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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