Cor pulmonaleCor pulmonale

• Cor pulmonale is right heart failure caused by chronic pulmonary hypertension

Causes of cor pulmonaleCauses of cor pulmonale

• Lung disease

Asthma (severe, chronic)

COPD

Bronchiectasis

Pulmonary fibrosis

Lung resection

• Pulmonary vascular disease

Pulmonary emboli

Pulmonary vasculitis

Primary pulmonary hypertension

ARDS

Sickle-cell disease

Parasite infestation

• Thoracic cage abnormality

Kyphosis

Scoliosis

Thoracoplasty

• Neuromuscular disease

Myasthenia gravis

Poliomyelitis

Motor neurone disease

• Hypoventilation

Sleep apnoea

Enlarged adenoids in children

Cerebrovascular disease

Clinical featuresClinical features

• Symptoms include dyspnoea, fatigue, or syncope. Signs: cyanosis; tachycardia; raised JVP with prominent a and v waves; RV heave; loud p2, pansystolic murmur (tricuspid regurgitation); early diastolic Graham Steell murmur; hepatomegaly and oedema.

InvestigationsInvestigations

• FBC: Hb and haematocrit ↑(secondary polycythaemia). ABG; hypoxia, with or without hypercapnia. CXR; enlarged right atrium and ventricle, prominent pulmonary arteries. ECG; P pulmonale; right axis deviation; right ventricular hypertrophy/ strain.

ManagementManagement

• Treat underlying cause – e.g. COPD and pulmonary infections

• Treat respiratory failure – in the acute situation give 24% oxygen if PaO2 <8kPa. Monitor ABG and gradually increase oxygen concentration if Pa CO2 is stable. In COPD patients, long-term oxygen therapy (LTOT) for 15h/d increases survival. Patients with chronic hypoxia when clinically stable should be assessed for LTOT.

• Treat cardiac failure with diuretics such as frusemide (=furosemide, e.g. 40-160mg/24h PO). Monitor U&E and give amiloride or potassium supplements if necessary. Alternative: spironolactone.

• Consider vensection if the haematocrit is > 55%.• Consider heart-lung transplantation in young

patients.

Prognosis Poor 50% die within 5yrs.