Brief Answer: please see details. Detailed Answer: Dear Sir 1. PAH or pulmonary artery hypertension, is not the final diagnosis. It has many reasons. Mainly Mitral valve disease, holes in heart, lung diseases, diseases of left side of heart etc. Although, usually, when we say patient is suffering from PAH, we mean that all these causes have been ruled out and patient has idiopathic (without any recognizable reason) PAH. 2. For treatment of all those patients who have a recognized cause for PAH, basic cause is treated in most of case scenarios. Following paragraphs discuss management of idiopathic or primary PAH. 3. These patients should be managed by experts who have special interest in managing patients of PAH. They may be cardiologists or pulmonary medicine specialist. There is no cure for idiopathic or primary PAH. Basic management is to treat symptoms of patient and delay development of bad effects on heart. These involve a number of medications like prostaglandins, endothelin receptor antagonist like bosentan, PDE receptor antagonist like sildenafil/ tadalafil, anticoagulants, oxygen. Diuretics and digoxin is used to treat associated heart failure. Your partner is receiving bosentan and tadalafil. 4. Calcium channel blockers may also be helpful if reactivity test suggests so. 5. In advance cases, surgeries like atrial septostomy , lung transplant or heart lung transplant can be done. 6. Beside, she must avoid heavy exertion and all kind of infections especially chest infection. She should not be exposed to dust and dirty, smoky environment. Hope this gives you some insight. Feel free to discuss further. Sincerely Sukhvinder

---

Brief Answer: please see details Detailed Answer: Dear Sir 1. A number of tests are performed before labeling the condition as idiopathic or primary pulmonary hypertension. They are governed by the clinical possibilities. These include Chest x-ray, ECG, pulmonary function tests, ECHO, contrast ECHO, exercise testing, Cardiac catheterization, CT scan of chest, screening for collagen vascular disorder, testing for suspicious drugs or toxins. Some of these are done in all patients and some of these are guided by clinical evaluation. 2. Life expectancy depends upon the cause and symptomatic status of patient. Those who are severely symptomatic do worse as compared to nearly asymptomatic patients. In a single given patient none can exactly tell the prognosis, as data is applicable to a group of patients. No definite cure exists for idiopathic PAH except for heart-lung transplant, which has its own problems. 3. These two drugs belong to the most commonly used class of drugs in primary pulmonary hypertension. I hope your physician has ruled out all possible etiologies for PAH and has confirmed the diagnosis of idiopathic PAH. I have named other drugs in my last answer and can be used depending upon the symptoms and patients response. An anticoagulant is usually added to the therapy in most individuals. You can discuss it out with your doctor. 4. These surgeries are usually reserved for those patients who have severe symptoms despite maximal drug therapy. Your treating physician will guide you whenever need for surgery arise. 5. The doctor who is treating the congested chest must be aware of all medications you are taking and no medication should be taken without informing and consulting your doctor. Some of the medicines may interact with other medicines adversely, especially the anti-coagulants. 6. Chest heaviness may have many reasons in given case. It can be due to current congestion, drug effect or related to the feeling of breathlessness due to PAH. It requires clinical examination to ascertain exact cause. Sincerely Sukhvinder

---