Primary Right Heart Failure

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Primary Right Heart Failure

• (Cor Pulmonale)

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Cor Pulmonale (CP) defined

• Alteration of the right ventricular structure or
  function that is due to pulmonary hypertension
  (PHTn) caused by diseases affecting the lung or
  its vasculature.
• Excludes
• Left sided heart disease with 2nd changes
• Congenital heart disease

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Etiologies

• Pulmonary Artery vasoconstriction
• Alveolar hypoxia
• Blood acidosis
• Anatomic redn of pulmonary vascular bed
• Emphysema
• Pulmonary emboli
• Increased blood viscosity
• Erythrocytosis (Includes polycythemia)
• Sickle-cell disease
• Increased pulmonary blood flow

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Patients with COPD

• Most frequent cause of cor pulmonale
• Right ventricular hypertrophy (RVH) in
• 40 of patients with FEV1
• 70 of patients with FEV1
• Independent predictors of RVH
• Hypoxemia
• Hypercapnea
• Erythrocytosis (not Polycythemia)

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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-sec/cm
  rarely survive more than 3 years
• May just reflect the degree of underlying COPD

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Symptoms of CP

• Directly attributable to PHTn
• Dyspnea on exertion, fatigue, lethargy
• Chest pain, syncope with exertion
• Typical exertional angina
• Occurs in patients with primary or secondary PHTn
  even in the absence of epicardial CAD
• Subendocardial RV ischemia induced by hypoxemia
  and increased transmural wall tension
• Dynamic compression of left main coronary by
  enlarged PA
• Less common
• Cough, hemoptysis, hoarseness
• With severe right ventricular (RV) failure
• Passive hepatic congestion
• Anorexia, right upper quadrant discomfort

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Physical Findings

• Cardiac findings
• RVH
• Prominent A wave in the jugular venous pulse.
  with R sided 4th heart sound
• RV failure leads to systemic venous HTn
• Elevated jugular venous pressure with a prominent
  V wave
• RV S3
• High pitched tricuspid regurgitant (TR) murmur
• Extra cardiac changes
• Hepatomegaly, pulsatile liver
• peripheral edema-often related to hypercarbia and
  passive Na and water retention

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Other Areas of Fluid Retention

• Pleural effusion, often bilateral
• Right heart failure until proved otherwise
• Also kidney and liver
• Engorged inferior vena cava
• Hepatic congestion
• Ascites
• Anasarca

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Right Atrial Pressure Tracing

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Jugular Pulsations

• 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.

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Hepatojugular Reflux

• 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
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Peripheral Edema

• Edema formation requires
• Alteration in capillary hemodynamics that favors
  the movement of fluid from the vascular space
  into the interstitium (IS)
• The retention of dietary or IV administered
  sodium and water by the kidneys.
• Requires 2.5 to 3.0 liters of extra volume
• Sequence of events
• Movement of fluid from vascular space into the IS
  reduces the plasma volume and consequently tissue
  perfusion
• The kidney then compensates by retaining sodium
  and water

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Evaluation

• Laboratory CBC, chem 7, LFTs, BNP
• Chest radiograph
• Electrocardiogram
• Two D and Doppler echocardiography
• Pulmonary function tests
• Radionuclide ventriculography
• Magnetic resonance imaging
• Right heart catheterization
• Lung biopsy

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Laboratory

• CBC-depressed or elevated Hgb, Hct
• Chem 7-relationship of BUN to Creatinine
• Normal ratio BUN/Cr approximates 20/1
• Prerenal azotemia 20/1
• Intrinsic renal disease
• Estimated glomerular filtration rate (eGFR)
• Liver function tests
• SGOT
• SGPT

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Brain Natriuretic Peptide (BNP)

• A hormone released from myocardial cells
• Both atria and both ventricles
• Inhibits weakly
• Renin-angiotensin system (Angiotensin II)
• Endothelin secretion
• Systemic and renal sympathetic activity
• Plasma aldosterone production

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BNP Continued

• Higher in
• Older younger
• Women men
• Normal weight obese
• Renal failure
• Congestive heart failure (right and/or left)
• Patient is his own reference point
• Baseline
• Post treatment

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BNP Continued, Prognosis

• HF pts.- Highest quartile at baseline had higher
  mortality over 2 years at baseline (32.4 vs 9.7)
  than lowest quartile.
• Following optimal medical treatment mortality
  increased proportionately to the level of the BNP
  elevation.

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Normal Chest Radiograph
Normal chest film
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Radiograph in Cor Pulmonale
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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

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Normal Electrocardiogram
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Right Atrial Enlargement on ECG

Right atrial enlargement                       
                                                  
 
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ECG in Cor Pulmonale
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Normal
Cor Pulmonale
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Two Dimensional Echocardiogram

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Two D Echo, continued
Tricuspid Regurgitation (TR)
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Doppler Echocardiography

• Most reliable noninvasive estimate of the
  Pulmonary Artery Pressure (PAP)
• Dependent on identifying an adequate tricuspid
  regurgitant jet
• More sensitive as PAP increases

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2D Echo with Color Flow Doppler
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Pulmonary Function Testing (PFTs)

• Primer and overview
• Satisfactory effort
• Obstruction
• Restriction
• Malingering

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PFT Expiratory Maneuver
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Expiratory Flow/Volume Loop
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Right Sided Cardiac Catheterization

• When echo does not permit measurement of TR
• When symptoms are exertional and left sided
  pressures are unremarkable
• When therapy will be determined by precise
  measurement of pulmonary vascular resistance
  (PVR) and the response to vasodilators
• When left heart catheterization is also required
  (patients 40 y/o and or with CAD)

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Lung Biopsy

• Rarely, if ever required
• High risk procedure (elevated PVP, PAP)
• Transbronchial lung biopsy first
• Fiber optic thoracoscopy
• Never open thoracotomy

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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)

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Effects of O2 Therapy in COPD
Survival benefit of LTOT in COPD
                                                  
             
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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

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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

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Treatment, Continued

• Digoxin is NOT indicated in pure CP
• These PA Vasodilators are of NO benefit
• Hydralazine
• Nitrates
• Nifedipine
• Verapamil

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Theophylline/Terbutaline

• Has effects other than direct bronchial
  dilatation and diuresis
• Improves myocardial contractility
• Provides some degree of pulmonary vasodilatation
• Enhances diaphragmatic endurance
• Narrow range of efficacy

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Phlebotomy

• When hematocrit 55
• Goal is hematocrit
• Secondary Erythrocytosis vs Polycythemia
• Treat underlying condition

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Treatment, The Future?

• The following is for completeness and is not
  considered applicable for most pts with PHTn due
  to
• The route of administration
• Cost
• Side effects
• ? Long term efficacy

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Nitric Oxide (INOmax)

• Initially in neonates with PHTn
• Relaxes vascular smooth muscle
• Binds to cytosolic guanylate cyclase
• Activates guanylate cyclase
• Increases intracellular levels of cyclic
  quanosine 3,5-monophosphate
• Produces vasodilatation
• Can only be used in inhalational form
• ½ life 15-30 seconds

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Epoprostenol (Flolan)

• Also called Prostacyclin (PG 12)
• Strong vasodilator of all vascular beds
• Decreases platelet aggregation and thrombogenesis
• Increases cyclic Adenosine Monophosphate
• Stimulates intracellular adenate cyclase
• ½ Life elimination six minutes
• Requires continuous central line and pump
• Cost to VA is 29,400/year

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Sildenafil (Viagra, Revatio)

• Phosphodiesterase 5 enzyme inhibitor (PDE 5)
• 20-60 mgm TID oral dosing schedule
• Yearly cost to VA 3180 to 9540/year
• Same benefits do not occur with other PDE5
  Inhibitors

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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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Questions or comments? Have at it!
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Glossary COPD Chronic Obstructive Pulmonary
Disease CP Cor Pulmonale DALYs Disability
Adjusted Life Years EC(K)G Electrocardiogram eGF
R Estimated Glomerular Filtration Rate HF Heart
Failure HTn Hypertension IS Interstitium JV Jug
ular Vein JVP Jugular Venous Pressure LV Left
Ventricle PA Pulmonary Artery PFT Pulmonary
Function Test PHTn Pulmonary Hypertension PDE5
Phosphodiesterase-5 Enzyme
Inhibitor RAP Right Atrial Pressure RV Right
Ventricle RVH Right Ventricular
Hypertrophy TR Tricuspid Regurgitation
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That'll do it!