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 PaO 2 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 system tend 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)

Diagnosis CXR : Moderate sized PAVMs appear as rounded, well circumscribed lesions, band shaped shadows resulting from dilated feeding and draining vessels. It is now recognised that a normal PA and lateral chest radiograph does not rule out PAVMs, particularly in patients with small or diffuse malformations.

CT scan: Helical CT scanning with three-dimensional reconstructions conveniently identifies small, multiple lesions; it can also identify thrombosed and, with contrast, recanalised structures. At present NMR screening is less effective than computed tomographic (CT) scanning or pulmonary angiography as small PAVMs with rapid blood flow are not visualised, but methodology is improving.

100% inspired oxygen breathing method: gold standard for non-invasive methods of estimating the size of the shunt (using A-VO 2 differences of 5 ml/100 ml and 3.5/100 ml, respectively)

Radionuclide scanning -technetium-99m ( 99m Tc)-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.

Treatment Embolisation(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 DateIssuesnote 1997(?)CXR: RLL nodule 三重 Hosp Discomfort during HD CXR 、 chest CT, cardiac echo Brain CT 、 Abd. sono Pul. MRA: RIPA→RIB of PVNTUH Angiography: can ’ t emboli Thoracotomy c wedge resect.

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