Eisenmenger Syndrome

symptoms of ES? (10)

Cyanosis & Clubbing Dyspnea on exertion Palpitations Onset of atrial fibrillation or atrial flutter Edema Hemoptysis due to pulmonary infarction or rupture of dilated pulmonary vessels Syncope RVH Chronic hypoxemia stimulates erythrocytosis leading to ↑d blood viscosity Bleeding: Thrombocytopenia, platelet dysfunction, clotting cascade abnormalities

what problems are also associated with Eisenmenger's? (8)

Chronic hypoxemia symptoms Polycythemia Coagulopathy Platelet consumption Brain abscesses Cerebral microemboli Hemoptysis: d/t rupture of pulmonary vessels Renal dysfunction

true or false? initial L to R changes are reversible?

true (anywhere from 3 months to years as the vessels stiffen, the disease progresses and can no longer dilate and contract as needed)

pathophysiology of Eisenmenger's syndrome?

- Normally SVR is greater than PVR - This pressure difference maintains the left-to-right shunt direction - A chronic pattern of left-to-right shunt follows for a sustained period of time - This results in chronic ↑s in PBF and pulmonary circulation overload - Replacement of normal pulmonary vessel tissue with fibrosis & smooth muscle tissue - Tissue replacement leads to narrowing & closure of pulmonary vessels - This leads to further ↑s in PVR - L to R shunt then becomes R to L shunt

are IV inductions fast or slow in ES patients? are inhalational agents fast or slow? why?

Arm-to-brain circulation times are rapid d/t R-to-L shunt IV drugs have rapid onset Inhalational agents have slow onset d/t ↓d PBF

what is Eisenmenger syndrome?

Eisenmenger's syndrome occurs when a long-term left-to-right shunt becomes bidirectional or is converted to a right-to-left shunt - pt either has an ASD or VSD defect

EKG changes with ES

RVH with right axis deviation

TEE findings with ES

TEE: Right sided volume overload RA & RV enlargement Decreased RV & LV contractility

true or false? you should always place a PA catheter in Eisenmenger's patients

false. Will ↑ risk of air emboli, thrombi, infection, hematoma. - Risk of pulmonary artery rupture & hemorrhage is high - The patient may not tolerate catheter-induced arrhythmias. - CO & PAP measurements are not helpful because values are generally fixed.

in late stages of ES, will nitric oxide and vasodilating drugs work?

no, the vessels no longer react and the only definitive treatment is heart/lung transplant

is anesthesia safe for pts with ES?

not really. - Avoid changes in SVR (↓d SVR =↑d R-to-L shunt) - Use inhalational agents cautiously - Valsalva maneuvers during labor may ↑ SVR, ↓ systemic blood flow, & cause fatal syncope - Use regional cautiously by bringing epidural levels up slowly & balancing with neo - Avoid spinals - Prevent air in IV lines - Maintain fluid balance & avoid dehydration - Maintain venous return - Prevent ↑s in PVR

when does Eisenmenger's syndrome occur?

when pulmonary vascular resistance (PVR) equals or exceeds systemic vascular resistance (SVR)