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This talk is about
Pulmonary Arterial
Hypertension in
infants, children,
and young adults.
My name is Georg Hansmann.
I'm a Professor of Pediatrics
in Pediatric Cardiology at
the Friedrich Alexander
University in
Erlangen-Nuremberg,
Bavaria, Germany.
I'm also the Founding Chair of
the European Pediatric Pulmonary
Vascular Disease Network.
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These are my disclosures.
0:26
Here's the outline,
you can have a look at it,
but we won't go through it.
0:31
The mechanisms involved in
pulmonary arterial
hypertension include,
at the beginning, endothelial
dysfunction and apoptosis,
pulmonary arterial smooth
muscle cell proliferation,
inflammation, and fibrosis.
That leads then to
vascular remodeling,
peripheral PA
obliteration and also
pulmonary artery loss
in the periphery.
Followed by increased
vascular resistance and
pulmonary arterial hypertension.
Over time, that results in
right ventricular
dysfunction due to
the pressure overload
of the right ventricle.
As you can see here
with the arrow,
the interventricular septum
bulges into the left ventricle.
There is LV compression
and underfilling,
and ultimately, heart
failure and death.
1:17
Here you see the histology of
a grade 4 advanced
PAH patient who
underwent lung transplantation.
You see concentric lesions,
also plexiform lesions,
even remodeling of
the airways and
also fresh intra-alveolar
hemorrhage.
1:39
This is an echocardiography.
On the left upper corner
four-chamber view,
you see the small
left ventricle and
the interventricular
septum being,
in end-systole, convexed
to the left side.
In the upper middle,
there is vector
velocity imaging.
In the right upper corner,
there is a short axis view
in echocardiography where
you can see the
typical features:
dilated hypertrophic
right ventricle,
a D-shape of the left ventricle,
and there is
pericardial effusion.
In the right lower corner,
there's a cardiac MRI image.
It showed excess fill of
the patient's right
ventricle prior to
lung transplantation—a huge
right ventricle, dilated.
Again, there is a D-sign and
underfilling and compression
of the left ventricle
as well as pericardial effusion.
In the left lower corner,
there is a typical estimation:
a right ventricular
systolic pressure by
Doppler echocardiography.
