par Naeije, Robert
Référence Pulmonary Vascular Disorders, Nova Science Publishers, Inc., page (247-270)
Publication Publié, 2021-04
Partie d'ouvrage collectif
Résumé : Cor pulmonale can be defined by right heart failure secondary to diseases affecting the structure and function of the lungs. It used to be a postmortem pathological definition but now relies on imaging combined or not with a right heart catheterization. The most common cause of cor pulmonale is chronic obstructive pulmonary disease. The sine qua non condition of cor pulmonale is an excessive afterload on an increase in pulmonary artery pressures. The right ventricle initially adapts increased loading by increased contractility and hypertrophy without increased dimensions. There may even be decreased dimensions due to dynamic hyper-inflation impeding systemic venous return, then creating a condition recently identified as cor pulmonale parvus. With evolution of the disease, chronic inflammation, hypoxemia and hormonal disturbances, salt and water retention concur with exhaustion of systolic function adaptation to only mild pulmonary hypertension to produce the clinical picture of systemic congestion with hepatomegaly, turgescent jugular veins and eventual ascites. Cor pulmonale is associated with a limitation to exercise capacity and a poor prognosis. Treatment of cor pulmonale includes correction of hypoxemia with supplemental oxygen, relief of hypercapnic acidosis by ventilatory support and diuretics to control fluid retention. Vasodilators including those interfering with the endothelin, nitric oxide-soluble guanylate cyclase and prostacyclin pathways effective in the treatment of pulmonary arterial hypertension have repeatedly failed to show benefit. The ultimate treatment for refractory cor pulmonale is lung transplantation as right heart structure and function return to normal or near-normal after correction of the causal lung condition.