This has been best conceptualized in COPD ( Figure 1). PH in hypoxemic lung diseases is likely the result of multiple factors including pulmonary vasoconstriction caused by alveolar hypoxia, 7 acidemia, 7 hypercarbia, 8 the distortion of pulmonary vessels by parenchymal changes, and increased cardiac output and blood viscosity from polycythemia secondary to hypoxia. 5,6 Other lung diseases known to cause cor pulmonale include interstitial lung diseases, restrictive ventilatory defects caused by thoracic cage deformities or neuromuscular diseases that cause respiratory muscle weakness, and disorders of ventilatory control including sleep-disordered breathing and obesity hypoventilation syndrome (OHS). 4 Between 10% and 30% of heart failure admissions in the US are the result of cor pulmonale with the most common cause in the United States being COPD, in 1 study accounting for 84% of cases. 2 Cor pulmonale was defined by the World Health Organization in 1963 as “hypertrophy of the right ventricle (RV) resulting from diseases affecting the function and/or structure of the lungs, except when these pulmonary alterations are the result of diseases that primarily affect the left side of the heart, as in congenital heart disease.” 3 Since then the definition has come to encompass both right ventricular hypertrophy, dilation, or both secondary to PH caused by pulmonary disorders. The classification of pulmonary hypertension (PH) has recently been revised, and PH associated with hypoxemic pulmonary disorders falls into World Health Organization group III. Pulmonary Vascular Disease in Respiratory Illness In addition, the complex nature of cardiac and lung disease will also be explored, particularly with respect to the relationship between chronic obstructive pulmonary disease, systemic inflammation, atherosclerosis, and cardiovascular death, which is currently a very active focus of research. This article will discuss the most common pulmonary diseases and disorders of ventilatory control that cause pulmonary vascular abnormalities and cor pulmonale, with particular concentration on how treatment of these diseases may affect the heart. Systemic manifestations of lung disease, particularly obstructive disorders, are also particularly relevant because they are associated with increased cardiac death and impaired health status.
The development of cor pulmonale is generally associated with poorer prognosis and increased death. Pulmonary hypertension, classified as group III in the World Health Organization classification scheme for pulmonary hypertension, may result in severe right ventricular dysfunction caused by lung disease, also known as cor pulmonale. Pulmonary vascular abnormalities are frequently present in patients with respiratory disorders, including chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, sarcoidosis, neuromuscular or chest wall disorders, and disorders of ventilatory control including sleep apnea syndromes and obesity hypoventilation syndrome. The complex nature of interactions between the pulmonary and cardiovascular systems is becoming increasingly appreciated.
Journal of the American Heart Association (JAHA).Circ: Cardiovascular Quality & Outcomes.
Arteriosclerosis, Thrombosis, and Vascular Biology (ATVB).
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