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Pulmonary Hypertension

Pulmonary hypertension

When the mean pulmonary artery pressure is at least 25mmHg at rest, as measured at right heart catheterisation.

Causes

Respiratory failure due to intrinsic pulmonary disease is the most common cause.

Complication of connective tissue disease like systemic sclerosis.

Or as result of chronic thromboembolic events.

Pathological features

There occurs hypertrophy of both the media and the intima of the blood vessel wall and there occurs clonal expansion of endothelial cells which take on the appearance of plexiform lesions.
There is marked narrowing of the vessel lumen, this leads to an increase in pulmonary vascular resistance and therefore pulmonary hypertension occurs.

Clinical features

Breathlessness, chest pain, fatigue, palpitation and syncope.

Signs:

Elevation of the jugular venous pulse, with prominent 'a' wave if in sinus rhythm.
Parasternal heave in right ventricular hypertrophy.
Accentuation of the pulmonary component of the second heart sound and a right ventricular third heart sound.

Investigations

Echo
X-ray
Doppler
Transthoracic echocardiography 
right heart catheterisation

Management

General measures:

Diuretic therapy for right heart failure.
Long-term oxygen therapy for those with chronic hypoxia.
In the risk of bleeding, anticoagulation should be considered.
Digoxin for patients who develop atrial tachyarrhythmias.
Pneumococcal and influenza vaccination should be done.

Specific treatment:

High dose calcium channel blockers.
Prostaglandins like epoprostenol or iloprost therapy, the phosphodiesterase type 5 (pde5) inhibitor sildenafil.
Oral endothelin antagonist bosentan.
Heart-lung transplantation.
Pulmonary thrombo endarterectomy.
Atrial septostomy.


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