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Cardio History

Pulmonary Hypertension



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    • Diagnosis - when pulmonary hypertension was diagnosed, symptoms, cause
    • Severity - functional impact, last echo / right heart catheter result
    • Management - treatment of underlying disease, PAH drugs
    • Complications - particularly right ventricular failure
    • Causes of Pulmonary Hypertension

    • Type 1: Pulmonary Arterial Hypertension

    • Idiopathic
    • Inherited
    • Drugs / toxins
    • HIV-related
    • Connective tissue disease - scleroderma, mixed connective tissue disease
    • Portopulmonary hypertension
    • Congenital heart disease
    • Schistosomiasis
    • Type 2: Left Heart Disease

    • Left ventricular systolic / diastolic dysfunction
    • Valvular disease - aortic stenosis, aortic regurgitation, mitral stenosis
    • Type 3: Lung Disease

    • Chronic obstruction pulmonary disease
    • Interstitial lung disease
    • Mixed lung disease
    • Sleep-disordered breathing
    • Alveolar hypoventilation
    • Type 4: Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

    • Chronic pulmonary emboli
    • Type 5: Other

    • Haematologic - chronic haemolytic anaemia, myeloproliferative disorders, splenectomy
    • Systemic - sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis
    • Metabolic - glycogen storage disorders, Gaucher disease
    • Other - tumour obstruction, chronic kidney disease


    • Symptoms of Pulmonary Hypertension

    • Fatigue
    • Exertional dyspnoea
    • Exertional chest pain
    • Exertional syncope
    • Symptoms of right heart failure - peripheral oedema, ascites


    • Complications of Pulmonary Hypertension

    • Pulmonary haemorrhage
    • Pulmonary arterial thrombosis
    • Right ventricular failure
    • Arrhythmias


    • Management Options

    • Treat / optimise the underlying disease
    • Pulmonary Arterial Hypertension Only

    • Endothelin receptor antagonists - bosentan, macitentan, ambrisentan
    • Phosphodiesterase 5 (PDE-5) inhibitors - sildenafil, tadalafil
    • Prostacyclin receptor agonists - prostacyclin, iloprost, tropostinil
    • Guanylate cyclase stimulants - riociguat
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