Pulmonary Hypertension And Cor Pulmonale Cheng Zhang , Respiratory Medicine , Affiliated Hospital of Jining Medicine college 23,Feb
What is Pulmonary Hypertension • Pulmonary hypertension ( PH ) is a disorder of the pulmonary circulation in which elevated pressure in the pulmonary vascular circuit can , when severe , lead to right heart failure and eventually cause death • The diagnostic criterion for PH is controversial • It is commonly accepted that at sea level,a mean pulmonary artery pressure(mPAP) ≥20mmHg at rest or >30mmHg with exercise along with a pulmonary artery occlusion pressure of ≤15 mmHg are required to establish the diagnosis of pulmonary arterial hypertension(PAH),a subtype of PH
Classification and Staging • For many years,PH was classified as either “primary” or “secondary” • In 2003 the Third World Symposium updated the classification system,notably dropping the term”primary” • They also stressed that the staging of patients with PH should be based on the functional capacity of the patient rather than on hemodynamic parameters • The World Health Organization classification system,a modified form of the New York Heart Association functional classification system,was recommended by the Symposium as the preferred staging system
Secondary Pulmonary Hypertension • Secondary pulmonary hypertension(SPH) defined as PH due to a known cause is far more common than primary pulmonary hypertension(PPH) • Secondary pulmonary hypertension can be caused by any disease that impedes the flow of blood through the lungs of that causes sustained periods of low oxygen in the blood • The predominant causes are cardiac and pulmonary diseases • This focus of this section is pulmonary hypertension associated with disorders of the respiratory system and/or hypoxemia
Secondary Pulmonary Hypertension
Causes of Secondary Pulmonary Hypertension
Obstructive Pulmonary Disease • Chronic obstructive pulmonary disease is the most common cause • When the lungs are impaired by disease,it takes more effort to pump blood through them • Over time, chronic obstructive pulmonary disease destroys the small air sacs together with their small vessels in the lungs
Secondary Pulmonary Hypertension • The most important cause of pulmonary hypertension in chronic obstructive pulmonary disease is the narrowing of the pulmonary artery that occurs as a result of hypoxemia Parenchymal Lung Diseases Including Alveolar Diseases And Interstitial Lung Diseases Alveolar diseases,such as pneumonedema and acute respiratory distress syndrome,which often precede SPH
Secondary Pulmonary Hypertension • Are not required specific treatment since the high pulmonary artery pressure caused by this group diseases will decrease as the primary diseases ameliorate • Interstitial lung diseases,such as pneumonoconiosis,sarcoidosis,idiopathic pulmonary fibrosis,and etc,can cause diffusion abnormality resulting in hypoxemia • The pulmonary arterial vasoconstriction develops as a result of hypoxemia and the inflammation of this group diseases
Secondary Pulmonary Hypertension Abnormalities of Pulmonary Arteries or Arterioles • Among abnormalities of Pulmonary arteries or arterioles, pulmonary thromboembolism is the most common cause • There are some other relatively rare diseases,such as pulmonary capillary hemangioma,pulmonary veno-occlusive disease,collagen vascular diseases,etc
Secondary Pulmonary Hypertension Neuromuscular Diseases • The pathophysiologic character of neurologic diseases,such as chest wall abnormalities,GuillainBarre syndrome,poliomyelitis and etc,is hypoventilation leading to hypoxemia and hypercapnia,which eventually result in the pulmonary hypertension
Secondary Pulmonary Hypertension
Clinical Presentation and Treatment The early clinical manifestations of SPH are frequently masked by the undering etiology Obtaining a careful history may help exclude some of the numerous causes of secondary pulmonary hypertension The late clinical manifestations are labeled with right heart failure The treatment of SPH is primonary directed at treatment of the undering disease
Secondary Pulmonary Hypertension • Effective therary should be instituted in the early stages,before irreversible changes in pulmonary vasculature occur • Once the cause of SPH has been established,the management consists of specific interventional therary, specific medical therary,or general supportive therary • In the late stages,the therapeutic regimen is targeted to Cor Pulmonale
Primary(idiopathic) Pulmonary Hypertension • Primary(idiopathic) pulmonary hypertension ( PPH ) is a rare disorder of the pulmonary circulation occurring mostly in young and middle aged wemen • Untreated , it is characterized by progressive dyspnea , a rapid downhill course , and an invariably fatal outcome • This condition is also called plexogenic pulmonary arteriopathy , in reference to the characteristic histopathologic plexiform lesion found in muscular pulmonary arteries
Primary(idiopathic) Pulmonary Hypertension
Epidemiology The estimated incidence of primary pulmonary hypertension is 1-2/million in the general population PPH accounts for 0.008‰-1.3 ‰ of all the nonselective autopsy cases in America and Europe There is no exact data about the Chinese prevalence PPH can occur in the population with any age
Primary(idiopathic) Pulmonary Hypertension
Aetiology
• The aetiology of PPH remains undefined • The disease can occur in a familial or sporadic form • The sporadic form of the disease may be caused by interaction between a genetic factor , possibly related to the gene encoding the familial form , with one or more triggering factors • The interaction between genetic and triggering factor could result in endothelial injury and vasoconstriction leading to vascular remodelling
Primary(idiopathic) Pulmonary Hypertension
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Genetics The Familial form of PPH is inherited as an autosomal dominant and is associated with genetic anticipation In this form of inheritance,there is worsening of the disease in subsequent generations The gene for PPH has been localized to a 27centimorgan region on chromosome 2q31-32 Both the familial and sporadic forms of PPH have been recently associated with mutations of the bone morphogenetic protein receptor type II gene (BMPR 2)
Primary(idiopathic) Pulmonary Hypertension
Autoimmunity There are a number of associations between various autoimmune diseases and PPH Patients with scleroderma,the CREST syndrome,dermatomyositis,rheumatoid arthritis and systemic lupus erythematosus as well as hypothyroidism have been known to develop pulmonary hypertension However no specific biomarker has been found for the diagnosis of PPH
Primary(idiopathic) Pulmonary Hypertension
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Vasoactive Mediators The tone of pulmonary vascular tree is reliant upon a balance between vasoconstrictor and vasodilator machanisms The vasoconstrictors include II dehydrothromboxane B2,endothelin-1,Serotomn and etc The vasodilators include nitric oxide synthase,prostacyclin receptor protein,etc An imbalance of vasoactive mediators has been demonstrated in patients with pulmonary hypertension
Primary(idiopathic) Pulmonary Hypertension
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Potassium Channels Disregulation of potassium channels can lead to vasoconstriction Inhibition of membrane potassium channels lead to depolarization of pulmonary artery smooth muscle cells and an increase in intracellular calcium resulting in vasoconstriction Patients with PPH have been shown to have abnormalities of potassium channels resulting in depolarization of the pulmonary vascular smooth muscle cells Furthermore,aminorex,fenfluramine and dexfenfluramine have been shown to inhibit potassium channels
Primary(idiopathic) Pulmonary Hypertension
Clinical presentation
Symptoms • The onset of symptoms is usually insidious with several years elapsing before the diagnosis is actually made • As the pulmonary hypertension increases,patients can present with any of the following systemic symptoms:dyspnoea,presyncope or syncope,chest pain and hemoptysis
Primary(idiopathic) Pulmonary Hypertension
• Other symptoms are fatigue,palpitations,sudden death,cough, Ortner’s syndrome and etc • Raynaud phenomenon has beeb reported as many as 10% patients • Physical Signs • The physical signs in pulmonary hypertension patients are often advanced at the time of presentation resulting from pulmonary hypertension and the overload of right heart
Primary(idiopathic) Pulmonary Hypertension
Lab and other findings
Blood tests
• Full blood count,ESR,clotting screen,abnormal haemoglobin,glucose,lipids,liver function tests,serum ACE,thyroid function
Electrocardiogram
• Signs of right ventricular hypertrophy or strain may be observed • These include right axis deviation,an R-to-S wave ratio greater than 1 in lead V1,increased P-wave amplitude,and an incomplete or complete right bundle-branch block pattern
Primary(idiopathic) Pulmonary Hypertension Chest radiograph • The classic finding on a chest radiograph from a patient with PPH is enlargement of central pulmonary arteries,attenuation of peripheral vessels,and oligemic lung fields • Findings of right ventricular and right atria dilatation are possible Two-dimensional echocardiography and doppler echocardiography • Reflection of pulmonary hypertention and other associated presentations
Primary(idiopathic) Pulmonary Hypertension
Pulmonary function tests • Diffusing of capacity is universally reduced in patients with pulmonary hypertention Arterial blood gas • Arterial blood gas determinations should be performed to assess for hypoxemia Cardiac catheterization • This investigation remains the gold standard for establishing the diagnosis of pulmonary hypertention
Primary(idiopathic) Pulmonary Hypertension
• At the time of cardiac catheterization,full haemodynamic mornitoring should be made as well as testing for acute vasodilator response Ventilation-perfusion lung scan • Ventilation-perfusion scan should be performed to exclude chronic thromboembolic pulmonary hypertention • A high-or low-probability scan result is more useful,whereas intermediate-probability results should lead to performing pulmonary angiography
Primary(idiopathic) Pulmonary Hypertension Lung biopsy • The pathological changes are the result of longstanding hypertension rather than a consequence of different causes • The plexiform lesion is observed in patients with all types of PPH • These lesions consist of medial hypertrophy,eccentric or concentric laminar intimal proliferation and fibrosis,fibrinoid degeneration,and thrombotic lesions
Primary(idiopathic) Pulmonary Hypertension
Diagnosis and differentials The diagnosis of PPH cannot be made until possible diseases,which might cause pulmonary hypertention,has been ruled out It is usually made during the process of evaluating symptoms such as dyspnea,exercise intolerance,chest pain,or syncope Electrocardiographic changes,elevated brain natriuretic peptide(BNP)levels,and echocardiographic changes may suggest the diagnosis Differentials:All those diseases that might cause pulmonary hypertention
Primary(idiopathic) Pulmonary Hypertension • However,current remmendations state that a rightheart catheterization(RHC)is “strongly advised”to formally make the diagnosis of PPH • It is the author’s belief that all patients who are suspected to have PPH should have RHC prior to initiation of therapy • A mean pulmonary artery pressure≥20mmHg at or >30mmHg with exercise along with a pulmonary artery occlusion pressure of <15 mmHg are required to establish the diagnosis of PPH
Primary(idiopathic) Pulmonary Hypertension
Treatment
Since the causes of PPH remain unknown,the therapy is directed to vasoconstriction,endothelial impairment,thrombosis and right heart failure,in order to improve the pulmonary arteries or arterioles function,cardiac function and life
Vasodilator therapy a. Calcium-Channel Blochers.About 20% of the PPH patients are responsive to Calcium-Channel Blockers.The high dose in recommended,for instance,Nifedipine,240mg/d,and adverse reaction should be under the surveillance
Primary(idiopathic) Pulmonary Hypertension
• b. Prostanoids.Prostacyclin is a potent vasodilator with antiplatelet effects,and therapy with prostacyclin analogs ( prostanoids ) has become a cornerstone in the treatment of patients with PPH.To data, different prostanoids have been studied,each with unique delivery systems,side effects,and evidence of efficacy • c. Endothelin Antagonists. Endothelin-1 has been shown to be a potent vasoconstrictor and a mediator of the pulmonary vascular remodeling seen in PAH.Two receptors for endothelin-1 have been indentified,ETA and ETB
Primary(idiopathic) Pulmonary Hypertension • ETA mediates vasoconstriction and remodeling and ETB is involved in the clearance of endothelin-1 and perhaps also in vasodilatation and oxide release • Many endothelin receptors antagonists,are being studied in PPH;however,only bosentan in currently available for use in the United States • d. Anticoagulation.In PPH there is a predisposition to thrombosis because of right ventricular failure.Futhermore,thrombotic lesions have been demonstrated in the pulmonary vascular tree
Primary(idiopathic) Pulmonary Hypertension
• The anticoagulation therapy cannot improve the clinical symptom while it can impede the process of deterioration.Wrafarin has been shown to prolong survival in patients with pulmonary hypertension Others Other treatments available for the treatment of PPH include oxygen,diuretics and digokin.The latter has been shown to improve right-ventricular function in such patients and can be commenced when patients remain symptomatic whilst on medical therapy
Primary(idiopathic) Pulmonary Hypertension Interventional and surgical therapy • Lung or heart transplantation can be applied in the late stages
Cor pulmonale • The term cor pulmonale denotes right ventricular enlargement(hypertrophy and/or dilatation)secondary to abnormal lungs,chest bellows,or control of breathing • It has been commonly used as a synonym for pulmonary heart disease • Cor pulmonale may be acute or chronic • The most common cause of the acute cor pulmonale is massive embolization of the lungs • Chronic cor pulmonale,in which hypertrophy generally predominates,is more common than acute cor pulmonale,in which dilatation is the rule
Cor pulmonale
Chronic cor pulmonale
• Chronic pulmonary heart disease or chronic cor pulmonale is defined as right ventricular hypertrophy ,dilation,or both as a result of pulmonary hypertension caused by pulmonary disorder involving the lung parenchyma,impaired pulmonary bellows function,or altered ventilatory drive,except when this condition occurs because of left heart disease or congenital heart diseases
Cor pulmonale
Epidemiology
• Chronic cor pulmonale is a common disorder of respiratory system in China • A study performed in China in 1970’s demonstrated that in the population,aged>14 years,the incidence is 4.8% • Another study carried out in 1992 showed that the incidence remained high in China,with 6.72% in the population aged>15 years
Cor pulmonale • However,the incidence of Chronic cor pulmonale varies from country to country,between urban and rural areas,and with exposure to air pollutants • Chronic cor pulmonale accounts for more cases in the older and smoker population than in the younger and non-smokers • With chronic cor pulmonale patients more easily contract acute exacerbations in winter and spring,or in the abruptly changeable weather • There is no significant difference between male and female population
Cor pulmonale
Aetieology of cor pulmonale The chronic respiratory diseases which may lead to cor pulmonale,pulmonary thromboembolic disease,and diseases of the pulmonary vascular bed,are categorized into three major groups Obstructive lung diseases Those characterized by a limitation to airflow,such as COPD(chronic obstruction bronchitis,emphysema and their association),cystic fibrosis,bronchiectasis,bronchiolitis obliterans,etc
Cor pulmonale Restrictive lung diseases • Those characterized by a restriction of pulmonary volumes from extrinsic or parenchymatous origin(restrictive lung diseases),such as neuromuscular diseses,scarcoidosis,kyphoscoliosis,drug related lung diseases,idiopathic interstitial pulmonary fibrosis, interstitial pulmonary fibrosis of known origin,etc
Cor pulmonale Respiratory insufficiency of “central”origin • Those where the relatively well preserved mechanical properties of the lungs and chest wall contrast with pronounced gas exchange abnormalities which are partially exlained by poor ventilatory drive ( respiratory insufficiency of “central”origin ) ,such as central alveolar hypoventilation,obesity-hypoventilation syndrome,sleep apnoea syndrome,etc
Cor pulmonale • COPD is the major cause of chronic respiratory insufficiency and cor pulmonale,and it probably accounts for 80-90% of the cases • COPD includes chronic obstructive bronchitis and emphysema which are often associated • Among the restrictive lung diseases,kyphosconiosis,,idiopathic pulmonary fibrosis,and pneumoconiosis are the main causes of cor pulmonale • Among the aetiologies of respiratory respiratory insufficiency of “central”origin the obesityhypoventilation syndrome is a relatively frequent cause of cor pulmonale
Cor pulmonale
Mechanism and pathology
• The mechanism of cor pulmonale are first those of pulmonary hypertension • In chronic respiratory diseases pulmonary hypertension results from increased pulmonary vascular resistance(PVR) Development of pulmonary hypertension • A. pulmonary vascular resistance due to functional factors
Cor pulmonale • The factors leading to an increased PVR in chronic respiratory disease are numerous but alveolar alveolar hypoxia is by far the most predominant • The district mechanism of action of alveolar hypoxia is that acute hypoxia causes pulmonary vasoconstriction and pulmonary hypertension develops • It is commonly believed that the balance between vasoconstrictors and vasodilators play the vital role in hypoxia pulmonary vasoconstriction(HPV)
Cor pulmonale • The well known vasoconstrictors mainly include 11dehydro-thromboxane B2,endothelin1,serotonin,vascular endothelin growth factor and platelet-derived growth factors A and B,etc • The vasodilators include nitric oxide synthase(NOs),prostacyclin receptor protein,etc • The overproduction and release of vasoconstrictors lead to PVR and cause HPV,subsequently cor pulmonale develops
Cor pulmonale • Impaired NO production or release by endothelial cells in chronic hypoxia vasoconstriction may account for the inability of the pulmonary vasculature to relax impaired NO production may also be responsible for the proliferation of vascular smooth muscle cells,medial hypertrophy,and eccentric intimal fibrosis • These structural changes may increase platelet aggregation and in situ thrombi,thereby further increasing pulmonary vascular resistance
Cor pulmonale • Eventually,the cross-sectional areaof the pulmonary vascular bed decreases and pulmonary hypertension bacomes irreversible • Acidosis and secondary polycythemia also contribute to resistance to blood flow through the restricted pulmonary vascular bed • B. Pulmonary vascular resistance due to anatomic factors • There are several anatomic factors that explain PVR
Cor pulmonale • Among them the theory of pulmonary vascular remodeling is believed to be the well-understood mechanism in explaination of the PVR pathogenesis • It is accepted that chronic alveolar hypoxia leads to hypertrophy of the muscular media of the small pulmoanary arteries,muscularisation of pulmoanary arteries,and intimal fibrosis • This remodelling leads to elevation of PVR and to pulmonary hypertention
Cor pulmonale • Some mediators play a role in the development of pulmonary vascular remodeling • For instances,endothelin-1 release by endothelial cells in increased in hypoxic states • The presence of endothelin-1 may increase calcium entry into the vascular smooth muscle cells leading to hypoxic vasoconstriction • Vascular endothelin growth factor and plateletderived growth factors A and B are up-regulated under conditions of chronic hypoxia
Cor pulmonale • These growth factors are involved in endothelial cell proliferation and vascular injury and remodeling of the pulmonary vascular bed • In addition,occlusion or narrowing of medium-size to large pulmonary arteries is the basis for increased pulmonary vascular resistance in such disorders as hilar or mediastinal compressive metastatic tumors or fibrosis,nonspecific arteritis,primary pulmonary tumors,major vessel chronic thromboembolic disease,and infection
Cor pulmonale • C. Increased blood volume and blood viscosity • Other functional factors must be considered,namely hypercapnic acidosis and hyperviscosity caused by polycythaemia and erythrocythemia,but their role seems small when compared to that of alveolar hypoxia • In the setting of hypoxia and acidosis,activation of the renin-angiotensin-aldosterone system leads to increase in plasma renin activity and increased levels of aldosterone,vasopressin,and catecholamines.Natriuretic peptide levels are also elevated
Cor pulmonale
Dysfunction of right heart Pulmonary hypertension increases the work of the right ventricle,which leads more or less rapidly to right ventricular enlargement which can result in ventricular dysfunction(systolic,diastolic) Later,right heart failure(RHF)characterized by the presence of peripheral edema can be observed Finally,left ventricular dysfunction in cor pulmonale occurs through ventricular interdependence in the setting of a limiting pericardium
Cor pulmonale Impairment of other vital organs • Hypoxia and hypercapnic acidosis also affect other vital organs beside heart,such as liver,kidney,brain,intestine,endocrine system and hematology system
Cor pulmonale
CLINICAL PRESENTATION Symptoms Clinical manifestations of cor pulmonale generally are nonspecific The symptoms may be subtle, especially in early stages of the disease, and mistakenly may be attributed to the underying pulmonary pathology The patients may complain of fatigue, exertional dyspnea, and syncope with exertion-symptoms reflecting a relative inability to increase cardiac output during exercise with a subsequent drop in the systemic arterial pressure
Cor pulmonale Exertional chest pain aslo can occur and may be due to pulmanory artery stretching and right ventricular ischemia • Other symptoms mainly related to pulmonary artery hypertention include cough, hemoptysis, and, rarely, hoarseness due to compression of the left recurrent laryngeal nerve by a dilated pulmonary artery • In advanced stages, passive heptic congestion secondary to severe right ventricular failure may lead to anorexia, right upper quadrant abdominal discomfort, and jaundice • Swelling of the legs also can occur, and syncope is a late and ominous occurrence
Cor pulmonale Physical Signs • The most obvious physical findings in cor pulmonale reflect the underlying lung disease, with an increase in chest diameter, labored respiratory efforts with retractions of the chest wall, hyperresonance to percussion, diminished breath sounds, wheezing, distant heart sounds, and, rarely, cyanosis • Physical findings characteristic of pulmonary hypertension, RVH, and right ventricular failure aslo may be present • Early signs characteristic of pulmonry artery hypertension include splitting of the second heart sound with accentuation of the pulmonic component
Cor pulmonale • A systolic ejection murmur with sharp ejection click over the region of the pulmonary artery may be heard in advanced disease, along with a diastolic pulmonary regurgitation murmur • RVH is charaterized by a left parasternal or subxiphoid heave • A prominent A wave in the jugular venous pulse associated with a right-sided fourth heart sound aslo can be present and reflects the increase in the filling pressure of the right ventricle
Cor pulmonale • Right ventricular failure leads to systemic venous congestion, which is manifested as distended neck veins with a prominent V wave, a right ventricular third heart sound, a holosystolic tricuspid insufficiency murmur accentuated during inspirating and occasionally associated with pulsatile liver, and peripheral edema. Pleural effusions and ascites are uncommon, even in severe cor pulmonale
Cor pulmonale
LAB AND OTHER FINDINGS
Chest Roentgenogram The chest X-ray in patients with chronic cor pulmonale may show enlargement of the central pulmonary arteries with oligemic peripheral lung fields Pulmonary hypertension should be suspected when the 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 leads to an increase of the transverse diameter of the heart shadow to the right on the posterior view and filling of the retrosternal air space on the lateral view
Cor pulmonale Electrocardiogram • ECG abnormalities in chronic cor pulmonale refect the presence of RVH or strain • ECG criteria for RVH have high specificity but low sensitivity, particularly patients with COPD and biventricular hypertromphy • P-pulmonale paterten due to right artrial enlargement and right bundle branch conduction abnormalities may be present
Cor pulmonale
Echocardiography
• Two-dimensional echocardiography usually demonstrates signs of chronic right ventricular wall with paradoxical motion of the interventricular septum during systole occurs. At an advanced stage, right ventricular dilatation occurs, and the septum shows abnormal diastolic flattening • In extreme cases, the septum actually may bulge into the left ventricular cavity during diastole
Cor pulmonale • Doppler echocardiography: Doppler echocardiography is considered the most reliable noninvasive technique to estimate pulmonary artery pressure. The efficacy of Doppler echocardiography may be limited by the ability to identify an adequate tricuspid regurgitant jet, which may be further enhanced by using saline contrast
Cardiac Catheterization • Right-heart catheterization is considered the most precise method for diagnosis and quantification of pulmonary hypertension
Cor pulmonale • It is indicated when echocardiography cannot assess the severity a tricuspid regurgitant jet, thus excluding an assessment of pulmonary hypertension • Right-heart catheterization occasionally is important for differentiating cor pulmonal from occult left ventricular dysfunction. Especially when the presentation is confusing • Another indication may be for evaluation of the potential reversibility of pulmonary arterial hypertension with vasodilator therapy
Cor pulmonale • In selected cases, ventilation-perfusion lung scaning, pulmonary angiography, chest CT scanning and lung biopsy may be indicated to determind underlying etiology. Lung biopsy occasionally may be indicated to determin underlying etiology
Cor pulmonale
DIAGNOSIS
• The diagnosis is based on the medical history, underlying diseases, pulmonary hypertension, right ventricular enlargement or dysfunction of right heart, such as,P2>A2, jugular vein distention, prominent right ventricular impulse, hepatomegaly peripheral edema, and lab findings that indicate the right ventricular enlargement
Cor pulmonale
DIFFERENTIAL DIAGNOSIS
• Prior to the diagnosis of chronic cor pulmonale,some other problem must be considered,such as ventricular septal defect,congestive heart failure,primary pulmonic stenosis,primary pulmonary hypertention
Treatment
Cor pulmonale
Goals of successful treatment of chronic cor pulmonale include eviating right failure and associated left heart failure,improving the patient’s functional capacity,and lengthening survival The specific measurements include as follow Antimicrobial treatment When the suspected or defined sepsis was found,the antimicrobial therapy should be initiated as early as possible The empirical therapy must based on the local microbial prevalence and step down after the referential lab data is available
Cor pulmonale
Oxygen Therapy Oxygen therapy is of most importance in patients with underlying COPD,particularly when administered on a continuous basis With cor pulmonale,the partial pressure of oxygen(PO2)is likely to be below 55mmHg and decreases further with exercise and during sleep Oxygen therapy relieves hypoxemic pulmonary vasoconstriction,which then improves cardiac output,lessens sympathetic vasoconstriction,alleviates tissue hypoxemia,and improves renal perfusion
Cor pulmonale • Thus, treatment pulmonary hypertention is part of an overall program to manage severe obstructive lung disease • Two separate clinical trial have shown that intellectual function and survival are improved in chronically hypoxemic patients (PaO2<55mmHg)and edematous and show Ppulmonale on the electrocardiogram • To be effective oxygen must be administered for at least 18 h per day.The Nocturnal Oxygen Therapy Trial(NOTT) a multicenter randomized trial,showed that continuous lowflow oxygen therapy for patients with severe COPD resulted in significant reduction in the mortality rate
Cor pulmonale
Treatment of Haert Failure A. Diuretics Diuretics are used in the management of chronic cor pulmonale , particularly when the right ventricular filling volume is markedly elevated Diuretics may result in improvement of the function of both the right and left ventricles However, diuretics may produce hemodynamic adverse effects if they are not used cautiously
Cor pulmonale • Excessive volume depletion can lead to decline in cardiac output • Another potential complication of diuresis is the production of a hypokalemic metabolic alkalosis,which diminishies the effectiveness of carbon dioxide stimulation on the respiatory centers and lessens ventilatory drive • Therefore, diuresis,while recommended in the management of chronic cor pulmonale,needs to be used with great caution
Cor pulmonale • B.Inotropic Agent • The use of cardiac glycosides,such as digitalis,in patients with cor pulmonale has been controversial,and the beneficial effect of these drugs is not as obvious as in the setting of left heart failure • Nevertheless,studies have confirmed a modest effect of digitalis on the failing right ventricle in patients with chronic cor pulmonale
Cor pulmonale • It must be used cautiously,however,and should not be used during the acute phases of respiratory insufficiency when large fluctuations in levels of hypoxia and acidosis may occur • Patients with hypoxemia or acidosis are at increased risk of developing arrhythmias due to digitalis • C. Vasodilators • Vasodilator drugs have been advocated in the longterm management of chronic cor pulmonale with modest results
Cor pulmonale • Calcium channel blockers,particularly oral sustained-release nifedipine and diltiazem,can lower pulmonary pressures,although they appear more effective in primary rather than secondary pulmonary hypertension • Right heart catheterization has been recommended during initial administration of vasodilators to objectively assess the efficacy and detect the possible adverse hemodynamic consequences of vasodilators
Cor pulmonale • D. Theophylline • Theophylline has been reported to reduce pulmonary vascular resistance and pulmonary arterial pressures acutely in patients with chronic cor pulmonale secondary to COPD • Theophylline has a weak inotropic effect and thus may improve right and left ventricular ejection. As a result, considering the use of theophylline as adjunctive therapy in the management of chronic or decompensated cor pulmonale is reasonable in patients with underlying COPD
Cor pulmonale • E.Anticoagulation • Anticoagulation with warfarin is recommended in patients at high risk for thromboembolism • The beneficial role of anticoagulation in improving the symptoms and mortality in patients with primary pulmonary arterial hypertension clearly was demonstrated in a variety of clinical trials • The evidence of benefit, however, has not been established in patients with secondary pulmonary arterial hypertension.
Cor pulmonale • Therefore, anticoagulation therapy may be used in patients with cor pulmonale secondary to thromboembolic phenomena and underlying primary pulmonary arterial hypertension • F.Miscellaneous • Treatment of arrhythmia, intensive nursing care, treatment targeted to the complications, and patient education must be considered
Cor pulmonale • PROGNOSIS • The prognosis of cor pulmonale is variable depending upon underlying pathology. Patients with cor pulmonale due to COPD have a high 2-year mortality • The mortality accounts for about 10%-15% of all the cor pulmonale
Cor pulmonale • PROPHYLAXIS • The goal aims to prevent the disorders of bronchi, the lung disease and the pulmonary vascular impairment • The measurements include education on smoking, avoidance of the causative factors resulting the underlying diseases which lead cor pulmonale