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Pulmonary Hypertension

Pulmonary hypertension is a rare disorder in which the arteries of the lungs become narrowed and thickened, making it difficult for blood to flow through the vessels. As a result, the blood pressure in the pulmonary arteries increases. Over time, the right side of the heart has difficulty pumping blood against the high pressure and resistance, and this leads to right heart strain, dilation, and then right heart failure.

Many can conditions cause pulmonary hypertension, which affects individuals of all ages, races, and ethnic backgrounds.  Idiopathic pulmonary hypertension, for which the cause is unknown, is more common in young adults and is approximately twice as common in women as in men.  One third of patients with left heart failure have pulmonary hypertension. 

Symptoms of pulmonary hypertension are usually only evident when the disease is advanced and the right side of the heart begins to fail.  The first symptom of pulmonary hypertension is usually shortness of breath with everyday activities.  In very advanced cases, some people experience fatigue, dizziness, and fainting spells.  For patients with right heart failure, symptoms include ankle and leg swelling, increased abdominal girth, and even chest pain.  Every patient feels the effects of pulmonary hypertension differently, especially as there is a broad spectrum of severity of illness.

Since there are many different causes of pulmonary hypertension, it is critical before starting treatment to establish the correct diagnosis and to understand the contributing conditions.  Secondary causes of pulmonary hypertension include: portopulmonary hypertension from cirrhosis, lung disease (including pulmonary fibrosis, COPD/emphysema, obstructive sleep apnea), mixed connective tissue disease (such as lupus, scleroderma), chronic thromboembolic disease (blood clots going to the lung, or “pulmonary embolisms”), congenital heart disease (such as ASDs and VSDs),  left heart failure (from valvular disease, dilated cardiomyopathy, ischemic heart disease, systolic or diastolic left ventricular dysfunction), high altitude living, drug toxicity (such as the diet drug “fen-phen”), HIV, sickle cell disease, end-stage renal disease, and genetic conditions (such as the BMPR2 mutation).   After an extensive evaluation for secondary causes, if none are found, then patients are considered to have idiopathic (formerly called “primary”) pulmonary hypertension. 

Westchester Heart and Vascular offers a multidisciplinary, comprehensive pulmonary hypertension evaluation and then tailors treatment to each patient depending on their diagnosis and severity of their illness.The specialists of the Pulmonary Hypertension Program will coordinate a patient’s diagnostic evaluation, which involves a complete history and physical examination, laboratory tests, specialized echocardiography, and often other tests including right heart catheterization and imaging. All forms of treatment are available for pulmonary arterial hypertension, including intravenous and subcutaneous infusion therapy. Westchester also treats patients with chronic thromboembolic pulmonary hypertension (CTEPH) and has a highly successful surgical experience performing pulmonary thromboendartectomies.

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As pulmonary hypertension may also be caused by many different conditions, the first step prior to initiating treatment is establishing the correct diagnosis and understanding the contributing conditions that have lead to the increased pulmonary pressure. 

Westchester Heart and Vascular’s comprehensive pulmonary hypertension evaluation begins with a complete medical history and physical exam.  Depending on each person’s history, a series of several tests will be performed and obtained, including: blood tests, ECG, Doppler echocardiogram, a functional assessment with a 6 minute walk test or cardiopulmonary exercise test, pulmonary function test (PFT), polysomnogram (sleep study), chest x-ray or high resolution CT of the chest, lung ventilation and perfusion nuclear scan (VQ scan), and right heart catheterization with a vasodilator study using inhaled nitric oxide.  Of course, not every person needs every study listed above.  We make every effort to only pursue the tests necessary to exclude secondary causes of pulmonary hypertension and to help further guide treatment.

After the initial evaluation is completed, treatment is initiated according to the etiology of the pulmonary hypertension and severity of illness.  In patients with volume overload and swelling from right heart failure, dietary sodium restriction and diuretics (water pills) are often used.  Most therapies are done to improve daily functioning and to decrease shortness of breath and fatigue.  For most people, initial treatment is with an oral medication such as sildenafil or a calcium channel blocker.  If symptoms are very significant, then two or more medications are necessary, such as an additional oral pill or inhaled, subcutaneous, or intravenous infusion treatments. 

Our center is equipped with the support staff and specialty pharmacies required to initiate the following pulmonary hypertension medications: sildenafil (Revatio®), tadalafil (Adcirca®)  bosentan (Tracleer®), ambrisentan (Letairis®), epoprostenol (Flolan®), treprostinil sodium (Remodulin®, Tyvaso®), and iloprost (Ventavis®).  Most patients with advanced pulmonary hypertension are given anticoagulation with warfarin.  Supplemental oxygen is also very helpful, especially for patients with pulmonary hypertension secondary to intrinsic lung disease. Serial 6-minute walk tests, cardiopulmonary exercise tests and right heart catheterizations are performed to follow the effect of treatment. 

Perhaps most importantly, Westchester Heart and Vascular has the dedicated medical staff and trained specialists necessary to coordinate the frequent outpatient follow-up visits, tests, monitoring, and drug titration that is essential in providing excellent pulmonary hypertension care.

To learn more about the services provided by Westchester Heart and Vascular please contact us at:

866-WMC-HEART (866.962.4327).