Pathophysiology: Patients with pulmonary hypertension develop changes in the small blood vessels of the lungs that can lead to increased blood pressure in the right side of the heart. This makes it harder for the heart to pump blood to the lungs. If the pressure continues , the strain on the right side of the heart leads to cor pulmonale.
Causes: include:
1. Lung diseases:
- Asthma ( severe and chronic)
- COPD
- Bronchiectasis
- Pulmonary fibrosis
- Lung resection
2. Pulmonary vascular disease:
- Pulmonary emboli
- Pulmonary vasculitis
- Primary pulmonary hypertension.
- Sickle cell disease
- Acute respiratory distress syndrome.
- Parasite infestation
3. Thoracic cage abnormality:
- Kyphosis
- Scoliosis
- Thoracoplasty
4. Neuromuscular disease:
- Myasthenia gravis
- Poliomyelitis
- Motor neuron disease
5. Hypoventilation:
- Sleep apnoea
- Enlarged adenoids in children.
Clinical Features:
Symptoms include:
- Breathlessness
- Fatigue
- Syncope (fainting)
Signs include:
- Cyanosis
- Tachycardia
- raised JVP with prominent a and v waves
- loud P2 pansystolic murmur (tricuspid regurgitation)
- early diastolic Graham Steell murmur.
- hepatomegaly
- edema
Investigations:
1. Complete blood count will show increased hematocrit and Hb (secondary polycythemia)
2. ABG will show hypoxia with or without hypercapnia
3. Chest X ray shows an enlarged right atrium and right ventricle, with prominent pulmonary arteries.
4. ECG: p pulmonale, right axis deviation, right ventricular hypertrophy/strain.
Management:
1. Treat the underlying cause.
2. In case of acute respiratory failure patients may need urgent care with ABG monitoring and giving oxygen accordingly.
3. Patient with COPD may require long term oxygen therapy (15 hrs/day) .
4. Treat cardiac failure with diuretics such as furosemide 40-160 mg/24hrs PO.
5. Monitor Urea and electrolytes and give amiloride or potassium supplements if needed,
Consider venesection if hematocrit is > 55%
6. Heart-lung transplantation is considered for younger patients.
Prognosis: is poor with 50% of patients dying within 5 years.
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