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Pulmonary hypertension and cor pulmonale

Last updated: July 27, 2021

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Pulmonary hypertension (PH) is elevated pressure in the pulmonary arteries ≥ 20 mmHg at rest. It can be idiopathic or due to chronic pulmonary (e.g., COPD, chronic sleep apnea) and/or cardiac disease (e.g., mitral valve disease). Over time, the rise in pressure may result in structural changes (e.g., dilation or hypertrophy) or impaired function of the right ventricle. If these changes are secondary to diseases of the lungs or the pulmonary artery system, the condition is referred to as cor pulmonale. While PH and cor pulmonale are often asymptomatic in early stages, symptoms like dyspnea on exertion, fatigue, cyanosis, and syncope appear in later stages. In decompensated cor pulmonale, the risk of arrhythmias and death is high. Echocardiograms are used as an initial non-invasive test to estimate the pulmonary artery pressure and to demonstrate an altered structure of the right ventricle. Right heart catheterization provides the definite diagnosis and the exact mean pulmonary arterial pressure (mPAP). Treatment mainly consists of management of the underlying causes of PH and cor pulmonale. Supplemental oxygen, pulmonary vasodilators, and diuretics may be given to reduce pulmonary artery hypertension and improve blood oxygenation. Lung transplantation is the treatment of last resort in refractory cases.

Pulmonary hypertension [1]

Cor pulmonale [2]

  • PAH: more commonly affects female individuals

Epidemiological data refers to the US, unless otherwise specified.

Classification of pulmonary hypertension (WHO) by cause

Cor pulmonale

Increased pressure in pulmonary circuit → ↑ right ventricular afterload dilatation and/or hypertrophy of the right heart right heart failure and arrhythmias death.

Clinical symptoms may be inconspicuous, especially, in early stages (the symptoms of underlying diseases such as COPD may eclipse those of PH).

The diagnostic evaluation of cor pulmonale is inseparable from the evaluation of PH. Testing aims at confirming PH with or without cor pulmonale and determining its severity and underlying pathology.

Treatment of the underlying cause

Therapy should be initiated early before irreversible changes in the pulmonary vessels occur.

Additional treatment

Pulmonary vasodilator therapy

Overview of pulmonary vasodilator agents
Agents Indications Mechanism of action Adverse effects Contraindications Interactions
Calcium channel blockers

Endothelin receptor antagonists (e.g., bosentan, macitentan, ambrisentan) [6]

Phosphodiesterase-5 inhibitors (e.g., sildenafil)
Prostacyclin analogs (iloprost, treprostinil)
  • None [7]
Long-acting synthetic prostacyclin (epoprostenol) [8]

Special patient groups

Over-diuresis may result in under-filling of the right ventricle and a decrease in cardiac output, leading to further complications such as prerenal failure!

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  2. Cor Pulmonale, Acute and Chronic. https://www.mdguidelines.com/cor-pulmonale-acute-and-chronic. Updated: February 19, 2017. Accessed: February 19, 2017.
  3. Fakhoury K, Kanu A. Clinical manifestations and evaluation of bronchiectasis in children. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/clinical-manifestations-and-evaluation-of-bronchiectasis-in-children?search=bronchiectasis%20cor%20pulmonale&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1.Last updated: March 2, 2018. Accessed: February 7, 2019.
  4. Le T, Bhushan V,‎ Sochat M, Chavda Y, Abrams J, Kalani M, Kallianos K, Vaidyanathan V. First Aid for the USMLE Step 1 2019. McGraw-Hill Medical
  5. Mahmud E, Raisinghani A, Keramati S, Auger W, Blanchard DG, DeMaria AN. Dilation of the coronary sinus on echocardiogram: prevalence and significance in patients with chronic pulmonary hypertension. J Am Soc Echocardiogr. 2001; 14 (1): p.44-9.
  6. TRACLEER® (bosentan) tablets, for oral use.
  7. VENTAVIS® (iloprost) inhalation solution.
  8. FLOLAN (epoprostenol sodium) for injection, for intravenous use.
  9. Kasper DL, Fauci AS, Hauser SL, Longo DL, Lameson JL, Loscalzo J. Harrison's Principles of Internal Medicine. McGraw-Hill Education ; 2015