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Pulmonary Hypertension

Primary pulmonary hypertension occurs in familial and sporadic forms and has been linked to the use of certain diet pills. Secondary pulmonary hypertension occurs as a result of many disorders including COPD, chronic hypoventilation, sleep apnea, left ventricle dysfunction, mitral stenosis, small multiple pulmonary emboli, and congenital cardiac shunts. Women represent the majority of cases of primary pulmonary hypertension. Symptoms tend to be present for some time, up to 1 to 3 years before diagnosis.


  • Most common cause of pulmonary hypertension is left heart failure leading to pulmonary venous hypertension. This may be due to systolic or diastolic malfunction of the left ventricle or due to valvular dysfunction includes mitral regurgitation or mitral stenosis.
  • Common causes of pulmonary arterial hypertension (PAH) include HIV, scleroderma and other autoimmune disorders, cirrhosis and portal hypertension, sickle cell disease, congenital heart disease, thyroid diseases.
  • Other factor that may cause Pulmonary Hypertension include sarcoidosis, histiocytosis X, and fibrosing mediastinitis.


  • Dyspnea +++ is the most common presenting symptom and occurs in all patients as the disease progresses.
  • Syncope or near-syncope +++, particularly with exertion
  • Fatigue ++
  • Chest pain ++
  • Raynaud's phenomenon +


  • Cardiac auscultation: loud P2; may have a systolic ejection murmur, S3 or S4
  • Signs of right ventricular failure (late)


  • Treatment is determined by whether the PH is arterial, venous, hypoxic, thromboembolic, or miscellaneous. Since pulmonary venous hypertension is synonymous with congestive heart failure, the treatment for pulmonary venous hypertension perform by the use of diuretics, beta blockers, ACE inhibitors, etc., or to repair the mitral valve.
  • Another treatment for pulmonary venous hypertension is Anticoagulants Therapy, In which the patients are used regularly with primary PAH because they help reduce symptoms and may provide a survival benefit. Nonetheless, anticoagulation with warfarin is indicated in patients with chronic pulmonary emboli, pulmonary venoocclusive disease, and atrial fibrillation induced by left or right heart failure. Long-term anticoagulation therapy should be considered in patients who are at high risk for developing venous thromboembolism.

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