대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Chief Complaint

Rt. Side weakness

Definitive Diagnosis

Pulmonary arteriovenous malformation

Reviewy

Structurally abnormal vessels that provide direct capillary-free communication between the pulmonary and systemic circulations
Anatomic “right-to-left” shunt
? Gas exchange, filtration, and other processing of systemic
venous blood are impaired
Frequency : 1 in 2630 (population-wide cancer screening programs)
Etiology
(1) Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber syndrome)
① 30%-40% of family members ; multiple pulmonary AVMs in 5% (AD)
? ENG, endoglin (HHT1), ACVLI/ALK1 (HHT2), or Smad4 (HTJP)
② AVMs and smaller telangiectatic vessels : nasal,
mucocutaneous, pulmonary, hepatic, gastrointestinal,
cerebrovascular beds
(2) Non-HHT
① Sporadic PAVMs
② Treatment of cyanotic congenital heart disease
③ Gestational trophoblastic disease
④ Trauma (arteriovenous fistula)
Classification
Simple (m/c) : a single segmental artery feeding the malformation
Complex : multiple segmental feeding arteries
Diffuse : hundreds of malformations, combination of simple and complex

Diagnosis
Gold standard : CT (contrast enhanced)
Chest radiography : diagnosis & follow up (abnormalities : 98% of patients)
Round or oval sharply defined mass of uniform density, frequently lobulated, and ranging in size from 1-5cm in diameter
Two thirds are located in the lower lobes
Connecting vessels radiating from the hilum
Angiography (angioarchitecture of individual PAVMs)

Keywords