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Eisenmenger's Syndrome

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Topic updated on 08/14/17 12:49pm

Overview
 

 
Snapshot
  • A 32-year-old man who showed exercise intolerance, cyanosis, heart failure, and hemoptysis prior to death is found to have a large congenital cardiac defect on autopsy.
Introduction
  • Consists of
    • pulmonary hypertension
    • RVH
    • reversal of left-to-right shunt
  • Associated with
    • large congenital cardiac defects that have been untreated including
      • ASD
      • VSD
    • surgically-created extracardiac left-to-right shunts
  • Epidemiology
    • usually develop before puberty
    • but can be in late adolescence or early adulthood
  • Physiology
    • causes increased pulmonary blood flow
    • results from inability to increase pulmonary blood flow under physiologic stress
  • Prognosis
    • often irreversible
Presentation
  • Symptoms
    • patients commonly present with
      • exercise intolerance
      • shortness of breath
      • fatigue/lethargy
      • presyncope/syncope
      • accompanied by other heart failure symptoms
    • less common symptoms include
      • erythrocytosis
      • easy bleeding
      • cholelithiasis
      • nephrolithiasis
  • Physical exam
    • ischemia
      • central cyanosis
      • clubbing 
    • cardiac exam
      • right ventricular heave
      • single, palpable S2
      • high-pitched early diastolic murmur
        • indicative of pulmonary insufficiency
      • right-sided S4
      • may not have discernable ASD or VSD murmurs
      • fade as shunt reverses

Evaluation
  • Imaging
    • echocardiogram is diagnostic in
      • locating the cardiac defect responsible for the shunt visualizing the left-to-right blood flow via color Doppler imaging
    • CXR shows
      • right-sided enlargement
      • signs of pulmonary hypertension including
        • dilated pulmonary artery
        • increased hilar vascular markings
        • pruned peripheral vessels


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Qbank (1 Questions)

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(M1.CV.116) A 27-year-old woman with a history of a "heart murmur since childhood" presents following a series of syncopal episodes over the past several months. She also complains of worsening fatigue over this time period, and notes that her lips have begun to take on a bluish tinge, for which she has been using a brighter shade of lipstick. You do a careful examination, and detect a right ventricular heave, clubbing of the fingers, and 2+ pitting edema bilaterally to the shins. Despite your patient insisting that every doctor she has ever seen has commented on her murmur, you do not hear one. Transthoracic echocardiography would most likely detect which of the following? Topic Review Topic

1. Aortic stenosis
2. Mitral insufficiency
3. Positive bubble study
4. Ventricular aneurysm
5. Dynamic left ventricular outflow tract obstruction

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