single-ventricle palliation. We performed a longitudinal follow-up of the original cohort of patients who underwent Glenn shunt. We performed a retrospective study of the original cohort of patients who underwent Glenn shunt at Yale between 1958 and 1988. Electronic medical records and chart review up to current era were used to collect data. Ninety-one patients underwent a Glenn shunt at an average age of 6.6 ± 2.5 years, of which 89 were classic Glenn shunts. Median overall survival was 43 years (range, 2 to 56; 95% confidence interval [CI], 39.5 to 46.5) while median survival from the Glenn shunt was 31.4 years (range, 0 to 45; 95% CI, 23.9 to 38.9). Forty-six patients died, 7 in the early postoperative period and 39 late deaths. Twenty-six patients were lost to follow-up. Nineteen patients remain alive with active clinical follow-up, 6 of whom still live with their classic Glenn shunt without conversion to bidirectional Glenn. Twenty-six patients (31%) developed pulmonary arteriovenous fistula with 11 patients (42%) requiring coil embolization. No patient developed thrombosis of the Glenn shunt. There were a total of 28 patients who developed arrhythmias, mostly in the tricuspid atresia group (n = 16), with the majority being atrial tachyarrhythmias (48%). Sixteen patients required permanent pacemaker placement for sinus node dysfunction. The Glenn shunt continues to provide excellent staged palliation in single-ventricle patients and a bridge to two-ventricle repair. Arrhythmias and pulmonary arteriovenous fistulas were common among single-ventricle cohort. Quality-of-life evaluation of the surviving patients would be an important outcome measure for future investigation.

Segmentectomy for bilateral pulmonary arteriovenous fistula with significant right to left shunt—a case report

Pulmonary arteriovenous fistulas (PAVFs) are rare vascular malformations of the lung and bilateral lung involvement adds further to it. We are presenting a case report of 33-year-old male patient who presented with cyanosis (SpO2 76 %) and breathlessness. Investigations revealed bilateral pulmonary arteriovenous fistula involving both the lower lobes. PAVFs were large, isolated and had aneurismal changes (8 × 6 cm on the right side and 3 × 3 cm on the left side). Staged lung resection was planed and right anterior and medial basal segmentectomy was done. Patient got rid of cyanosis and saturation has improved to 96 %. He was advised second surgery on the left side after 1 month but patient turned up after 7 years with massive haemoptysis and pain chest. Investigation showed that there was fistula on the left side which has increased in size to 5 × 4 cm. Left lateral basal segmentectomy was done. Patient did well and became asymptomatic. This report highlights that large and localized pulmonary arteriovenous fistula should be treated surgically sparing as much as lung tissue to preserve pulmonary function.

Percutaneous occlusion of vascular malformations in pediatric and adult patients: 20-year experience of a single center.

A case series on different vascular malformations (VM) treated with percutaneous occlusion in children and adults is presented. Percutaneous occlusion is usually the preferred treatment method for VM. Previous series have mostly focused on single types of devices and/or VM. Retrospective analysis of all patients who underwent percutaneous occlusion of VM in a single center, from 1995 to 2014, excluding patent ductus arteriosus. Clinical and angiographic data, procedural details, implanted devices, and complications were assessed. Procedural success was defined as effective device deployment with none or minimal residual flow. Predictors of procedural failure and complications were determined by multivariate analysis. A total of 123 VM were intervened in 47 patients with median age of 12 years (25 days-76 years). The VM included 55 pulmonary arteriovenous fistulae, 39 aortopulmonary collaterals, 10 systemic venovenous collaterals, 8 peripheral arteriovenous fistulae, 5 Blalock-Taussig shunts, 4 coronary fistulae, and 2 Fontan fenestrations. The 143 devices used included 80 vascular plugs, 38 coils, 22 duct occluders, and 3 foramen ovale or atrial septal defect occluders. Median vessel size was 4.5 (2.0-16.0) mm and device/vessel size ratio was 1.4 (1.1-2.0). Successful occlusion was achieved in 118 (95.9%) VM, including three reinterventions. Four (3.3%) clinically relevant complications occurred, without permanent sequelae. Lower body weight was independently associated with procedural failure and complications. To our knowledge, this is the largest series on different VM occluded percutaneously in children and adults, excluding patent ductus arteriosus. Percutaneous occlusion was effective and safe, using different devices.

Off-pump hepatic to azygos connection via thoracotomy for relief of fistulas after a Kawashima procedure: Ten-year results

ObjectivesAn almost universal incidence of developing pulmonary arteriovenous fistulas after the Kawashima operation has been reported. Exclusion of the hepatic venous flow from the pulmonary circulation causes the development of these malformations. Redirection of hepatic venous flow to the pulmonary circulation mostly leads to the regression of the arteriovenous fistulas. MethodsWe analyzed 11 patients with arteriovenous fistulas that developed after the Kawashima operation. The hepatic-to-azygos shunts were performed with an off-pump technique through a lateral thoracotomy in all but one. Operative and postoperative data were retrospectively collected. ResultsNo intraoperative complications occurred, and no patient died in the hospital. Up to 10-year follow-up showed a significant postoperative improvement of patients' oxygen saturation and New York Heart Association class. Apart from 2 re-thoracotomies for bleeding in 1 patient, no complications occurred and no patient died during follow-up. Two other patients underwent reoperation for an undiagnosed additional hepatic vein. The improvement of patients' oxygen saturation and New York Heart Association class persisted during the follow-up period. ConclusionsThe surgical connection can be performed safely with an off-pump technique that avoids the risks related to extracorporeal circulation and circulatory arrest. The results at 10 years follow-up confirmed the efficacy and safety of the surgical technique described.

Neuroradiological Manifestations of Hereditary Hemorrhagic Telangiectasia in 139 Japanese Patients.

The purpose of this study is to report the neuroradiological manifestations of hereditary hemorrhagic telangiectasia (HHT). One hundred and thirty-nine Japanese HHT patients (73 men and 66 women, aged 2–78 years) were included in this study. Diagnosis of HHT was based on genetic analysis and/or clinical diagnosis of Curaçao. They included 68 HHT1 and 37 HHT2 patients. Essentially, all patients underwent brain magnetic resonance imaging (MRI) and pulmonary computed tomography (CT). Contrast enhanced studies of brain MRI and hepatic CT were performed in a subset of patients. Catheter cerebral angiography was performed when indicated. Their neuroradiological features were reviewed retrospectively. Various imaging abnormalities were found. Brain arteriovenous malformations (AVMs) were observed in 27/136 patients (19.9%, 21 patients with HHT1 and 1 patient with HHT2). Pulmonary arteriovenous fistulas (AVFs) were found in 73/137 patients (65.2%, 45 patients with HHT1 and 6 patients with HHT2). Cerebral infarction and brain abscess were found in 17 patients and 3 patients with pulmonary AVFs, respectively. T1 high lesions in the basal ganglia suggestive of porto-venous shunts were observed in 51/136 patients (37.5%, 9 patients with HHT1 and 28 patients with HHT2). Hepatic AVMs were observed in 61/136 patients (44.9%, 15 patients in HHT1 and 29 patients in HHT2). Brain AVMs and pulmonary AVFs were more common in HHT1 than in HHT2 (both p < 0.01), but hepatic AVMs were conversely more common in HHT2 than in HHT1 (p < 0.01). In conclusion, HHT patients present with a variety of neuroradiological manifestations, which are related to substantial causes of morbid-mortality in HHT.