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:warning: Pulmonary Hypertension :warning: (Idiopathic Pulmonary Arterial…
:warning: Pulmonary Hypertension :warning:
Idiopathic Pulmonary Arterial Hypertension
(IPAH)
occurs without an
apparent cause
if untreated, can rapidly progress,
causing right-sided heart failure
& death within a few years
new drug therapy improves
survival, but disease is still incurable
Etiology & Patho
cause is unknown & affects
females more than males
some type of insult occurs
(hormonal, mechanical, other)
pulmonary endothelial injury
vascular scarring
endothelial dysfunction
smooth muscle proliferation
sustained pulmonary hypertension
right ventricular hypertrophy
cor pulmonale
right-sided heart failure
Clinical Manifestations
dyspnea on exertion
fatigue
exertional chest pain
dizziness
exertional syncope
r/t inability of CO to increase in
response to increase O2 demand
eventually, dyspnea occurs at rest
Diagnostic Studies
right-sided cardiac
catheterization
provides accurate measurement
of pulmonary artery pressures
determines CO & pulmonary vascular resistance
ECG
chest x-ray
pulmonary function tests
echocardiogram
CT scans
mean time b/w onset of symptoms
& diagnosis is about 2 years
by the time pt.'s become symptomatic, disease
is already in advanced stages & pulmonary
artery pressure is 2-3 times the norm
Management
drug therapy
vasodilators:
vasodilation of pulmonary blood vessels
reduce right ventricular overload
reverse remodeling
Table 28-27 of Lewis
diuretics to manage peripheral edema
anticoagulants r/t thrombus formation
low-flow O2
keep O2 sat > 90%
surgical
atrial septostomy (AS)
creation of an intraatrial right-to-left shunt to decompress the right ventricle
lung transplantation
for those who do not respond to drug therapy & progress to severe right-sided heart failure
elevated pulmonary
artery pressure
from an increase in resistance to blood
flow through the pulmonary circulation
pulmonary pressures
are elevated
with mean pulmonary artery
pressure > 25 mmHg at rest
or > 30 mmHg with exercise
normal is 12-16 mmHg
Secondary Pulmonary Arterial Hypertension
(SPAH)
occurs
when a primary disease causes a chronic increase in pulmonary
artery pressures
parenchymal
lung disease
left ventricular dysfunction
intracardiac shunts
chronic pulmonary thromboembolism
systemic connective
tissue disease
The initial physical findings can include
right ventricular hypertrophy and signs of right ventricular
failure (increased pulmonic heart sound, right-sided fourth
heart sound, peripheral edema, and hepatomegaly).
Treatment
consists mainly of treating the underlying primary
disorder.
When irreversible pulmonary vascular damage