ACUTE AND CHRONIC

COR PULMONALE
dr. I Kadek Susila Surya Darma, Sp.JP, FIHA
OUTLINES
DEFINITION

EPIDEMIOLOGI

ETIOLOGI

CLINICAL MANIFESTATION

DIAGNOSIS

MANAGEMENT

PROGNOSIS
DEFINITION
• Cor pulmonale: the altered structure and/or impaired
function of the right ventricle that results from
pulmonary hypertension that is associated with:
 diseases of the lung (eg, chronic obstructive
pulmonary disease),
 vasculature (eg, idiopathic pulmonary arterial
hypertension),
 upper airway (eg, obstructive sleep apnea),
 chest wall (eg, kyphoscoliosis)
EPIDEMIOLOGI
Cor pulmonale is estimated to account for 6-7% of all types of
adult heart disease in the United States, with chronic obstructive
pulmonary disease (COPD) due to chronic bronchitis or
emphysema the causative factor in more than 50% of cases

acute cor pulmonale is usually secondary to massive pulmonary

embolism

Acute massive pulmonary thromboembolism is the most

common cause of acute life-threatening cor pulmonale in adults

50,000 deaths in the United States are estimated to occur per

year from pulmonary emboli
OVERVIEW CIRCULATORY SYSTEM
ETIOLOGI
ETIOLOGI: acute cor pulmonale
Acute cor pulmonale is a disorder in which the right ventricle (RV) is dilated and the muscular
wall is stretched thin

Two main conditions can cause acute cor pulmonale: pulmonary embolism (more common) and
acute respiratory distress syndrome (ARDS)

The underlying pathophysiology in massive pulmonary embolism causing cor pulmonale is the
sudden increase in pulmonary resistance

In ARDS, 2 factors cause right ventricular (RV) overload: the pathologic features of the syndrome
itself and mechanical ventilation. Mechanical ventilation, especially higher tidal volume, requires
a higher transpulmonary pressure.

This may present with often life-threatening cardiogenic shock, or death, but if the patient
survives the initial event, then the RV often recovers and cor pulmonale no longer exists after
several weeks.
ETIOLOGI

Chronic cor pulmonale is hypertrophy of the right ventricle caused by increased afterload in the
pulmonary circuit

Mechanisms leading to pulmonary hypertension and thus predisposing to the development of

cor pulmonale:

1. Pulmonary vasoconstriction leading to increased RV afterload, often resulting from conditions

causing alveolar hypoxia and/or respiratory acidosis (e.g., high altitude/, obstructive sleep
apnea/obesity-hypoventilation syndrome, chronic obstructive pulmonary disease)

2. Anatomic reduction or remodeling of the pulmonary vascular bed leading to increased RV

afterload (e.g., emphysema, interstitial lung disease, pulmonary emboli, pulmonary arterial
hypertension)

3. Increased blood viscosity leading to increased RV afterload (e.g., polycythemia related to

chronic hypoxemia, polycythemia vera, Waldenström’s macroglobulinemia)

4. Chronic thrombotic and/or embolic disease

 In idiopathic pulmonary arterial
hypertension (IPAH), the right
ventricle (RV) is characterized by
increased end-diastolic volume,
change of the normal ventricular
conformation tetrahedron to a
crescentic trapezoid, and varying
degrees of right ventricular
hypertrophy (B).
 The right ventricle in severe idiopathic
pulmonary arterial hypertension
assumes a spherical shape with a
greater cross-sectional area than the
left ventricle (LV), which is normally
larger (A).
 The more spherical-shaped right
ventricle results in abnormal septal
function that also impairs left ventricle
performance.
CLINICAL MANIFESTATION: symptoms
• Usually chronic and slowly progressive, but can be acute

• They are often not recognized as being due to right-sided heart failure because many
can also be caused by the underlying disease (ex.COPD)

• Symptoms: dyspnea on exertion, fatigue, lethargy, exertional syncope, and exertional

angina

• Dyspnea on exertion, fatigue, lethargy, and exertional syncope: the results of an

inability to sufficiently increase cardiac output during exertion because of increased
pulmonary vascular resistance

• Dyspnea on exertion generally emerges first, followed by fatigue, lethargy, and,

eventually, exertional syncope.
CLINICAL MANIFESTATION: symptoms
• Exertional angina can occur in patients with cor pulmonale, even in the absence of
coronary artery disease

• There are two major mechanisms.

• 1. Increased myocardial demand: when myocardial demand increases further due to

elevated transmural wall tension during exertion, the demand may exceed the supply,
precipitating subendocardial right ventricular ischemia

• 2. Dynamic compression of the left main coronary artery due to an enlarged pulmonary
artery (uncommon until the pulmonary artery trunk diameter > 40 mm )

• Anorexia and right upper quadrant discomfort due to passive congestion of the liver
and bowel may occur when right ventricular dysfunction becomes severe.
CLINICAL MANIFESTATION: signs
Physical findings: related to both pulmonary hypertension and right-sided heart disease

Pulmonary hypertension:
• increased intensity of the pulmonic component of the second heart sound (which may be palpable)
• a narrowly split second heart sound (which may be absent if there is a right bundle branch block)
• a holosystolic murmur at the left lower sternal border (tricuspid insufficiency)
• more severe disease: a diastolic pulmonary regurgitation murmur
As the right ventricle becomes hypertrophic:
• elevated right-sided pressures
• right-sided fourth heart sound and either a left parasternal heave or a downward subxiphoid thrust.

When the right ventricle becomes dilated and fails

• resultant systemic venous hypertension can produce an elevated jugular venous pressure
• a right ventricular third heart sound,
• peripheral edema
• Rarely:ascites.
• liver can become enlarged

End-stage cor pulmonale :

• signs of cardiogenic shock, (hypotension, tachycardia, oliguria, and cool extremities) due to low poor stroke
volume.
• Pulmonary edema
LABORATORY INVESTIGATIONS
Defining the potential underlying etiologies as well as evaluating complications of cor
pulmonale.

COMPLETE BLOOD • Hematocrit for polycythemia; Erythrocytosis from chronic

hypoxia.
COUNT
BRAIN NATRIURETIC • BNP may be elevated from RV dilation.
PEPTIDE (BNP)
ARTERIAL BLOOD GAS • May provide important information about the level of
oxygenation and type of acid-base disorder
MEASUREMENTS
CONNECTIVE TISSUE • To exclude scleroderma, lupus, rheumatoid arthritis
DISORDER WORKUP
COAGULATIONS • To evaluate hypercoagulability states
STUDIES
• HIV testing, Serum alpha1-antitrypsin, if deficiency is suspected,
DLL antinuclear antibody level for collagen vascular disease, such as
scleroderma
Chest X-Ray
May show underlying pulmonary disease and evidence of pulmonary
hypertension

Chronic cor pulmonale: enlargement of the central pulmonary arteries

with oligemic peripheral lung fields.

Suspect pulmonary hypertension: 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

Chest X-Ray
• Chest radiography in a
patient with severe intrinsic
pulmonary vascular disease
demonstrating enlargement
of the main pulmonary
artery, right ventricle, and
right atrium.
ELECTROCARDIOGRAPHY (ECG)
• ECG abnormalities  right ventricular hypertrophy (RVH), RV
strain, or underlying pulmonary disease
• Right ventricular hypertrophy: a dominant R wave in V1 and V2
with prominent S waves in V5 and V6, or small R waves and
deep S waves across the precordium
• Right atrial enlargement: increase in the amplitude of the p
wave in lead II (ie, p pulmonale) or a p wave axis of ≥90 degrees
• An S1Q3 pattern, with or without T wave inversion in lead 3,
may be present if the cor pulmonale is acute
• Low-voltage QRS because of underlying COPD with
hyperinflation
• Such electrocardiographic findings are highly specific, but have
low sensitivity, for cor pulmonale.
Doppler echocardiography
Echocardiography directly visualizes the right ventricle; thus, it is useful for detecting
structural changes and estimating function

Pulmonary hypertension without cor pulmonale is characterized by signs of increased

right ventricular pressure

As the disease progresses, right ventricular hypertrophy develops with paradoxical

bulging of the septum into the left ventricle during systole

Right ventricular dilatation eventually occurs with abnormal flattening of the

interventricular septum

The right ventricular dilatation is likely to lead to tricuspid regurgitation, which may
cause right atrial enlargement
Chest CT Scan

• Chest CT Scan assess for pulmonary parenchymal disease

and embolus in the pulmonary vasculature.
WORK-UP DIAGNOSIS
Cardiac magnetic • superior to echocardiography for assessment of right
ventricular size and function
resonance • Right ventricular pressures can also be estimated via
imaging (MRI) cardiac MRI.

• frequently abnormal
Pulmonary • >> impaired diffusion capacity due to the associated
pulmonary hypertension.
function tests • restrictive ventilatory defect.

Right heart • Gold standard for the diagnosis of cor pulmonale

• Evidence of right ventricular dysfunction
catheterization • Evidence of pulmonary hypertension
WORK-UP DIAGNOSIS
• Indications for right heart catheterization
 Symptoms and signs of cor pulmonale exist but the non-
invasive diagnostic testing is either non-diagnostic or normal
 Confirmation of non-invasive findings that suggest cor
pulmonale and pulmonary hypertension
 Determination of whether the right ventricular abnormalities
and elevated pulmonary artery pressures are due to left
heart disease;
 Initiation of therapy with the guidance of direct
hemodynamic measurements
DIAGNOSIS APPROACH
• The diagnostic evaluation of cor pulmonale is inseparable
from the evaluation for pulmonary hypertension
• Doppler echocardiography as the initial diagnostic test
• If suggestive pulmonary hypertension, with or without
cor pulmonale  right heart catheterization
• If the echocardiogram is technically inadequate or is
normal despite a high clinical suspicion  a diagnostic
right heart catheterization.
• Cardiac MRI to evaluate the right ventricle is a
reasonable option for patients who request additional
noninvasive testing before agreeing to right heart
catheterization
DIFFERENTIAL DIAGNOSIS
• Atrial myxoma
• Blood disorders that are associated with increased
blood viscosity
• Congestive (biventricular) heart failure
• Costrictive pericarditis
• High-output heart failure
• Infiltrative cardiomyopathies
• Primary pulmonic stenosis
• Right heart failure due to right ventricular infarction
• Right-sided heart failure due to congenital heart
diseases
MANAGEMENT
• The treatment of cor pulmonale is directed toward the
underlying etiology while also reversing hypoxemia, improving
right ventricular contractility, decreasing pulmonary artery
vascular resistance, and improving pulmonary hypertension
Nonpharmacologic Therapy
• Supplemental oxygen to correct hypoxemia is an important
• Goal O 2 sat of more than 90% is recommended.
• Continuous positive airway pressure is used in patients with
obstructive sleep apnea.
• Sodium and fluid restriction in setting of edema from RV
failure
• Phlebotomy is reserved as adjunctive therapy in patients with
polycythemia (hematocrit >55%)
• Low-level aerobic exercise, avoiding heavy physical exercise,
or isometric exercises that may cause syncope
MANAGEMENT: Acute General Rx

Pulmonary • the most common cause of acute cor pulmonale ->

anticoagulation, hemodynamic support, and consideration of
thrombolytics

embolism • Cathether-based thrombectomy or surgical embolectomy.

• Related to hypercapnia/acidosis, hypoxic pulmonary

(ARDS) vasoconstriction, and the effects of mechanical ventilation
• Tx: supportive care for ARDS with low tidal volume ventilation
MANAGEMENT: Acute General Rx

Treat the underlying exacerbating conditions

Fluid balance is important in the management of acute decompensated

right heart failure.

monitoring of intake and output, electrolytes and creatinine, and

hemodynamics.

Intravenous inotropes

In acutely decompensated right heart failure due to pulmonary arterial

hypertension, IV prostanoid medications are the treatment of choice.
MANAGEMENT: Chronic Rx
Long-term oxygen supplementation improves survival in hypoxemic patients with COPD.
Indications for oxygen in COPD patients include the following:

• 1. Resting PaO 2 ≤55 mm Hg on arterial blood gas

• 2. Resting PaO 56–59 mm Hg if clinical presence of right heart failure with dependent edema,
P pulmonale on EKG
• 3. Desaturation to SpO 2 ≤88% on oximetry with activity or at night

Right ventricular volume overload should be treated with loop diuretics (e.g., furosemide)
and potassium-sparing diuretics such as spironolactone.

Anticoagulation with Warfarin a target international normalized ratio (INR) of 1.5-2.5

Digoxin, though controversial, may be employed as an oral inotropic agent to improve right
heart contractility and control atrial arrhythmias.
MANAGEMENT: Chronic Rx
• Selective oral or parenteral pulmonary vasodilators such
as oral calcium channel blockers (CCBs), prostanoids, and
endothelin receptor blockers may be used after a right
heart catheterization that establishes pulmonary
hypertension.
• If vasoreactivity to calcium channel blockers is
established, CCBs may be used to combat pulmonary
hypertension.
• However, if there is no vasoreactivity, prostanoids such as
epoprostenol or treprostinil or endothelin receptor
blockers such as bosentan may be tried.
• Phosphodiesterase inhibitors such as sildenafil and
tadalafil are newer vasodilators that improve symptoms,
functional class, and mean pulmonary artery pressures as
alternatives or in addition to prostanoids.
MANAGEMENT: Surgical Management of Cor Pulmonale
• Phlebotomy: indicated in patients with chronic cor pulmonale and chronic hypoxia causing severe
polycythemia, defined as hematocrit of 65% or more

• Phlebotomy : decrease in mean pulmonary artery pressure, a decrease in mean pulmonary vascular
resistance, and an improvement in exercise performance in such patients

• Phlebotomy should be reserved as an adjunctive therapy for patients with acute decompensation of cor
pulmonale and who remain significantly polycythemic despite appropriate long-term oxygen therapy.

• Pulmonary embolectomy : for unresolved pulmonary emboli.

• Uvulopalatopharyngoplasty (UPPP) in selected patients with sleep apnea and hypoventilation may relieve cor
pulmonale.

• Atrial septostom: salvage option for patients with ongoing decompensated right heart failure from PAH
despite pulmonary vasodilators  rapid assessment for possible transplantation.

• Single-lung, double-lung, and heart-lung transplantation

• Lung transplantation may lead to a reversal of right ventricular dysfunction from the chronic stress of
pulmonary hypertension.
MANAGEMENT: Outpatient Monitoring
• Require close attention in the outpatient setting. Regularly assess the
patient’s oxygen needs and pulmonary function
• Consider a formal program of pulmonary rehabilitation, as many patients
benefit from this therapy.
• Regular assessment of their functional class (e.g., NYHA or WHO
functional class); worse functional class indicates a poorer prognosis.
• Other poor prognostic features: high right atrial pressure, impaired cardiac
output, presence of hyponatremia, elevated BNP levels, low systemic
blood pressure, and poor exercise tolerance as demonstrated by a 6-
minute walk test.
• Referral : Patients with pulmonary disease who have progressed to cor
pulmonale should be followed up by a pulmonologist and a tertiary care
center that specializes in treatment of advanced pulmonary hypertension.
PROGNOSIS
Cor pulmonale is the most common cause of
hospitalization among patients with pulmonary arterial
hypertension

In-hospital mortality was 14 percent, but this increased

to 45 to 50 percent among patients who required
inotropic medications or were admitted to the intensive
care unit.

Predictors of in-hospital mortality included connective

tissue disease, a systolic blood pressure <100 mmHg, and
a serum sodium ≤136 mEq/L.

Mortality following discharge was 13, 26, and 35 percent

at 3, 6, and 12 months, respectively.
SUMMARY AND RECOMMENDATIONS
• Cor pulmonale refers to the altered structure and/or impaired function of the right ventricle that results from
pulmonary hypertension associated with diseases of the lung, upper airway, or chest wall.

• Right-sided heart disease due to left-sided heart disease or congenital heart disease is NOT considered cor
pulmonale.

• Cor pulmonale tends to be chronic and slowly progressive, but can be acute. Symptoms attributable to cor
pulmonale include dyspnea on exertion, fatigue, lethargy, exertional syncope, and exertional angina.

• Patients with end-stage cor pulmonale may develop evidence of cardiogenic shock, including hypotension,
tachycardia, oliguria, and cool extremities.

• The diagnostic evaluation of cor pulmonale is inseparable from the evaluation for pulmonary hypertension.

• All patients with cor pulmonale should have the underlying cause of the cor pulmonale and pulmonary
hypertension treated
SUMMARY AND RECOMMENDATIONS
• Those patients whose cor pulmonale and pulmonary hypertension persist despite treatment of the underlying
cause should be referred to a specialized center to be evaluated for possible pulmonary hypertension-specific
therapy.

• Pulmonary hypertension-specific therapy should not be administered outside of this setting.

• For patients with cor pulmonale who are hypoxemic  supplemental oxygen therapy (Grade 1B)

• For patients with cor pulmonale who are hemodynamically stable but have high right ventricular filling
pressures -> diuretic therapy

• For patients with cor pulmonale who are in cardiogenic shock with right ventricular volume overload 
diuretic therapy

• For patients with cor pulmonale who do not have coexisting atrial fibrillation or left ventricular dysfunction:
AGAINST prescribing an oral inotropic agent (eg, digoxin)

• For patients with cardiogenic shock due to cor pulmonale  administration of an intravenous inotropic agent:
dobutamine, milrinone