Pulmonary heart disease (Cor Pulmonale)

Definition Right heart disease caused by pulmonary arterial hypertension

Clinical presentation May have an acute or chronic presentation

Acute form

Pathophysiology

Thrombus form the systemic vein

Thrombus from the right heart (<10% of cases of pulmonary embolism)

This means there is ventilation but no perfusion

After a few hours non-infused area of lung will collapse

Reduced cross sectional area of pulmonary arterial bed leads to pulmonary arterial hypertension

Area may not infarct due to local oxygenation and bronchial circulation

Clinical features

Sudden dyspnoea

Pleuritic chest pain and haemoptysis only occur if there is pulmonary infarction

Clinical DVT is often not observed Fever may present

Large embolus

Chest pain as a result of myocardial hypoperfusion Shock Raised JVP

Investigations

Ultrasound CXR and ECG may be normal D – dimer Blood gases Pulmonary angiography Ventilation / perfusion V / Q scan

Treatment

Oxygen Intravenous heparin, bolus of 10 000 units then infusions of 1 – 2 000 iu. per hour

Fibrinolytic therapy Sometimes surgery

Chronic form

Pathophysiology

Right ventricular hypertrophy causes by increased afterload

Chronic lung disease ---- areas of hypoxia and acidosis ------ localised reactionary vasoconstriction ------- increased pulmonary arterial resistance ------ increased pulmonary arterial pressure ------- right ventricular hypertrophy ------- right ventricular failure

Normal PA pressure is 18 – 25 / 6 – 10 PA hypertension is over 30/20

Hypoxia also reduced RV function Eventually the LV is also affected

RV failure increases systemic venous pressure causing chronic systemic venous hypertension ----- systemic oedema ------ Bloated Blue – polycythaemia and cyanosis

Clinical features Chest pain Dyspnoea Syncope Fatigue Sudden death JVP

Investigations CXR ECG Echocardiography

Treatment Underlying condition Diuretics

Long term oxygen therapy (LTOT) improves symptoms and diagnosis in COPD

There is a primary form of pulmonary hypertension with a poor prognosis