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Pulmonary Hypertension

 
 
 
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On this page:Complications

Overview

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

Complications

  • Complications of Pulmonary Hypertension

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