Chronic thromboembolic pulmonary hypertension

Posted by e-Medical PPT Sunday, September 18, 2011
Epidemiology and PathophysiologyPresent late in the course of the disease.
Early natural history of the condition is not completely known.
Can occur without symptoms.
May remain asymptomatic for months or years.
May involve recurrent thromboembolism.
May involve in situ pulmonary-artery thrombosis.
Vascular remodeling.
Hypertensive pulmonary arteriopathy.
Inadequate anticoagulation.

Predisposing factors
Defective fibrinolytic systems
Presence of lupus-like anticoagulant
Deficiency of protein C, protein S, and antithrombin III
Malignancy
Atrial septal defects
Indwelling venous catheters

Selected etiologic conditions giving rise to pulmonary hypertension
1. Pulmonary Arterial hypertension
2. Pulmonary Venous hypertension
3. Pulmonary hypertension associated with disorders of the respiratory system and/or hypoxaemia
4. Pulmonary hypertension due to chronic thrombotic and/or    embolic disease
    4.1. Thromboembolic obstruction of proximal pulmonary arteries.
    4.2. Obstruction of distal pulmonary arteries
             a). Pulmonary embolism (thrombus, tumour, ova and/or parasites, foreign material)
              b). In-situ thrombosis
              c). Sickle cell disease
5.Pulmonary hypertension due to disorders directly affecting the pulmonary vasculature. 

Clinical Manifestation
Progressive exertional dyspnea
Exercise intolerance
Pulmonic component of the second heart sound
Chest pain on exertion
Presyncope
Syncope
Inability of a compromised right ventricle to meet the body’s demands for CO
Loud second heart sound,
Tricuspid regurgitation murmurs
Engorged liver and neck veins
Elevated jugular pressure with a positive hepatojugular reflex
The presence of peripheral oedema
Peripheral and central cyanosis
Prominent right ventricular impulse..

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