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
Cătălina 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, LFT’s, 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 PA’s

• 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 (PFT’s)
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–
ventilation–perfusion 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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