Images of the month 1: Ischaemic stroke due to pulmonary arteriovenous fistula (2024)

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Shunpeng Xu, Mingfan Hong and Zhihua Zhou

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DOI: https://doi.org/10.7861/clinmed.2022-0092

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Clin Med May 2022

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ABSTRACT

A 38-year-old man was admitted to the hospital due to a “suddenly developed right hemiplegia, unconsciousness and gaze to the right”. Pulmonary arteriovenous fistulas (PAVFs) are rare but an important cause of stroke in young people, which is easy to be clinically neglected. Therefore, for young patients with pulmonary diseases and cerebral infarction, the possibility of PAVF should be considered.

KEYWORDS:

  • ischaemic stroke
  • pulmonary arteriovenous fistula
  • right-to-left shunt

Case presentation

A 38-year-old man was admitted to the hospital due to a ‘suddenly developed right hemiplegia, unconsciousness and gaze to the right’. The patient had a 19-year history of recurrent pulmonary embolism. A neurological examination showed delirium and the right limb was plegic (grade 0). No obvious abnormality was found in the rest of the nervous system. Computed tomography (CT) of the head showed a high density of the left middle cerebral artery and swelling of left cerebral hemisphere (Fig 1a). Pulmonary angiography demonstrated a pulmonary arteriovenous fistula (PAVF) in the left bronchial artery (Fig 1b). Digital subtraction angiography (DSA) showed occlusion of the left internal carotid artery (Fig 1c). Laboratory examinations revealed abnormal coagulation, such as D-dimer of 20 μg/mL, activated partial thrombin time of 34.0 seconds and prothrombin time of 14.7 seconds. He was not tested for the thrombophilia gene. During the period of hospitalisation, the patient received comprehensive treatment, including vascular intervention therapy, reduce cranial pressure, lowering blood lipid, nerve rehabilitation and so on. Sadly, despite treatments, the patient died as a result of cerebral herniation.

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Fig 1.

a) Computed tomography of the head showing high density of left middle cerebral artery and swelling of left cerebral hemisphere. b) Pulmonary angiography demonstrating a pulmonary arteriovenous fistula in the left bronchial artery. c) Digital subtraction angiography revealing occlusion of the left internal carotid artery.

Discussion

Right-to-left shunt (RLS) is considered to be the main cause of death in young and middle-aged patients with ischaemic stroke. RLS can be divided into intracardiac and extracardiac types. The former includes patent foramen ovale, atrial septal defect and ventricular septal defect, the latter includes PAVF, patent ductus arteriosus and so on. Among them, ischaemic stroke due to PAVF is rare, so there is a lack of clinical awareness and vigilance.1 PAVF, a vessel alformation connecting the pulmonary circulation to the systemic circulation while bypassing the pulmonary capillaries, can cause paradoxical cerebral infarction.2

Pulmonary artery digital angiography is the gold standard for diagnosing PAVF, which can provide information such as the size and number of lesion sites. Multi-detector row CT enhancement can not only reveal the lesion itself and corresponding blood vessels but also detect smaller lesions, and can be used to interpret peripheral and complex PAVF more accurately.4 In this case, the patient had abnormal coagulation and pulmonary angiography showed PAVF in the left bronchial artery. Therefore, the patient had cerebral infarction due to an RLS formed by PAVF.

A study has shown that patients with ischaemic stroke due to PAVF are 15 years younger than patients with routine stroke. Patients who died were 13 years younger than those who died after routin stroke, overall losing 9 extra healthy life-years per patient.3

PAVFs are rare but an important cause of stroke in young people, which is easy to be clinical neglected. Therefore, for young patients with pulmonary diseases and cerebral infarction, the possibility of PAVF should be consid.

  • © Royal College of Physicians 2022. All rights reserved.

References

    1. Belopasova AV

    , Dobrynina LA, Kalashnikova LA, et al. [Pulmonary arteriovenous shunt - a rare cause of recurrent stroke due to paradoxical embolism]. Zh Nevrol Psikhiatr Im S S Korsakova 2020;120:10713.

    OpenUrl

    1. Hasegawa I

    , Abe T, Mino T, et al. Paradoxical Brain Embolism Caused by Isolated Pulmonary Arteriovenous Fistula Successfully Treated with Recombinant Tissue Plasminogen Activator. J Stroke Cerebrovasc Dis 2019;28:e1001.

    OpenUrlPubMed

    1. Topiwala KK

    , Patel SD, Pervez M, Shovlin CL, Alberts MJ. Ischemic stroke in patients with pulmonary arteriovenous fistulas. Stroke 2021;52:e3115.

    OpenUrl

    1. Suga K

    , Iwanaga H, Tokuda O, et al. Steal phenomenon-induced lung perfusion defects in pulmonary arteriovenous fistulas: assessment with automated perfusion SPECT-CT fusion images. Nucl Med Commun 2010;31:8219.

    OpenUrlPubMed

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Images of the month 1: Ischaemic stroke due to pulmonary arteriovenous fistula (2024)

FAQs

Can a fistula cause a stroke? ›

Depending on where the fistula is, it may lead to a stroke. Leg pain due to lack of blood flow (claudication). An arteriovenous fistula can block blood flow to muscles, causing leg pain.

What is pulmonary arteriovenous fistula? ›

A pulmonary arteriovenous fistula is an abnormal blood vessel that creates a potentially troublesome connection between a vein and an artery in the lung. It acts as a detour, sidestepping the normal route the blood is supposed to take. If a patient has symptoms or problems, medical attention is essential.

What are the symptoms of pulmonary arteriovenous shunting? ›

The second most common complaint is dyspnea on exertion, which is more common in patients with large or multiple pulmonary AVMs. Hemoptysis and rarely massive hemoptysis can occur. Less common complaints include chest pain, cough, migraine, headaches, dizziness, dysarthria, syncope, vertigo, and diplopia.

What causes pulmonary fistula? ›

Causes. Pulmonary arteriovenous fistulas are usually the result of abnormal development of the blood vessels of the lung. Most occur in people with hereditary hemorrhagic telangiectasia (HHT). These people often have abnormal blood vessels in many other parts of the body.

What is the most common complication of AV fistula? ›

The most common type of AVF was radiocephalic fistula (RCF). The most common complication associated with the patients with AVF was ischemic neuropathy (29.6%).

What is the most known consequence of fistulas? ›

These holes typically spread the infection that creates them, and life-threatening conditions such as peritonitis (inflammation of the lining of the abdomen) may occur. A fistula is an abnormal connection between an organ, vessel, or intestine and another organ, vessel or intestine, or the skin.

What is the survival rate of arteriovenous fistula? ›

Previous studies reported a wide range of AVF survival rates: 68% to 92% for one-year survival, 57% to 85% for two-year survival, and 4% to 71% for five-year survival.

What is the difference between pulmonary AVM and fistula? ›

Pulmonary arteriovenous fistula is an abnormal connection between an artery and vein in the lungs. As a result, blood passes through the lungs without receiving enough oxygen.

What is the recovery time for an arteriovenous fistula? ›

Arteriovenous fistulas can take between 6-12 weeks to mature and arteriovenous grafts can generally be used 2-3 weeks after surgery.

What is the life expectancy of a pulmonary AVM patient? ›

Survival is therefore significantly higher than the International Society for Heart Lung Transplantation 2016 median survival figures across all pathologies of 5.7 years for adults, 5.4 years for children67](p<0.01) and 10-year survival rates (48%) reported at one UK transplant centre68 (p<0.01).

How does pulmonary AVM cause stroke? ›

Pulmonary arteriovenous malformations may serve as a conduit for deep vein thrombosis to cerebral circulation, thereby causing an acute ischemic stroke.

How rare is pulmonary AVM? ›

PAVM is a rare disorder with an incidence of 2–3 per 1000000 population [1].

Where does a pulmonary arteriovenous fistula most often occur? ›

Most pulmonary arteriovenous malformations (AVMs) are congenital in origin and result when the blood vessels of the lung do not develop normally. There have been reports of acquired lesions, purportedly related to infections, neoplasma, or trauma. AVMs may be single or multiple. They are more common in the lower lobe.

What is the most common cause of an arteriovenous fistula? ›

Causes of Arteriovenous Fistulas

Acquired arteriovenous fistulas often occur when there is trauma to the body's tissue, such as a stabbing wound or gunshot injury. Some patients with late-stage kidney disease may have a fistula surgically created to help with dialysis.

How do you fix a fistula in the lungs? ›

BPFs do not typically spontaneously undergo closure and almost always require some surgical or bronchoscopic intervention. All patients with a BPF should have a chest tube placed to drain air. In cases of an empyema, infected pleural fluid should be drained, and antibiotics should be initiated.

What problems can a fistula cause? ›

Fistulas may result in malnutrition and dehydration, depending on their location in the intestine. They may also cause skin problems and infection.

How serious is a fistula in the brain? ›

Pial or cerebral arteriovenous fistulas occur in the brain. A large one can cause heart failure at or even before birth. Smaller ones can damage the brain around the fistula because it diverts blood flow away from the brain tissue and into the draining vein.

What are the symptoms of a severe fistula? ›

Symptoms of an anal fistula include:
  • skin irritation around the anus.
  • a constant, throbbing pain that may be worse when you sit down, move around, poo or cough.
  • smelly discharge from near your anus.
  • passing pus or blood when you poo.
  • swelling and redness around your anus and a high temperature if you also have an abscess.

How can a fistula be life threatening? ›

Sepsis (also known as blood poisoning or septicaemia) is a potentially life-threatening condition that can occur in people with infections. Sepsis is uncommon in people with simple fistulas, but can occur in more complex cases or where there is a large collection of pus (an abscess) that fails to discharge.

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