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Cor Pulmonale

Pulmonary Heart Disease

Cor pulmonale is defined as an alteration in the structure and function of the right ventricle of the heart as a response to a disorder of the respiratory system. The chronic form usually leads to right ventricular hypertrophy, the acute form results in dilatation.


Presentation

The symptoms of cor pulmonale, pulmonary heart disease vary as the disorder progresses. Individuals may be asymptomatic in the early stages or symptoms will suggest other milder conditions. However, as the disease progresses symptoms become more severe but vary between patients. Symptoms of pulmonary heart disease may include [2] [3] [14]:

Physical indicators of cor pulmonale on exam[3][14]:

Pulmonary heart disease can lead to congestive heart failure (CHF) [3]. The symptoms of congestive heart failure include[1][3]:

  • Worsening of respiratory symptoms
  • Pulmonary edema
  • Peripheral edema 
  • Congestive hepatomegaly

Complications of cor pulmonale include [1] [3] congestive heart failure and liver cirrhosis due to chronic congestion in the systemic venous system, liver hypoxia and fatty liver changes.

Cough
  • Symptoms: Some of the early signs of cor pulmonale include constant cough, difficulty breathing, fatigue and weakness.[medigoo.com]
  • Symptoms of CPM include fatigue, lethargy, dypsnoea on exertion, tachypnoea, chest pain, exertional syncope, dyspnoea at rest, nonproductive cough, haemoptysis, hoarseness, and anorexia.[link.springer.com]
  • Cough with sputum that is discolored or tinged with blood. Last updated 21 December 2011[nmihi.com]
  • Symptoms include chronic cough, shortness of breath on exertion, fatigue, and other signs of oxygen lack to the body tissues.[curlie.org]
Dyspnea
  • ) pronounced neck veins inability to tolerate exercise Pulmonary hypertension and cor pulmonale can lead to severe fluid retention which, in turn, can cause life-threatening dyspnea, shock, and even death.[verywell.com]
  • Cor pulmonale PANCE Blueprint Pulmonary (12%) Presentation Pearls Diagnostic studies Treatment Media Patient will present as a 65-year-old man who presents with a chronic productive cough, dyspnea, and wheezing.[smartypance.com]
  • Determine the amount and type of dyspnea and if it is related only to exertion or is continuous. Physical examination The patient may appear acutely ill with severe dyspnea at rest and visible peripheral edema .[medical-dictionary.thefreedictionary.com]
  • Nonspecific: fatigue, tachypnea, exertional dyspnea, cough, anginal chest pain, hemoptysis, hoarseness What nonspecific signs may indicate underlying lung disease Increase in chest diameter, labored respiratory efforts with chest wall retractions, distended[quizlet.com]
Chronic Cough
  • Symptoms include chronic cough, shortness of breath on exertion, fatigue, and other signs of oxygen lack to the body tissues.[curlie.org]
  • Cough Difficulty in breathing after exercise Wheezing Weak/Fatigue Fluid collection in the legs Pain in abdomen Digestive disturbances Swollen veins in neck Treatment Acute- Removal of the pulmonary blockage Chronic- Antibiotics to fight against respiratory[prezi.com]
  • A person with cor pulmonale has a chronic cough, experiences difficulty in breathing after exertion, wheezes, and is weak and easily fatigued.[britannica.com]
  • These may include chronic coughing, wheezing, weakness, fatigue, and shortness of breath. Edema (abnormal buildup of fluid), weakness, and discomfort in the upper chest may be evident in cor pulmonale.[encyclopedia.com]
Exertional Dyspnea
  • Nonspecific: fatigue, tachypnea, exertional dyspnea, cough, anginal chest pain, hemoptysis, hoarseness What nonspecific signs may indicate underlying lung disease Increase in chest diameter, labored respiratory efforts with chest wall retractions, distended[quizlet.com]
  • The patient may complain of fatigue, tachypnea, exertional dyspnea, and cough. Anginal chest pain also can occur and may be due to right ventricular ischemia or pulmonary artery stretching and usually does not respond to nitrates.[medictests.com]
  • Help reduce the patient’s fear of exertional dyspnea by providing thoughtful care that builds trust. Encourage the patient to progress in small increments.[medical-dictionary.thefreedictionary.com]
  • Symptoms Patients may report a combination of fatigue, tachypnea, exertional dyspnea, and cough.[emedicine.medscape.com]
Clubbed Finger
  • fingers CMV (cytomegalovirus) Coccidioidomycosis - chest x-ray Colds and the flu Colon cancer - series Colonoscopy Common asthma triggers Complete blood count - series Cor pulmonale Coronary artery blockage Craniotomy - series Crohn disease Crohn's disease[nicklauschildrens.org]
Fatigue
  • Symptoms of CPM include fatigue, lethargy, dypsnoea on exertion, tachypnoea, chest pain, exertional syncope, dyspnoea at rest, nonproductive cough, haemoptysis, hoarseness, and anorexia.[link.springer.com]
  • Symptoms include chronic cough, shortness of breath on exertion, fatigue, and other signs of oxygen lack to the body tissues.[curlie.org]
  • […] of the heart caused by pulmonary hypertension Cœur pulmonaire - insuffisance du côté droit du cœur causée par une hypertension artérielle pulmonaire Cor pulmonale : enlargement and strain on the right side of the heart Cœur pulmonaire : gonflement et fatigue[dictionary.reverso.net]
  • […] right ventricle to the lungs This pressure increases the size and work of the chamber leading to an enlargement of the heart, and if uncorrected quickly leads to heart failure Symptoms Chronic Cough Difficulty in breathing after exercise Wheezing Weak/Fatigue[prezi.com]
Pulmonary Valve Stenosis
  • Pulmonary valve stenosis. Congestive cardiac failure due to primary cardiological disease. Congenital right-sided cardiac impairment. Right-sided heart failure due to right-ventricular myocardial infarction. Ventricular septal defect.[medigoo.com]
  • Pulmonary valve stenosis . Congestive cardiac failure due to primary cardiological disease. Congenital right-sided cardiac impairment. Right-sided heart failure due to right ventricular myocardial infarction . Ventricular septal defect .[patient.info]
  • In the absence of right ventricular outflow or pulmonary valve stenosis pulmonary artery systolic pressure can be reliably deduced by summing the right ventricular-to-right atrial pressure gradient obtained via the peak tricuspid regurgitation velocity[wikiecho.org]
Heart Disease
  • Pulmonay heart disease Pulmonary Heart Disease Symptoms Clinical manifestations are nonspecific for pulmonary heart disease. They differ in acute pulmonary heart disease from chronic pulmonary heart disease.[doctortipster.com]
  • What is Pulmonary Heart Disease – Cor Pulmonale Statistics on Pulmonary Heart Disease – Cor Pulmonale Risk Factors for Pulmonary Heart Disease – Cor Pulmonale Progression of Pulmonary Heart Disease – Cor Pulmonale Symptoms of Pulmonary Heart Disease[myvmc.com]
  • , Pulmonary , Heart Diseases, Pulmonary , Pulmonary Heart Disease , Pulmonary Heart Diseases , COR PULMONALE , Pulmonary heart disease, unspecified , cardiopulmonary disease , DISEASES PULM HEART , PULM HEART DISEASES , HEART DIS PULM , HEART DISEASES[fpnotebook.com]
Cyanosis
  • Examination reveals cyanosis, distended neck veins, and a prominent epigastric pulsation Cor pulmonale is right ventricular enlargement and eventually failure secondary to a lung disorder that causes pulmonary artery hypertension.[smartypance.com]
  • Physical examination may reveal: Skin changes: cyanosis, pallor, sweating; Signs of cerebral hypoperfusion: agitation, confusion and even coma; Tachypnea; Tachycardia, gallop rhythm of the right ventricle,systolic murmur of tricuspid regurgitation; Signs[doctortipster.com]
  • , tachypnea, exertional dyspnea, cough, anginal chest pain, hemoptysis, hoarseness What nonspecific signs may indicate underlying lung disease Increase in chest diameter, labored respiratory efforts with chest wall retractions, distended neck veins, cyanosis[quizlet.com]
  • On examination, child had respiratory distress, cyanosis and edema. Tachycardia was present along with raised jugular venous pressure and hepatomegaly. Blood pressure was normal, and a grade 2 systolic murmur was present.[journals.lww.com]
  • Abnormal heart sounds Physical indicators of cor pulmonale on exam: Pulmonary hypertension Right ventricular hypertrophy Right ventricular failure Increased chest diameter Labored respirations with retractions Distended neck veins Cyanosis On auscultation[symptoma.com]
Distended Neck Veins
  • Examination reveals cyanosis, distended neck veins, and a prominent epigastric pulsation Cor pulmonale is right ventricular enlargement and eventually failure secondary to a lung disorder that causes pulmonary artery hypertension.[smartypance.com]
  • neck veins with prominent a or v waves, and cyanosis may be seen. the heart and lung are intricately related. whenever the heart is affected by disease, the lungs will follow and vice versa. pulmonary heart disease is by definition a condition when the[allnurses.com]
  • Distended neck veins. Bluish skin. Chest pain. Enlarged liver and swollen abdomen.[nmihi.com]
  • CLINICAL MANIFESTATIONS If heart failure accompanies cor pulmonale additional manifestations such as • Peripheral edema • Weight gain • Distended neck veins • Full bounding pulse • Enlarged liver 35.[slideshare.net]
Hepatojugular Reflux
  • Increased right atrial pressures may lead to dependent edema, hepatojugular reflux, and ascites.[javed-abbas.tripod.com]
  • Marked hepatojugular reflux due to hepatic congestion. Hepatomegaly liver pulsatility if there is significant associated tricuspid regurgitation. Jaundice in advanced cases. Ascites in advanced cases. Peripheral pitting oedema.[patient.info]
  • Hepatojugular reflux and pulsatile liver are signs of RV failure with systemic venous congestion. In severe disease, ascites can also be present. Examination of the lower extremities reveals evidence of pitting edema.[emedicine.medscape.com]
Pansystolic Murmur
  • On cardiovascular system examination, there was loud pulmonic component of second heart sound along with a pansystolic murmur suggestive of tricuspid regurgitation at a left lower parasternal area.[ijem.in]
  • Distended neck veins with raised and/or prominent JVP and visible a or v waves. 3rd/4th heart sounds and pansystolic murmur of tricuspid regurgitation over right heart. Split second heart sound with loud pulmonary component.[patient.info]
Hepatomegaly
  • Findings include peripheral edema, neck vein distention, hepatomegaly, and a parasternal lift. Diagnosis is clinical and by echocardiography. Treatment is directed at the cause.[merckmanuals.com]
  • Pulmonary artery hypertension then leads to right ventricular failure Findings include peripheral edema, neck vein distention, hepatomegaly, and a parasternal lift Diagnosis usually requires echocardiography or radionuclide imaging and sometimes right[smartypance.com]
  • Signs of decompensation of the right ventricle: jugular turgor, hepatomegaly, hepato-jugular reflux, edema.[doctortipster.com]
Pulsatile Liver
  • liver, a loud pulmonic component of the 2nd heart sound, or evidence of venous thrombosis.[clinicaladvisor.com]
  • Hepatojugular reflux and pulsatile liver are signs of RV failure with systemic venous congestion. In severe disease, ascites can also be present. Examination of the lower extremities reveals evidence of pitting edema.[emedicine.medscape.com]

Workup

The diagnosis of cor pulmonale clinically may be difficult because its symptoms are similar to those of any pulmonary or cardiac disorder. Diagnosis of pulmonary heart disease depends on the presence of both pulmonary hypertension and right ventricular hypertrophy. These two signs can be detected by the following studies [3] [8] [11]:

  • Routine laboratory tests: PaO2, PCO2, hematocrit, coagulation studies, renal function
  • Brain natriuretic peptide (BNP) levels
  • Electrocardiogram
  • Chest X-ray
  • Pulmonary function tests
  • Echocardiogram
  • Computed tomography
  • Magnetic resonance imaging
  • Cardiac catheterization which allows measurement of pressures in the lung and heart

The diagnosis of pulmonary hypertension traditionally is depended on measurements taken during invasive cardiac catheterisation [4] [11]. An increased mean pulmonary arterial pressure is characteristic of pulmonary hypertension [4]. The development of non-invasive methods to measure pulmonary hypertension using magnetic resonance imaging imaging alone is being researched [3]. Magnetic resonance imaging can measure mean pulmonary arterial pressure as well as evaluate vascular anatomy and blood flow patterns [4].
Plasma brain natriuretic peptide (BNP) may be a reliable and accurate diagnostic test for pulmonary hypertension and right ventricular dysfunction [3]. It is a cardiac hormone which is produced by the ventricle and secreted in response to increased ventricular stretch and tension [1] [3]. There is a significant correlation between brain natriuretic peptide levels and pulmonary hypertension determined by echocardiography [3].

Chest X-ray will show right ventricular hypertrophy, right atrial dilatation, and a prominent pulmonary artery [3]. Pulmonary fields show reduced vasculature, hyperinflation, increased translucency of the lung and/or a flattening of the diaphragm [3] [8]. Echocardiogram shows indications of right ventricular hypertrophy, right axis deviation, and mitral valve abnormalities[8]. Findings differ from patient to patient. Two-dimensional (2-D) echocardiography may demonstrate increased right ventricle wall thickness asign of chronic right ventricular pressure overload [8]. Doppler echocardiography can estimate pulmonary arterial pressure and is the most reliable noninvasive means to do this [2]. High-resolution echocardiography and magnetic resonance imaging can accurately measure pulmonary pressure, however, right heart catheterization is still considered the most precise method for diagnosis and evaluation of pulmonary hypertension [2] [3].

Right Axis Deviation
  • Electrocardiography shows the appearance of flat or inverted T waves in the right ventricular precordial leads, right axis deviation of greater than 30 in the mean electrical axis of the QRS, transient S-T depression of leads II, III, and aVF, and transient[rjoson.tripod.com]
  • ECG evidence of RV hypertrophy (eg, right axis deviation, QR wave in lead V 1 , and dominant R wave in leads V 1 to V 3 ) correlates well with degree of pulmonary hypertension.[merckmanuals.com]
  • axis deviation due to right ventricular hypertrophy Incomplete or complete right bundle branch block In some cases, P pulmonale S1Q3 pattern in acute cor pulmonale Chest x-ray Pronounced central pulmonary arteries Right heart hypertrophy ( prominent[amboss.com]
  • On electrocardiogram, there was right axis deviation, p-pulmonale, features of right ventricular hypertrophy along with presence of prominent Q waves in leads II, III and aVF.[ijem.in]
Poor R Wave Progression
  • R wave progression RAE with and RVH multifocal atrial tachycardia return to top Differential Diagnosis return to top Treatments Consequences and Course return to top The Case of...[sharinginhealth.ca]
Right Bundle Branch Block
  • In chronic pulmonary heart disease : right ventricular hypertrophy, right bundle branch block, P wave of pulmonary type, sinus tachycardia, atrial or ventricular arrhythmias.[doctortipster.com]
  • bundle branch block. 6.[rjoson.tripod.com]
  • bundle branch block In some cases, P pulmonale S1Q3 pattern in acute cor pulmonale Chest x-ray Pronounced central pulmonary arteries Right heart hypertrophy ( prominent right heart border and loss of retrosternal space due to right ventricular enlargement[amboss.com]
  • Electrocardiographic findings in 67,375 asymptomatic subjects, VI: right bundle branch block. Am J Cardiol . 1960 ; 6 : 143 –152. Fisch C. Electrocardiography and vectorcardiography. In: Braunwald E, ed. Heart Disease . 4th ed.[circ.ahajournals.org]
  • bundle branch block; right precordial T-wave inversions; or an S1Q3T3 pattern Radiographic features include a distended azygous vein on plain radiography or RV dilation and septal shift on helical CT Several blood markers may also be abnormal, such as[clinicaladvisor.com]
Incomplete Right Bundle Branch Block
  • ECG shows the following changes: In acute pulmonary heart disease : right axial deviation, complete or incomplete right bundle branch block, sinus tachycardia, reversal of T waves in right chest leads.[doctortipster.com]
  • right bundle branch block Changes in cardiac conduction due to right-sided hypertrophy Echocardiography Normal heart size To show ventricular hypertrophy, decreased contractility, and valvular disorders in both right and left ventricular failure Demonstrates[medical-dictionary.thefreedictionary.com]
P Pulmonale
  • Tall tented p-pulmonale may also be seen. 3) Echocardiography may show dilatation and also allows indirect measurement of pulmonary artery pressures.[myvmc.com]
  • pulmonale S1Q3 pattern in acute cor pulmonale Chest x-ray Pronounced central pulmonary arteries Right heart hypertrophy ( prominent right heart border and loss of retrosternal space due to right ventricular enlargement in lateral view ) Sudden change[amboss.com]
  • On electrocardiogram, there was right axis deviation, p-pulmonale, features of right ventricular hypertrophy along with presence of prominent Q waves in leads II, III and aVF.[ijem.in]
  • ECG changes in Cor Pulmonale include right axis deviation, R/S amplitude ratio in V1 greater than 1, R/S amplitude ratio in V6 less than 1 and P-Pulmonale pattern (Klinger et.al, 1991).[asrn.org]
Prominent A-Wave
  • On electrocardiogram, there was right axis deviation, p-pulmonale, features of right ventricular hypertrophy along with presence of prominent Q waves in leads II, III and aVF.[ijem.in]
  • The most typical ECG findings in emphysema are: Rightward shift of the P wave axis with prominent P waves in the inferior leads and flattened or inverted P waves in leads I and aVL.[lifeinthefastlane.com]
Right Ventricular Hypertrophy on ECG
  • Over time, this chronic elevation of pulmonary arterial pressures results in compensatory right atrial and right ventricular hypertrophy. Typical ECG Findings in COPD 1.[lifeinthefastlane.com]
Electrocardiogram Change
  • In both types, characteristic electrocardiogram changes occur, and in later stages there is usually right-sided cardiac failure. cor pulmonale (kôr′ po͝ol′mə-nä′lē, -năl′ē, pŭl′-) n.[medical-dictionary.thefreedictionary.com]

Treatment

Elimination of the cause(s) of cor pulmonale is the most important intervention [2] [3]. Smoking cessation and/or elimination of exposure to second-hand smoke is the major preventive measure. Exposure to dust, toxic fumes, asbestos, toxic chemicals, and known allergens are also necessary [2]. Treatment of the underlying causative conditions is paramount to the prevention and treatment of cor pulmonale [3] [6]. The goals of interventions focus on the treatment of the underlying pulmonary disease and improving oxygenation and right ventricular function by increasing right ventricular contractility and decreasing pulmonary vasoconstriction by improving pulmonary blood flow, decreasing pulmonary vascular pressure, increasing cardiac out-put, and preventing hypoxemia and therefore prevent pulmonary vascular changes [5] [6].
Treatment regimes may include [1] [3] [6] [7]:

  • Long-term oxygen therapy
  • Calciumm channel blockers 
  • Phosphodiesterase inhibitors
  • Diuretics
  • Bronchodilators
  • Mucolytic medications
  • Anticoagulant therapy
  • Antibiotics with respiratory infections

Currently, long-term oxygen therapy is the primary treatment to prevent pulmonary hypertension since the major causative factor is alveolar hypoxia [1] [3] [6]. Oxygen therapy can stabilize or partially reverse pulmonary hypertension [1] [7]. It is unclear if this is the result of improved oxygenation, reduced systemic inflammation or reversal or stabilization of pulmonary hypertension [3] [8].

Oxygen therapy is almost always required to prevent hypoxemia that contributes to pulmonary vascular changes and cardiac ischemia [1] [6]. Oxygen at a rate of 2 litres per minute is recommended. Excessive oxygen can in patients with chronic obstructive pulmonary disease who are chronically hypoxic. Sudden correction of hypoxia can remove the main stimulus to respiration and respiratory arrest can occur [3] [4] [7].

Treatment to prevent congestive heart failure requires diuretics, nitrates to improve cardiac out-put, and phosphodiesterase inhibitors to improve heart contractility [6] [8].
Anticoagulant therapy is recommended for all patients with primary pulmonary hypertension [11] [13] [15]. The goal of their use is to prevent thromboembolisms that are a frequent complication of pulmonary hypertension [13] [15] and a common cause of death in individuals suffering from cor pulmonale [11] [13].

Inhaled nitric oxide is a potent vasodilator. It must be used in conjunction with oxygen therapy it may worsen ventilation perfusion imbalance [3]. When it is used with oxygen there is significant improvement in pulmonary vascular resistance, pulmonary hypertension and cardiac output [3]. Sildenafil is a phosphodiesterase 5 inhibitor specific to pulmonary function. It causes vasodilation and increases the effects of inhaled nitric oxide [3] [5].

Diuretics are used to reduce right ventricular dilatation and improve cardiac output and reduce pulmonary edema [3]. They can cause volume depletion that decrease cardiac output so should be used cautiously [3]. Intravenous epoprostenol (Flolan) is used to improve symptoms and survival of patients with pulmonary hypertension [5]. It has been shown to be effective but their long-term efficacy is not known [5].

Bronchodilators are the primary treatment regime for COPD [6]. They can be combined with inhaled corticosteroids for better results and fewer side effects [6]. Nifedipine, a calcium antagonist, reduces both pulmonary and systemic vasomotor tone at rest and during exercise. It has been shown to increase resting cardiac output by 26% [7].
Theophylline has significant antiinflammatory effects in chronic obstructive pulmonary disease [10]. It has been shown to restore steroid sensitivity [10]. The use of digoxin in the treatment of cor pulmonale is controversial. Research has shown that heart failure was significantly worsened in some patients, paticularly those with atrial fibrillation, and showed no improvement in other patients [9].

Polycythemia, an increased number of red blood cells in response to chronic hypoxemia, is a common occurrence in chronic obstructive pulmonary disease [1] [3]. This increases blood viscosity decreasing pulmonary profusion and contributing to pulmonary hypertension. When the hematocrit is greater than 60% therapeutic phlebotomies is recommended to reduce the red blood cell count [3]. Losartan used weekly in escalating doses daily for 4 weeks caused a significant reduction in the hematocrit [3]. This ‘bloodless phlebotomy’ appears to be a promising therapy [3].

Lung volume reduction surgery (LVRS) reduces hyperinflation and improves gas exchange [1] [3]. It may result in better distribution of pulmonary blood flow and ventilation perfusion balance [3]. It is an extremely invasive intervention and should be reserved after other treatments have failed. Non-pharmacologic interventions for chronic pulmonary disease include long-term oxygen therapy, nasal positive pressure ventilation, pulmonary rehabilitation, lung-volume-reduction surgery and, most important, smoking cessation [6].

Prognosis

The development of cor pulmonale is associated with poor prognosis, increased cardiac death rate, and impaired health status from chronic respiratory disease [2] [3]. Two factors have been shown significant for prognosis in cor pulmonale; the pulmonary arterial oxygen saturation and the use of anticoagulant therapy [11]. In severe chronic obstructive pulmonary disease and cor pulmonale activities of daily living, such as climbing stairs or walking, can be difficult and cause an acute episode of increased pulmonary hypertension [3].

Long-term oxygen therapy is the only treatment, apart from smoking cessation, that has been shown to improve survival rates in severe cases of chronic pulmonary disease and cor pulmonale [6]. Cor pulmonale, hypoproteinemia and a high PaCO2 level have been identified as risk factors for early readmission of patients with chronic obstructive pulmonary disese [16]. Readmission rate of about 6.8% have been reported [16].

Etiology

Cor pulmonale and pulmonary hypertension are difficult to diagnose and to treat [12]. Untreated it is characterized by a progressive increase in pulmonary vascular resistance leading to right ventricular failure and death [12]. In the past decade there have been remarkable improvements in therapy for this disorder [12].
The heart and lungs are intricately connected. Pulmonary heart disease, by definition, indicates that the lungs cause the heart to fail [1] [2]. Two major causes of cor pulmonale are changes to the pulmonary vascular from tissue damage and chronic pulmonary vasoconstriction due to chronic hypoxemia [4]. Cigarette smoking and air pollution are the main risk factors for lung disease and the potential for developing cor pulmonale[1]. Cor pulmonale is a progressive disease and if left untreated results in significant disability, impaired quality of life, and eventually death [3].

The causes of cor pulmonale include [1] [4]:

Epidemiology

It is difficult to determine the exact incidence of cor pulmonale [3]. The reported prevalence varies depending on the definition of pulmonary hypertension, the group studied, and the method of measuring [3]. Approximately 6-7% of all heart disease in adults in the United States is due to cor pulmonale[4]. More than 50% of cases of cor pulmonale are due to chronic obstructive pulmonary disease (COPD), chronic bronchitis or emphysema [1] [2]. The primary cause of these conditions is cigarette smoking [1]. About 15 million people in the United States suffer from chronic pulmonary disease [4].

Each year there are approximately 20,000 deaths and 280,000 hospital admissions due to pulmonary heart disease [1]. In patients experiencing pulmonary emboli who have a co-morbidity of chronic cor pulmonale the mortality rate may be as high as 17 to 30% higher than in patients with the co-morbidity [15].

Sex distribution
Age distribution

Pathophysiology

The heart is responsible for circulating blood throughout the body with the two ventricles acting as the pumps. The left ventricle pumps blood throughout the body. The right ventricle pumps blood to the lungs where it is oxygenated and then returned to the left heart for distribution [2]. Normally the pulmonary system offers minimal resistance. However with chronic obstructive pulmonary disease (COPD) and disorders that increase pulmonary vascular resistance, pulmonary hypertension occurs [1] [2].

The pathology of pulmonary hypertension is the result of pulmonary vascular vasoconstriction, smooth muscle cell proliferation, and thrombosis [5] [8]. These changes are due to an imbalance between vasodilators (prostacyclin, nitric oxide, vasoactive intestinal peptide) and vasoconstrictors (thromboxane A2, endothelin, serotonin) [8]. Current treatment is directed at restoring the balance between these factors [8].

Pulmonary hypertension is always the underlying pathology for right ventricular hypertrophy in cor pulmonale [3]. Hypertrophy is an adaptive response to chronic hypoxia. In order to provide the force required to move blood against the greater resistance in the pulmonary vasculature the right ventricle enlarges and the musculature thickens [2]. Acute, rather than chronic, pulmonary heart disease results in right ventricular dilatation [1] [3]. Dilatation is stretching of the musculature of the ventricle as due to a sudden increase in pulmonary pressure. This occurs from a pulmonary embolism or acute respiratory distress syndrome (ARDS) [1]. Both of these occurrences result in decreased cardiac output and eventually right sided heart failure. If left untreated either will progress to congestive heart failure and death [2]. Several mechanisms lead to pulmonary hypertension and pulmonary heart disease including[5]:

Pulmonary hypertension in cor pulmonale is the result of hypoxia causing pulmonary vasoconstriction, polycythemia, and pulmonary vascular bed destruction [3] [5]. Pulmonary vasoconstriction is an adaptive response to divert blood from unventilated alveoli to maintain ventilation-perfusion balance and a normal PaO2 [3] [5] [8]. Pulmonary vascular remodeling due to chronic ischemia causes fibrosis and proliferation of smooth muscle of small pulmonary arteries [3]. These narrow, thickened pulmonary arteries are less compliant and increase resistance to blood flow [3].

In response to chronic hypoxemia the body produces an excess number of erythrocytes in an effort to increase the oxygen carrying capacity of the blood [1] [3]. The resulting polycythemia increases the viscosity of the blood decreasing blood flow throught the pulmonary vasculature even further. This impairs oxygenation and contributes to pulmonary hypertension[3] [5]. Pulmonary hyperinflation is also associated with pulmonary hypertension. Severe emphysema causes air-trapping that may play a role in the development of pulmonary hypertension [3]. Elevated brain natriuretic peptide (BNP) level may be a natural mechanism to compensate for pulmonary hypertension and right heart failure by promoting diuresis and natriuresis, vasodilating systemic and pulmonary vessels, and reducing circulating levels of endothelin and aldosterone[3].

In patients with advanced chronic obstructive pulmonary disease, oxygen saturation falls during REM sleep by 20%–30% [3]. Nocturnal desaturation in COPD can lead to the development of pulmonary hypertension and cor pulmonale [3].

Prevention

A majority of cases of cor pulmonale can be prevented by [1] [2]:

Correction of abnormal gas exchange and ventilation through oxygen supplementation and vasodilators can decrease pulmonary hypertension. Doing this will improve survival and quality of life for those with chronic pulmonary disease [3].

Summary

Cor pulmonale also known as pulmonary heart disease (Latin: cor- heart; pulmōnāle- of the lungs) refers to the interconnectedness of the pulmonary and cardiac systems. It is characterized by hypertrophy and failure of the right ventricle of the heart [1] [2]. The abnormalities in in the structure and function of the right ventricle in cor pulmonale occur in response to increased pulmonary vascular resistance and/or pulmonary hypertension [1] [3].

Pulmonary hypertension, a disabling chronic disorder of the pulmonary vasculature, is characterized by increased pulmonary artery pressure due to increased pulmonary vascular resistance [8]. Pulmonary hypertension is the most common cause of cor pulmonale, but a wide variety of cardiopulmonary disease processes can cause the condition [1] [3].
The course of cor pulmonale is usually chronic and it progresses slowly [1]. Cor pulmonale can be life-threatening if untreated. The major risk factors for lung disease are cigarette smoking and air pollution which are preventable [1]. Elimination of cigarette smoking, second-hand smoke and air pollution could significantly decreases the incidence of cor pulmonale.

Patient Information

What is cor pulmonale?

Cor pulmonale refers to heart disease that results from chronic pulmonary disease. It is characterized by pulmonary hypertension, elevated pressure within the blood vessels of the lungs, and enlargement and abnormal function of the right ventricle of the heart. The most common causes of cor pulmonale are chronic obstructive pulmonary disease (COPD), emphysema, and pulmonary emboli (blood clots). If left untreated cor pulmonale will progress to heart failure and death.

What are the symptoms?

The symptoms of cor pulmonale are not specific but are similar to any heart of lung conditions. They include:

What causes cor pulmonale?

The most common causes are cigarette smoking and exposure to toxic fumes and particulates (asbestos, coal dust, chemicals). Pulmonary hypertension in cor pulmonale is the result of destruction of the cells and blood vessels of the lungs that narrows the pathways to the flow of blood. Enlargement and decreased function of the right ventricle of the heart is due to the increased work required by the right ventricle to push blood through these narrowed vessels.

Who gets cor pulmonale?

Cor pulmonale generally occurs in adults with chronic lung disease. The highest incidence in smokers and those with COPD and blood clots in the lungs.

How is it diagnosed?

Cor pulmonale is diagnosed by the presence of pulmonary hypertension and right ventricle enlargement. Pulmonary hypertension can be detected by measuring the pressure within the pulmonary arteries. This is done using cardiac magnetic resonance imaging (MRI) and/or cardiac catherization. Right ventricular enlargement can be detected and measured using chest X-rays, electrocardiogram, and echocardiogram.

How is cor pulmonale treated?

Cor pulmonale is treated by treating the underlying pulmonary hypertension and preventing low oxygen levels and blot clot formation. Long-term oxygen therapy is the most efficient method of treatment. Other medications that may be used are hypertensive drugs, vasodilators, bronchodilators, diuretics, and anticoagulants.

What are the complications?

Complications of cor pulmonale include:

  • Difficulty completing activities of daily living
  • Decreased quality of life
  • Worsening of respiratory symptoms
  • Pneumonia
  • Edema of hands and feet and decreased circulation
  • Liver damage, liver failure

How can it  be prevented?

The primary way to prevent the condition is to prevent chronic ling disease. The best way of doing this is to:

  • Stop smoking
  • Prevent exposure to toxic chemicals and particulates which means using protective equipment
  • Control of COPD, chronic bronchitis, and asthma

References

Article

  1. Weitzenblum E, Chaouat A. Cor pulmonale. Chron Respir Dis. 2009;6(3):177-85. 
  2. Han MK, McLaughlin VV, Criner GJ, Martinez FJ. Pulmonary diseases and the heart. Circulation. 2007;116(25):2992-3005. 
  3. Shujaat A, Minkin R, Eden E. Pulmonary hypertension and chronic cor pulmonale in COPD. Int J Chron Obstruct Pulmon Dis. 2007;2(3):273-82. 
  4. Lungu A, Wild JM, Capener D, Kiely G, Swift AJ, Hose DR. MRI model-based non-invasive differential diagnosis in pulmonary hypertension. J Biomech. 2014;47(12):2941-7. 
  5. Hoeper MM. Drug treatment of pulmonary arterial hypertension: current and future agents. Drugs. 2005;65(10):1337-54. 
  6. Hanania NA, Ambrosino N, Calverley P, Cazzola M, Donner CF, Make B. Treatments for COPD. Respir Med. 2005; 99( Suppl B):S28-40. 
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