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Cardio History
 

Pulmonary Hypertension

 
 
 
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Overview

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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

Diagnosis

  • Symptoms of Pulmonary Hypertension

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

Complications

  • 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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