Transcript Pulmonary Hypertension

Dr.Mirdamadi
Cardiologist,Fellowship of Echocardiography
The lung has a unique double arterial blood
supply from the pulmonary and bronchial
arteries, as well as double venous drainage
into the pulmonary and azygos veins
 Pulmonary hypertension, an abnormal
elevation in pulmonary artery pressure,
may be the result of left heart failure,
pulmonary parenchymal or vascular
disease, thromboembolism, or a
combination of these factors.
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Pathophysiology
The right ventricle responds to an increase
in pulmonary vascular resistance by
increasing right ventricular (RV) systolic
pressure to preserve cardiac output
 In some patients, chronic changes occur in
the pulmonary circulation, resulting in
progressive remodeling of the vasculature,
which can sustain or promote pulmonary
hypertension even if the initiating factor is
removed.
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The ability of the RV to adapt to increased
vascular resistance is influenced by several
factors, including age and the rapidity of
the development of pulmonary
hypertension
 a large acute pulmonary thromboembolism
can result in RV failure and shock, whereas
chronic thromboembolic disease of equal
severity may result in only mild exercise
intolerance.
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Definitions
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PH has been defined as an increase in
mean pulmonary arterial pressure (PAP)
25 mmHg at rest as assessed by right
heart catheterization
Diagnosis
The most common symptom attributable to
pulmonary hypertension is exertional
dyspnea. Other common symptoms are
fatigue, angina pectoris, syncope, near
syncope, and peripheral edema
 The physical examination typically reveals
increased jugular venous pressure, a
reduced carotid pulse, and a palpable RV
impulse. Most patients have an increased
pulmonic component of the second heart
sound, a right-sided fourth heart sound,
and tricuspid regurgitation
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The chest x-ray generally shows enlarged
central pulmonary arteries. The lung fields
may reveal other pathology.
 The electrocardiogram usually shows right
axis deviation and RV hypertrophy.
 The echocardiogram commonly
demonstrates RV and right atrial
enlargement, a reduction in left ventricular
(LV) cavity size, and a tricuspid regurgitant
jet that can be used to estimate RV systolic
pressure by Doppler.
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Pulmonary function tests are helpful in
documenting underlying obstructive
airways disease, whereas highresolution chest computed tomography
(CT) is preferred to diagnose restrictive
lung disease.
 A perfusion lung scan is almost always
abnormal in patients with
thromboembolic pulmonary
hypertension
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Laboratory tests should include
antinuclear antibody and HIV testing.
 Because of the high frequency of thyroid
abnormalities in patients with idiopathic
pulmonary hypertension, it is
recommended that the thyroidstimulating hormone level be determined
periodically.
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Cardiac catheterization is mandatory for
accurate measurement of pulmonary
artery pressure, cardiac output, and LV
filling pressure as well as documentation
of an underlying cardiac shunt.
Pathobiology
Vasoconstriction, vascular proliferation,
thrombosis, and inflammation appear to
underlie the development of PAH
 Abnormalities in multiple molecular
pathways and genes that regulate the
pulmonary vascular endothelial and
smooth muscle cells have been
identified.
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Idiopathic Pulmonary Arterial
Hypertension
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Idiopathic pulmonary arterial
hypertension (IPAH), formerly referred to
as primary pulmonary hypertension, is
uncommon, with an estimated incidence
of two cases per million. There is a
female predominance, with most
patients presenting in the fourth and fifth
decades, although the age range is from
infancy to >60 years.

Familial IPAH accounts for up to 20% of
cases of IPAH and is characterized by
autosomal dominant inheritance and
incomplete penetrance.
Natural History
The natural history of IPAH is uncertain,
but the disease typically is diagnosed
late in its course. Before current
therapies, a mean survival of 2–3 years
from the time of diagnosis was reported.
 The cause of death is usually RV failure,
which is manifest by progressive
hypoxemia, tachycardia, hypotension,
and edema.
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Treatment
Because the pulmonary artery pressure
in PAH increases with exercise, patients
should be cautioned against
participating in activities that impose
physical stress.
 Diuretic therapy relieves peripheral
edema and may be useful in reducing
RV volume overload.

Anticoagulant therapy is advocated for
all patients with PAH based on studies
demonstrating that warfarin increases
survival of patients with PAH.
 Patients who respond to vasodilators at
the time of cardiac catheterization
should be treated with calcium channel
blockers. these patients require high
doses (e.g., nifedipine, 240 mg/d, or
amlodipine, 20 mg/d).
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The endothelin receptor antagonists
bosentan and ambrisentan are approved
treatments of PAH.
 Sildenafil and tadalafil,
phosphodiesterase-5 inhibitors, are
approved for the treatment of PAH.
 The prostacyclins have the greatest
efficacy as treatments for PAH
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Lung transplantation is considered for
patients who, while on an intravenous
prostacyclin, continue to manifest right
heart failure.
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Venous thromboembolism (VTE), which
encompasses deep venous thrombosis
(DVT) and pulmonary embolism (PE), is
one of the three major cardiovascular
causes of death, along with myocardial
infarction and stroke

In 1856, Rudolf Virchow postulated a
triad of factors that predispose to
intravascular coagulation: local trauma
to the vessel wall, hypercoagulability,
and stasis.
the pathogenesis of PE has been
dichotomized as caused by either
inherited (primary) or acquired
(secondary) risk factors.
 combination of thrombophilia and
acquired risk factors often precipitates
overt thrombosis.
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Risk Factors for Venous
Thromboembolism
Obesity
 Metabolic syndrome
 Cigarette smoking
 Hypertension
 Abnormal lipid profile
 High consumption of red meat and low
consumption of fish, fruits, and
vegetables
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Advancing age
Arterial disease, including carotid and coronary disease
Personal or family history of venous thromboembolism
Recent surgery, trauma, or immobility, including stroke
Congestive heart failure
Chronic obstructive pulmonary disease
Acute infection
Air pollution
Long-haul air travel
Pregnancy, OCP, or postmenopausal HRT
Pacemaker, ICD leads, or indwelling central venous
catheter
Hypercoagulable states
 Factor V Leiden
 Prothrombin gene mutation
 Antithrombin deficiency
 Protein C deficiency
 Protein S deficiency
 Antiphospholipid antibody syndrome
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increased risk of VTE with
malignancyes: adenocarcinomas of the
pancreas,stomach, lung, esophagus,
prostate, and colon,
 liquid tumors :
myeloproliferative disease,
lymphoma, and leukemia
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The two most common genetic causes of
thrombophilia are factor V Leiden and the
prothrombin gene mutation
 Normally, a specified amount of activated
protein C (aPC) can be added to plasma to
prolong the aPTT.
 Patients with aPC resistance have a
blunted aPTT prolongation and a
predisposition to development of PE and
DVT.
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The phenotype of aPC resistance is
associated with a single point mutation,
designated factor V Leiden, in the factor
V gene
Factor V Leiden triples the risk for
development of VTE,also a risk factor
for recurrent pregnancy loss, possibly
caused by placental vein thrombosis.
 Use of OCP by patients with factor V
Leiden increases the risk of VTE by at
least 10-fold
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The most common acquired
thrombophilia is the antiphospholipid
syndrome
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When venous thrombi are dislodged
from their site of formation, they
embolize to the pulmonary arterial
circulation or, paradoxically, to the
arterial circulation through a patent
foramen ovale or atrial septal defect.
VTE mimics other illnesses, and PE is
known as "the Great Masquerader,"
making diagnosis difficult.
 Occult PE is especially hard to detect
when it occurs concomitantly with overt
heart failure or pneumonia. In such
circumstances, clinical improvement
often fails to occur despite standard
medical treatment of the concomitant
illness
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Pathophysiology
- increased pulmonary vascular resistance caused by
vascular obstruction, neurohumoral agents, or pulmonary
artery baroreceptors;
- impaired gas exchange caused by increased alveolar
dead space from vascular obstruction and hypoxemia
from alveolar hypoventilation, low ventilation-perfusion
units, and right-to-left shunting
- impaired carbon monoxide transfer caused by
loss of gas exchange surface;
- alveolar hyperventilation caused by reflex stimulation of
irritant receptors
-increased airway resistance due to bronchoconstriction;
-decreased pulmonary compliance due to lung edema,
lung hemorrhage, and loss of surfactant.
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Dyspnea is the most frequent symptom
and tachypnea is the most frequent sign
of PE
Symptoms:
 Otherwise unexplained dyspnea
 Chest pain, either pleuritic or atypical
 Anxiety
 Cough
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Signs:
Tachypnea
Tachycardia
Low-grade fever
Left parasternal lift
Tricuspid regurgitant murmur
Accentuated P2
Hemoptysis
Leg edema, erythema, tenderness
Differential Diagnosis of DVT:
 Ruptured Baker's cyst
 Cellulitis
 Postphlebitic syndrome/venous
insufficiency
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Differential Diagnosis of PE:
Pneumonia, asthma, chronic obstructive
pulmonary disease
Congestive heart failure
Pericarditis
Pleurisy: "viral syndrome," costochondritis,
musculoskeletal discomfort
Rib fracture, pneumothorax
Acute coronary syndrome
Anxiety
Clinical Decision Rules
Clinical Decision Rules
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d-dimer rises in the presence of DVT or PE because
of the breakdown of fibrin by plasmin.
The sensitivity of the d-dimer is >80% for DVT and
>95% for PE.
The d-dimer assay is not specific. Levels increase in
patients with myocardial infarction, pneumonia,
sepsis, cancer, and the postoperative state and
those in the second or third trimester of pregnancy.
d-dimer rarely has a useful role among hospitalized
patients, because levels are frequently elevated due
to systemic illness.
The d-dimer is a useful "rule out" test.
Electrocardiogram
The most frequently cited abnormality:
 sinus tachycardia
 S1Q3T3 sign: an S wave in lead I, a Q
wave in lead III, and an inverted T wave
in lead III
 T-wave inversion in leads V1 to V4.
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Noninvasive Imaging Modalities
Venous Ultrasonography
 Chest Roentgenography
 Chest CT
 Lung Scanning(Ventilation,perfusion
scan)
 Magnetic Resonance (ContrastEnhanced)
 Echocardiography
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Invasive Diagnostic Modalities
Pulmonary Angiography
 Contrast Phlebography
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Thank you for
your attention