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.

Comments

Hereditory spherocytosis

• This is the abnormality of red blood cells. • This is usually inherited as an AUTOSOMAL DOMINANT condition. • The most common abnormalities are deficiencies of BETA SPECTRIN or ANKYRIN. • Most cases are associated with an asymptomatic compensated chronic haemolytic state with spherocutes present on the blood film, a reticulocytosis and mild hyperbilirubinaemia. • Pigment GALLSTONES are present in up to 50% of patients and may cause chlolecystitis. The clinical course may be complicated by crisis : • A HAEMOLYTIC CRISIS occurs when the severity of haemolysis increases. • A MEGALOBLASTIC CRISIS follows the development of folate deficiency. • An APLASTIC CRISIS occurs in association with PARVOVIRUS B19 infection. Investigations • The blood film will show spherocytes but the direct Coombs test is negative, excluding immune haemolysis. • An OSMOTIC FRAGILITY TEST may show increased sensitivity to lysis in hypotonic saline solutions but is limited by lack of sensitivity and specificity. • ...

PLEURAL EFFUSION

Pleural Effusion: Causes, Clinical features, Investigations And Management Pleural effusion is the accumulation of serous fluid in the pleural cavity. Mechanism of Pleural Fluid Formation Elevation of venous pressure (rare in pure Right Ventricular failure) Decreased plasma oncotic pressure (except in congenital hypoalbuminuaemia) Increased capillary permiability due to local inflammation, toxins or vasoactive substances as occurs in collagen-vascular diseases, pancreatitis, pulmonary emboli and pneumonitis Increase in pleural space oncotic pressure as a result of : Protein leak through capillaries, Protein exudation due to local pleural inflammation, Defective lymphatic absorption. Simple transfer of ascitic fluid across diaphragmatic defect and also through transdiaphragmatic lymphatics as occurs in cirrhosis and Meig's syndrome. Increased negativity of pressure in the pleural also results in pleural effusion as occurs in atelectasis. Obstruction of lymphatics. Pleural fluid, o...

Rheumatoid arthritis (RA)

Rheumatoid arthritis is a chronic inflammatory, destructive and deforming symmetrical polyarthritis associated with systemic involvement. The individuals with HLA-D4 and HLA-DR4 are more prone to RA. It starts when your immune system, which is supposed to protect you, goes awry and begins to attack your body’s own tissues. It causes inflammation in the lining of your joints (the synovium). As a result, your joints may get red, warm, swollen, and painful. Symptoms of Rheumatoid Arthritis Joint pain and swelling Stiffness, especially in the morning or after you sit for a long time Fatigue Who Gets Rheumatoid Arthritis? Anyone can get RA. It affects about 1% of Americans. The disease is 2 to 3 times more common in women than in men, but men tend to have more severe symptoms. It usually starts in middle age. But young children and the elderly also can get it. Pathogenesis Synovitis (synovial cell hyperplasia, hypertrophy with CD4 lymphocytic infiltration and synovi...

MBBS question bank

Search This Blog