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/

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/