Pulmonary AVM
R1 陳世昱
Name :林○○
Gender :女
Age : 71 Y/O
Date of admission : 90/08/15
Chief complaint : SOB & general weakness during hemodialysis
Present illness :
Discomfort (dyspnea, general weakness and loss of appetite) developed while HD recently
CXR & CT revealed a Pul. nodule over RLL (same with 1997)
Cardiac echo: MR, TR and PHTN
PaO2 in room air: 58mmHg
Past history :
ESRD for 5~6 yrs, under HD 3 times/week for 3 yrs
CHF : Af with LVH
DM : (-) HTN : (?)
Myoma s/p ATH 20+ yrs ago
Renal stones s/p op
Gout attack 4~5 yrs ago
Special findings :L’t arm ecchymosis, but no telangiectasis.
Brain CT : no brain AVM
Abd. Sono : no intrahepatic arterio-portal shunting
Pulmonary MRA : feeding a. from the right inferior PA directly into inferior branch of RPVs.
Catheterization : fail to perform embolization because of the huge size of the PAVM
Impression :
Pulmonary Arterio-venous malformation
麻照 麻單
Review :
Pulmonary Arterio-venous malformation
Incidence: 1/50000
Etiology: unknown(genetic)
Range: diffuse telangiectases to large complex structures consisting of a bulbous aneurysmal sac
Origin: 95% from pulmonary systemtend to increase in size (multiple)
Auto-regress: rare
Mortality: 4~22% (untreat-sympatom)40% (severe case)
S/S:
Complications:
Bleed into a bronchus or pleural cavity
Right-to-left shunts (most common, with
the following embolisms into systemic)
Pulmonary congestion (↓PVR)
DiagnosisCXR:
Moderate sized PAVMs appear as rounded, well circu
mscribed lesions, band shaped shadows resulting fro
m dilated feeding and draining vessels.
It is now recognised that a normal PA and lateral che
st radiograph does not rule out PAVMs, particularly in
patients with small or diffuse malformations.
CT scan:
Helical CT scanning with three-dimensional re
constructions conveniently identifies small, m
ultiple lesions; it can also identify thrombosed
and, with contrast, recanalised structures. At
present NMR screening is less effective than
computed tomographic (CT) scanning or pulm
onary angiography as small PAVMs with rapi
d blood flow are not visualised, but methodolo
gy is improving.
100% inspired oxygen breathing method:
gold standard for non-invasive methods of estimating the size of the shunt (using A-VO2 differences of
5 ml/100 ml and 3.5/100 ml, respectively)
Radionuclide scanning
-technetium-99m (99mTc)-labelled albumin
-87% sensitivity and 61% specificity
All non-invasive methods occasionally fail to detect PAVMs which are subsequently diagnosed by angiography
More commonly the inverse is seen; an abnormally high shunt is detected by non-invasive methods but not at formal pulmonary artery catheter angiography.
TreatmentEmbolisation(1)
-Material:metallic coils, or as a result of blood stasis due to an occluding balloon.
-Safety and efficacy
-Dramatic improvements
-Embolisation is currently recommended for all PAVMs with feeding arteries greater than 3 mm in diameter (some center 2~3mm).
Embolism(2)
-removal of a low resistance shunt may unmask or provoke the development of new PAVMs
-no adequate numerical data support ↓cerebral events
-19% ~ 60% have residual shunts
Surgery
-Surgical resection might be indicated for patients in whom a persistent right-to-left shunt (and embolic risk) persists following embolisation of all feasible vessels.
-Lung transplantation has been proposed for patients with diffuse disease.
Clinical course
Date Issues note1997(?) CXR: RLL nodule 三重 Hosp.
2001.8 Discomfort during HD
CXR、 chest CT, cardiac echo
Brain CT 、 Abd. sono
Pul. MRA: RIPA→RIB of PV NTUH
2001.8.29 Angiography: can’t emboli.
2001.9.03 Thoracotomy c wedge resect.
Thank you for your Attention!!