The Journal of International Medical Research

2005; 33: 467 – 471

Surgical Treatment of Pulmonary

Arteriovenous Malformation:
Report of Two Cases and Review
of the Literature
K METIN1, M KARAÇELIK3, Ö YAVAÇCAN4, M ÇELIK2, N ÇETIN4,
MC DORAK4, Ö OTO1 AND N AKSU4

1
Department of Cardiovascular Surgery and 2Department of Anaesthesiology, Ege Sağlik
Hospital, Izmir, Turkey; 3Department of Cardiovascular Surgery, SSK Tepecik Hospital, Izmir,
Turkey; 4Department of Paediatrics, SSK Tepecik Training and Education Hospital, Izmir, Turkey

Pulmonary arteriovenous malformations diameter PAVM with one feeding artery

(PAVMs) are rare anomalies. The degree
of right-to-left shunting, which can lead
to cyanosis and paradoxical embolism
causing neurological complications,
determines the prognosis. We report two
cases of PAVM and review the literature. A
45-year-old woman with clinical signs and
symptoms of PAVM was examined using
several different scanning techniques,
which showed a large PAVM in the lower
lobe of her right lung. A lobectomy was
performed, which revealed a 5 cm
and multiple veins. Intravenous angio-
graphy of a 7-year-old girl with symptoms
of fatigue and acro-cyanosis confirmed the
presence of a large PAVM in her right lower
lobe. The PAVM had two major arteries
arising from the aorta, which were ligated
during a lobectomy. Both patients
recovered well following surgery. Although
PAVMs are rare, their neurological and
haemodynamic consequences may be
fatal. Interventional treatment techniques,
including surgery, are usually curative.

KEY WORDS: PULMONARY ARTERIOVENOUS MALFORMATION; HEREDITARY HAEMORRHAGIC

TELANGIECTASIA; CYANOSIS

Introduction in neurological complications, determines the

Pulmonary arteriovenous malformations
(PAVMs) are rare, inherited anomalies, mostly
manifested in young adults.1,2 They may be
very small connections or a complex of
arteriovenous shunts between the pulmonary
arterial tree and pulmonary veins. The degree
of right-to-left shunting, which can lead to
cyanosis and paradoxical embolism resulting
clinical status of the patient and prognosis of
the pathology.1 – 3
Pulmonary arteriovenous malformation
is only reported in sporadic case reports as it
is rare and seldom accurately diagnosed pre-
mortem. Here we report on two cases where
the patients had symptoms of PAVM and
who were treated successfully with surgery.

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 K Metin, M Karaçelik, Ö Yavaçcan et al.
Surgery for pulmonary arteriovenous malformation

Case reports underdeveloped (weight 22 kg [25 –

50 percentile] and height 123 cm [50 –
CASE 1
75 percentile]). All findings during the
A 45-year-old woman with fatigue, headache,
physical examination were normal except
swollen legs and numbness in her lower
for cyanosis and digital clubbing. The
extremities had right-sided hemiparesia and
patient had a high haematocrit level
attacks of epilepsy for more than 30 years. She
(58.9%), and her arterial oxygen saturation
was tachypneic and mildly cyanotic with
was 73% with a PO2 value of 44.9 mmHg in
digital clubbing. A continuous murmur below
standard room air (21% oxygen).
the right scapula and non-pitting oedema on
A chest X-ray revealed a right lower lobe
both legs were noted. She was slightly
density and a helical CT scan showed a mass
anaemic (haemoglobin: 11.2 g/dl).
in her right lower lobe (Fig. 1). Intravenous
A standard chest X-ray showed an
angiography confirmed the presence of a
increased sub-pleural density on the lower
rapidly opacified large PAVM in the right
right lung at the supradiaphragmatic
lower lobe. The total body Tc-99m macro-
junction. A calcified, lobular lesion 4 cm in
aggregated albumin scintigraphy excluded
diameter was visualized on a computed
the diagnosis of hereditary haemorrhagic
tomography (CT) scan, and a ventilation-
telangiectasia (HHT).
perfusion scan of the lungs showed large
amounts of radiopharmaceutical media in
her liver. A duplex scan of her lower
extremities revealed chronic clotting on the
right common femoral vein wall with a
reduction in blood flow in the one-third
distal portions of the right femoral and
popliteal veins. An intravenous digital
subtraction angiography study revealed a
large PAVM in the lower lobe of her right
lung. There were chronic ischaemic patterns
on the left occipitoparietotemporal lobe of Heart
the brain, i.e. the feeding area of the left
internal carotid artery, on CT and magnetic
resonance imaging scans. PAVM
Right-sided lower lobectomy was performed A
o
and a pathological examination revealed a r
large (5 cm diameter) arteriovenous mal- t
a
formation with one feeding artery and
multiple veins. The clinical status of the pa-
tient improved immediately following surgery. FIGURE 1: A helical computed
tomography image of the chest of
a 7-year-old girl showing a mass
CASE 2 in the right lower lobe of her lung,
A 7-year-old girl who complained of fatigue which was confirmed by intra-
(for 1 year) and acro-cyanosis (for 4 years) venous angiography to be a
large pulmonary arteriovenous
was admitted as a result of her severely
malformation (PAVM)
limited capacity for exercise. She was slightly

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468
 K Metin, M Karaçelik, Ö Yavaçcan et al.
Surgery for pulmonary arteriovenous malformation
A right lower lobectomy was performed.
Two major arteries arising from the aorta
were detected and ligated (Fig. 2). The
hypoxic status of the patient improved
immediately following surgery; she was
discharged on post-operative day 6 with
normal arterial oxygen saturation, and is
currently doing well 5 months post-surgery.
Discussion
Following the first description of a post-
mortem PAVM on a 12-year-old boy in 1897,
this pathology has been described using
various nomenclature, including: pulmonary
arteriovenous fistula; pulmonary arterio-
venous aneurysm; haemangioma of the
lung; cavernous angioma of the lung;
pulmonary telangiectasia; and PAVM.4,5 The
congenital form has two types: a cavernous
angioma, usually fed by one or more
tortuous dilated pulmonary artery branches;
or capillary telangiectasia, which is formed
by a complex of capillaries and is usually
associated with HHT (Rendu-Osler-Weber
syndrome), an autosomal-dominant
vascular dysplasia affecting one in 10 000
individuals.1,2,6 Approximately one-third of
Major artery Vena azygos
Major artery
FIGURE 2: A photograph showing
the operative field and two major
arteries arising from the aorta,
which were ligated during the
surgical procedure to remove
the right lower lobe in a 7-year-old
girl with a large pulmonary
arteriovenous malformation
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469
patients with HHT have pulmonary vascular
malformations and 70% of PAVMs are
associated with HHT.1,2,6,7 In both of our
patients the PAVM was cavernous, as
observed during surgery and confirmed by
histological examination.
The development of PAVMs is linked to
the mutation of two genes, endoglin and
ALK-1.2,6 The embryological differentiation
of the vascular bed occurs between the fifth
and tenth intrauterine weeks, which results
in separate arterial and venous vessels
interconnected with capillaries.1 PAVMs are
a result of an error in or premature
termination of this process. Haemodynamic
disturbances may occur in patients with
larger shunts where massive capillary
involvement is present.1 The consequences
of an intrapulmonary shunt are arterial
desaturation, cyanosis, clubbing of the
fingertips and polycythaemia (or anaemia
in rare cases).1 Due to the pathological right-
to-left shunting and absence of capillary
filtration, small blood clots and bacteria
may pass directly into the systemic
circulation and PAVMs may lead to clinically
dangerous paradoxical septic emboli.6,8
Pulmonary arteriovenous malformations
occur twice as often in women as in men, but
there is a male predominance reported in
newborns.7 Although PAVMs may occur in
childhood, their clinical symptoms appear
during adolescence or adult life.2,6 Usually,
PAVMs < 2 cm in size are asymptomatic.7 As
observed in our two cases, the vast majority
(53 – 70%) of PAVMs are located in the lower
lobes, where 75% are unilateral.5,8 – 12 Eighty
to 90% of cases are simple PAVMs with single
feeding and draining vessels.7 Classically,
drainage is to the left atrium, but in rare
cases, inferior vena caval or innominate
venous drainage has been reported.5,13
The most common symptom of PAVMs is
epistaxis. Dyspnoea, the second most
 K Metin, M Karaçelik, Ö Yavaçcan et al.
Surgery for pulmonary arteriovenous malformation
common complaint, reflects the size or
number of PAVMs. Haemoptysis, rarely
massive and fatal, might also be seen. Chest
pain, headache, cough, tinnitus, dizziness,
dysarthria, syncope, diplopia and vertigo are
more rare complaints.7 Murmurs over the
chest are audible in approximately 50%
of cases. Cyanosis, digital clubbing and
dyspnoea have been reported as a diagnostic
triad in classical textbooks.
A round or oval mass commonly in the
lower lobes, between 1 and 5 cm in diameter,
is the classical view on a standard X-ray,7
although CT scanning is much more useful at
identifying the vascular structure of the lesion.
The feeding artery may be visible as radiating
from the hilus, and the draining veins are
usually directed to the left atrium.7 PAVMs
consist of two basic elements: thin walled
vascular channels, which are lined with
a single layer of endothelium; and the
connective tissue stroma.5 There are three
typical appearances of these malformations:
a large single sac; a plexus of vascular
channels; or a direct and tortuous communi-
cation between artery and vein.10,13,14
Neurological, rather than pulmonary,
complications are the rule in patients
with PAVMs. 3 Important neurological
complications (particularly stroke or brain
abscess) are due to embolic, haemorrhagic
and infectious consequences. 1,3 Approxi-
mately 30% of patients with PAVMs have a
history of stroke, 10% have brain abscess
and 10% have pulmonary or pleural
haemorrhage. 3 Other complications are
migraine headache, haemothorax (rupture
of a sub-pleural PAVM), life-threatening
haemoptysis (ruptured PAVM or endo-
bronchial telangiectasia), pulmonary
hypertension, congestive heart failure,
polycythaemia (25%), anaemia (17%) and
infectious endocarditis.7
The diagnosis of HHT is based on a clinical
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470
questionnaire and findings, including:
epistaxis; telangiectasias; visceral lesions; and
a family history.1 The clinical characteristics
of these patients when left untreated are not
known. Generally these cases have an
exercise intolerance and profound cyanosis.
Most of them appear to have a poor
prognosis. Brain abscess is the result of
microbacterial seeding, and polycythaemia is
secondary to hypoxia.3 Cases with diffuse
PAVM have a high risk of developing brain
abscesses (38%).3 The right-to-left shunt
fraction is highly elevated in patients with a
severe and diffuse pattern of PAVMs, being
88 – 100% (≤ 5% in normal individuals).15 – 17
Contrast echocardiography with an injection
of 5 – 10 ml of indocyanine is a highly
selective diagnostic tool. Three-dimensional
helical CT is a superior non-invasive method
with an accuracy of up to 95%.18 Pulmonary
angiography is still the gold standard in
diagnostic techniques7 and is mandatory if
surgical or obliterative therapy is planned in
patients with PAVMs.
When left untreated, the majority of
PAVMs remain unchanged in size, although
25% of cases gradually enlarge.9,19 PAVM-
related mortality ranges from 0% to 55%,
with the vast majority of fatalities due to
cerebral complications.7 Treatment of a PAVM
is indicated in patients with persistent hypox-
aemia and for prevention of neurological
sequelae. Extended and complex PAVMs must
be treated surgically, but interventional
embolotherapy may be an alternative
technique for simple PAVM with relatively
narrower feeding arteries.6 Embolization is
usually limited to cases with relatively smaller
sized PAVMs. Surgery involves ligation or
excision of the affected part (segment, lobe or
hemi-lung) and carries the same risks of any
similar thoracic surgical procedure. Post-
operative follow-up shows recurrence in
0 – 12% of surgically treated cases.7
 K Metin, M Karaçelik, Ö Yavaçcan et al.
Surgery for pulmonary arteriovenous malformation
Although PAVMs are rare, their neuro-
logical and haemodynamic consequences
may be fatal, so it is important to offer
interventional treatment techniques,
including surgery, which are usually curative.
The clinical status of both our patients, who
had large PAVMs in their lower right lobes,
• Received for publication 31 January 2005 • Accepted subject to revision 25 February 2005
• Revised accepted 11 March 2005
Copyright © 2005 Cambridge Medical Publications
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Address for correspondence
Dr K Metin
Mithatpaşa Cad. No:653/3, 35280 Izmir, Turkey.
E-mail: kmetin@yahoo.com
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Conflicts of interest
No conflicts of interest were declared in
relation to this article.
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