Cardiovascular Disease· Topic 2.7 Myocardial disease· 2.7.8

Cor pulmonale

Cor pulmonale is right ventricular structural and functional impairment caused by group 3 pulmonary hypertension from chronic lung disease or hypoxemia. Diagnosis rests on echocardiographic screening confirmed by right heart catheterization, and management centers on treating the underlying lung disease, correcting hypoxemia, and cautious diuresis.

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What this article covers

  • Overview

    Right ventricular disease driven by the lungs, not the left heart: cor pulmonale is an alteration in the structure and function of the right ventricle (RV) caused by a primary disorder of the…

  • Epidemiology

    COPD is the most common cause of cor pulmonale in adults, reflecting both the prevalence of COPD and the combination of hypoxic vasoconstriction and capillary bed destruction (emphysema) that raises pulmonary vascular resistance.

  • Risk factors

    COPD and emphysema: hypoxic vasoconstriction plus capillary bed destruction raise pulmonary vascular resistance; risk tracks with airflow obstruction severity.

  • Pathophysiology

    Chronic alveolar hypoxia triggers hypoxic pulmonary vasoconstriction: precapillary arteriolar constriction, normally localized to shunt blood from poorly ventilated units, becomes diffuse and…

  • Clinical features

    Exertional dyspnea is the earliest and most common symptom, reflecting both the underlying lung disease and an RV unable to augment output with exercise.

  • Differential diagnosis

    Left-sided heart failure (HFrEF or HFpEF): the dominant cause of PH overall; wedge pressure 15 mmHg on catheterization (rather than an isolated precapillary process) confirms left heart disease and excludes cor pulmonale.

  • Diagnosis

    The diagnosis requires demonstrating PH (resting mean PA pressure =20 mmHg on right heart catheterization) attributable to a chronic lung disease or hypoxemic disorder (group 3), plus evidence of RV structural change or failure.

  • Management

    First, treat the underlying lung disease aggressively, since group 3 PH management centers on the causative disorder, not the pulmonary vasculature: optimize inhaled bronchodilators in COPD…

  • Complications

    Decompensated right heart failure with low cardiac output, from progressive RV dilation, is the most serious complication and can precipitate cardiogenic shock.

  • Prognosis

    WHO functional class predicts survival: median survival is approximately 6 years for class I or II, versus approximately 6 months for class IV.

  • Prevention

    Smoking cessation is the single highest-yield intervention to slow progression of the COPD that most commonly causes cor pulmonale.

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