Definition

Alteration of the right ventricular structure or

function that is due to pulmonary hypertension
(PHTn) caused by diseases affecting the lung or
its vasculature,
vasculature, airways, thorax, or respiratory

Cor Pulmonale
control mechanisms.
Excludes
Left sided heart dis.
with 2nd changes
Ctlina Lionte, MD, PhD Congenital heart dis.

Cor pulmonale may be The most common cause of chronic cor

pulmonale - COPD
acute or chronic. Etiology of Cor Pulmonale ( I )
Lung and Airways Vascular
The most common COPD Occlusion
cause of acute cor Asthma Multiple Emboli
pulmonale Bronchiectasis Schistosomiasis
DILD (Diffuse
Massive or multiple Infiltrative Lung Filariasis
pulmonary emboli Disease) Sickle Cell
Pulmonary P. Pulmonary
tuberculosis
Hypertension

Etiology of Cor Pulmonale ( II ) Etiology of Cor Pulmonale ( III )

Thoracic Cage N-M Disease Abnormal Respiratory Control
Kyphosis > 100 o Polio Myelitis Idiopathic hypoventilation Syndrome
Scoliosis > 120 o Myasthenia Gravis Obesity hypoventilation syndrome
ALS (Amyotrophic
(Amyotrophic (Pick-
(Pick-Wickian syndrome)
Thoracoplasty lateral sclerosis )
Pleural fibrosis Muscular
Cerebrovascular disease
Dystrophy

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 Hypercapnea
PATHOPHYSIOLOGY Hypoxia Anatomic changes
Pathophysiological mechanisms causing pulmonary Acidemia
hypertension include:
Pulmonary Artery vasoconstriction Pulmonary Vessel
Alveolar hypoxia
Blood acidosis
Restriction
Anatomic reduction
reduction of pulmonary vascular bed
Increased
secondary to lung disorders Viscosity Increased C.O.
Emphysema
Pulmonary emboli Acidosis
Increased blood viscosity Chronic Cor Pulmonale
Erythrocytosis (Includes polycythemia)
Sickle-
Sickle-cell disease
Idiopathic primary pulmonary hypertension
Right Ventricular Failure

PATHOPHYSIOLOGY Patients with COPD

Failure of right ventricle:
Pulmonary hypertension Most frequent cause of cor pulmonale
Myocardial anoxia Right ventricular hypertrophy (RVH) in
40% of patients with FEV1 < 1.0 L
Repeatedly pulmonary infection: 70% of patients with FEV1 < 0.6 L
effect of bacterial toxin to the heart Independent predictors of RVH
Acid base disorder:
disorder: Hypoxemia
Hypercapnea
arrhythmia
Erythrocytosis (not Polycythemia)

Pathologic Features
Lung : consistent with Natural History
Specific diseases
Common Features:
Features: Several months to years to develop
hypertrophy of
microvasculatures All ages from child to old people
Hallmark : Rt. Ventricular Repeated infections aggravate RV strain
Hypertrophy into RV failure
60g 200g, > 0.5 CM,
RV/LV <2.5 Initilly respondes well to therapy but
Left Ventricular progressively becomes refractory
Hypertrophy
Hypertrophy of Carotid
Body

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Symptoms of CP Physical Findings
Directly attributable to PHTn Cardiac findings
Dyspnea on exertion, fatigue, lethargy RVH
Chest pain, syncope with exertion
Prominent A wave in the jugular venous pulse.
Typical exertional angina with right sided 4th heart sound
Occurs in patients with primary or secondary PHTn even in the
absence of epicardial CAD RV failure leads to systemic venous HTn
Subendocardial RV ischemia induced by hypoxemia and Elevated jugular venous pressure with a prominent
increased transmural wall tension V wave
Dynamic compression of left main coronary by enlarged PA
RV S3
Less common High pitched tricuspid regurgitant (TR) murmur
Cough, hemoptysis, hoarseness
With severe right ventricular (RV) failure Extra cardiac changes
Passive hepatic congestion Hepatomegaly, pulsatile liver
Anorexia, right upper quadrant discomfort peripheral edema-
edema-often related to hypercarbia and
passive Na+ and water retention

Other Areas of Fluid Retention Right Atrial Pressure Tracing

Pleural effusion, often bilateral

Right heart failure until proved otherwise
Also kidney and liver
Engorged inferior vena cava
Hepatic congestion
Ascites
Anasarca

Jugular Pulsations Hepatojugular Reflux

A wave
RAP transmitted to jugular veins (JV)
during right atrial systole
V wave
Rise in RA and JVP due to continued
inflow of blood to the venous system
during late ventricular systole when the
tricuspid valve is still closed
May also be elevated in heart failure
and renal failure, but not cirrhosis.
Assessed by applying firm sustained
pressure over the upper abdomen with pt.
breathing quietly.
Response
Transient elevation by approximately 1 cm in
normal response
In RHF sustained elevation
Low specificity and sensitivity

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 Major Physical Finding in Edematous States Peripheral Edema
Disorder
Pulmonary Central venous Ascites and/or Edema formation requires
edema pressure pedal edema
Alteration in capillary hemodynamics that favors the
Left-
Left-sided heart + Variable - movement of fluid from the vascular space into the
failure
interstitium (IS)
Right-
Right-sided - Variable + The retention of dietary or IV administered sodium
heart and water by the kidneys.
failure
Cirrhosis - Normal + Requires 2.5 to 3.0 liters of extra volume
Renal disease Variable + Sequence of events
Nephrotic - Variable +
Movement of fluid from vascular space into the IS
syndrome reduces the plasma volume and consequently tissue
Idiopathic - -Normal + perfusion
edema
The kidney then compensates by retaining sodium
Venous - Normal +, edema may be
insufficiency asymmetric and water

Symptoms & Signs - Acute

cor pulmonale Evaluation
Sudden onset of severe dyspnea and
cardiovascular collapse Laboratory CBC, chem.test, LFTs, BNP
Occurs in the setting of massive pulmonary Chest radiograph
embolism Electrocardiogram
Pallor Two D and Doppler echocardiography
Sweating Pulmonary function tests
Hypotension Radionuclide ventriculography
Rapid pulse of small amplitude Magnetic resonance imaging
Neck vein distention Right heart catheterization
Pulsatile distended, tender liver Lung biopsy
Systolic murmur of tricuspid regurgitation along the
left sternal border
Presystolic (S4) gallop

Laboratory Brain Natriuretic Peptide (BNP)

CBC-
CBC-depressed or elevated Hgb, Hct A hormone released from myocardial cells
Chem.test-
Chem.test-relationship of BUN to Creatinine
Both atria and both ventricles
Normal ratio BUN/Cr approximates 20/1
Prerenal azotemia > 20/1 Inhibits weakly
Intrinsic renal disease < 20/1 Renin-
Renin-angiotensin system (Angiotensin II)
Estimated glomerular filtration rate (eGFR)
Endothelin secretion
Liver function tests
SGOT
Systemic and renal sympathetic activity
SGPT Plasma aldosterone production

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BNP Continued BNP Continued, Prognosis
Higher in HF pts.-
pts.- Highest quartile at baseline had
Older >younger higher mortality over 2 years at baseline
Women > men (32.4 vs 9.7%) than lowest quartile.
Normal weight > obese
Renal failure
Following optimal medical treatment
Congestive heart failure (right and/or left) mortality increased proportionately to the
Patient is his own reference point level of the BNP elevation.
Baseline
Post treatment

Normal Chest Radiograph Radiograph in Cor Pulmonale

Normal chest film

Radiograph and Cor Pulmonale

Radiograph and Cor Pulmonale

Enlargement of Central PAs

In 95% of Pts with PHTn from COPD the
diameter of the descending branch of the
right PA is > 20 mm in width
Peripheral vessels are attenuated leading
to peripheral oligemia

Posteroanterior chest radiograph showing severe Lateral chest radiograph showing severe
pectus excavatum and the complete pectus excavatum and the complete
displacement of the heart into left the displacement of the heart into the left
hemithorax. hemithorax.

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Normal Electrocardiogram Right Atrial Enlargement on ECG

Right atrial enlargement

ECG in Cor Pulmonale

Normal

Cor Pulmonale

Two Dimensional Echocardiogram

Two D Echo, continued
Tricuspid Regurgitation (TR)

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 Doppler Echocardiography 2D Echo with Color Flow Doppler

Most reliable noninvasive estimate of the

Pulmonary Artery Pressure (PAP)
Dependent on identifying an adequate
tricuspid regurgitant jet
More sensitive as PAP increases

Differentiating features between RHF

with or without cor pulmonale/
pulmonale/
pulmonary arterial hypertension Pulmonary Function Testing (PFTs)
Cor pulmonale/
pulmonale/pulmonary hypertension
RHF without pulmonary hypertension present
Chest x-
x-ray: Enlargement of Chest x-
x-ray: Right-
Right-sided cardiac Primer and overview
pulmonary arteries (uncommon), enlargement, enlargement of
oligemic peripheral lung fields (rare) pulmonary arteries, oligemic peripheral Satisfactory effort
lung fields
Echocardiography: No evidence of
increased pulmonary pressure. Echocardiography: Evidence of increased
Obstruction
Septal flattening during diastole but pulmonary pressure. Septal flattening Restriction
not systole during systole
Physical examination: Evidence of Malingering
underlying pulmonary pathology if cor
pulmonale present (but not in primary
PAH)

Howlett JG, McKelvie RS, Arnold JMO et al. Can J Cardiol 2009;25(2):85-105.

PFT Expiratory Maneuver Expiratory Flow/Volume Loop

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 Right Sided Cardiac Catheterization Lung Biopsy
When echo does not permit measurement of TR Rarely, if ever required
When symptoms are exertional and left sided High risk procedure (elevated PVP, PAP)
pressures are unremarkable
When therapy will be determined by precise
Transbronchial lung biopsy first
measurement of pulmonary vascular resistance Fiber optic thoracoscopy
(PVR) and the response to vasodilators Never open thoracotomy
When left heart catheterization is also required
(patients > 40 y/o and or with CAD)

Acute cor pulmonale Differential Diagnosis

Arterial blood gas Coronary artery disease:
disease: Angina pectoris,
Reduced partial pressure of arterial oxygen (PaO2)
Myocardial infarction history &
due to ventilation
ventilationperfusion mismatch Left ventricular hypertrophy (EKG)
Low partial pressure of carbon dioxide (PaCO2) due Rheumatic heart disease:
disease:
to hyperventilation
History & echocardiogram
BNP and N-
N-terminal BNP levels are: Myocardial disease:
disease:
dramatically elevated in acute pulmonary embolism
Without chronic respiratory disease
Spiral CT of the chest Echocardiogram
Useful in diagnosing acute thromboembolic disease
The total heart enlarged

Differential Diagnosis Complication:

Pulmonary encephalopathy
Liver cirrhosis Acid-base disorder and electrolyte disturbances
Nephrotic syndrome Shock
Renal failure with significant volume DIC
overload
Arrhythmia
Gastrointestinal hemorrhage

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 Prognosis of Cor Pulmonale
When due to COPD, PHTn plus peripheral
edema
5 year survival 30%, mean 3 years from dx
Pulmonary vascular resistance >550 dynes-
dynes-
sec/cm rarely survive more than 3 years
May just reflect the degree of underlying
COPD
Effects of O2 Therapy in COPD
Survival benefit of LTOT in COPD
Complimentary Treatments Related to Severity of
PHTn and its Systemic Effects
Furosemide/Bumetanide/Torsemide
loop diuretics
Hydrochlorothiazide
blocks sodium reabsorption
Spironolactone/Eplerinone
Blocks aldosterone effect on both kidney and heart
Angiotensin Converting Enzyme (ACE) inhibitor/ACE
Receptor Blockers
Blocks Renin and Angiotensin
Beta blockers (metroprolol, Atenolol, Carvedilol)
Blocks effect of norepinephrine
Treatment
Oxygen
Relieves pulmonary vasoconstriction
Decreases PVR
Increases RV Stroke volume and cardiac output
Renal vasoconstriction may be relieved with
increase in urinary sodium excretion
Improves arterial oxygen tension with
enhanced delivery to
Heart
Brain
Other vital organs (kidneys)
Treatment-Diuretics
Increasing RV filling volume using diuretics
Improve function of both RV and LV
As RV dilatation is reduced LV filling improves
May improve cardiovascular performance
Monitor for excessive volume depletion
BUN (blood urea nitrogen) Prerenal
Creatinine Renal
Estimated glomerular filtration rate (eGFR)
Watch for metabolic alkalosis
May suppress ventilation
Treatment, Continued
Digoxin is NOT indicated in pure CP
Digitalis is used by some clinicians to support
the failing right ventricle.
The indications of digitalis are:
1.uncontrolled heart failure therapy of diuretics
after infection controlling and respiratory
function improved;
2.evident signs of right heart failure without
severe infection;
3.accompanied
3.accompanied acute left heart failure.
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Treatment, Continued Theophylline/Terbutaline

These PA Vasodilators are of NO benefit Has effects other than direct bronchial
Hydralazine dilatation and diuresis
Nitrates Improves myocardial contractility
Nifedipine Provides some degree of pulmonary
Verapamil vasodilatation
Enhances diaphragmatic endurance
Narrow range of efficacy

Treatment
Phlebotomy Acute exacerbation period
Controlling infection, clearing airways, elevating
respiratory function, improving hypoxia and
When hematocrit > 55 hypercapnia, and correcting respiratory failure and
Goal is hematocrit < 50 cardiac failure are the priority.

Secondary Erythrocytosis vs Polycythemia
Treat underlying condition
*Antibiotics: commonly used antibiotics include penicillin, aminoglycosides,
quinolones, and cephalosporins.
- select antibiotics on the basis of surrounding and sputum smear
Gram stain to do the empiric treatment.
- Gram stain positive pathogens are predominant in community-
acquired infection mostly, while Gram stain negative pathogens
are predominant in hospital-acquired infection.

Treatment
Compensation: Treatment
Preventive measure:
measure:
Breath training
Elevate the power of resistance:
resistance:
Improve nutritional status Prevent respiratory infection
Home oxygen therapy Physical exercise
Long term oxygen therapy is indicated for patients
with persistent arterial hypoxemia at rest or after Environmental health
exercise (arterial oxygen tension consistently Stop smoking
below 55mmHg while breathing room air.
Lung function monitoring

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Acute cor pulmonale
Treatment of pulmonary embolism
Cautious expansion of blood volume to
maintain cardiac output
Inhalation of 100% oxygen
Primary therapy
Clot dissolution with thrombolysis or
Removal of pulmonary embolus by embolectomy
Secondary prevention
Anticoagulation with heparin and warfarin and/or
Placement of an inferior vena cava filter
Summary
Cor Pulmonale
is an end stage manifestation of primary right
sided heart failure.
For the most part, treatment is supportive.
In COPD, oxygen is a mainstay of therapy.
Diuretics, ACEI, ARB, beta blockers may add
efficacy.
Better drug therapy, directed at pulmonary
artery relaxation, may be on the horizon.
Whatever the etiology the prognosis remains
poor
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