Diagnosis Of Pulmonary Arteriovenous Fistula Research Articles

Abstract TP577: Prevalence and Associations of Ischemic Stroke in Pulmonary ArterioVenous Fistulas

Introduction: Pulmonary arteriovenous fistula (PAVF) is an abnormal intrapulmonary right-to-left shunt. About 1 in 4 patients with untreated PAVF may suffer a paradoxical embolic acute ischemic stroke (AIS) by 65 years of age. Current expert recommendations are to treat all CT scan visible PAVF, even if asymptomatic. The American Heart Association Stroke Guidelines recommend using antiplatelet agents for secondary prophylaxis of AIS related to PAVF. Objective: To determine from the Nationwide Inpatient Sample (NIS), the prevelance and management trends of AIS in patients diagnosed with PAVF. Methods: Using the NIS from 2001-2014, patients carrying a secondary diagnosis of PAVF (ICD-9 CM code: 417.0) and a subsequent hospitalization with AIS were identified. SAS 9.4 was used for data analysis with categorical and continuous variables tested using the Chi-Square test of proportions and Student’s t-test, respectively. Cox proportional hazard regression was used to adjust for confounders. Results: A total of 4430 (weighted) patients (>18 years) were diagnosed with PAVF at a mean age of 55.6 years (SE±0.7). About 7% (n=312, p<0.0001) patients with PAVF were admitted with AIS at a mean age of 58.5 years (SE ±2.5). From 2001 to 2014, there was a two-fold increase in the prevalence of PAVF (204 vs. 455) as well as AIS related admissions (5% vs. 12%). On univariate analysis, epistaxis (p= 0.0184 OR 5.7), migraine (p= 0.0007, OR 5.7), patent foramen ovale (PFO) (p=0.0002, OR 3.2) and cannabis use (p= 0.009, OR 5.8) were significantly associated with AIS. Migraine (p=0.0062, OR 4.17) and PFO (p=0.0004, OR 3.68) retained significance on multivariate analysis. None of the ischemic strokes received reperfusion therapy. Only about 8% (n=24) were on long-term antiplatelet therapy. Approximately 3.2% (n= 141) of all patients with PAVF had a percutaneous embolization of their fistula. Conclusion: In a nationally representative sample, the prevalence of AIS in patients with PAVF has roughly doubled from 2001 to 2014. Utilization of reperfusion therapy and secondary stroke prophylaxis with antiplatelet agents is low. Consensus guidelines and randomized control trials are need to establish the role of fistula embolization in these patients.

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.

Late Presented Posttraumatic Pulmonary Arteriovenous Fistulea Occlusion With Septal Occluder Device

Posttraumatic pulmonary arteriovenous fistula is a rare complication of penetrating injury. Cases may remain asymptomatic for years prior to diagnosis. We present a case of pulmonary arteriovenous fistula related to a penetrating injury of the thorax 6 years previously that was occluded with a septal occluder device. Consistent use of CT or conventional catheter pulmonary angiography following penetrating injury to the thorax is essential to the early diagnosis of pulmonary arteriovenous fistula during the asymptomatic stage. Modern occlude devices facilitate the transcatheter approach as a viable alternative to surgery for the closure large, high pressure defects.

Communication between the right pulmonary artery and left atrium with aneurysm formation

Communication between the pulmonary artery and the left atrium is a rare congenital anomaly. A 7-year-old boy was referred to our hospital with a diagnosis of pulmonary arteriovenous fistula. Referral to a pediatric cardiologist had been delayed because he had only slight cyanosis and no heart murmur. Catheterization and computed tomography scans confirmed that the right pulmonary branches and pulmonary venous returns were normal and that an additional aneurysmal communication was located between the right pulmonary artery and the left atrium. Surgery proceeded uneventfully together with a cardiopulmonary bypass. The connection from the right pulmonary artery to the aneurysm was ligated, and the connection from the left atrium to the aneurysm was closed with a polytetrafluoroethylene patch. We describe this rare anomaly with cyanosis and no heart murmur.

The value of 64-slice CT angiography in diagnosing pulmonary arteriovenous fistulas

目的 探讨64层螺旋CT血管成像技术对肺动静脉瘘的诊断价值.方法 对12例临床怀疑肺动静脉瘘的患者行CT血管成像检查.对所有扫描图像行容积再现(VR)、最大密度投影(MIP)以及冠状多平面重组(MPR).结果 12例患者均确诊为肺动静脉瘘,7例为单发,5例为多发,共发现20处肺动静脉瘘,除漏诊1例合并位于近心位置的3处肺动静脉直接交通外,其余病变均诊断正确且无遗漏.结论 64层螺旋CT血管成像技术可以无创地诊断肺动静脉瘘,为治疗方案的制定及术后随访提供依据。

Successful coil embolization for diffuse, multiple pulmonary arteriovenous fistulas

Although many patients with pulmonary arteriovenous fistulas (PAVF) remain asymptomatic in infancy and early childhood,‘* 2 symptoms and serious complications may occur in later childhood or adulthood and may necessitate some type of intervention. Previously, surgical resection was the accepted treatment for symptomatic patients with PAVF.” More recently, however, it has been shown that some of these lesions are amenable to less invasive therapy4-g such as balloon10-13 or coil spring emboiization’4-*6 that spares normal pulmonary parenchyma.r3-I5 A 24-year-old woman became aware of cyanosis and clubbing about 1 year prior to consultation after she had begun to experience fatigue on exertion. Physical examination confirmed cyanosis and marked clubbing but revealed a normal heart size and heart sounds with no cardiac or pulmonary murmur. Blood pressure was 102170 mm Hg. She had no cutaneous vascular lesions or other abnormalities. Red blood cell (RBC) count was 6.88 million, the hemoglobin was 17.5 gm, and the hematocrit was 55.9 (Table I). Oxygen saturation in room air by pulse oximetry was 87 %. The electrocardiogram and echocardiogram were normal, Chest roentgenogram revealed a heart of normal size with average pulmonary vascularity in the right lung but diffuse interstitial markings in the mid and lower left lung field, where a prominent vascular shadow was seen within the cardiac silhouette (Fig. 1, A). A diagnosis of pulmonary arteriovenous fistulas was made. Prior to cardiac catheterization, the patient underwent a ventilation-perfusion lung scan. The results, consistent with abnormality of the left lung, are seen in Table II. The images from the scan showed no segmental or subsegmental defects. There was slightly decreased perfusion in the region of the left major fissure. The distribution of radionuclide medium suggested the presence of a right-to-left shunt. Cardiac catheterization and selective arteriography of the left and right pulmonary arteries and their major branches was then carried out using intraarterial digital

Pulmonary arteriovenous fistulas in hereditary hemorrhagic telangiectasia.

We reviewed all available chest X-rays of 95 patients with hereditary hemorrhagic telangiectasia (HHT). The diagnosis of pulmonary arteriovenous fistula (PF) had previously been made in 13 patients, nine of whom had been operated on. Another three cases were found in the review. The 16 patients with HHT and PF came from eight families, one of which contributed seven patients. Median observation time between the first and the latest chest X-ray examination was 11 years (range 1/2-35). Growth of the PF was seen in four patients and spontaneous regression in one. Four of the 16 patients with PF had symptoms consistent with cerebral embolism, while only two of the 79 patients without PF had such symptoms. This study is part of an epidemiological investigation of HHT--to our knowledge not carried out before. The calculated period prevalence of simultaneous HHT and PF in the decade 1964-74 in the county of Fyn (429 207 inhabitants) was 2.6 per 100 000.

Echocardiography and perfusion scintigraphy in the diagnosis of pulmonary arteriovenous fistula: A. B. Lewis, G. F. Gates, and P. Stanley. Chest 73:675–677 (May), 1978

Echocardiography and perfusion scintigraphy in the diagnosis of pulmonary arteriovenous fistula: A. B. Lewis, G. F. Gates, and P. Stanley. Chest 73:675–677 (May), 1978

Diagnosis of pulmonary arteriovenous fistula by contrast echocardiography

That the anatomic substrate of the Wolff-ParkinsonWhite syndrome can be inherited without the ECG evidence is illustrated by Family 1. It is likely that the other three families also represent examples of this phenomenon. These findings explain why previous genetic studies have been inconclusive. It was assumed, erroneously, that many subjects did not have Kent bundles when, in fact, they did. It will be difficult to overcome this problem since at present, cardiac catheterization is necessary to find Kent bundles which conduct only in the retrograde direction. If all subjects with PSVT are assumed to have Kent bundles, then false positives may be included since some PSVT might be due to other mechanisms. The mechanism of inheritance of Kent bundles seems to most closely follow that of autosomal dominant with variable expression. This paper has identified one mechanism of the variability. Another might be a patient with a retrograde conducting bundle of Kent who never has PSVT. Orinious et al e reported results which support ours in his 20-year follow-up of 150 members of two families with WPW. Although he found only two additional patients with WPW, he found 17 new examples of SVT, eight of Which were in parent-child combinations. In a patient with PSVT without WPW who has a family member with WPW, a concealed bundle of Kent should be suspected. This finding has obvious therapeutic implications, since patients With concealed bundles of Kent may respond to different medical treatment or may be cured by surgical division of the anomalous pathway. r

Echocardiography and Perfusion Scintigraphy in the Diagnosis of Pulmonary Arteriovenous Fistula

The echocardiographic and perfusion scintigraphic evaluation of an adolescent boy with a pulmonary arteriovenous fistula is reported. Contrast echocardiography following the rapid intravenous injection of indocyanine green dye was utilized to document extracardiac right-to-left shunting. Perfusion lung scintigraphy demonstrated the presence of a single large pulmonary arteriovenous fistula. Contrast echocardiography and perfusion scintigraphy are minimally invasive, safe and easily performed techniques for the rapid diagnosis of pulmonary arteriovenous fistula.

Recurrent Brain Abscess: Manifestation of Pulmonary Arteriovenous Fistula and Hereditary Hemorrhagic Telangiectasia

A unique case of recurrent brain abscess as the primary manifestation of pulmonary arteriovenous fistula is presented. In the absence of hematologic abnormalities and characteristic physical findings, the diagnosis of pulmonary arteriovenous fistula is particularly elusive. Patients with brain abscess must be carefully evaluated for the presence of pulmonary arteriovenous fistula or hereditary hemorrhagic telangiectasia.

Pulmonary arteriovenous fistula. Diagnosis and prognosis in noncomplainant patients.

Data are reported on 27 patients with pulmonary arteriovenous fistula; 18 were mem­ bers of one family who were suffering from hereditary hemorrhagic telangiectasia. Only 2 patients had consulted their doctor because of complaints related to the pulmonary ar­ teriovenous fistula; the disease in the other 25 patients was discovered accidentally, mainly as a result of mass radiographic surveys. In general, the chest radiographs were not helpful, but a tentative diagnosis of pulmonary arteriovenous fistula was often made when heredi­ tary hemorrhagic telangiectasia and an extracardiac murmur were present. The value of bilateral pulmonary angiography is stressed. In patients with no complaints, an abnormal right-left shunt (calculated with the aid of arterial oxygen tension measurements) was often demonstrated. Progression of the lesions was mainly confined to patients with multiple fistulas. In 2 patients, serious complications, possibly iatrogenic, occurred. Indications for and results of surgical treatment are discussed.

Varicosities of the Pulmonary Veins Simulating Arteriovenous Fistulas

In four cases, the original diagnosis of pulmonary arteriovenous fistula proved to be incorrect. Pulmonary angiography readily established the correct diagnosis of varicosity of the pulmonary vein in each case. Such varicosities are congenital in origin and probably have little significance, although cases of spontaneous rupture have been reported. Awareness of this lesion should prevent a misdiagnosis of pulmonary arteriovenous fistula or other serious, radiographically evident lesion.

Arteriovenous fistula of the lung.

Pulmonary arteriovenous fistulas are anomalous connections between the arteries and veins of a portion of the pulmonary vascular bed. Since the first clinical diagnosis of pulmonary arteriovenous fistula by Smith and Horton in 1939 (13), 200 cases have been reported. The detection by routine chest roentgenograms and photofluorograms of clinically silent spheroid pulmonary lesions has been largely responsible for an increasing frequency of diagnosis. It is now mandatory that a radiologist, confronted with a density in the lung parenchyma, include arteriovenous fistula in the differential diagnosis. This is of prime importance, since the surgical approach may differ from that for the usual “coin lesion.” Case Reports Case I: On Feb. 15, 1958, a 27-year-old Caucasian sailor was admitted to the St. Albans Naval Hospital. A routine chest roentgenogram for discharge from the service revealed a density in the right lower lobe. History and physical examination were unremarkable. A murmur within the right chest could not be detected and examination of the mucous membranes and skin failed to reveal telangiectasia. There was no history of familial disease. Oxygen saturation and pulmonary function studies were normal. The roentgenograms are shown in Figures 1–4. A segmental resection of the fistula in the right lower lobe was performed with an uneventful postoperative course. Case II: A 24-year-old Caucasian sailor was admitted to the U. S. Naval Hospital in December 1955 because of a mass in the left lung, detected on an annual chest roentgenogram. He was asymptomatic and had never had any operation or serious illnesses. System review and familial history were unremarkable. Physical examination failed to reveal telangiectasia or other abnormalities except for a soft, sharply localized systolic murmur in the left posterior axillary line over the eighth rib. This did not change with respiration. Results of laboratory studies were within normal limits. Oxygen saturation of the arterial blood was normal. Roentgen studies are shown in Figures 5–7. At surgery, a wedge resection removed the fistula intact and no murmurs or thrills were detectable in the remaining lung. The postoperative course was uneventful. Case III: A 33-year-old Caucasian female was admitted to the St. Albans Naval Hospital in September 1945. During a bout of acute tonsillitis a chest roentgenogram revealed a mass in the left lung, adjacent to the hilus. History and physical examination were otherwise unremarkable and there were no familial diseases. Angiocardiography confirmed the diagnosis of a pulmonary arteriovenous fistula but surgery was refused by the patient.

The use of angiocardiography as an aid in the diagnosis of pulmonary disease.

Angiocardiography, with 70 % sodium acetrizoate as the contrast medium, has been carried out in more than 600 patients with pulmonary disease without a single fatality. It permits the visualization of the lobar and segmental branches of the pulmonary arteries and thus reveals conditions that often escape detection by other diagnostic methods. Seven cases are reported illustrating its usefulness in distinguishing between vascular and nonvascular lesions and its importance in deciding between surgical and medical treatment. It is essential for the diagnosis of pulmonary arteriovenous fistula and helpful in evaluating thoracoplasties. The author believes that it should be more frequently employed in the study of pulmonary diseases.

Pulmonary arteriovenous fistula: Angiocardiographic observations in nine cases

Pulmonary arteriovenous fistulas are hereditary hemangiomatous malformations of the pulmonary vascular bed. Almost 40 per cent of the reported cases also have hemangiomas elsewhere in the body, indicating that a close relationship to familial hemorrhagic telangiectasis (Rendu-Osier-Weber's disease) exists. When pulmonary arteriovenous fistulas are large and do not have systemic arterial connections, dyspnea, cyanosis, clubbing of the digits and polycythemia result from unsaturation of the blood. Hemoptysis may occur when there is rupture of the paper-thin pulmonary vascular anomaly. There is also a high incidence of cerebral symptoms due either to cerebral thrombosis, secondary to the polycythemia, or to infection, especially brain abscess. The heart remains normal in size because pulmonary arteriovenous fistulas do not cause increased pulmonary vascular resistance. In our series of nine cases the classical syndrome of cyanosis, clubbing of digits, polycythemia and a vascular murmur heard over the pulmonary fistula was present in one case only. One patient with dyspnea, fatigue and familial telangiectasis had bilateral pulmonary arteriovenous fistulas; chronic anemia prevented intense cyanosis and polycythemia. In three instances the patients were asymptomatic; in two the pulmonary arteriovenous fistula was an incidental finding; in the remaining two, acute cerebral conditions (brain abscess and meningoencephalitis) were the presenting symptoms. Vascular bruits were heard in eight patients. All patients had abnormal chest roentgenograms. The diagnosis of pulmonary arteriovenous fistula can often be made by conventional roentgenography, particularly if the index of suspicion is high. Pulmonary densities with hilar vascular connections localized in lobes or segments of the lung, or increased hilar vascularities, should arouse suspicion of an arteriovenous fistula. Change in size of the lesions after respiratory maneuvers (Valsalva, Müller) and delineation of the afferent arterial and efferent venous connections by body section radiography may be confirmatory. Angiocardiography, however, establishes the diagnosis and is recommended for preoperative evaluation. The treatment of pulmonary arteriovenous fistulas is surgical excision, with preservation of as much lung tissue as possible in order to preserve pulmonary function, especially if multiple lesions are present. Surgery is usually curative. It is recommended by some even in the asymptomatic case since the risk of brain abscess, fatal hemoptysis or development of cerebral thrombosis when polycythemia occurs is far greater than the morbidity and mortality of lobectomy or segmental resection.

The Angiographic Demonstration of Pulmonary Arteriovenous Fistula

Contrast angiography in the diagnosis of pulmonary arteriovenous fistula was first recorded by Smith and Horton (24) in 1939. Their patient, a forty-seven-year-old male in whom cyanosis and clubbing of the fingers had been noted since the age of twenty-four, was examined first in 1932. At that time he was thought to have polycythemia vera. Five years later a bruit was heard at the base of the right lung and a density in the right lower lobe was demonstrable roentgenographically. Following the injection of a radiopaque medium into the basilic vein to visualize the pulmonary blood vessels, roentgenograms showed the density to be a vascular tumor and the diagnosis of hemangioma of the lung acting as an arteriovenous fistula was made. The patient was not treated. The first successful surgical cure of a pulmonary arteriovenous fistula was reported by Hepburn and Dauphinee (12) in 1942. A twenty-three-year-old female complained of dizziness, faintness, and dyspnea. She was cyanotic, and was known to have had clubbing of the fingers since she was fifteen. Roentgenograms revealed a shadow in the right middle and lower lobes, which was diagnosed as a pulmonary arteriovenous fistula. A right pneumonectomy was done by Shenstone (21), and the patient made a rapid recovery, with prompt disappearance of the cyanosis and polycythemia and gradual improvement in the clubbing of the fingers. In the accompanying table are listed data on 22 cases of pulmonary arteriovenous fistula recorded in the literature accessible to us. It will be noticed that the first accurately described case was recorded by Wilkens (28) in 1918. At the postmortem examination of a twenty-three-year-old girl it was noted that two vessels which emerged from a dilated branch of the artery to the left lower lobe joined to enter the pulmonary vein just before its entrance into the left auricle. Similar changes were found in the parenchyma of the right lung. The ages in the reported cases, as shown in the table, varied from two days to fortyfive years. There were 13 males and 9 females. Some degree of cyanosis was noted in 17 cases, and clubbing of the fingers and toes was found in 15. Nine patients had small hemangiomata of the skin or petechiae. In more than half of the cases some type of murmur was heard over the affected area. Red cell counts varied from normal to 11.4 million. Hemorrhage or epistaxis was reported in 9 cases. Other symptoms were dyspnea, cough, persistent headache, and a “lightheaded feeling.” The familial character of the lesion was stressed by Goldman (10), who observed its occurrence in two brothers. Whitaker reported (27) a family history of telangiectasis accompanied in one member by a proved pulmonary arteriovenous fistula. Maier, Himmelstein, Riley, and Bunin (18) described a bacterial endarteritis in association with their case of pulmonary arteriovenous fistula.