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Pulmonary Hypertension (Treatment (Oral calcium channel blockers as…
Pulmonary Hypertension
Key Facts
Normal mean pulmonary artery pressure is 14 +/- 3mmHg with an upper limit of normal of 20 mmHg
Pulmonary hypertension is defined as an mPAP of above 25mmHg as measured at right heart catheterisation and secondary right ventricular failure
The lung circulation offers a low resistance to flow compared to the systemic circulation (about 90mmHg)
Epidemiology
MSK - kyphoscoliosis, poliomyelitis, myasthenia gravis
Disturbance of respiratory drive - obstructive sleep apnoea, morbid obesity, cerebrovascular disease
Diseases of lung and parenchyma - COPD, chronic lung disorders
Cardiac - mitral stenosis, left ventricle failure, left atrial myxoma, congenital heart disease
Pulmonary vascular disorders - pulmonary embolism, primary pulmonary hypertension, veno-occlusive disease
Other - appetite suppression drugs, type 1 glycogen storage diseases, lipid storage disease, rheumatic autoimmune disease, hepatic mass, sickle cell disease, familial, idiopathic
Occurs due to an increase in pulmonary vascular resistance or an increase in pulmonary blood flow
Pathophysiology
Damages of the pulmonary endothelium, resulting in the release of vasoconstrictors
Increased platelet and leukocyte adhesion, elevated serotonin
Hypoxic vasoconstriction, inflammation, cell proliferation resulting in narrower vessels and increased right ventricular pressure caused pulmonary hypertension
Patients with progressive pulmonary hypertension develop right ventricular hypertrophy, dilation and eventually failure resulting in death
Clinical Presentation
Syncope - temporary loss of consciousness caused by a sudden drop in blood pressure
As right ventricular failure develops there will be oedema & abdominal pain from hepatic congestion
Chest pain
Loud pulmonary second sound
Ankle swelling
Right parasternal heave caused by right ventricular hypertrophy
Exertional dyspnoea, lethargy and fatigue are the initial features due to an inability to increase cardiac output with exercise
In advanced disease there are features of right heart failure (cor pulmonate)
Elevated jugular venous pressure
Prominent V wave if tricuspid regurgitation present
Hepatomegaly
Pulsatile liver
Peripheral oedema
Ascites
Pleural effusion
Differential Diagnosis
Cor pulmonale, cardiomyopathies, primary right ventricular heart failure, congestive cardiac failure, portal hypertension
Diagnosis
Echocardiogram
Right ventricular dilation and/or hypertrophy
Can show cause of pulmonary hypertension
LFTs to detect portal hypertension
ECG
Right ventricular hypertrophy and P pulmonale (tall & peaked P wave in)
Autoimmune screening
CXR
Enlarged heart
May also reveal cause of pulmonary hypertension e.g. emphysema or calcified mitral valve
Enlarged proximal pulmonary arteries which taper distally
Treatment
Oral calcium channel blockers as pulmonary vasodilators
Oral endothelia receptor antagonist e.g. bosenten
Diuretics for oedema
Phosphodiesterase-5 inhibitors
Warfarin - due to intrapulmonary thrombosis
Prostanoid (mediators of vasoconstrictor) analogues
Oxygen
Consider heart-lung transplant in young patients
Treat underlying cause