Page 153 - Clinical Pearls in Cardiology

Congenital Heart Disease 141

3. What is Eisenmenger’s syndrome?
In 1958, Paul Wood coined the term Eisenmenger
complex to describe pulmonary hypertension at the
systemic level due to high pulmonary vascular resistance,
secondary to reversed or bidirectional shunting
through a large ventricular septal defect or VSD”.
Subsequently, the term “Eisenmenger’s syndrome” has
been used to describe pulmonary vascular disease (i.e.
pulmonary hypertension) and cyanosis resulting from any
systemic-to-pulmonary circulation connection such as
an ASD, VSD or PDA.
Physical examination of a patient with the
Eisenmenger’s syndrome reveals central cyanosis and
clubbing. If systemic vascular resistance falls, as may
occur with hot weather, exercise, fever or systemic
infection, the magnitude of right-to-left shunting and
cyanosis increases. The jugular venous pressure may
be normal or elevated, with prominent “v” waves in JVP.
Other signs of pulmonary arterial hypertension are also
invariably present. The usual murmurs associated with
VSD or PDA usually disappear with the development of
pulmonary artery hypertension.
4. Which are the parts from which the various cardiac
chambers evolve?
The development of various cardiac chambers from the
primitive heart tube is as follows:
Development of cardiac chambers
• Truncus arteriosus—aorta and pulmonary artery
• Conus arteriosus—right ventricle
• Primitive ventricle—left ventricle
• Primitive atrium—trabeculated portion of left and
right atrium