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Vascular Disease
 
 

Pulmonary Hypertension

 
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Overview

  • Pulmonary hypertension refers to a heterogeneous and functionally limiting group of disorders that result in increased pressure within the pulmonary arteries.

Pathogenesis

    • 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

Manifestations

    • Symptoms of Pulmonary Hypertension

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

    • General Signs

    • Raised JVP
    • Parasternal heave
    • Palpable P2
    • Loud second heart sound (P2)
    • Evidence of tricuspid regurgitation - pansystolic murmur loudest at the lower left sternal edge on inspiration
    • Signs Suggesting a Cause

    • Evidence of connective tissue disease - Raynaud’s, sclerodactyly, skin thickening, telangiectasia, synovitis
    • Evidence of portal hypertension - caput medusae, ascites, splenomegaly
    • Evidence of left ventricular failure - pulsus alternans, presence of S3, pulmonary crepitations
    • Evidence of pulmonary disease - hypoxia, hyperexpansion, crepitations, wheeze
    • Signs of Complications

    • Evidence of right ventricular failure - raised JVP, ascites, pleural effusions, peripheral oedema
    • Complications of Pulmonary Hypertension

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

Management

    • 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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