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2014 ; 190
(11
): 1217-28
Nephropedia Template TP
Am J Respir Crit Care Med
2014[Dec]; 190
(11
): 1217-28
PMID25420112
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Within the past decade, pulmonary arteriovenous malformations (PAVMs) have
evolved from rare curiosities to not uncommon clinical states, with the latest
estimates suggesting a prevalence of ~1 in 2,600. PAVMs provide anatomic
right-to-left shunts, allowing systemic venous blood to bypass gas exchange and
pulmonary capillary bed processing. Hypoxemia and enhanced ventilatory demands
result, although both are usually asymptomatic. Paradoxical emboli lead to
strokes and cerebral abscesses, and these commonly occur in individuals with
previously undiagnosed PAVMs. PAVM hemorrhage is rare but is the main cause of
maternal death in pregnancy. PAVM occlusion by embolization is the standard of
care to reduce these risks. However, recent data demonstrate that currently
recommended management protocols can result in levels of radiation exposure that
would be classified as harmful. Recent publications also provide a better
appreciation of the hematologic and cardiovascular demands required to maintain
arterial oxygen content and oxygen consumption in hypoxemic patients, identify
patient subgroups at higher risk of complications, and emphasize the proportion
of radiologically visible PAVMs too small to treat by embolization. This review,
therefore, outlines medical states that exacerbate the consequences of PAVMs.
Chief among these is iron deficiency, which is commonly present due to concurrent
hereditary hemorrhagic telangiectasia: iron deficiency impairs hypoxemia
compensations by restricting erythropoiesis and increases the risk of ischemic
strokes. Management of periodontal disease, dental interventions, pulmonary
hypertension, and pregnancy also requires specific consideration in the setting
of PAVMs. The review concludes by discussing to what extent previously
recommended protocols may benefit from modification or revision.
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