Eisenmenger syndrome blue book article Flashcards
What’s eisenmenger syndrome?
a severe phenotype of pulmonary HTN resulting in R) to L) flow through an intracardiac or aorto-pulmonary shunt, usually from congenital cardiac disease
What proportion of patients with congenital heart disease survive into adulthood?
> 85%
While most children with CHD do not develop pulm HTN due to advances in diagnostic & surgical procedures, who may some surviving into adulthood still develop PAH?
ineffective pulmonary artery banding or residual shunts, rare in high income countries (increased rates of detection & Rx of abnormal circulatory communications)
When eisenmenger syndrome develops, is repair of the underlying defect indicated?
No- largely contraindicated
What WHO class of pregnancy would eisenmenger syndrome fall into? maternal mortality?
IV- pregnancy contraindicated (as it’s pulmonary HTN)- high risk maternal mortality & severe morbidity. If pregnancy occurs termination should be discussed. If it proceeds, care for as per class III (intensive specialist cardiac & obstetric monitoring required throughout pregnancy, childbirth & postnatally)
30-70%
What’s the commonest presentation of congenital cyanotic heart disease in adults & children?
eisenmenger syndrome in adults
tetralogy of fallot in children
Within which of the WHO pHTN categories does Eisenmenger syndrome fall?
group 1
What’s the first step in the pathophysiological cascade leading to eisenmenger syndrome? how does it proceed?
systemic-to-pulmonary shunt
increases pulmonary blood flow/pressure, endothelial dysfunction & pulm vascular remodelling, incr PVR, inverted shunt: pulmonary to systemic, cyanosis, eisenmenger
Roughly what proportion of pts with unrepaired VSD, unrepaired ASD & unrepaired truncus arteriosus are @ risk of developing Eisenmenger syndrome?
50%, 10% & almost all
Is the incidence of pulmonary arterial HTN greater in pts with pre-tricuspid or post-tricuspid shunts?
post-tricuspid, as there’s both a combined pressure & volume overload on the RV (as opposed to pre-tricuspid such as ASD or anomalous pulmonary venous return, where it’s predominantly a volume overload); post-tricuspid shunts generally diagnosed in infancy, pre-tricuspid shunts in adulthood. Eg. post-tricuspid include VSD, PDA or complex such as AV septal.
Which syndrome has particular propensity to develop pulm HTN & Eisenmenger?
Down syndrome, although with early screening & Rx this has decreased
What are poor prognostic predictors in pts with eisenmenger?
age pre-tricuspid shunt lower resting SpO2 absence of sinus rhythm pericardial effusion
Which factors are not identified to confer prognostic risk in eisenmenger?
functional status (NYHA)
RV function (TAPSE on TOE)
lab data
use of pulm arterial vasodilators
Describe the clinical picture of Eisenmenger syndrome?
Multi-system disease central cyanosis dyspnoea fatigue haemoptysis syncope RHF with progressive functional deterioration over time
Renal dysfunction common: incl pre-renal from low CO & secondary to glomerular abnormalities due to hypoxaemia & hyperuricaemia
Coagulation derangements: plt dysfunction (incr turnover & aggregation)
elevated plasma vWF:Ag
Vit K-dependent factor deficiency
Factor V & vWF deficiency
hypofibrinoginaemia & increased fibrinolysis
Erythrocytosis from chronic hypoxaemia; predisposed to bleeding & thrombosis
Increased risk to calcium bilirubinate gallstones (from incr haem turnover with erythrocytosis) so more predisposed to cholecystitis
clubbing & hypertrophic pulmonary osteoarthropathy from megacaryocytes bypassing the pulmonary vascular bed
Among patients of a comparable functional class, who has better survival? Eisenmenger or idiopathic PAH? why may this be?
Eisenmenger
The R)-L) shunt creates a “pressure relief valve” for the RV which, while at the expense of arterial hypoxaemia, sustains CO by reducing pressure load on the RV & increasing LV preload
Also, there’s a degree of reversibility of the incr PVR in Eisenmenger
Haemodynamic goals for Eisenmenger:
maintain balance between SVR & PVR
AVOID sudden drop in SVR: increases R)–> L) shunt, hypoxaemia & worsening cyanosis, may–> CV collapse & death
AVOID sudden incr SVR which increases afterload & reduces ventricular function, may transmit to incr PVR
avoid factors increasing PVR
AVOID arrhythmias: poorly tolerated
Why are pts with Eisenmenger @ incr risk for sudden cardiac death during strenuous physical activity?
reduced capacity to increase pulm blood flow; exercise increases R)–> L) shunt & cyanosis
patExample of post-tricuspid shunts
VSD
PDA
Why may pts with PDA be less symptomatic? What does differential cyanosis in these pts mean?
carotid chemoreceptors are exposed to higher PaO2 ejected to upper body
Detectable difference in cyanosis- lower SpO2 in LLs
Why may pts with Eisenmenger be @ higher risk of CVA & brain abscess?
paradoxical thromboemboli, air emboli or septic emboli
