Cor Pulmonale and Heart-Lung Interactions

 

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Cor pulmonale is the alteration in the structure and function of the right ventricle that results from pulmonary artery hypertension caused by various disorders affecting the lung. Cor pulmonale is itself a cause of significant morbidity and mortality and adversely affects the natural history of many of these causative disorders. The clinical manifestations of cor pulmonale as well as the laboratory and radiographic findings are relatively insensitive, thereby complicating its diagnosis and management. In this issue we provide an up-to-date review of cor pulmonale, emphasizing advances in its diagnosis and therapy.

The initial overview of cor pulmonale by Drs. Budev, Arroliga, Wiedemann, and Matthay, along with the subsequent article by Drs. Kosiborod and Wackers on the assessment of right ventricular morphology and function, establish the background and context for the subsequent discussion of the clinical entities that are associated with pulmonary hypertension and cor pulmonale. The overview by Budev and colleagues discusses the pathophysiology of pulmonary hypertension, right ventricular anatomy and physiology, and the interaction of the right and left ventricle in cor pulmonale.

Drs. Kosiborod and Wackers elucidate some recent advances in echocardiography and radionuclide and magnetic resonance imaging that enable clinicians to assess accurately and noninvasively the structure and function of the right ventricle.

Following these two introductory articles are seven reviews that focus primarily on the epidemiology, etiology, manifestations, evaluation, and therapy of the clinical disorders complicated by cor pulmonale. The first such article, by Drs. Lee-Chiong and Matthay, discusses cor pulmonale in chronic obstructive pulmonary disease (COPD). This article emphasizes that pulmonary hypertension is the primary cardiovascular complication in COPD and that the development of pulmonary hypertension and cor pulmonale reduces the survival rate in COPD.

Dr. Fedullo and colleagues discuss recent progress in the surgical management and postoperative care of patients with chronic thromboembolic pulmonary hypertension. The advances in this rapidly evolving field have helped to improve the once dismal prognosis of this disorder.

Dr. Tanoue extensively reviews the relation of collagen vascular diseases and pulmonary artery hypertension, including those disorders, such as scleroderma and mixed connective tissue disease, that have a high prevalence of pulmonary hypertension and other disorders, such as systemic lupus erythematosus and rheumatoid arthritis, that are less commonly associated with pulmonary hypertension.

Drs. Krachman, Criner, and Chatila examine the association of obesity, kyphoscoliosis, and such neuromuscular disorders as muscular dystrophy, myotonic dystrophy, poliomyelitis, and myasthenia gravis and the development of cor pulmonale.

Dr. Angtuaco provides an excellent synopsis of current information on congenital heart diseases and right ventricular dysfunction. Although cor pulmonale is limited to cardiac dysfunction resulting from pulmonary disease rather than primary cardiac diseases, the manifestations of congenital heart diseases can be indistinguishable from those of pulmonary heart disease.

The relationship between sleep disordered breathing and daytime pulmonary hypertension remains controversial. Drs. Judd, Liu, and Sateia discuss recent findings on the physiological changes in the cardiovascular system that occur during sleep disordered breathing.

The final article by Drs. Eckles and Anderson reviews cor pulmonale as a complication of cystic fibrosis, emphasizing the pathophysiology, diagnosis, and treatment of heart disease in patients with cystic fibrosis. Secondary pulmonary hypertension and cor pulmonale are likely to develop as lung disease in cystic fibrosis progresses, and pulmonary hypertension increases the risk for death in affected patients.

We thank the contributors of this seminar for the excellent texts and the expert counsel they have generously provided us, and we encourage our readers to share with us their thoughts regarding this issue.

Richard A. Matthay, M.D.

eofilo L. Lee-Chiong Jr., M.D.