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Definition: a consistently increased pulmonary arterial pressure (> 20mmHg) under resting conditions, which can lead to cor pulmonale- right ventricular failure secondary to pulmonary hypertension.
Occurs due to an increase in pulmonary vascular resistance or an increase in pulmonary blood flow.
Primary pulmonary hypertension: idiopathic
Left heart disease– mitral valve disease, left ventricular failure, left atrial myxoma/thrombosis
Chronic lung disease– COPD, bronchiectasis, pulmonary fibrosis, severe chronic asthma, lung resection
Pulmonary vascular disorders– pulmonary emboli, pulmonary vasculitis, ARDS, sickle-cell disease, parasite infestation
Increased pulmonary blood flow– atrial septal defect, ventral septal defect, patent ductus arteriosus
Neuromuscular disease– myasthenia gravis, poliomyelitis, motor neuron disease
Skeletal disease– kyphosis, scoliosis, thoracoplasty (rib resection)
Connective tissue disease– SLE, systemic sclerosis
Hypoventilation– sleep apnoea, enlarged adenoids in children
Epidemiology: primary pulmonary hypertension is usually seen in young females
-dyspnoea (on exertion), fatigue, lethargy = inability to increase cardiac output with exercise
-symptoms of underlying cause e.g. chronic cough
As right ventricular failure develops:
-abdominal pain from hepatic congestion
-raised JVP (with prominent a and v waves)
-right ventricular/left parasternal heave (hypertrophy)
–pansystolic murmur (tricuspid regurgitation)
–early diastolic Graham Steell murmur (pulmonary regurgitation secondary to pulmonary hypertension)
Signs of underlying condition e.g. hyper-resonant chest in COPD
Signs of right heart failure if severe:
-hepatomegaly, pulsatile liver
FBC- raised Hb and haematocrit (secondary polycythaemia in chronic lung disease)
ABG- hypoxia +/- hypercapnia
CXR- enlarged right atrium and ventricle, prominent pulmonary arteries
signs of cause e.g. COPD, calcified mitral valve
- P pulmonale (peaked P wave = right atrial hypertrophy, usually in lead II)
- Right axis deviation
- Right ventricular hypertrophy/ strain (RVH- dominant R wave in V1 + deep S wave in V6, T wave inversion in V1-V3 or V4. RV strain- dominant R wave + T wave inversion/ST depression in V1-2. Leads II, III, aVF may show similar changes.)
- Limb leads exhibit low voltage in COPD
Echo- to visualise right ventricular hypertrophy or dilation and possible underlying case e.g. mitral disease
Lung function tests- to assess for chronic lung disease
Cardiac catheterisation– to assess severity, right heart pressures and response to vasodilators
High-resolution CT thorax- images pulmonary arteries and to diagnose lung disease
Lung biopsy- assesses structural lung changes
-treat underlying cause e.g. COPD and pulmonary infections
-for primary pulmonary hypertension consider:
- anticoagulation (warfarin)- due to high risk of thrombosis
- calcium channel blockers (e.g. verapamil, nifedipine)
- prostacyclin analogues (e.g. iloprost) Epoprostenol = IV prostacyclin .
- endothelin receptor antagonist (bosentan) blocks vasoconstriction but teratogenic so only considered in later stage
- phosphodiester inhibitors (e.g. sildenafil) promote pulmonary smooth muscle relaxation reducing pulmonary hypertension
–treat respiratory failure
= acutely, 24% oxygen if PaO2 < 8kPa.
= monitor ABG and gradually increase O2 concentration if PaCO2 is stable.
=long-term oxygen therapy for 15h/d in COPD patients increases survival
NICE guidelines suggest LTOT for COPD patients with PaO2 < 7.3 kPa or < 8kPa if they have pulmonary hypertension.
–treat cardiac failure
=diuretics e.g. furosemide, spironolactone
=monitor U&Es and give amiloride (potassium-sparing diuretic) or potassium supplements if necessary.
-consider venesection if haematocrit > 55% (percentage of blood volume that is red blood cells)
-consider heart-lung transplantation in young patients
Complications: respiratory failure, right heart failure (cor pulmonale), arrhythmias (AF/VT/VF), sudden death
Prognosis: poor. Chronic and incurable with unpredictable survival rate. Length of survival has improves to up to 15-20 years.
50% with cor pulmonale die within 5 years.
References: Cheese & Onion, Rapid Medicine