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Prof. Sevda Özdoğan
 Define
pulmonary hypertension
 List and explain the types of pulmonary
 List the symptoms and signs of pulmonary
 Define the diagnostic approach in pulmonary
 Define the treatment approach in pulmonary
Pulmonary hypertension (PH) is characterized by
elevated pulmonary arterial pressure and
secondary right ventricular failure (ie, cor
pulmonale). It is a progressive, fatal disease if
untreated, although the rate of progression is
highly variable.
PAH is defined according to right heart
catheterization. Mean pulmonary artery pressure
(mPAP) >25 mmHg at rest
A mean pulmonary artery pressure of 8 to 20 mmHg at rest is considered
normal, while a mean pulmonary artery pressure of 21 to 24 mmHg at
rest has uncertain clinical implications.
 This
group consists of sporadic IPAH,
heritable IPAH, and PAH due to diseases that
localize to small pulmonary muscular
(eg: connective tissue diseases, HIV infection,
portal hypertension, congenital heart disease,
schistosomiasis, chronic hemolytic anemia,
persistent pulmonary hypertension of the
newborn, pulmonary veno-occlusive disease,
and pulmonary capillary hemangiomatosis)
 Abnormal
bone morphogenetic protein
receptor type II (BMPR2) appears to play an
important role in the pathogenesis of IPAH.
 In hereditary IPAH, the gene that encodes
BMPR2 (IPAH1) appears to be transmitted as
an autosomal dominant trait. It has been
estimated that up to 80 percent of
hereditary IPAH is due to mutations in BMPR2
 Connective
tissue diseases
Rheumatoid arthritis and systemic lupus
erythematosus (SLE) can also lead to fibrous
obliteration of the pulmonary vascular bed, with
a marked female predominance
 Drug-
and toxin-induced PAH is also
considered group 1 PAH.
(eg: aminorex, fenfluramine, dexfenfluramine,
(appetite supressants) and toxic rapeseed oil)
Also amphetamines, cocaine (chronic use), L-tryptophan,
phenylpropanolamine, chemotherapeutic agents (thyrosine
kinase inhibitors, interferon), and selective serotonin
reuptake inhibitors are considered likely to cause PAH
The cause of HIV-related PAH is uncertain, but viral
and host factors both likely play an important role.
PAH associated with portal hypertension is referred to
as portopulmonary hypertension (PPHTN), The most
widely accepted hypothesis is that a humoral
substance (which would normally be metabolized by
the liver) is able to reach the pulmonary circulation
through portosystemic collaterals, resulting in
PAH due to congenital heart disease is due to
pulmonary blood volume overload due to intracardiac
shunting. Left to right shunts are most common,
especially due to large (nonrestrictive) ventricular
septal defects (VSD). PAH also occurs in patients with
atrial septal defects (ASD).
 Pulmonary
hypertension owing to left heart
disease. (systolic dysfunction, diastolic
dysfunction, or mitral and aortic valve
disease, restrictive cardiomyopathy,
constrictive pericarditis, and left atrial
 Pulmonary
hypertension owing to lung
diseases or hypoxemia.
This group includes PH due to severe chronic
obstructive pulmonary disease, interstitial lung
disease, pulmonary diseases with a mixed
restrictive and obstructive pattern, sleepdisordered breathing, alveolar hypoventilation
Chronic thromboembolic pulmonary hypertension
Pulmonary hypertension with unclear
multifactorial mechanisms.
In: hematologic disorders (eg, myeloproliferative
disorders), systemic disorders (eg, sarcoidosis),
metabolic disorders (eg, glycogen storage disease),
or miscellaneous causes (eg, sickle cell disease)
 the
prevalence of pulmonary hypertension in
patients with Sickle Cell Disease is 20 to 40
 Red
cell hemolysis resulting in increased
plasma levels of cell-free hemoglobin, which
rapidly depletes the potent pulmonary
vasodilator nitric oxide (NO) is the proposed
mechanism of PH in SCD
exertional dyspnea,
 lethargy, and fatigue
Symptoms and signs of PH may be difficult to
recognize because they are nonspecific. These
symptoms are frequently attributed incorrectly
to age, deconditioning, or a coexisting medical
The initial symptoms of PH are the result of an
inability to adequately increase cardiac output
during exercise
 Exertional
chest pain
Subendocardial hypoperfusion
Compression of the enlarged pulmonary artery to
left main coronary artery
 Exertional
syncope is due to the inability to
increase cardiac output during activity.
 Peripheral
edema is due to increased right
sided cardiac pressures
 Anorexia
and/or abdominal pain in the right
upper quadrant due to passive hepatic
 Less
common symptoms:
Hoarseness (caused by compression of the left
recurrent laryngeal nerve by a dilated main
pulmonary artery)
 increased
intensity of the pulmonic
component of the second heart sound
 Right-sided systolic ejection murmur
 A high-pitched systolic murmur of tricuspid
 Signs of right ventricular failure:
Signs of elevated jugular venous pressure
An enlarged and pulsatile liver,
peripheral edema,
ascites and pleural effusion may also exist
 Diagnostic
testing is indicated whenever PH
is suspected.
 The
purpose of the diagnostic testing is to
confirm that PH exists, determine its
severity, and identify its cause
 It
has been estimated that more than 20
percent of patients have symptoms of PH for
longer than two years before the PH is
 Chest
X ray
enlargement of the central pulmonary arteries
with attenuation of the peripheral vessels,
resulting in oligemic lung fields
Right ventricular enlargement (diminished
retrosternal space) and right atrial dilatation
(prominent right heart border)
Signs of the underlying cause of the PH (eg,
interstitial lung disease).
 Echocardiography
 Pulmonary Function Tests (to identify and
characterize underlying lung disease that may be contributing to
 Polysomnography
 V/Q
scan/ CT Angiography/ Pulmoner
 Laboratory tests:
HIV serology
Liver function tests
Antinuclear antibody (ANA), rheumatoid factor
(RF), and antineutrophil cytoplasmic antibody
(ANCA) titers
 Exercise
Screens for alternative causes of the patient's
Determines the patient's functional capacity
which guides therapy
Provides prognostic information, since a longer
distance walked during the 6MWT is associated
with longer survival
 Right
Hearth Catheterization
Necessary to confirm the diagnosis of PH
Helpful in distinguishing patients who have PH
due to left heart disease
The diagnosis of PAH also requires right heart catheterization:
Several criteria must be met:
Mean pulmonary artery pressure is ≥25 mmHg at rest
Mean pulmonary capillary wedge pressure <15 mmHg (to exclude PH
owing to left heart disease)
Chronic lung diseases and other causes of hypoxemia are mild or
Venous thromboembolic disease is absent
Certain miscellaneous disorders are absent, including systemic
disorders (eg, sarcoidosis), hematologic disorders (eg,
myeloproliferative diseases), and metabolic disorders (eg, glycogen
storage disease).
 Asthma
 Chronic
obstructive pulmonary disease
 Interstitial lung disease
 Myocardial dysfunction
 Obesity/deconditioning
 Liver disease
 Budd-Chiari syndrome (Thrombosis of hepatic veins)
PH is a progressive, fatal condition if untreated.
However, the rate of progression is highly variable
and depends upon the type and severity of the PH.
Symptomatic patients with IPAH who do not receive
treatment have a median survival of approximately
three years. Symptomatic patients with PAH
associated with another disease (eg, liver disease,
systemic sclerosis [also called scleroderma])
generally have a worse prognosis
Patients with severe PAH or right heart failure die
sooner without treatment (usually within one year)
than patients with mild PAH or no right heart failure
The main cause of death in patients with PAH is
thought to be right heart failure with circulatory
collapse and superimposed respiratory failure.
Primary therapy is directed at the underlying
cause of the PH.
Some patients progress to advanced therapy,
which is therapy directed at the PH itself. It
includes treatment with prostanoids, endothelin
receptor antagonists, phosphodiesterase 5
inhibitors, or, rarely, certain calcium channel
Diuretics — Diuretics are used to treat fluid
retention due to PH because diuresis will
diminish hepatic congestion and peripheral
Continuous oxygen administration remains the
cornerstone of therapy in patients with group 3
It is generally accepted that anticoagulation is
indicated in patients with IPAH, hereditary
pulmonary artery hypertension, drug-induced
PAH, or group 4 PH (CTEPH).
Exercise training appears to be beneficial for
patients with PH