Pulmonary Hypertension

And Cor Pulmonale

Cheng Zhang ， Respiratory Medicine ， Affiliated
Hospital of Jining Medicine college
23,Feb
What is Pulmonary Hypertension
• Pulmonary hypertension （ PH ） is a disorder of
the pulmonary circulation in which elevated
pressure in the pulmonary vascular circuit
can ， when severe ， lead to right heart failure
and eventually cause death
• The diagnostic criterion for PH is controversial
• It is commonly accepted that at sea level,a mean
pulmonary artery pressure(mPAP) ≥20mmHg at
rest or >30mmHg with exercise along with a
pulmonary artery occlusion pressure of ≤15 mmHg
are required to establish the diagnosis of pulmonary
arterial hypertension(PAH),a subtype of PH
 Classification and Staging
• For many years,PH was classified as either
“primary” or “secondary”
• In 2003 the Third World Symposium updated the
classification system,notably dropping the
term”primary”
• They also stressed that the staging of patients with
PH should be based on the functional capacity of
the patient rather than on hemodynamic parameters
• The World Health Organization classification
system,a modified form of the New York Heart
Association functional classification system,was
recommended by the Symposium as the preferred
staging system
Secondary Pulmonary Hypertension
• Secondary pulmonary hypertension(SPH) defined
as PH due to a known cause is far more common
than primary pulmonary hypertension(PPH)
• Secondary pulmonary hypertension can be caused
by any disease that impedes the flow of blood
through the lungs of that causes sustained periods
of low oxygen in the blood
• The predominant causes are cardiac and pulmonary
diseases
• This focus of this section is pulmonary hypertension
associated with disorders of the respiratory system
and/or hypoxemia
Secondary Pulmonary Hypertension
 Causes of Secondary Pulmonary
Hypertension
 Obstructive Pulmonary Disease
• Chronic obstructive pulmonary disease is the most
common cause
• When the lungs are impaired by disease,it takes
more effort to pump blood through them
• Over time, chronic obstructive pulmonary disease
destroys the small air sacs together with their small
vessels in the lungs
Secondary Pulmonary Hypertension
• The most important cause of pulmonary
hypertension in chronic obstructive pulmonary
disease is the narrowing of the pulmonary artery
that occurs as a result of hypoxemia
 Parenchymal Lung Diseases Including Alveolar

Diseases And Interstitial Lung Diseases


Alveolar diseases,such as pneumonedema and acute
respiratory distress syndrome,which often precede
SPH
Secondary Pulmonary Hypertension
• Are not required specific treatment since the high
pulmonary artery pressure caused by this group
diseases will decrease as the primary diseases
ameliorate
• Interstitial lung diseases,such as
pneumonoconiosis,sarcoidosis,idiopathic pulmonary
fibrosis,and etc,can cause diffusion abnormality
resulting in hypoxemia
• The pulmonary arterial vasoconstriction develops as
a result of hypoxemia and the inflammation of this
group diseases
Secondary Pulmonary Hypertension
 Abnormalities of Pulmonary Arteries or Arterioles
• Among abnormalities of Pulmonary arteries or
arterioles, pulmonary thromboembolism is the most
common cause
• There are some other relatively rare diseases,such
as pulmonary capillary hemangioma,pulmonary
veno-occlusive disease,collagen vascular
diseases,etc
Secondary Pulmonary Hypertension
 Neuromuscular Diseases
• The pathophysiologic character of neurologic
diseases,such as chest wall abnormalities,Guillain-
Barre syndrome,poliomyelitis and etc,is
hypoventilation leading to hypoxemia and
hypercapnia,which eventually result in the
pulmonary hypertension
Secondary Pulmonary Hypertension
 Clinical Presentation and Treatment

The early clinical manifestations of SPH are
frequently masked by the undering etiology

Obtaining a careful history may help exclude some
of the numerous causes of secondary pulmonary
hypertension

The late clinical manifestations are labeled with
right heart failure

The treatment of SPH is primonary directed at
treatment of the undering disease
Secondary Pulmonary Hypertension
• Effective therary should be instituted in the early
stages,before irreversible changes in pulmonary
vasculature occur
• Once the cause of SPH has been established,the
management consists of specific interventional
therary, specific medical therary,or general
supportive therary
• In the late stages,the therapeutic regimen is targeted
to Cor Pulmonale
 Primary(idiopathic) Pulmonary
Hypertension
• Primary(idiopathic) pulmonary
hypertension （ PPH ） is a rare disorder of the
pulmonary circulation occurring mostly in young
and middle aged wemen
• Untreated ， it is characterized by progressive
dyspnea ， a rapid downhill course ， and an
invariably fatal outcome
• This condition is also called plexogenic pulmonary
arteriopathy ， in reference to the characteristic
histopathologic plexiform lesion found in muscular
pulmonary arteries
 Primary(idiopathic) Pulmonary
Hypertension

Epidemiology

The estimated incidence of primary pulmonary
hypertension is 1-2/million in the general
population

PPH accounts for 0.008‰-1.3 ‰ of all the non-
selective autopsy cases in America and Europe

There is no exact data about the Chinese prevalence

PPH can occur in the population with any age
 Primary(idiopathic) Pulmonary
Hypertension
 Aetiology
• The aetiology of PPH remains undefined
• The disease can occur in a familial or sporadic form
• The sporadic form of the disease may be caused by
interaction between a genetic factor ， possibly
related to the gene encoding the familial
form ， with one or more triggering factors
• The interaction between genetic and triggering
factor could result in endothelial injury and
vasoconstriction leading to vascular remodelling
 Primary(idiopathic) Pulmonary
Hypertension
 Genetics
• The Familial form of PPH is inherited as an
autosomal dominant and is associated with genetic
anticipation
• In this form of inheritance,there is worsening of the
disease in subsequent generations
• The gene for PPH has been localized to a 27-
centimorgan region on chromosome 2q31-32
• Both the familial and sporadic forms of PPH have
been recently associated with mutations of the bone
morphogenetic protein receptor type II gene (BMPR
2）
 Primary(idiopathic) Pulmonary
Hypertension
 Autoimmunity

There are a number of associations between various
autoimmune diseases and PPH

Patients with scleroderma,the CREST
syndrome,dermatomyositis,rheumatoid arthritis and
systemic lupus erythematosus as well as
hypothyroidism have been known to develop
pulmonary hypertension

However no specific biomarker has been found for
the diagnosis of PPH
 Primary(idiopathic) Pulmonary
Hypertension
 Vasoactive Mediators
• The tone of pulmonary vascular tree is reliant upon
a balance between vasoconstrictor and vasodilator
machanisms
• The vasoconstrictors include II dehydro-
thromboxane B2,endothelin-1,Serotomn and etc
• The vasodilators include nitric oxide
synthase,prostacyclin receptor protein,etc
• An imbalance of vasoactive mediators has been
demonstrated in patients with pulmonary
hypertension
 Primary(idiopathic) Pulmonary
Hypertension
 Potassium Channels
• Disregulation of potassium channels can lead to
vasoconstriction
• Inhibition of membrane potassium channels lead to
depolarization of pulmonary artery smooth muscle cells and
an increase in intracellular calcium resulting in
vasoconstriction
• Patients with PPH have been shown to have abnormalities of
potassium channels resulting in depolarization of the
pulmonary vascular smooth muscle cells
• Furthermore,aminorex,fenfluramine and dexfenfluramine
have been shown to inhibit potassium channels
 Primary(idiopathic) Pulmonary
Hypertension

Clinical presentation

Symptoms
• The onset of symptoms is usually insidious with
several years elapsing before the diagnosis is
actually made
• As the pulmonary hypertension increases,patients
can present with any of the following systemic
symptoms:dyspnoea,presyncope or syncope,chest
pain and hemoptysis
 Primary(idiopathic) Pulmonary
Hypertension
• Other symptoms are fatigue,palpitations,sudden
death,cough, Ortner’s syndrome and etc
• Raynaud phenomenon has beeb reported as many
as 10% patients
• Physical Signs
• The physical signs in pulmonary hypertension
patients are often advanced at the time of
presentation resulting from pulmonary hypertension
and the overload of right heart
 Primary(idiopathic) Pulmonary
Hypertension

Lab and other findings

Blood tests
• Full blood count,ESR,clotting screen,abnormal
haemoglobin,glucose,lipids,liver function tests,serum
ACE,thyroid function
 Electrocardiogram
• Signs of right ventricular hypertrophy or strain may be
observed
• These include right axis deviation,an R-to-S wave ratio
greater than 1 in lead V1,increased P-wave amplitude,and an
incomplete or complete right bundle-branch block pattern
 Primary(idiopathic) Pulmonary
Hypertension
 Chest radiograph
• The classic finding on a chest radiograph from a
patient with PPH is enlargement of central
pulmonary arteries,attenuation of peripheral
vessels,and oligemic lung fields
• Findings of right ventricular and right atria
dilatation are possible

Two-dimensional echocardiography and doppler
echocardiography
• Reflection of pulmonary hypertention and other
associated presentations
 Primary(idiopathic) Pulmonary
Hypertension
 Pulmonary function tests
• Diffusing of capacity is universally reduced in
patients with pulmonary hypertention
 Arterial blood gas

• Arterial blood gas determinations should be

performed to assess for hypoxemia
 Cardiac catheterization

• This investigation remains the gold standard for

establishing the diagnosis of pulmonary
hypertention
 Primary(idiopathic) Pulmonary
Hypertension
• At the time of cardiac catheterization,full
haemodynamic mornitoring should be made as well
as testing for acute vasodilator response
 Ventilation-perfusion lung scan

• Ventilation-perfusion scan should be performed to

exclude chronic thromboembolic pulmonary
hypertention
• A high-or low-probability scan result is more
useful,whereas intermediate-probability results
should lead to performing pulmonary angiography
 Primary(idiopathic) Pulmonary
Hypertension
 Lung biopsy
• The pathological changes are the result of
longstanding hypertension rather than a
consequence of different causes
• The plexiform lesion is observed in patients with all
types of PPH
• These lesions consist of medial
hypertrophy,eccentric or concentric laminar intimal
proliferation and fibrosis,fibrinoid degeneration,and
thrombotic lesions
 Primary(idiopathic) Pulmonary
Hypertension
 Diagnosis and differentials

The diagnosis of PPH cannot be made until possible
diseases,which might cause pulmonary
hypertention,has been ruled out

It is usually made during the process of evaluating
symptoms such as dyspnea,exercise
intolerance,chest pain,or syncope

Electrocardiographic changes,elevated brain
natriuretic peptide(BNP)levels,and
echocardiographic changes may suggest the
diagnosis

Differentials:All those diseases that might cause
pulmonary hypertention
 Primary(idiopathic) Pulmonary
Hypertension
• However,current remmendations state that a right-
heart catheterization(RHC)is “strongly advised”to
formally make the diagnosis of PPH
• It is the author’s belief that all patients who are
suspected to have PPH should have RHC prior to
initiation of therapy
• A mean pulmonary artery pressure≥20mmHg at or
>30mmHg with exercise along with a pulmonary
artery occlusion pressure of <15 mmHg are
required to establish the diagnosis of PPH
 Primary(idiopathic) Pulmonary
Hypertension
 Treatment

Since the causes of PPH remain unknown,the therapy is
directed to vasoconstriction,endothelial
impairment,thrombosis and right heart failure,in order to
improve the pulmonary arteries or arterioles
function,cardiac function and life
 Vasodilator therapy

a. Calcium-Channel Blochers.About 20% of the PPH
patients are responsive to Calcium-Channel Blockers.The
high dose in recommended,for
instance,Nifedipine,240mg/d,and adverse reaction should be
under the surveillance
 Primary(idiopathic) Pulmonary
Hypertension
• b. Prostanoids.Prostacyclin is a potent vasodilator
with antiplatelet effects,and therapy with
prostacyclin analogs （ prostanoids ） has become
a cornerstone in the treatment of patients with
PPH.To data, different prostanoids have been
studied,each with unique delivery systems,side
effects,and evidence of efficacy
• c. Endothelin Antagonists. Endothelin-1 has been
shown to be a potent vasoconstrictor and a mediator
of the pulmonary vascular remodeling seen in
PAH.Two receptors for endothelin-1 have been
indentified,ETA and ETB
 Primary(idiopathic) Pulmonary
Hypertension
• ETA mediates vasoconstriction and remodeling and
ETB is involved in the clearance of endothelin-1
and perhaps also in vasodilatation and oxide release
• Many endothelin receptors antagonists,are being
studied in PPH;however,only bosentan in currently
available for use in the United States
• d. Anticoagulation.In PPH there is a predisposition
to thrombosis because of right ventricular
failure.Futhermore,thrombotic lesions have been
demonstrated in the pulmonary vascular tree
 Primary(idiopathic) Pulmonary
Hypertension
• The anticoagulation therapy cannot improve the
clinical symptom while it can impede the process of
deterioration.Wrafarin has been shown to prolong
survival in patients with pulmonary hypertension

Others

Other treatments available for the treatment of PPH
include oxygen,diuretics and digokin.The latter has
been shown to improve right-ventricular function in
such patients and can be commenced when patients
remain symptomatic whilst on medical therapy
 Primary(idiopathic) Pulmonary
Hypertension

 Interventional and surgical therapy

• Lung or heart transplantation can be applied in the
late stages
 Cor pulmonale
• The term cor pulmonale denotes right ventricular
enlargement(hypertrophy and/or
dilatation)secondary to abnormal lungs,chest
bellows,or control of breathing
• It has been commonly used as a synonym for
pulmonary heart disease
• Cor pulmonale may be acute or chronic
• The most common cause of the acute cor pulmonale
is massive embolization of the lungs
• Chronic cor pulmonale,in which hypertrophy
generally predominates,is more common than acute
cor pulmonale,in which dilatation is the rule
 Cor pulmonale

Chronic cor pulmonale
• Chronic pulmonary heart disease or chronic cor
pulmonale is defined as right ventricular
hypertrophy ,dilation,or both as a result of
pulmonary hypertension caused by pulmonary
disorder involving the lung parenchyma,impaired
pulmonary bellows function,or altered ventilatory
drive,except when this condition occurs because of
left heart disease or congenital heart diseases
 Cor pulmonale

Epidemiology
• Chronic cor pulmonale is a common disorder of
respiratory system in China
• A study performed in China in 1970’s demonstrated
that in the population,aged>14 years,the incidence
is 4.8%
• Another study carried out in 1992 showed that the
incidence remained high in China,with 6.72% in the
population aged>15 years
 Cor pulmonale
• However,the incidence of Chronic cor pulmonale
varies from country to country,between urban and
rural areas,and with exposure to air pollutants
• Chronic cor pulmonale accounts for more cases in
the older and smoker population than in the
younger and non-smokers
• With chronic cor pulmonale patients more easily
contract acute exacerbations in winter and spring,or
in the abruptly changeable weather
• There is no significant difference between male and
female population
 Cor pulmonale
 Aetieology of cor pulmonale

The chronic respiratory diseases which may lead to
cor pulmonale,pulmonary thromboembolic
disease,and diseases of the pulmonary vascular
bed,are categorized into three major groups

Obstructive lung diseases

Those characterized by a limitation to airflow,such
as COPD(chronic obstruction
bronchitis,emphysema and their association),cystic
fibrosis,bronchiectasis,bronchiolitis obliterans,etc
 Cor pulmonale
 Restrictive lung diseases
• Those characterized by a restriction of pulmonary
volumes from extrinsic or parenchymatous
origin(restrictive lung diseases),such as
neuromuscular
diseses,scarcoidosis,kyphoscoliosis,drug related
lung diseases,idiopathic interstitial pulmonary
fibrosis, interstitial pulmonary fibrosis of known
origin,etc
 Cor pulmonale
 Respiratory insufficiency of “central”origin
• Those where the relatively well preserved
mechanical properties of the lungs and chest wall
contrast with pronounced gas exchange
abnormalities which are partially exlained by poor
ventilatory drive （ respiratory insufficiency of
“central”origin ） ,such as central alveolar
hypoventilation,obesity-hypoventilation
syndrome,sleep apnoea syndrome,etc
 Cor pulmonale
• COPD is the major cause of chronic respiratory
insufficiency and cor pulmonale,and it probably
accounts for 80-90% of the cases
• COPD includes chronic obstructive bronchitis and
emphysema which are often associated
• Among the restrictive lung
diseases,kyphosconiosis,,idiopathic pulmonary
fibrosis,and pneumoconiosis are the main causes of
cor pulmonale
• Among the aetiologies of respiratory respiratory
insufficiency of “central”origin the obesity-
hypoventilation syndrome is a relatively frequent
cause of cor pulmonale
 Cor pulmonale

Mechanism and pathology
• The mechanism of cor pulmonale are first those of
pulmonary hypertension
• In chronic respiratory diseases pulmonary
hypertension results from increased pulmonary
vascular resistance(PVR)

Development of pulmonary hypertension
• A. pulmonary vascular resistance due to functional
factors
 Cor pulmonale
• The factors leading to an increased PVR in chronic
respiratory disease are numerous but alveolar
alveolar hypoxia is by far the most predominant
• The district mechanism of action of alveolar
hypoxia is that acute hypoxia causes pulmonary
vasoconstriction and pulmonary hypertension
develops
• It is commonly believed that the balance between
vasoconstrictors and vasodilators play the vital role
in hypoxia pulmonary vasoconstriction(HPV)
 Cor pulmonale
• The well known vasoconstrictors mainly include 11-
dehydro-thromboxane B2,endothelin-
1,serotonin,vascular endothelin growth factor and
platelet-derived growth factors A and B,etc
• The vasodilators include nitric oxide
synthase(NOs),prostacyclin receptor protein,etc
• The overproduction and release of vasoconstrictors
lead to PVR and cause HPV,subsequently cor
pulmonale develops
 Cor pulmonale
• Impaired NO production or release by endothelial
cells in chronic hypoxia vasoconstriction may
account for the inability of the pulmonary
vasculature to relax impaired NO production may
also be responsible for the proliferation of vascular
smooth muscle cells,medial hypertrophy,and
eccentric intimal fibrosis
• These structural changes may increase platelet
aggregation and in situ thrombi,thereby further
increasing pulmonary vascular resistance
 Cor pulmonale
• Eventually,the cross-sectional areaof the pulmonary
vascular bed decreases and pulmonary hypertension
bacomes irreversible
• Acidosis and secondary polycythemia also
contribute to resistance to blood flow through the
restricted pulmonary vascular bed
• B. Pulmonary vascular resistance due to anatomic
factors
• There are several anatomic factors that explain
PVR
 Cor pulmonale
• Among them the theory of pulmonary vascular
remodeling is believed to be the well-understood
mechanism in explaination of the PVR pathogenesis
• It is accepted that chronic alveolar hypoxia leads to
hypertrophy of the muscular media of the small
pulmoanary arteries,muscularisation of pulmoanary
arteries,and intimal fibrosis
• This remodelling leads to elevation of PVR and to
pulmonary hypertention
 Cor pulmonale
• Some mediators play a role in the development of
pulmonary vascular remodeling
• For instances,endothelin-1 release by endothelial
cells in increased in hypoxic states
• The presence of endothelin-1 may increase calcium
entry into the vascular smooth muscle cells leading
to hypoxic vasoconstriction
• Vascular endothelin growth factor and platelet-
derived growth factors A and B are up-regulated
under conditions of chronic hypoxia
 Cor pulmonale
• These growth factors are involved in endothelial cell
proliferation and vascular injury and remodeling of
the pulmonary vascular bed
• In addition,occlusion or narrowing of medium-size
to large pulmonary arteries is the basis for
increased pulmonary vascular resistance in such
disorders as hilar or mediastinal compressive
metastatic tumors or fibrosis,nonspecific
arteritis,primary pulmonary tumors,major vessel
chronic thromboembolic disease,and infection
 Cor pulmonale
• C. Increased blood volume and blood viscosity
• Other functional factors must be considered,namely
hypercapnic acidosis and hyperviscosity caused by
polycythaemia and erythrocythemia,but their role
seems small when compared to that of alveolar
hypoxia
• In the setting of hypoxia and acidosis,activation of
the renin-angiotensin-aldosterone system leads to
increase in plasma renin activity and increased
levels of aldosterone,vasopressin,and
catecholamines.Natriuretic peptide levels are also
elevated
 Cor pulmonale
 Dysfunction of right heart

Pulmonary hypertension increases the work of the
right ventricle,which leads more or less rapidly to
right ventricular enlargement which can result in
ventricular dysfunction(systolic,diastolic)

Later,right heart failure(RHF)characterized by the
presence of peripheral edema can be observed

Finally,left ventricular dysfunction in cor
pulmonale occurs through ventricular
interdependence in the setting of a limiting
pericardium
 Cor pulmonale
 Impairment of other vital organs
• Hypoxia and hypercapnic acidosis also affect other
vital organs beside heart,such as
liver,kidney,brain,intestine,endocrine system and
hematology system
 Cor pulmonale

CLINICAL PRESENTATION
 Symptoms

Clinical manifestations of cor pulmonale generally
are nonspecific

The symptoms may be subtle, especially in early
stages of the disease, and mistakenly may be
attributed to the underying pulmonary pathology

The patients may complain of fatigue, exertional
dyspnea, and syncope with exertion-symptoms
reflecting a relative inability to increase cardiac
output during exercise with a subsequent drop in
the systemic arterial pressure
 Cor pulmonale

Exertional chest pain aslo can occur and may be
due to pulmanory artery stretching and right
ventricular ischemia
• Other symptoms mainly related to pulmonary artery
hypertention include cough, hemoptysis, and, rarely,
hoarseness due to compression of the left recurrent
laryngeal nerve by a dilated pulmonary artery
• In advanced stages, passive heptic congestion
secondary to severe right ventricular failure may
lead to anorexia, right upper quadrant abdominal
discomfort, and jaundice
• Swelling of the legs also can occur, and syncope is
a late and ominous occurrence
 Cor pulmonale
 Physical Signs
• The most obvious physical findings in cor
pulmonale reflect the underlying lung disease, with
an increase in chest diameter, labored respiratory
efforts with retractions of the chest wall,
hyperresonance to percussion, diminished breath
sounds, wheezing, distant heart sounds, and, rarely,
cyanosis
• Physical findings characteristic of pulmonary
hypertension, RVH, and right ventricular failure
aslo may be present
• Early signs characteristic of pulmonry artery
hypertension include splitting of the second heart
sound with accentuation of the pulmonic
component
 Cor pulmonale
• A systolic ejection murmur with sharp ejection click
over the region of the pulmonary artery may be
heard in advanced disease, along with a diastolic
pulmonary regurgitation murmur
• RVH is charaterized by a left parasternal or
subxiphoid heave
• A prominent A wave in the jugular venous pulse
associated with a right-sided fourth heart sound aslo
can be present and reflects the increase in the filling
pressure of the right ventricle
 Cor pulmonale
• Right ventricular failure leads to systemic venous
congestion, which is manifested as distended neck
veins with a prominent V wave, a right ventricular
third heart sound, a holosystolic tricuspid
insufficiency murmur accentuated during
inspirating and occasionally associated with
pulsatile liver, and peripheral edema. Pleural
effusions and ascites are uncommon, even in severe
cor pulmonale
 Cor pulmonale
 LAB AND OTHER FINDINGS
 Chest Roentgenogram

The chest X-ray in patients with chronic cor
pulmonale may show enlargement of the central
pulmonary arteries with oligemic peripheral lung
fields

Pulmonary hypertension should be suspected when
the right descending pulmonary artery is larger than
16 mm in diameter and the left pulmonary artery is
larger than 18 mm in diameter

Right ventricular enlargement leads to an increase
of the transverse diameter of the heart shadow to the
right on the posterior view and filling of the
retrosternal air space on the lateral view
 Cor pulmonale
 Electrocardiogram
• ECG abnormalities in chronic cor pulmonale refect
the presence of RVH or strain
• ECG criteria for RVH have high specificity but low
sensitivity, particularly patients with COPD and
biventricular hypertromphy
• P-pulmonale paterten due to right artrial
enlargement and right bundle branch conduction
abnormalities may be present
 Cor pulmonale

Echocardiography
• Two-dimensional echocardiography usually
demonstrates signs of chronic right ventricular wall
with paradoxical motion of the interventricular
septum during systole occurs. At an advanced stage,
right ventricular dilatation occurs, and the septum
shows abnormal diastolic flattening
• In extreme cases, the septum actually may bulge
into the left ventricular cavity during diastole
 Cor pulmonale
• Doppler echocardiography: Doppler
echocardiography is considered the most reliable
noninvasive technique to estimate pulmonary artery
pressure. The efficacy of Doppler echocardiography
may be limited by the ability to identify an adequate
tricuspid regurgitant jet, which may be further
enhanced by using saline contrast
 Cardiac Catheterization
• Right-heart catheterization is considered the
most precise method for diagnosis and
quantification of pulmonary hypertension
 Cor pulmonale
• It is indicated when echocardiography cannot assess
the severity a tricuspid regurgitant jet, thus
excluding an assessment of pulmonary hypertension
• Right-heart catheterization occasionally is
important for differentiating cor pulmonal from
occult left ventricular dysfunction. Especially when
the presentation is confusing
• Another indication may be for evaluation of the
potential reversibility of pulmonary arterial
hypertension with vasodilator therapy
 Cor pulmonale
• In selected cases, ventilation-perfusion lung
scaning, pulmonary angiography, chest CT
scanning and lung biopsy may be indicated to
determind underlying etiology. Lung biopsy
occasionally may be indicated to determin
underlying etiology
 Cor pulmonale

DIAGNOSIS
• The diagnosis is based on the medical history,
underlying diseases, pulmonary hypertension, right
ventricular enlargement or dysfunction of right
heart, such as,P2>A2, jugular vein distention,
prominent right ventricular impulse, hepatomegaly
peripheral edema, and lab findings that indicate the
right ventricular enlargement
 Cor pulmonale

DIFFERENTIAL DIAGNOSIS
• Prior to the diagnosis of chronic cor
pulmonale,some other problem must be
considered,such as ventricular septal
defect,congestive heart failure,primary pulmonic
stenosis,primary pulmonary hypertention
 Cor pulmonale
 Treatment

Goals of successful treatment of chronic cor
pulmonale include eviating right failure and
associated left heart failure,improving the patient’s
functional capacity,and lengthening survival

The specific measurements include as follow

Antimicrobial treatment

When the suspected or defined sepsis was found,the
antimicrobial therapy should be initiated as early as
possible

The empirical therapy must based on the local
microbial prevalence and step down after the
referential lab data is available
 Cor pulmonale
 Oxygen Therapy

Oxygen therapy is of most importance in patients
with underlying COPD,particularly when
administered on a continuous basis

With cor pulmonale,the partial pressure of
oxygen(PO2)is likely to be below 55mmHg and
decreases further with exercise and during sleep

Oxygen therapy relieves hypoxemic pulmonary
vasoconstriction,which then improves cardiac
output,lessens sympathetic
vasoconstriction,alleviates tissue hypoxemia,and
improves renal perfusion
 Cor pulmonale
• Thus, treatment pulmonary hypertention is part of an overall
program to manage severe obstructive lung disease
• Two separate clinical trial have shown that intellectual
function and survival are improved in chronically hypoxemic
patients (PaO2<55mmHg)and edematous and show P-
pulmonale on the electrocardiogram
• To be effective oxygen must be administered for at least 18 h
per day.The Nocturnal Oxygen Therapy Trial(NOTT) a
multicenter randomized trial,showed that continuous low-
flow oxygen therapy for patients with severe COPD resulted
in significant reduction in the mortality rate
 Cor pulmonale
 Treatment of Haert Failure

A. Diuretics

Diuretics are used in the management of chronic
cor pulmonale ， particularly when the right
ventricular filling volume is markedly elevated

Diuretics may result in improvement of the function
of both the right and left ventricles

However, diuretics may produce hemodynamic
adverse effects if they are not used cautiously
 Cor pulmonale
• Excessive volume depletion can lead to decline in
cardiac output
• Another potential complication of diuresis is the
production of a hypokalemic metabolic
alkalosis,which diminishies the effectiveness of
carbon dioxide stimulation on the respiatory centers
and lessens ventilatory drive
• Therefore, diuresis,while recommended in the
management of chronic cor pulmonale,needs to be
used with great caution
 Cor pulmonale
• B.Inotropic Agent
• The use of cardiac glycosides,such as digitalis,in
patients with cor pulmonale has been
controversial,and the beneficial effect of these drugs
is not as obvious as in the setting of left heart failure
• Nevertheless,studies have confirmed a modest effect
of digitalis on the failing right ventricle in patients
with chronic cor pulmonale
 Cor pulmonale
• It must be used cautiously,however,and should not
be used during the acute phases of respiratory
insufficiency when large fluctuations in levels of
hypoxia and acidosis may occur
• Patients with hypoxemia or acidosis are at increased
risk of developing arrhythmias due to digitalis
• C. Vasodilators
• Vasodilator drugs have been advocated in the long-
term management of chronic cor pulmonale with
modest results
 Cor pulmonale
• Calcium channel blockers,particularly oral
sustained-release nifedipine and diltiazem,can lower
pulmonary pressures,although they appear more
effective in primary rather than secondary
pulmonary hypertension
• Right heart catheterization has been recommended
during initial administration of vasodilators to
objectively assess the efficacy and detect the
possible adverse hemodynamic consequences of
vasodilators
 Cor pulmonale
• D. Theophylline
• Theophylline has been reported to reduce
pulmonary vascular resistance and pulmonary
arterial pressures acutely in patients with chronic
cor pulmonale secondary to COPD
• Theophylline has a weak inotropic effect and thus
may improve right and left ventricular ejection. As a
result, considering the use of theophylline as
adjunctive therapy in the management of chronic or
decompensated cor pulmonale is reasonable in
patients with underlying COPD
 Cor pulmonale
• E.Anticoagulation
• Anticoagulation with warfarin is recommended in
patients at high risk for thromboembolism
• The beneficial role of anticoagulation in improving
the symptoms and mortality in patients with primary
pulmonary arterial hypertension clearly was
demonstrated in a variety of clinical trials
• The evidence of benefit, however, has not been
established in patients with secondary pulmonary
arterial hypertension.
 Cor pulmonale
• Therefore, anticoagulation therapy may be used in
patients with cor pulmonale secondary to
thromboembolic phenomena and underlying
primary pulmonary arterial hypertension
• F.Miscellaneous
• Treatment of arrhythmia, intensive nursing care,
treatment targeted to the complications, and patient
education must be considered
 Cor pulmonale
• PROGNOSIS
• The prognosis of cor pulmonale is variable
depending upon underlying pathology. Patients with
cor pulmonale due to COPD have a high 2-year
mortality
• The mortality accounts for about 10%-15% of all
the cor pulmonale
 Cor pulmonale
• PROPHYLAXIS
• The goal aims to prevent the disorders of bronchi,
the lung disease and the pulmonary vascular
impairment
• The measurements include education on smoking,
avoidance of the causative factors resulting the
underlying diseases which lead cor pulmonale