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Cor Pulmonale •
control mechanisms.
Excludes
– Left sided heart dis.
with 2nd changes
Cătălina Lionte, MD, PhD – Congenital heart dis.
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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
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
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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
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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
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
Cor Pulmonale
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Doppler Echocardiography 2D Echo with Color Flow Doppler
Howlett JG, McKelvie RS, Arnold JMO et al. Can J Cardiol 2009;25(2):85-105.
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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)
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Prognosis of Cor Pulmonale Treatment
• Oxygen
• When due to COPD, PHTn plus peripheral – Relieves pulmonary vasoconstriction
• Decreases PVR
edema
• Increases RV Stroke volume and cardiac output
– 5 year survival 30%, mean 3 years from dx • Renal vasoconstriction may be relieved with
– Pulmonary vascular resistance >550 dynes-
dynes- increase in urinary sodium excretion
sec/cm rarely survive more than 3 years – Improves arterial oxygen tension with
– May just reflect the degree of underlying enhanced delivery to
COPD • Heart
• Brain
• Other vital organs (kidneys)
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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 *Antibiotics: commonly used antibiotics include penicillin, aminoglycosides,
• Treat underlying condition 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 Summary
Cor Pulmonale
• Treatment of pulmonary embolism • is an end stage manifestation of primary right
sided heart failure.
• Cautious expansion of blood volume to • For the most part, treatment is supportive.
maintain cardiac output
• In COPD, oxygen is a mainstay of therapy.
• Inhalation of 100% oxygen • Diuretics, ACEI, ARB, beta blockers may add
• Primary therapy efficacy.
– Clot dissolution with thrombolysis or • Better drug therapy, directed at pulmonary
– Removal of pulmonary embolus by embolectomy artery relaxation, may be on the horizon.
• Secondary prevention • Whatever the etiology the prognosis remains
– Anticoagulation with heparin and warfarin and/or poor
– Placement of an inferior vena cava filter
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