Eisenmenger Syndrome
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Introduction
Eisenmenger syndrome occurs with chronic left-to-right shunting, resulting in severe pulmonary vascular obstruction.
Elevated pulmonary resistance causes reversal of the shunt, leading to entry of deoxygenated blood into the systemic circulation and resulting in cyanosis.
Pulmonary arteriolar media hypertrophies and the intima proliferates, and over time vessels become thrombosed. This causes a rise in pulmonary reistance.
Hypoxemia results in exertional dyspnea and fatigue. Reduced hemoglobin saturation leads to erythropiesis, causing hyperviscosity, headaches, and occasional stroke.
Infarction or rupture of pulmonary vessels can result in hemoptysis.
The Case of...
a simple case introducing clincial presentation and calling for a differential diagnosis to get students thinking.
Causes and Risk Factors
Pathophysiology
Signs and Symptoms
- history
- physical exam
History
Physical Exam
cysnosis, clubbing
prominent a wave on JVP
loud P2
absent murmur
Investigations
- lab investigations
- diagnostic imaging
Lab Investigations
Diagnostic Imaging
Differential Diagnosis
Treatments
avoid exacerbationary activities, including strenuous exercise, high altitude, and peripheral vasodilator drugs.
Consequences and Course
Pregancy is especially dangerous; rate of spotaneous abortion is 20
The Case of...
Case #2 - a small story wrapping it all up and asking especially about management.
Additional Resources
Topic Development
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