An arteriovenous fistula is an abnormal connection or passageway between an artery and a vein. It may be congenital, surgically created for hemodialysis treatments, or acquired due to pathologic process, such as trauma or erosion of an arterial aneurysm. These communications can occur at any point in the vascular system; varying in size, length, location, and number.
Historical Perspective
Arteriovenous fistula (AVF) was first discovered by William Hunter, in 1758, during a venupuncture with accidental piercing of the artery.[1][2][3][4]
Congenital arteriovenous fistula (AVF) of the coronary arteries, a rare cardiac anomaly, was first described by Krause W. Uber, in 1865.[5][6][7]
Classification
Arteriovenous fistula (AVF) can be classified into:
Congenital, a developmental anomaly in which there are always multiple fistulas between the arteries and veins[8][3]
Acquired, usually a single communication, which can be the result of an injury or can be a part of treatment for the purpose of hemodialysis.[3][9][10][11]
Pathophysiology
The abnormal communication causes shunting of blood from high-pressure arterial side to the low-pressure venous side.
An arteriovenous fistula (AVF) involving a major artery such as the abdominal aorta can lead to a large decrease in peripheral resistance, which in turn causes the heart to increase cardiac output in order to maintain proper blood flow to all tissues.
The physical manifestations of this would be a relatively normal systolic blood pressure with a decreased diastolic blood pressure resulting in a wide (large) pulse pressure.
Large arteriovenous fistulae (AVF) can lead to congestive heart failure associated with increased activity of vasoconstrictor neurohormonal systems, renin-angiotensin, sympathetic nervous system, endothelin system, and Arginine vasopressin.[12]
The increased activity of vasoconstrictor neurohormonal systems is in concurrence with compensatory activation of systemic and vasodilating systems, atrial natriuretic peptide (ANP) and nitric oxide (NO).
A fistula can progress to an aneurysm, usually in the setting of a trauma, carrying a risk of rupture and necessitating surgical intervention.[13]
The malformation can result in hemorrhage and the risk is significantly higher with small size malformation because of the significantly higher feeding artery pressures.[14]
Clinical Features
In the case of coronary arteriovenous fistula (AVF) patients can be asymptomatic or can present with:
Fistulae involving the spinal vasculature can present with neurologic symptoms of the lower extremities which may include weakness and sensory disturbance.[18]
Acquired arteriovenous fistula (AVF) can result in digital clubbing.[19]
Patients on hemodialysis with arteriovenous fistula (AVF) can develop pseudo-Kaposi's sarcoma.[20]
Abdominal arteriovenous fistulae can present with:
Ilio-iliac arteriovenous fistula (AVF) presents with progressive abdominal distention, dyspnea, and swollen leg, which makes it difficult to distinguish from deep venous thrombosis (DVT) and can lead to a delay in the diagnosis.[22]
Tentorial dural arteriovenous fistula (AVF) can rarely manifest as trigeminal neuralgia.[23]
Differentiating Arteriovenous Fistula (AVF) from other Conditions
It is important to differentiate an arteriovenous fistula (AVF) from other conditions that cause a hyperdynamic circulation such as:
Prognosis of arteriovenous fistulae is dependent on the system involved with many congenital fistulae undergoing regression and large fistulae may progress to cardiac decompensation and death.
Diagnosis
Diagnostic Criteria
An arteriovenous fistula (AVF) can be detected by a stethoscope and can present with a machinery murmur.[120][121][18][122]
A more superficial fistula can also be detected by palpation, presenting as continuous vibration.[123]
Imaging is the modality of choice to effectively diagnose an arteriovenous fistula (AVF) and it includes:
Decreased platelets and fibrinogen in the case of large fistulae[155]
Swan-Ganz catheter findings in pulmonary arteriovenous fistula (AVF) include decreased arterial oxygenation and shunting of cardiac output.[156]
Swan-Ganz catheter findings in aorto-caval fistula include higher vena caval pressure and higher than normal oxygen content.[154]
Patients on hemodialysis suffering from occlusive and thrombotic events of the arteriovenous fistula (AVF) may exhibit significant increase of platelet surface glycoproteins GPIb and GPIIb/IIIa.[157]
Large fistulae can also present with laboratory findings of consumptive coagulopathy such as, low platelets, increased bleeding time, elevated prothrombin time (PT) and partial thromboplastin time (PTT), and signs of enhanced fibrinolysis.[155][158]
Imaging Findings
Computed tomography angiography (CTA) and magnetic resonance angiogrpahy (MRA) are the imaging modalities of choice for arteriovenous fistula (AVF) with modest evidence that computed tomography angiography (CTA) is better than magnetic resonance (MR) angiogrpahy.[129]
On plain radiograph, an arteriovenous fistula (AVF) is characterized by a round or oval lobulated homogeneous mass and the identification of feeding and draining vessels can also be be possible.[159]
On ultrasound, an arteriovenous fistula (AVF) is characterized by a pseudoaneurysm, high flow velocity, increased diastolic arterial flow, abnormal arterial pulsitility in the vein, and enlargement of the downstream vein.[160][161][162][163]
On computed tomography angiogrphy (CTA) and magnetic resonance angiography (MRA), an arteriovenous fistula is characterized by abnormal dilatation along with early and flow-related enhancements.[164][129]
On digital subtraction arteriography (DSA), an arteriovenous fistula (AVF) is characterized by abnormal early filling of an adjacent vein in the region of the fistula.[165]
Other Diagnostic Studies
An arteriovenous fistula (AVF) involving the heart and pulmonary vasculature in some cases, can also be diagnosed via echocardiography.[166][167][168]
Findings on echocardiography may include, dilated lumen and abnormal unidirectional continuous flow signals with broad velocity spectra[168]
An aorto-caval fistula may also be diagnosed using Swan-Ganz catheter, demonstrating higher vena caval pressure and higher than normal oxygen content.
Treatment
Medical Therapy
There is no medical treatment for an arteriovenous fistula (AVF).
Iatrogenic arteriovenous fistulae, presenting only as pain, can resolve after a trial of compression and observation.[169][169]
Asymptomatic fistulae can resolve spontaneously and most lesions can be monitored after detection especially if the size of the lesion is small and they do not cause adverse effects.[170]
Conservative management has been tried with a successful outcome in the case of an arteriovenous fistula (AVF) as a complication of vascular catheterization.[169]
Surgery
Surgery is the mainstay of therapy for an arteriovenous fistula (AVF).
Endovascular embolization is the most common approach to the treatment of an arteriovenous fistula (AVF), where a catheter is inserted into an artery and, guided by fluoroscopic or x-ray imaging, contrast is injected to visualize the vasculature followed by the injection of an embolization material (such as, particles, liquid embolics, embolization glue, detachable balloons, vascular plugs, and coils) into the exact location where the artery and the vein meet.[171][172][173][174][175][176][177]
Microsurgery, with or without endovascular embolization, is the most appropriate treatment for dural, brain, or spinal arteriovenous fistulae, where a titanium clip is placed to cut off the connection between the artery and the vein.[175][178][179][180]
Stereotactic radiosurgery, the treatment of choice to successfully obliterate an arteriovenous fistula (AVF) with low complication rates and hence preferred over endovascular embolization and microsurgery in the case of an arteriovenous fistula close to important brain structures.[181][180][182][183][184]
”2. A large coronary arteriovenous fistula (CAVF), regardless of symptomatology, should be closed via either a transcatheter or surgical route after delineation of its course and its potential to fully obliterate the fistula. (Level of Evidence: C)"
”3. A small to moderate CAVF in the presence of documented myocardial ischemia, arrhythmia, otherwise unexplained ventricular systolic or diastolic dysfunction or enlargement, or endarteritis should be closed via either a transcatheter or surgical approach after delineation of its course and its potential to fully obliterate the fistula. (Level of Evidence: C)"
"1. Clinical follow-up with echocardiography every 3 to 5 years can be useful for patients with small, asymptomatic CAVF to exclude development of symptoms or arrhythmias or progression of size or chamber enlargement that might alter management. (Level of Evidence: C)"
"1. Surgeons with training and expertise in CHD should perform operations for management of patients with CAVF. (Level of Evidence: C)"
”2. Transcatheter closure of CAVF should be performed only in centers with expertise in such procedures. (Level of Evidence: C)"
”3. Transcatheter delineation of CAVF course and access to distal drainage should be performed in all patients with audible continuous murmur and recognition of CAVF. (Level of Evidence: C)"
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