Pulmonary arteriovenous malformation (PAVM), a rare pulmonary condition, is defined as a structurally abnormal communication between the pulmonary artery and pulmonary vein, creating a pathologic intrapulmonary right-to-left shunt. This, in turn, impairs regular gas exchange and filtration of systemic venous blood. PAVMs are also known as pulmonary arteriovenous fistulae, pulmonary arteriovenous aneurysms, and pulmonary hemangiomas. These lesions were initially described by Churton in 1897 and were later identified to be associated with hereditary hemorrhagic telangiectasia (HHT) in 1938.
A vast majority of patients with PAVMs can be asymptomatic; however, patients that remain undiagnosed can later present with life-threatening complications such as ischemic stroke, myocardial infarctions, cerebral abscesses, massive hemoptysis, and hemothorax. Although there is a relatively low incidence of this condition, it is essential to consider in the differential diagnosis for patients presenting with epistaxis, hypoxemia, and dyspnea with exertion.
Pulmonary arteriovenous malformations (PAVMs) can either be congenital or sporadic, with the majority of congenital cases being secondary to HHT. PAVMs can be present from birth and ultimately develop by adult life. Up to 80 to 90% of individuals with PAVMs will later go on to develop HHT.
Sporadic causes of PAVMs are rare and include prior chest surgery, trauma, schistosomiasis, actinomycosis, mitral stenosis, Fanconi’s syndrome, hepatic cirrhosis related hepatopulmonary syndrome, and metastatic carcinoma. They have also been demonstrated to be a complication from congenital heart disease surgery in children. The remainder of PAVMs is thought to be idiopathic.
Pulmonary arteriovenous malformations (PAVMs) are generally uncommon in the population. In 1953 Johns Hopkins hospital performed an autopsy study with 15,000 cases with only 3 cases of PAVM detected. Investigators believed that small PAVMs could easily be missed during routine biopsies after that new case series by Mayo clinic revealed an incidence of approximately 4.3 cases per year.
PAVMs are largely associated with hereditary hemorrhagic telangiectasia (HHT), which itself affects approximately 1 in 5,000 to 8,000 people. HHT is an autosomal dominant disorder characterized by the development of AVMs at multiple sites, including mucocutaneous, pulmonary, hepatic, gastrointestinal, and cerebrovascular systems. Approximately 80 to 90% of individuals with PAVMs will have HHT. On the contrary, approximately 15% to 35% of individuals with HHT will develop PAVMs.
Pulmonary arteriovenous malformations (PAVMs) are predominantly found in the pleura of the lower lobes likely due to increased pulmonary blood flow. Bosher and colleagues reviewed the pathologic anatomy in 350 patients with PAVMs and found that 75% had unilateral disease, whereas 36% had bilateral disease. These lesions can further be classified into simple versus complex, with up to 95% being simple type. A simple type is defined as perfusion by a single segmental artery and venous drainage by a single vein. About 95% of cases are supplied by pulmonary arteries, with the remaining 5% supplied by systemic arteries by way of the aorta, bronchial artery, or intercostal arteries. Sizes of these lesions can also vary considerably but are typically 1 to 5 cm in size, with more extensive lesions being the culprit of more severe complications.
PAVM consists of thin-walled vascular channels that are built by elastic fibers and remnants of smooth muscle cells. The intima can be thickened and partly covered by mural thrombi.
Although the etiology of PAVM is unknown, recent discoveries in genetic mutations of hereditary hemorrhagic telangiectasias have shown relevance in the potential causes. Mutations in the endoglin (ENG) gene, activin receptor-like-kinase-1 (ACVRLI/ALK1) gene, and SMAD4 gene have all been associated with inheritance of HHT. These proteins are components involved in transforming growth factor-β (TGF-β) signaling pathways. Vascular abnormalities seen in PAVM are thought to arise from mutations in these proteins and the resultant imbalances in response to angiogenic factors like vascular endothelial growth factor and defective vascular repair. It has been postulated that mutations in endoglin may result in an abnormal response to TGF-β during vascular remodeling, resulting in the formation of arteriovenous malformations.
There have been numerous proposed theories on the pathogenesis of these lesions. Anabtawi and colleagues hypothesized that lesions were the result of incomplete resorption of the vascular septae that separate arterial and venous plexuses during fetal development. Others have hypothesized that the lesions are due to a defect in terminal arterial loops that allow dilatation of thin-walled capillary sacs.
Congenital cases of pulmonary arteriovenous malformations (PAVMs), although rare, usually present at birth with cyanosis, murmur, and congestive heart failure.
The majority of cases often develop by the fourth and sixth decades of life. However, patients with hereditary hemorrhagic telangiectasia (HHT) typically develop symptoms by the second decade of life.
The most common complaint in patients with underlying HHT is epistaxis, caused by bleeding from mucosal telangiectasias.
Dyspnea is the second most common symptom, with an incidence of 13% to 56%. It is most common among patients that have clubbing or whose PAVMs are large or multiple. Some patients have platypnea, which is thought to be secondary to decreased blood flow through PAVM in the dependent lung portions while supine.
Other features include hypoxemia, exercise intolerance, chest pain, cough, murmurs, bruits, clubbing, and cyanosis.
Patients presenting with radiographic evidence of pulmonary nodules, suspected or known diagnosis of hereditary hemorrhagic telangiectasia or unexplained findings such as hypoxemia, dyspnea, hemoptysis, cyanosis, clubbing, or brain abscess should prompt further investigation.
Shunt Fraction Measurement
The normal fraction of cardiac output that shunts from right-to-left is less than 5%. In patients with pulmonary arteriovenous malformations (PAVMs), this fraction is increased. Dines and colleagues found that out of 21 patients with PAVM who underwent shunt determination were found to have shunts between 9.5 and 42%. Shunt fraction is done by breathing 100% oxygen for 15 to 20 minutes, then measuring the PaO2 and SaO2. Those measurements are then used to determine the shunt fraction-a shunt fraction of more than 5% warrants further evaluation.
Chest radiography is a simple, inexpensive, low radiation technique that should be used for the initial screening of a PAVM. Frontal and lateral views should be obtained. Typical PAVMs should appear as well-defined lesions with feeding vessels on radiography. This imaging modality, however, has a low sensitivity for detecting smaller sized PAVMs.
Transthoracic Contrast Echocardiography (TTCE)
The screening test of choice is transthoracic contrast echocardiography (TTCE), which has a sensitivity of 95%-100%. This test is simple, non-invasive, and associated with low false-negative rates, in one study, positive and negative predictive values of 96% and 100%, respectively. It involves introducing 10 mL of agitated saline into the peripheral circulation and observing its course through the cardiac system with echocardiography. A positive result is seen with the presence of bubbles in the left cardiac chamber after three to eight cardiac cycles. TTCE can also provide information on the likelihood of future neurologic events based on pulmonary shunt grading. Pulmonary shunt grade 1 or (<30 microbubbles), is not associated with an increased prevalence of CNS events, whereas grade 2 (30-100 microbubbles) and grade 3 (>100 microbubbles) are predictors of CNS events.
Radionuclide Perfusion Lung Scanning
This test can be useful in situations where echocardiography or the 100 percent oxygen method is not readily available. It involves the peripheral injection of macro aggregated albumin labeled with technetium-99m (99mTc). In healthy individuals, the radiolabeled particles will be filtered by pulmonary capillaries. When a right-to-left shunt is present, such as a pulmonary arteriovenous malformation, the radiolabeled particles pass through the lungs and are subsequently filtered by capillary beds in other organs such as the brain and kidneys. The shunt fraction is calculated by quantifying the renal uptake as a percentage of the total dose given. The disadvantages are that it is an expensive test and not routinely available at most institutions. Besides, this test is unable to differentiate between intrapulmonary and intracardiac shunts.
Computed Tomography (CT)
CT is the imaging modality of choice for confirmation of PAVM and should be performed in patients with either high suspicion of PAVM or grade 2 or 3 shunts by TTCE. Contrast enhancement will show a PAVM sac with both feeding and draining vessels. Generally, if the CT scan shows one or more PAVM with a feeding artery diameter ≥ 2 to 3 mm, the patient should be referred for pulmonary angiography. If the feeding artery diameter is < 2 mm, pulmonary angiography can be deferred unless there are clinical features suggestive of symptomatic PAVM.
Pulmonary angiography is the gold standard for diagnosis of PAVM and is utilized to define the vascular anatomy for cases that are suitable for embolization therapy. Contrast is directly introduced into the feeding artery to determine the vasculature of the lesion accurately. Contrast is also injected into the left and right main pulmonary arteries to detect additional lesions that are also amenable to embolization therapy.
Other Diagnostic Tests
Contrast-enhanced magnetic resonance angiography (MRA) is not currently used for screening of PAVM. This modality is accurate in the detection of PAVM; however, it is expensive, not routinely available, and requires specialized expertise in the interpretation of imaging.
Screening for HHT
In patients without a known diagnosis of HHT with high suspicion of a PAVM, it is vital to perform a thorough history and physical exam for supporting evidence of the diagnosis. Family members of patients with HHT should also be routinely screened, as there is a high incidence of unsuspected pulmonary and cerebral AVM in family members of patients with HHT. It has been reported that in HHT families in which one person has PAVM, the incidence of another family member having PAVMs in about 35%.
If available, routine genetic screening is preferred in all family members. In patients found to have HHT genotype, further investigation would include chest radiography and shunt fraction measurement. In those instances where genetic testing is not available, screening protocols can be used as described by Haitjema and colleagues.
The main implication for the treatment of pulmonary arteriovenous malformations (PAVMs) is to prevent cerebral abscesses, stroke, and improve exercise tolerance and hypoxemia.
Patients are best managed at facilities such as hereditary hemorrhagic telangiectasia centers, where there are experts in PAVM treatment. This is done to facilitate the best outcome possible, given the potential complexity of each case.
Deciding whether treatment should be offered to patients is dependent on factors such as the presence of symptoms, the feeding artery diameter, and if the patient is capable of tolerating the procedure. Patients who are asymptomatic with a feeding artery diameter (FAD) of less than 2 mm can be observed clinically, whereas patients who are symptomatic with lesions less than 2 mm or presence of grade 3 shunting warrant further treatment.
The most widely used and successful form of treatment is percutaneous transcatheter embolization, which involves the occlusion of the feeding artery of the PAVM. Previously in the 1990s, only lesions with a feeding artery diameter of 3 mm or more were considered for embolization therapy. Since then, it has been reported that paradoxical emboli can occur independently of the FAD. Given these findings, embolization is now being recommended in lesions with a feeding artery diameter of less than 3 mm.
Embolotherapy can be done using either a coil or a balloon. Both techniques involve the localization of the feeding artery via angiography. A steel coil or balloon is advanced through the catheter and released at the site of the artery to disrupt blood flow. A pulmonary angiogram is repeated to ensure PAVM occlusion.
The most common complication of embolization therapy is self-limiting pleuritic chest pain, reported in 5% to 13% of cases. Less common complications include stroke, transient ischemic attack, and transient air embolization.
Follow-up is recommended three to six months post-embolization with contrast-enhanced chest computed tomography and transthoracic echocardiography and is used to confirm closure of the PAVM. If a PAVM persists at one year following initial treatment, repeat pulmonary angiography, and transcatheter embolization are indicated. Patients can develop new pulmonary hypertension or a worsening of baseline pulmonary hypertension after embolization or extensive PAVM resection.
Surgical excision is recommended in patients who fail embolization therapy or in patients with life-threatening pulmonary hemorrhage from a ruptured PAVM.
Lung transplantation is an option for those patients who are refractory to repeated embolization, usually in those with bilateral disease or those considered to have a high risk of mortality.
Given the high risk of air embolism in these patients, the administration of intravenous fluids or medications should be done with caution. Scuba diving should also be avoided in this population.
Lastly, all patients should be given lifelong antibiotic prophylaxis before dental or surgical procedures to avoid the development of bacteremia and brain abscesses. This is recommended, even after embolization therapy. A preliminary study has shown a decrease in the duration and the number of epistaxis episodes with bevacizumab.
The clinical features and radiological findings of pulmonary arteriovenous malformations (PAVMs) resemble many other diseases. The following differentials should be kept in mind when assessing a patient of PAVM.
Pulmonary arteriovenous malformations (PAVMs) do not spontaneously resolve. Most remain stable in size, although approximately 25% of PAVMs can enlarge in size at a rate of 0.2 to 0.3 mm a year.
Although correct estimates of morbidity and mortality are lacking, PAVMs are known to carry high morbidity and mortality precisely due to the development of serious complications such as stroke and brain abscess. PAVM that remain untreated is associated with a mortality of up to 50% compared to 3% in those that are treated.
Complications of pulmonary arteriovenous malformations (PAVMs) are thought to be secondary to paradoxical emboli. The most common neurologic sequelae include stroke and brain abscesses. The prevalence of these complications is seen in patients with multiple PAVMs.
Other complications include:
Given the potential for life-threatening complications, patients with pulmonary arteriovenous malformations (PAVMs) should undergo thorough evaluation including history and physical exam, along with the appropriate genetic testing for hereditary hemorrhagic telangiectasia. Clinical signs, symptoms, and complications should be reviewed with patients beforehand. Close follow-up is also recommended in order to monitor disease progression or possible complications from interventions performed.
The diagnosis of pulmonary arteriovenous malformation (PAVM) requires a high index of suspicion, as this a rare condition. It is crucial to consider PAVM in the differential of patients presenting with complaints of epistaxis, hemoptysis, shortness of breath, and exercise intolerance. Besides, any patient presenting with radiographic evidence of pulmonary nodules, suspected or known diagnosis of hereditary hemorrhagic telangiectasia, or unexplained findings such as hypoxemia, dyspnea, hemoptysis, cyanosis, clubbing, or brain abscess should prompt further investigation.
A thorough evaluation with history, physical exam, ancillary imaging such as contrast-enhanced CT to further support the diagnosis. As this condition is highly associated with HHT, patients should routinely undergo genetic testing, especially those with a family history. Lesions that are identified on CT should undergo further evaluation with pulmonary angiography. Although there are no clear guidelines in the definitive management of PAVMs, it has been recommended that those with a feeding artery diameter of less than 3 mm should undergo embolization therapy (Level II). Future randomized control studies are necessary further to delineate guidelines for the management of these patients.
The importance of diagnosis and treatment in this condition is to prevent the life-threatening and debilitating complications of untreated lesions such as stroke, seizures, and brain abscesses. All patients should be educated on these complications and the importance of interval follow-up and imaging. It is recommended that patients with HHT should be followed at an HHT center.
|||Gossage JR,Kanj G, Pulmonary arteriovenous malformations. A state of the art review. American journal of respiratory and critical care medicine. 1998 Aug; [PubMed PMID: 9700146]|
|||SLOAN RD,COOLEY RN, Congenital pulmonary arteriovenous aneurysm. The American journal of roentgenology, radium therapy, and nuclear medicine. 1953 Aug; [PubMed PMID: 13065567]|
|||Tellapuri S,Park HS,Kalva SP, Pulmonary arteriovenous malformations. The international journal of cardiovascular imaging. 2019 Aug; [PubMed PMID: 30386957]|
|||Pollak JS,Saluja S,Thabet A,Henderson KJ,Denbow N,White RI Jr, Clinical and anatomic outcomes after embolotherapy of pulmonary arteriovenous malformations. Journal of vascular and interventional radiology : JVIR. 2006 Jan; [PubMed PMID: 16415131]|
|||Moussouttas M,Fayad P,Rosenblatt M,Hashimoto M,Pollak J,Henderson K,Ma TY,White RI, Pulmonary arteriovenous malformations: cerebral ischemia and neurologic manifestations. Neurology. 2000 Oct 10; [PubMed PMID: 11061251]|
|||DE FARIA JL,CZAPSKI J,LEITE MO,DE PENNA DO,FUJIOKA T,DE CINTRA AB, Cyanosis in Manson's schistosomiasis; role of pulmonary schistosomatic arteriovenous fistulas. American heart journal. 1957 Aug; [PubMed PMID: 13444184]|
|||Prager RL,Laws KH,Bender HW Jr, Arteriovenous fistula of the lung. The Annals of thoracic surgery. 1983 Aug; [PubMed PMID: 6349562]|
|||Taxman RM,Halloran MJ,Parker BM, Multiple pulmonary arteriovenous malformations in association with Fanconi's syndrome. Chest. 1973 Jul; [PubMed PMID: 4717446]|
|||Lange PA,Stoller JK, The hepatopulmonary syndrome. Annals of internal medicine. 1995 Apr 1; [PubMed PMID: 7872588]|
|||Mathur M,Glenn WW, Long-term evaluation of cava-pulmonary artery anastomosis. Surgery. 1973 Dec; [PubMed PMID: 4127196]|
|||Wong HH,Chan RP,Klatt R,Faughnan ME, Idiopathic pulmonary arteriovenous malformations: clinical and imaging characteristics. The European respiratory journal. 2011 Aug; [PubMed PMID: 21177836]|
|||Dines DE,Arms RA,Bernatz PE,Gomes MR, Pulmonary arteriovenous fistulas. Mayo Clinic proceedings. 1974 Jul; [PubMed PMID: 4834927]|
|||Dines DE,Seward JB,Bernatz PE, Pulmonary arteriovenous fistulas. Mayo Clinic proceedings. 1983 Mar; [PubMed PMID: 6219255]|
|||Swanson KL,Prakash UB,Stanson AW, Pulmonary arteriovenous fistulas: Mayo Clinic experience, 1982-1997. Mayo Clinic proceedings. 1999 Jul; [PubMed PMID: 10405695]|
|||Cottin V,Dupuis-Girod S,Lesca G,Cordier JF, Pulmonary vascular manifestations of hereditary hemorrhagic telangiectasia (rendu-osler disease). Respiration; international review of thoracic diseases. 2007; [PubMed PMID: 17641482]|
|||Allen SW,Whitfield JM,Clarke DR,Sujansky E,Wiggins JW, Pulmonary arteriovenous malformation in the newborn: a familial case. Pediatric cardiology. 1993 Jan; [PubMed PMID: 8456028]|
|||Shovlin CL, Pulmonary arteriovenous malformations. American journal of respiratory and critical care medicine. 2014 Dec 1; [PubMed PMID: 25420112]|
|||Vase P,Holm M,Arendrup H, Pulmonary arteriovenous fistulas in hereditary hemorrhagic telangiectasia. Acta medica Scandinavica. 1985; [PubMed PMID: 4050544]|
|||HODGSON CH,BURCHELL HB,GOOD CA,CLAGETT OT, Hereditary hemorrhagic telangiectasia and pulmonary arteriovenous fistula: survey of a large family. The New England journal of medicine. 1959 Sep 24; [PubMed PMID: 14402245]|
|||BOSHER LH Jr,BLAKE DA,BYRD BR, An analysis of the pathologic anatomy of pulmonary arteriovenous aneurysms with particular reference to the applicability of local excision. Surgery. 1959 Jan; [PubMed PMID: 13624986]|
|||White RI Jr,Lynch-Nyhan A,Terry P,Buescher PC,Farmlett EJ,Charnas L,Shuman K,Kim W,Kinnison M,Mitchell SE, Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy. Radiology. 1988 Dec; [PubMed PMID: 3186989]|
|||MURI JW, Arteriovenous aneurysm of the lung. American journal of surgery. 1955 Jan; [PubMed PMID: 13218240]|
|||Berg JN,Gallione CJ,Stenzel TT,Johnson DW,Allen WP,Schwartz CE,Jackson CE,Porteous ME,Marchuk DA, The activin receptor-like kinase 1 gene: genomic structure and mutations in hereditary hemorrhagic telangiectasia type 2. American journal of human genetics. 1997 Jul; [PubMed PMID: 9245985]|
|||McAllister KA,Grogg KM,Johnson DW,Gallione CJ,Baldwin MA,Jackson CE,Helmbold EA,Markel DS,McKinnon WC,Murrell J, Endoglin, a TGF-beta binding protein of endothelial cells, is the gene for hereditary haemorrhagic telangiectasia type 1. Nature genetics. 1994 Dec; [PubMed PMID: 7894484]|
|||ANABTAWI IN,ELLISON RG,ELLISON LT, PULMONARY ARTERIOVENOUS ANEURYSMS AND FISTULAS. ANATOMICAL VARIATIONS, EMBRYOLOGY, AND CLASSIFICATION. The Annals of thoracic surgery. 1965 May; [PubMed PMID: 14301427]|
|||STORK WJ, Pulmonary arteriovenous fistulas. The American journal of roentgenology, radium therapy, and nuclear medicine. 1955 Sep; [PubMed PMID: 13249010]|
|||STRINGER CJ,STANLEY AL,BATES RC,SUMMERS JE, Pulmonary arteriovenous fistula. American journal of surgery. 1955 May; [PubMed PMID: 14361892]|
|||Robin ED,Laman D,Horn BR,Theodore J, Platypnea related to orthodeoxia caused by true vascular lung shunts. The New England journal of medicine. 1976 Apr 22; [PubMed PMID: 1256486]|
|||Chilvers ER,Whyte MK,Jackson JE,Allison DJ,Hughes JM, Effect of percutaneous transcatheter embolization on pulmonary function, right-to-left shunt, and arterial oxygenation in patients with pulmonary arteriovenous malformations. The American review of respiratory disease. 1990 Aug; [PubMed PMID: 2382905]|
|||Lacombe P,Lacout A,Marcy PY,Binsse S,Sellier J,Bensalah M,Chinet T,Bourgault-Villada I,Blivet S,Roume J,Lesur G,Blondel JH,Fagnou C,Ozanne A,Chagnon S,El Hajjam M, Diagnosis and treatment of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: An overview. Diagnostic and interventional imaging. 2013 Sep; [PubMed PMID: 23763987]|
|||Kjeldsen AD,Oxhøj H,Andersen PE,Elle B,Jacobsen JP,Vase P, Pulmonary arteriovenous malformations: screening procedures and pulmonary angiography in patients with hereditary hemorrhagic telangiectasia. Chest. 1999 Aug; [PubMed PMID: 10453873]|
|||Karam C,Sellier J,Mansencal N,Fagnou C,Blivet S,Chinet T,Lacombe P,Dubourg O, Reliability of contrast echocardiography to rule out pulmonary arteriovenous malformations and avoid CT irradiation in pediatric patients with hereditary hemorrhagic telangiectasia. Echocardiography (Mount Kisco, N.Y.). 2015 Jan [PubMed PMID: 24813063]|
|||Nanthakumar K,Graham AT,Robinson TI,Grande P,Pugash RA,Clarke JA,Hutchison SJ,Mandzia JL,Hyland RH,Faughnan ME, Contrast echocardiography for detection of pulmonary arteriovenous malformations. American heart journal. 2001 Feb; [PubMed PMID: 11174338]|
|||Velthuis S,Vorselaars VMM,van Gent MWF,Westermann CJJ,Snijder RJ,Mager JJ,Post MC, Role of transthoracic contrast echocardiography in the clinical diagnosis of hereditary hemorrhagic telangiectasia. Chest. 2013 Dec; [PubMed PMID: 23907523]|
|||Remy J,Remy-Jardin M,Wattinne L,Deffontaines C, Pulmonary arteriovenous malformations: evaluation with CT of the chest before and after treatment. Radiology. 1992 Mar; [PubMed PMID: 1535899]|
|||McAllister KA,Lennon F,Bowles-Biesecker B,McKinnon WC,Helmbold EA,Markel DS,Jackson CE,Guttmacher AE,Pericak-Vance MA,Marchuk DA, Genetic heterogeneity in hereditary haemorrhagic telangiectasia: possible correlation with clinical phenotype. Journal of medical genetics. 1994 Dec; [PubMed PMID: 7891374]|
|||Haitjema T,Disch F,Overtoom TT,Westermann CJ,Lammers JW, Screening family members of patients with hereditary hemorrhagic telangiectasia. The American journal of medicine. 1995 Nov; [PubMed PMID: 7485210]|
|||Faughnan ME,Palda VA,Garcia-Tsao G,Geisthoff UW,McDonald J,Proctor DD,Spears J,Brown DH,Buscarini E,Chesnutt MS,Cottin V,Ganguly A,Gossage JR,Guttmacher AE,Hyland RH,Kennedy SJ,Korzenik J,Mager JJ,Ozanne AP,Piccirillo JF,Picus D,Plauchu H,Porteous ME,Pyeritz RE,Ross DA,Sabba C,Swanson K,Terry P,Wallace MC,Westermann CJ,White RI,Young LH,Zarrabeitia R, International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. Journal of medical genetics. 2011 Feb; [PubMed PMID: 19553198]|
|||Trerotola SO,Pyeritz RE, PAVM embolization: an update. AJR. American journal of roentgenology. 2010 Oct; [PubMed PMID: 20858807]|
|||Iqbal M,Rossoff LJ,Steinberg HN,Marzouk KA,Siegel DN, Pulmonary arteriovenous malformations: a clinical review. Postgraduate medical journal. 2000 Jul; [PubMed PMID: 10878194]|
|||White RI Jr,Pollak JS,Wirth JA, Pulmonary arteriovenous malformations: diagnosis and transcatheter embolotherapy. Journal of vascular and interventional radiology : JVIR. 1996 Nov-Dec; [PubMed PMID: 8951745]|
|||Poulikakos D,Theti D,Pau V,Banerjee D,Jones D, The impact of arteriovenous fistula creation in pulmonary hypertension: measurement of pulmonary pressures by right heart catheterization in a patient with respiratory failure following arteriovenous fistula creation. Hemodialysis international. International Symposium on Home Hemodialysis. 2012 Oct [PubMed PMID: 22360582]|
|||Shovlin C,Bamford K,Wray D, Post-NICE 2008: Antibiotic prophylaxis prior to dental procedures for patients with pulmonary arteriovenous malformations (PAVMs) and hereditary haemorrhagic telangiectasia. British dental journal. 2008 Nov 22; [PubMed PMID: 19023305]|
|||Faughnan ME,Lui YW,Wirth JA,Pugash RA,Redelmeier DA,Hyland RH,White RI Jr, Diffuse pulmonary arteriovenous malformations: characteristics and prognosis. Chest. 2000 Jan; [PubMed PMID: 10631195]|
|||Dupuis-Girod S,Ginon I,Saurin JC,Marion D,Guillot E,Decullier E,Roux A,Carette MF,Gilbert-Dussardier B,Hatron PY,Lacombe P,Lorcerie B,Rivière S,Corre R,Giraud S,Bailly S,Paintaud G,Ternant D,Valette PJ,Plauchu H,Faure F, Bevacizumab in patients with hereditary hemorrhagic telangiectasia and severe hepatic vascular malformations and high cardiac output. JAMA. 2012 Mar 7 [PubMed PMID: 22396517]|
|||Saboo SS,Chamarthy M,Bhalla S,Park H,Sutphin P,Kay F,Battaile J,Kalva SP, Pulmonary arteriovenous malformations: diagnosis. Cardiovascular diagnosis and therapy. 2018 Jun; [PubMed PMID: 30057879]|
|||Gill SS,Roddie ME,Shovlin CL,Jackson JE, Pulmonary arteriovenous malformations and their mimics. Clinical radiology. 2015 Jan; [PubMed PMID: 25443645]|
|||Sluiter-Eringa H,Orie NG,Sluiter HJ, Pulmonary arteriovenous fistula. Diagnosis and prognosis in noncomplainant patients. The American review of respiratory disease. 1969 Aug; [PubMed PMID: 4896450]|
|||Puskas JD,Allen MS,Moncure AC,Wain JC Jr,Hilgenberg AD,Wright C,Grillo HC,Mathisen DJ, Pulmonary arteriovenous malformations: therapeutic options. The Annals of thoracic surgery. 1993 Aug; [PubMed PMID: 8347006]|
|||Chick JFB,Reddy SN,Pyeritz RE,Trerotola SO, A Survey of Pulmonary Arteriovenous Malformation Screening, Management, and Follow-Up in Hereditary Hemorrhagic Telangiectasia Centers of Excellence. Cardiovascular and interventional radiology. 2017 Jul; [PubMed PMID: 28188364]|