Who Are We
What is PH?
Pulmonary hypertension (PH) is a group of diseases characterized by high pulmonary artery pressures and pulmonary vascular resistance. Pulmonary hypertension may be idiopathic (primary), inherited or secondary to an identifiable underlying pulmonary, cardiac or systemic disease.
Idiopathic pulmonary arterial hypertension (IPAH), previously known as primary pulmonary hypertension, is a progressive disease that affects predominantly young to middle aged individuals, male or female and traditionally had an average survival rate of several months to a few 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.
Disorders associated with PH evaluated at our Pulmonary Vascular Disease Program include:
- Pulmonary Arterial Hypertension link (Idiopathic, Inherited)
- Connective Tissue Disorders (Scleroderma, Lupus, Rheumatoid Arthritis, Sjogren’s Disease)
- Heart Disease (Congenital and Diastolic Dysfunction)
- Liver Disease (Portal Hypertension)
- HIV infection
- Hemolytic Anemia (Sickle Cell Disease, Thalassemia)
- Drugs and Toxins (Diet pills, Amphetamines, Methamphetamines, Cocaine)
- Interstitial lung disease
- Sleep apnea
- Pulmonary embolism
Your initial evaluation
Before your first visit with us, all relevant medical records should be faxed to our office. Please bring to the visit or mail ahead, copies of your most recent chest x-rays, CAT scans of the chest and ventilation-perfusion lung scan, if already performed. The imaging center where performed will be happy to make a copy of the images on a CD upon your request. At the time of your visit, additional tests may be ordered. And depending upon the results, a right heart catheterization might be recommended. Treatment plans will be developed based upon confirmation of the diagnosis and the preferences of the patient and family. Family members are often important to help develop treatment plans, so we encourage all patients to bring a close family member to the visit.
Diagnostic testing available as part of the evaluation at Jefferson:
- Pulmonary function testing (PFTs)
- 6 minute walk test
- Ventilation-Perfusion (V/Q) lung scan
- CT angiogram of chest
- Sleep Study (Polysomnography)
- Echocardiogram (may include saline bubble contrast study)
- Right heart catheterization link with acute pulmonary vasodilator trial using inhaled Nitric Oxide.
Treatments available at Jefferson
- Prostacyclins: This most potent class of PH medications, may be delivered continuously IV or subcutaneously via a pump worn at home (Epoprostenol, Treprostinil) or by an inhaled preparation (Iloprost, Treprostinil) every few hours while awake.
- Endothelin receptor antagonists (Bosentan, Ambrisentan Macitentan): Oral therapies taken once or twice daily.
- Phosphodiesterase-5 inhibitors (Sildenafil, Tadalafil): Oral therapies taken one-three times daily requiring avoidance of the nitrate class of medications.
- Soluble guanylase cyclase stimulator (Riociguat): oral therapy FDA-approved for PH due to prior pulmonary thromboembolism.
- Diuretics (Furosemide, Spironolactone): Oral therapies to reduce remove excess water from the pulmonary circulation.
- Anticoagulation (Warfarin Factor X): inhibitors Blood thinners.
- Support Services:
- Medications for PH may be very expensive. Our nurses and medical assistants work very closely with the specialty pharmacies to find our patients financial support, when necessary.