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]:

• Fatigue
• Shortness of breath with exertion or at rest
• Dyspnea at rest or with exertion
• Anorexia
• Wheezing
• Chronic productive cough
• Ascites
• Pedal/peripheral edema
• Angina due to right ventricular ischemia
• Neurologic symptoms due to decreased cardiac output and hypoxemia 
• Decrease in glomerular filtration rate (GFR)
• Engorgement of jugular and other neck and facial veins
• Increased jugular venous pressure
• Hepatomegaly
• Cyanosis
• Abnormal heart sounds

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

• Pulmonary hypertension
• Right ventricular hypertrophy
• Right ventricular failure
• Increased chest diameter
• Labored respirations with retractions
• Distended neck veins
• Cyanosis
• On auscultation wheezes and crackles
• On percussion hyperresonance of the lungs 
• Ascites

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.

• Fatigue

  Symptoms include chest pain, fatigue, syncope, dyspnea on exertion, cough, hypoxemia, hemoptysis, cyanosis, and peripheral edema. Acute presentations may also present with pallor, diaphoresis, hypotension, and tachycardia. [visualdx.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]

  Symptoms: Some of the early signs of cor pulmonale include constant cough, difficulty breathing, fatigue and weakness. As the disease progress, breathing difficulties may become more severe. [medigoo.com]

  Due to inadequate perfusion of peripheral tissues (fatigue, dyspnea) and elevated intracardiac filling pressures (orthopnea, paroxysmal nocturnal dyspnea, peripheral edema). [accessmedicine.mhmedical.com]

• Ankle Swelling

  (p. 207) Patients often present with their first episode of ankle swelling during an exacerbation of their COPD when, for the first time, the PaCO2 rises and the PaO2 falls, far enough to provoke the above events. [oxfordmedicine.com]

  The exam may find: Fluid buildup in your belly Abnormal heart sounds Bluish skin Liver swelling Swelling of the neck veins, which is a sign of high pressure in the right side of the heart Ankle swelling These tests may help diagnose cor pulmonale as well [account.allinahealth.org]

• Falling

  Excessive diuresis can lead to a true fall in right-sided output due to inadequate filling of the RV. Top Previous Management Minimal ankle oedema needs no treatment. [oxfordmedicine.com]

  Patient A (PHD I phenotype) had a fall in 6 MW from 320 m to 300 m and a worsening in NYHA functional class from 3 to 4. RV function improved as evidenced by an increase in TAPSE from 2.0 cm to 2.4 cm and a 40% fall in the BNP level. [doi.org]

  This must be done with care as decreased right ventricular filling can be associated with a catastrophic fall in cardiac output. Digoxin is not effective for treating right heart failure. [javed-abbas.tripod.com]

  Contact us online or call Avera McKennan Hospital & University Health Center in Sioux Falls, SD, at 605-322-8000. [mckennan.org]

  Each child developed slowly, with the weight falling below the tenth percentile. On physical examination during the acute episodes, a low grade fever was usually present. Tachycardia and tachypnea were marked. [pubs.rsna.org]

• Pulmonary Valve Stenosis

  Diagnosis: Differential diagnosis may include: Primary pulmonary hypertension. Pulmonary valve stenosis. Congestive cardiac failure due to primary cardiological disease. Congenital right-sided cardiac impairment. [medigoo.com]

  […] circulation Pulmonary embolism - Cor pulmonale Pericardium Pericarditis - Pericardial effusion - Cardiac tamponade Endocardium/heart valves Endocarditis - mitral valves (regurgitation, prolapse, stenosis) - aortic valves (stenosis, insufficiency) - pulmonary [bionity.com]

  Differential diagnosis Primary pulmonary hypertension (can be considered a cause of cor pulmonale). Pulmonary valve stenosis. Congestive cardiac failure due to primary cardiological disease. Congenital right-sided cardiac impairment. [patient.info]

  The Right Ventricle in Pulmonary Hypertension The right ventricle is exposed to pressure overload by pulmonary valve stenosis or by chronic pulmonary hypertension from any cause ( Table 1 ). [circ.ahajournals.org]

• Cough

  Symptoms: Some of the early signs of cor pulmonale include constant cough, difficulty breathing, fatigue and weakness. As the disease progress, breathing difficulties may become more severe. [medigoo.com]

  Cough with sputum that is discolored or tinged with blood. Last updated 3 April 2018 [nmihi.com]

  Cor pulmonale Presentation Pearls Diagnosis Treatment Media Patient will present as → a 65-year-old man who presents with a chronic productive cough, dyspnea, and wheezing. [smartypance.com]

  Symptoms include chest pain, fatigue, syncope, dyspnea on exertion, cough, hypoxemia, hemoptysis, cyanosis, and peripheral edema. Acute presentations may also present with pallor, diaphoresis, hypotension, and tachycardia. [visualdx.com]

   Chronic wet cough  Chest discomfort  Bluish discoloration of skin  Distention of neck veins  Enlargement of liver 10.  History collection  Physical examination  ECG ( prominent R wave and inverted T wave)  Chest X Ray – enlargement of the [slideshare.net]

• Dyspnea

  We report the case of a 40-year-old woman who died after 3 days of progressive dyspnea, cough, and pulmonary hypertension. [ncbi.nlm.nih.gov]

  Dyspnea secondary to pulmonary venous hypertension (often referred to as cardiac dyspnea) usually involves orthopnea and paroxysmal nocturnal dyspnea, two symptoms usually absent in patients with pulmonary dyspnea. [tele.med.ru]

  Due to inadequate perfusion of peripheral tissues (fatigue, dyspnea) and elevated intracardiac filling pressures (orthopnea, paroxysmal nocturnal dyspnea, peripheral edema). [accessmedicine.mhmedical.com]

  Furthermore, she rapidly developed progressive dyspnea and hypoxia. [kjim.org]

• Exertional Dyspnea

  The patient developed progressive exertional dyspnea, with clinical and laboratory findings of right-sided heart failure and pulmonary hypertension. [ncbi.nlm.nih.gov]

  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 predominant symptoms of compensated cor pulmonale are related to the pulmonary disorder and include chronic productive cough, exertional dyspnea, wheezing respirations, easy fatigability, and weakness. [accessmedicine.mhmedical.com]

  Symptoms and Signs The predominant symptoms of compensated cor pulmonale are related to the pulmonary disorder and include chronic productive cough, exertional dyspnea, wheezing respirations, easy fatigability, and weakness. [health.am]

  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]

• Respiratory Insufficiency

  Respiratory insufficiency may be suspected from the history. Physical and roentgenologic examinations may be confirmatory, but pulmonary function tests are necessary for a precise evaluation of the functional abnormalities. [ahajournals.org]

  The surviving infant ventilated for meconium aspiration, now two years old, has persistent pulmonary hyperexpansion and has been readmitted twice for respiratory insufficiency and right heart failure. [nature.com]

  Abstract A male patient with pycnodysostosis suffered from chronic respiratory insufficiency and pulmonary hypertension. [ncbi.nlm.nih.gov]

  insufficiency of “central” origin). [doi.org]

• Chronic Cough

  A person with cor pulmonale has a chronic cough, experiences difficulty in breathing after exertion, wheezes, and is weak and easily fatigued. [britannica.com]

  History : Symptoms include shortness of breath with exercise, chronic cough that produces sputum, blue tinge around the mouth and fingernails (cyanosis), wheezing, weakness, tiring easily, and swelling of the lower extremities (edema). [nmihi.com]

  Symptoms include chronic cough, shortness of breath on exertion, fatigue, and other signs of oxygen lack to the body tissues. [curlie.org]

  These include wheezing and weakness as well as chronic coughing and fatigue. Who to See and Types of Treatments Available Cor pulmonale is always suspected in patients with COPD. [docdoc.com.sg]

  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]

• Abdominal Pain

   Tussive or effort-related syncope  because of the inability of the RV to deliver blood adequately to the left side of the heart  Abdominal pain and ascites  Lower extremity edema  Shortness of breath  wheezing 8.  tachypnea  elevated jugular [slideshare.net]

  dyspnea, orthopnea, and hemoptysis (rare in PAH) Gastrointestinal: nausea, anorexia, abdominal bloating and fullness, occasionally severe abdominal pains Neurologic: lightheadedness with positional change: presyncope and syncope with effort, cough, [thoracickey.com]

• Heart Disease

  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 – [healthinfo.healthengine.com.au]

  ICD-10-CM Codes › I00-I99 Diseases of the circulatory system › I26-I28 Pulmonary heart disease and diseases of pulmonary circulation › I27- Other pulmonary heart diseases › Cor pulmonale (chronic) 2016 2017 2018 2019 Billable/Specific Code I27.81 is a [icd10data.com]

  ‘Without treatment, pulmonary hypertension can progress to cor pulmonale: right heart failure associated with pulmonary disease and pulmonary vascular disease in the absence of congenital heart disease or left sided heart disease.’ [oxforddictionaries.com]

• Cyanosis

  The patient with acute cor pulmonale 4 days after coronary bypass for unstable angina pectoris suddenly developed severe breathlessness with cyanosis and had fatal cardiac arrest and necropsy disclosed massive pulmonary embolism. [ncbi.nlm.nih.gov]

  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]

  Symptoms include chest pain, fatigue, syncope, dyspnea on exertion, cough, hypoxemia, hemoptysis, cyanosis, and peripheral edema. Acute presentations may also present with pallor, diaphoresis, hypotension, and tachycardia. [visualdx.com]

  Cyanosis is more prevalent if there is right to left shunting via a PFO. C. ECG and Chest Radiography The ECG may show right axis deviation and peaked P waves. Deep S waves are present in lead V6. [accessmedicine.mhmedical.com]

• Hepatojugular Reflux

  Increased right atrial pressures may lead to dependent edema, hepatojugular reflux, and ascites. [javed-abbas.tripod.com]

  At admission the patient required continuous oxygen support, with a tolerance of 1 hour without supplemental oxygen, tachypnea, postural limitation due to breathlessness, jugular engorgement at 90° and hepatojugular reflux, decreased breathing noises, [elsevier.es]

  Tender hepatomegaly,Hepatojugular reflux, lower extremity edema, ascites 21. signs 22. [slideshare.net]

  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]

• Gallop Rhythm

  Later, an RV gallop rhythm (3rd [S3] and 4th [S4] heart sounds) augmented during inspiration, distended jugular veins (with a dominant a wave unless tricuspid regurgitation is present), hepatomegaly, and lower-extremity edema may occur. [msdmanuals.com]

  Later, an RV gallop rhythm (3rd [S 3 ] and 4th [S 4 ] heart sounds) augmented during inspiration, distended jugular veins (with a dominant a wave unless tricuspid regurgitation is present), hepatomegaly, and lower-extremity edema may occur. [merckmanuals.com]

• Increased Jugular Venous Pressure

  […] and other neck and facial veins Increased jugular venous pressure Hepatomegaly Cyanosis Abnormal heart sounds Physical indicators of cor pulmonale on exam: Pulmonary hypertension Right ventricular hypertrophy Right ventricular failure Increased chest [symptoma.com]

  CLINICAL ASSESSMENT OF COR PULMONALE: PLACE OF NON-INVASIVE METHODS The clinical signs of cor pulmonale are relatively insensitive 6 and some of them (signs related to an increased jugular venous pressure) are often obscured by hyperinflation of the chest [doi.org]

  CLINICAL ASSESSMENT OF COR PULMONALE: PLACE OF NON-INVASIVE METHODS The clinical signs of cor pulmonale are relatively insensitive6 and some of them (signs related to an increased jugular venous pressure) are often obscured by hyperinflation of the chest6 [heart.bmj.com]

• Hepatomegaly

  PERIPHERAL EDEMA 3. hepatic congestion 4. altered tricuspid and pulmonic valve sounds 5. hepatomegaly 6. jugular distension. what is diagnosis of cor pulmonale chornic depend on? 1. physical and radiological exams 2. [quizlet.com]

  Findings include peripheral edema, neck vein distention, hepatomegaly, and a parasternal lift. Diagnosis is clinical and by echocardiography. Treatment is directed at the cause. [msdmanuals.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]

  Chronic presentations are harder to detect and may present with clubbed fingernails, wheezing, crepitations, papilledema, tender hepatomegaly, and ascites. Treatment and prognosis depend on the causal underlying disease. [visualdx.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]

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].

• Pulmonary Arterial Pressure Increased

  Indeed, from a pathophysiologic and clinical standpoint, any disease in which pulmonary arterial pressures increase sufficiently to affect right ventricular function results in a similar clinical syndrome of right heart failure. [accessmedicine.mhmedical.com]

  Elevated pulmonary artery pressure increases right ventricular workload with resulting myocardial hypertrophy and diastolic dysfunction ultimately leading to diastolic right-heart failure. [acphospitalist.org]

• Central Venous Pressure Increased

  At which point, central venous pressure increases in proportion to the increase in RV EDV [26]. [emcrit.org]

• Right Axis Deviation

  ECG shows tall, peaked P waves (P pulmonale), right axis deviation, and RVH. Echocardiogram excludes primary LV dysfunction. [accessmedicine.mhmedical.com]

  Electrocardiography and Chest X-Ray The ECG may show right axis deviation and peaked P waves. Deep S waves are present in lead V6. Right axis deviation and low voltage may be noted in patients with pulmonary emphysema. [health.am]

  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]

  Right axis deviation, dominant R wave in V1 and deep S wave in V6 suggest right ventricular hypertrophy due to cor pulmonale. [lifeinthefastlane.com]

• Hypercapnia

  The presence of peripheral edema in cor pulmonale is almost invariably associated with hypercapnia. Correction of abnormalities of gas exchange and ventilation can ameliorate pulmonary hypertension and improve survival. [ncbi.nlm.nih.gov]

  There was no significant difference in hypercapnia between groups. [journals.tbzmed.ac.ir]

  Adverse effect: low blood pressure, tachycardia and hypoxemia and hypercapnia 38. [slideshare.net]

• 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

  right bundle branch block. 6. [rjoson.tripod.com]

  V leads; (3) ST depressions in leads II, III, and aV F ; and (4) transitory right bundle-branch block. [ahajournals.org]

  […] axis deviation Right bundle branch block R:S ratio > 1 in V1 TWI in V1-3 in acute right heart strain S1 Q3 T3 in acute right heart strain Large P wave in II, III, aVF Arrhythmia (PAC, SVT, MFAT, A-fib, A-flutter) Echocardiography Increased RV thickness [wikem.org]

  […] a prolongation of the QRS; the onset of a complete right bundle branch block (QRS > 120 ms) is rare, whereas an incomplete block pattern is much more common; the QRS axis may be normal or deviated to the right; when emphysema is predominant (COPD patients [cardiocases.com]

  Related Topics Right axis deviation Low voltage QRS complexes Right atrial enlargement (P pulmonale) Right ventricular hypertrophy Right bundle branch block References Harrigan RA, Jones K. ABC of clinical electrocardiography. [lifeinthefastlane.com]

• Incomplete Right Bundle Branch Block

  […] deviation and incomplete 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 [nursing.unboundmedicine.com]

• P Pulmonale

  “P pulmonale” was found in one third of the emphysema group but was inconstant. Paroxysmal atrial arrhythmias were common in the group with emphysema and cor pulmonale, but chronic atrial arrhythmia was absent in both groups. [ajconline.org]

  With development of cor pulmonale, the following additional changes are seen: Right atrial enlargement (P pulmonale) Right ventricular hypertrophy 3. [lifeinthefastlane.com]

  In ECG, P pulmonale was observed in 58 (99.66%) of cases. 48 (80%) were having right ventricular dilatation and right atrial dilation was observed in 30 (50%) of patients. [scopemed.org]

  Tall tented p-pulmonale may also be seen. 3) Echocardiography may show dilatation and also allows indirect measurement of pulmonary artery pressures. [healthinfo.healthengine.com.au]

• Prominent A-Wave

   History collection  Physical examination  ECG ( prominent R wave and inverted T wave)  Chest X Ray – enlargement of the main pulmonary artery and the descending right pulmonary artery andRt ventricular hypertrophy 11.  Echocardiogram: rt ventricular [slideshare.net]

  Clinical findings associated with pulmonary heart disease include jugular venous distension, prominent v-wave in the jugular venous pulsation, narrow S-2 split or no split (slight delay in closure of the pulmonic valve), and, in advanced stages, peripheral [acphospitalist.org]

  Investigations X-Ray Chest (Right Ventricular Enlargement, Right atrial dilatation, prominent pulmonary artery) ECG: RVH, Prominent R wave in lead VI Ech: RV dilatation Treatment Elimination of the cause is the most important intervention. [bionity.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]

  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]

• Prominent V-Waves

  Clinical findings associated with pulmonary heart disease include jugular venous distension, prominent v-wave in the jugular venous pulsation, narrow S-2 split or no split (slight delay in closure of the pulmonic valve), and, in advanced stages, peripheral [acphospitalist.org]

• 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. [medical-dictionary.thefreedictionary.com]

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].

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].

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]:

• Pulmonary embolism
• Acute respiratory distress syndrome (ARDS)
• Primary pulmonary hypertension
• Uncontrolled persistent asthma
• Trauma or surgery resulting in loss of lung tissue
• Congenital abnormalities/disorders
• Pneumoconiosis
• Sarcoidosis
• T1-4 vertebral subluxation
• Obstructive sleep apnea
• Interstitial lung disease
• Sickle cell anemia
• Bronchopulmonary dysplasia (in infants)
• Severe thoracic kyphosis

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].

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 vasoconstriction
• Anatomic changes in pulmonary vasculature
• Increased blood viscosity
• Idiopathic or primary pulmonary hypertension

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].

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

• Not smoking
• Cessation of smoking
• Preventing second-hand smoke exposure
• Avoidance of lung irritants: toxic fumes and chemicals, coal and other dust, asbestos
• Treatment and control of asthma
• Treatment of chronic obstructive pulmonary disease and chronic bronchitis

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].

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.

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:

• Fatigue
• Shortness of breath with exertion or at rest
• Loss of appetite
• Wheezing
• Chronic productive cough
• Abdominal swelling
• Swelling of hands and feet
• Chest pain
• Dizziness, fainting, disorientation
• Decreased kidney function
• Enlargement of the liver
• Cyanosis

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

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