- •Table of Contents
- •Copyright
- •Dedication
- •Introduction to the eighth edition
- •Online contents
- •List of Illustrations
- •List of Tables
- •1. Pulmonary anatomy and physiology: The basics
- •Anatomy
- •Physiology
- •Abnormalities in gas exchange
- •Suggested readings
- •2. Presentation of the patient with pulmonary disease
- •Dyspnea
- •Cough
- •Hemoptysis
- •Chest pain
- •Suggested readings
- •3. Evaluation of the patient with pulmonary disease
- •Evaluation on a macroscopic level
- •Evaluation on a microscopic level
- •Assessment on a functional level
- •Suggested readings
- •4. Anatomic and physiologic aspects of airways
- •Structure
- •Function
- •Suggested readings
- •5. Asthma
- •Etiology and pathogenesis
- •Pathology
- •Pathophysiology
- •Clinical features
- •Diagnostic approach
- •Treatment
- •Suggested readings
- •6. Chronic obstructive pulmonary disease
- •Etiology and pathogenesis
- •Pathology
- •Pathophysiology
- •Clinical features
- •Diagnostic approach and assessment
- •Treatment
- •Suggested readings
- •7. Miscellaneous airway diseases
- •Bronchiectasis
- •Cystic fibrosis
- •Upper airway disease
- •Suggested readings
- •8. Anatomic and physiologic aspects of the pulmonary parenchyma
- •Anatomy
- •Physiology
- •Suggested readings
- •9. Overview of diffuse parenchymal lung diseases
- •Pathology
- •Pathogenesis
- •Pathophysiology
- •Clinical features
- •Diagnostic approach
- •Suggested readings
- •10. Diffuse parenchymal lung diseases associated with known etiologic agents
- •Diseases caused by inhaled inorganic dusts
- •Hypersensitivity pneumonitis
- •Drug-induced parenchymal lung disease
- •Radiation-induced lung disease
- •Suggested readings
- •11. Diffuse parenchymal lung diseases of unknown etiology
- •Idiopathic pulmonary fibrosis
- •Other idiopathic interstitial pneumonias
- •Pulmonary parenchymal involvement complicating systemic rheumatic disease
- •Sarcoidosis
- •Miscellaneous disorders involving the pulmonary parenchyma
- •Suggested readings
- •12. Anatomic and physiologic aspects of the pulmonary vasculature
- •Anatomy
- •Physiology
- •Suggested readings
- •13. Pulmonary embolism
- •Etiology and pathogenesis
- •Pathology
- •Pathophysiology
- •Clinical features
- •Diagnostic evaluation
- •Treatment
- •Suggested readings
- •14. Pulmonary hypertension
- •Pathogenesis
- •Pathology
- •Pathophysiology
- •Clinical features
- •Diagnostic features
- •Specific disorders associated with pulmonary hypertension
- •Suggested readings
- •15. Pleural disease
- •Anatomy
- •Physiology
- •Pleural effusion
- •Pneumothorax
- •Malignant mesothelioma
- •Suggested readings
- •16. Mediastinal disease
- •Anatomic features
- •Mediastinal masses
- •Pneumomediastinum
- •Suggested readings
- •17. Anatomic and physiologic aspects of neural, muscular, and chest wall interactions with the lungs
- •Respiratory control
- •Respiratory muscles
- •Suggested readings
- •18. Disorders of ventilatory control
- •Primary neurologic disease
- •Cheyne-stokes breathing
- •Control abnormalities secondary to lung disease
- •Sleep apnea syndrome
- •Suggested readings
- •19. Disorders of the respiratory pump
- •Neuromuscular disease affecting the muscles of respiration
- •Diaphragmatic disease
- •Disorders affecting the chest wall
- •Suggested readings
- •20. Lung cancer: Etiologic and pathologic aspects
- •Etiology and pathogenesis
- •Pathology
- •Suggested readings
- •21. Lung cancer: Clinical aspects
- •Clinical features
- •Diagnostic approach
- •Principles of therapy
- •Bronchial carcinoid tumors
- •Solitary pulmonary nodule
- •Suggested readings
- •22. Lung defense mechanisms
- •Physical or anatomic factors
- •Antimicrobial peptides
- •Phagocytic and inflammatory cells
- •Adaptive immune responses
- •Failure of respiratory defense mechanisms
- •Augmentation of respiratory defense mechanisms
- •Suggested readings
- •23. Pneumonia
- •Etiology and pathogenesis
- •Pathology
- •Pathophysiology
- •Clinical features and initial diagnosis
- •Therapeutic approach: General principles and antibiotic susceptibility
- •Initial management strategies based on clinical setting of pneumonia
- •Suggested readings
- •24. Bacterial and viral organisms causing pneumonia
- •Bacteria
- •Viruses
- •Intrathoracic complications of pneumonia
- •Respiratory infections associated with bioterrorism
- •Suggested readings
- •25. Tuberculosis and nontuberculous mycobacteria
- •Etiology and pathogenesis
- •Definitions
- •Pathology
- •Pathophysiology
- •Clinical manifestations
- •Diagnostic approach
- •Principles of therapy
- •Nontuberculous mycobacteria
- •Suggested readings
- •26. Miscellaneous infections caused by fungi, including Pneumocystis
- •Fungal infections
- •Pneumocystis infection
- •Suggested readings
- •27. Pulmonary complications in the immunocompromised host
- •Acquired immunodeficiency syndrome
- •Pulmonary complications in non–HIV immunocompromised patients
- •Suggested readings
- •28. Classification and pathophysiologic aspects of respiratory failure
- •Definition of respiratory failure
- •Classification of acute respiratory failure
- •Presentation of gas exchange failure
- •Pathogenesis of gas exchange abnormalities
- •Clinical and therapeutic aspects of hypercapnic/hypoxemic respiratory failure
- •Suggested readings
- •29. Acute respiratory distress syndrome
- •Physiology of fluid movement in alveolar interstitium
- •Etiology
- •Pathogenesis
- •Pathology
- •Pathophysiology
- •Clinical features
- •Diagnostic approach
- •Treatment
- •Suggested readings
- •30. Management of respiratory failure
- •Goals and principles underlying supportive therapy
- •Mechanical ventilation
- •Selected aspects of therapy for chronic respiratory failure
- •Suggested readings
- •Index
Obstructive disease, interstitial disease, and a variety of neural, muscular, and chest wall diseases may produce PH and cor pulmonale.
The most important aim of treatment for cor pulmonale in the setting of obstructive and interstitial disease is correction of alveolar hypoxia and hypoxemia by the administration of supplemental O2. The goal is to maintain arterial PO2 at a level greater than approximately 60 mm Hg, above which hypoxic vasoconstriction is largely eliminated. Other forms of therapy aimed more specifically at the underlying disease are discussed in Chapters 6, 10, and 11.
In addition to these two categories of lung disease, other disorders of the respiratory apparatus associated with hypoxemia and hypercapnia may be complicated by the development of cor pulmonale. Specifically, disorders of the control of breathing, of the chest bellows, and of the neural apparatus controlling the chest bellows may be complicated by cor pulmonale. These disorders are discussed in more detail in Chapters 18 and 19.
Chronic thromboembolic pulmonary hypertension (group 4 PH)
The typical presentation of chronic thromboembolic pulmonary hypertension (CTEPH) is with insidious onset of dyspnea and findings related to PH, rather than with a history suggesting one or more known acute episodes of pulmonary embolism (see Chapter 13). Presumably, by the time a patient presents with CTEPH, the emboli have been occurring and organizing with fibrosis over months to years. Because chronic thrombi are organized and extensively remodeled with fibroblasts and connective tissue, anticoagulation alone is not an effective therapy. In most cases, the organized thromboemboli are primarily located within the large proximal pulmonary arteries, causing significant obstruction. In these patients, surgical removal of the proximal organized thrombi (pulmonary thromboendarterectomy) may be a feasible and highly effective therapeutic option. More recently, balloon pulmonary angioplasty is employed as an alternative to surgery in some cases. In other patients, there is extensive thromboembolic occlusion of smaller, inaccessible vessels. Although this type of small vessel occlusion has generally been assumed to result from multiple small pulmonary emboli, primary thrombosis of the microvasculature—perhaps secondary to endothelial damage—also has been suggested to play a role. For the small vessel or microvascular form of chronic pulmonary thromboembolism, therapy involves anticoagulation and agents similar to those used for IPAH.
Pulmonary hypertension with unclear multifactorial mechanisms (group 5 PH)
A miscellaneous group of diseases listed in Table 14.2 under Group 5 may be associated with PH. The most common disorder in this category is sarcoidosis. The underlying mechanisms responsible for PH in these disorders are not entirely clear and are often believed to be multifactorial.
Suggested readings
General reviews
Dweik R.A, Rounds S, Erzurum S.C, Archer S, Fagan K, Hassoun P.M., et al. An official American Thoracic Society statement: Pulmonary hypertension phenotypes American Journal of Respiratory and Critical Care Medicine 2014;189: 345-355.
Frost A, Badesch D, Gibbs J.S.R, Gopalan D, Khanna D, Manes A., et al. Diagnosis of pulmonary hypertension European Respiratory Journal 2019;53: 1801904.
Данная книга находится в списке для перевода на русский язык сайта https://meduniver.com/
Galiè N, Humbert M, Vachiery J.L, Gibbs S, Lang I, Torbicki A., et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) European Respiratory Journal 2015;46: 903-975.
Hassoun P.M. Pulmonary arterial hypertension New England Journal of Medicine 2021;385: 2361-2376.
Humbert M, Guignabert C, Bonnet S, Dorfmüller P, Klinger J.R, Nicolls M.R., et al.
Pathology and pathobiology of pulmonary hypertension: State of the art and research perspectives European Respiratory Journal 2019;53: 1801887.
Morrell N.W, Aldred M.A, Chung W.K, Elliott C.G, Nichols W.C, Soubrier F., et al. Genetics and genomics of pulmonary arterial hypertension European Respiratory Journal 2019;53: 1801899.
Poch D. & Mandel J. In the clinic. Pulmonary hypertension Annals of Internal Medicine 2021;174: ITC49ITC64.
Remy-Jardin M, Ryerson C.J, Schiebler M.L, Leung A.N.C, Wild J.M, Hoeper M.M., et al.
Imaging of pulmonary hypertension in adults: A position paper from the Fleischner Society European Respiratory Journal 2021;57: 2004455.
Simonneau G, Montani D, Celermajer D.S, Denton C.P, Gatzoulis M.A, Krowka M., et al.
Haemodynamic definitions and updated clinical classification of pulmonary hypertension European Respiratory Journal 2019;53: 1801913.
Pulmonary arterial hypertension and related disorders
Chaisson N.F. & Hassoun P.M. Systemic sclerosis-associated pulmonary arterial hypertension Chest 2013;144: 1346-1356.
Chan S.Y. & Loscalzo J. Pathogenic mechanisms of pulmonary arterial hypertension Journal of Molecular and Cellular Cardiology 2008;44: 14-30.
D’Alto M. & Diller G.P. Pulmonary hypertension in adults with congenital heart disease and Eisenmenger syndrome: Current advanced management strategies Heart 2014;100: 1322-1328.
Davies R.J. & Morrell N.W. Molecular mechanisms of pulmonary arterial hypertension: Role of mutations in the bone morphogenetic protein type II receptor Chest 2008;134: 1271-1277.
Fadini G.P, Avogaro A, Ferraccioli G. & Agostini C. Endothelial progenitors in pulmonary hypertension: New pathophysiology and therapeutic implications European Respiratory Journal 2010;35: 418-425.
Fritz J.S, Fallon M.B. & Kawut S.M. Pulmonary vascular complications of liver disease
American Journal of Respiratory and Critical Care Medicine 2013;187: 133-143.
Galiè N, Channick R.N, Frantz R.P, Grünig E, Jing Z.C, Moiseeva O., et al. Risk stratification and medical therapy of pulmonary arterial hypertension European Respiratory Journal 2019;53: 1801889.
Humbert M. & Ghofrani H.A. The molecular targets of approved treatments for pulmonary arterial hypertension Thorax 2015;71: 73-83.
Klinger J.R, Elliott C.G, Levine D.J, Bossone E, Duvall L, Fagan K., et al. Therapy for pulmonary arterial hypertension in adults: Update of the CHEST Guideline and Expert Panel Report Chest 2019;155: 565-586.
Krowka M.J. Portopulmonary hypertension Seminars in Respiratory and Critical Care Medicine 2012;33: 17-25.
Le Pavec J, Humbert M, Mouthon L. & Hassoun P.M. Systemic sclerosis-associated pulmonary arterial hypertension American Journal of Respiratory and Critical Care Medicine 2010;181: 1285-1293.
Ma L. & Chung W.K. The role of genetics in pulmonary arterial hypertension Journal of Pathology 2017;241: 273-280.
Maron B.A, Abman S.H, Elliott C.G, Frantz R.P, Hopper R.K, Horn E.M., et al. Pulmonary arterial hypertension: Diagnosis, treatment, and novel advances American Journal of Respiratory and Critical Care Medicine 2021;203: 1472-1487.
Morrell N.W, Adnot S, Archer S.L, Dupuis J, Lloyd Jones P, MacLean M.R., et al. Cellular and molecular basis of pulmonary arterial hypertension Journal of the American College of Cardiology Suppl. 1, 2009;54: S20S31.
Naeije R, Richter M.J. & Rubin L.J. The physiological basis of pulmonary arterial hypertension European Respiratory Journal 2022;59: 2102334.
Ruopp N.F. & Cockrill B.A. Diagnosis and treatment of pulmonary arterial hypertension. A review JAMA 2022;327: 1379-1392.
Pulmonary hypertension related to lung disease
Behr J. & Ryu J.H. Pulmonary hypertension in interstitial lung disease European Respiratory Journal 2008;31: 1357-1367.
Chaouat A, Naeije R. & Weitzenblum E. Pulmonary hypertension in COPD European Respiratory Journal 2008;32: 1371-1385.
Haran S, Elia D. & Humbert M. Pulmonary hypertension in parenchymal lung diseases: Any future for new therapies? Chest 2018;153: 217-223.
Klinger J.R. Group III Pulmonary hypertension: Pulmonary hypertension associated with lung disease: Epidemiology, pathophysiology, and treatments Cardiology Clinics 2016;34: 413-433.
Poor H.D, Girgis R. & Studer S.M. World Health Organization group III pulmonary hypertension Progress in Cardiovascular Diseases 2012;55: 119-127.
Seeger W, Adir Y, Barberà J.A, Champion H, Coghlan J.G, Cottin V., et al. Pulmonary hypertension in chronic lung diseases Journal of the American College of Cardiology Suppl. 25, 2013;62: D109D116.
Shlobin O.A, Brown A.W. & Nathan S.D. Pulmonary hypertension in diffuse parenchymal lung diseases Chest 2017;151: 204-214.
Singh I, Ma K.C. & Berlin D.A. Pathophysiology of pulmonary hypertension in chronic parenchymal lung disease American Journal of Medicine 2016;129: 366-371.
Chronic thromboembolic pulmonary hypertension
Delcroix M, Torbicki A, Gopalan D, Sitbon O, Klok F.A, Lang I., et al. ERS statement on chronic thromboembolic pulmonary hypertension European Respiratory Journal 2021;57: 2002828.
Kim N.H, Delcroix M, Jenkins D.P, Channick R, Dartevelle P, Jansa P., et al. Chronic thromboembolic pulmonary hypertension Journal of the American College of Cardiology Suppl. 25, 2013;62: D92D99.
Данная книга находится в списке для перевода на русский язык сайта https://meduniver.com/
Lang I.M. & Madani M. Update on chronic thromboembolic pulmonary hypertension
Circulation 2014;130: 508-518.
Piazza G. & Goldhaber S.Z. Chronic thromboembolic pulmonary hypertension New England Journal of Medicine 2011;364: 351-360.
Pulmonary hypertension associated with left heart disease
Guazzi M. & Labate V. Group 2 PH: Medical therapy Progress in Cardiovascular Diseases 2016;59: 71-77.
Naeije R. & D’Alto M. The diagnostic challenge of group 2 pulmonary hypertension
Progress in Cardiovascular Diseases 2016;59: 22-29.
Opitz C.F, Hoeper M.M, Gibbs J.S, Kaemmerer H, Pepke-Zaba J, Coghlan J.G., et al. Precapillary, combined, and post-capillary pulmonary hypertension: A pathophysiological continuum Journal of the American College of Cardiology 2016;68: 368-378.
Pulmonary hypertension associated with multifactorial mechanisms
Baughman R.P. Pulmonary hypertension associated with sarcoidosis Arthritis Research and Therapy Suppl. 2, 2007;9: S8.
Golbin J.M, Somers V.K. & Caples S.M. Obstructive sleep apnea, cardiovascular disease, and pulmonary hypertension Proceedings of the American Thoracic Society 2008;5: 200206.
Gordeuk V.R, Castro O.L. & Machado R.F. Pathophysiology and treatment of pulmonary hypertension in sickle cell disease Blood 2016;127: 820-828.