What is Pulmonary Hypertension?
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Pulmonary hypertension (PH) is a group of diseases characterized by high pulmonary artery pressures and pulmonary vascular resistance. Pulmonary hypertension can be idiopathic (primary) or secondary to an identifiable underlying pulmonary, cardiac or systemic disease.
Idiopathic pulmonary arterial hypertension (IPAH), previously known as primary pulmonary hypertension, is unfortunately a progressive disease that affects predominantly young adults, is more common in females, and untreated has an average survival rate of two to three years from the time of diagnosis.
Thanks to the availability of targeted or advanced therapies for pulmonary hypertension, this survival rate has improved in recent years.
Types of Pulmonary Hypertension
Types of Pulmonary Hypertension
- PH originating at the pulmonary arteries (i.e., pulmonary arterial hypertension or PAH)
- PH originating in the heart (i.e., pulmonary venous hypertension or PVH)
- PH originating in the lung tissue or due to poor oxygenation
- PH due to clots in the pulmonary arteries (i.e., chronic thromboembolic pulmonary hypertension or CTEPH)
Causes of Pulmonary Hypertension
Causes of Pulmonary Hypertension
Pulmonary hypertension is associated with several conditions including:
- Unknown causes (i.e., idiopathic)
- Familial pulmonary hypertension (several genetic abnormalities identified)
- Scleroderma, lupus or other inflammatory diseases
- Heart, lung, liver, kidney and blood diseases
- Blood clots - this condition is called chronic thromboembolic pulmonary hypertension (CTEPH)
- And many others
Treatment
Treatment
At the Cottage Pulmonary Hypertension Care Center, our patients are on various advanced pulmonary hypertension therapies. These include:
- Intravenous epoprostenol (Flolan and Veletri)
- Intravenous, subcutaneous and inhaled treprostinil (Remodulin and Tyvaso)
- Oral bosentan, ambrisentan and macitentan
- Oral sildenafil, tadalafil and riociguat
- Oxygen therapy as needed
- Diuretic therapy
- Cardiac arrythmia therapies
- Exercise and nutritional programs
- Newer treatments and experimental therapies under research investigation
Support Services
Support Services
Our Registered Nurse coordinator specializes in navigating issues that patients with PH face. We also provide other support services with an emphasis on nutrition and exercise counseling.
Many of our patients partake in our local patient support group. Standard medical therapies for patients suffering with lung diseases such as oxygen, diuretics and anticoagulation may also be incorporated into your care.
