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!!