Palevsky H I, Fishman A P
Department of Medicine, University of Pennsylvania School of Medicine, Philadelphia 19104.
JAMA. 1990 May 2;263(17):2347-53.
Cor pulmonale is right ventricular enlargement secondary to pulmonary hypertension. Although most often caused by parenchymal lung disease, derangements of the ventilatory drive, the respiratory pumping mechanism, or the pulmonary vascular bed may also result in right ventricular hypertrophy and dilatation. Arterial hypoxemia (and resultant polycythemia), hypercapnia, and respiratory acidosis all contribute to the increased afterload on the right ventricle. Diagnosis is often difficult, since pulmonary vascular disease, pulmonary hypertension, and cor pulmonale have few specific manifestations, especially early in their evolution. Treatment is primarily directed at the underlying pulmonary or ventilatory disorder, rather than at the right ventricular failure per se. Supplemental oxygen is essential to avoid hypoxia; corticosteroids, anticoagulants, vasodilators, and other specific therapies are used as indicated to treat the underlying pulmonary disorders. When medical therapies fail, lung or heart-lung transplantation has become a possibility for selected patients.
肺心病是继发于肺动脉高压的右心室扩大。虽然最常见的病因是实质性肺部疾病，但通气驱动、呼吸泵机制或肺血管床的紊乱也可能导致右心室肥厚和扩张。动脉血氧不足（以及由此导致的红细胞增多症）、高碳酸血症和呼吸性酸中毒都会导致右心室后负荷增加。诊断往往很困难，因为肺血管疾病、肺动脉高压和肺心病几乎没有特异性表现，尤其是在疾病早期。治疗主要针对潜在的肺部或通气障碍，而不是右心室衰竭本身。补充氧气对于避免缺氧至关重要；根据需要使用皮质类固醇、抗凝剂、血管扩张剂和其他特定疗法来治疗潜在的肺部疾病。当药物治疗无效时，对于选定的患者，肺移植或心肺移植已成为一种选择。
