Familial Primary Pulmonary Hypertension

Posted by Jeanette Smith on July 10, 1999 at 12:19:23
Please can you send me as much info on FPPH as we have this condition in our family. Thank you
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Posted by CCF CARDIO MD - CRC on July 12, 1999 at 10:10:10
Dear  Jeanette,
Thank you for your question. I don't have any specific information on familial pulmonary hypertension but here is some general information about pulmonary hypertension.  Pulmonary artery (PA) pressure is much like systemic blood pressure except that it is the blood pressure in the lungs.  PA pressure is a low pressure system with the normal pressure being about 25/12 mmHg with a mean pressure of 16 mmHg.  
There are many causes of high pulmonary pressures.  The most likely cause is long standing heart valve problems. Other potential causes are chronic blood clots (pulmonary thrombosis),  congenital heart disease, obstructive lung disease, collagen vascular disease, parasitic disease involving the lung, sickle cell anemia, IV drug abuse and chronic liver disease.  If no cause of the hypertension can be found the diagnosis of primary pulmonary hypertension is made.  
The treatment of pulmonary hypertension is difficult.  Vasodilator drugs such as calcium channel blockers can be used.  Nitric oxide lowers PA pressure but only lasts for a very short time.  Flolan (Prostacyclin) lasts for a longer time but must be given intravenously and is very expensive.   Anticoagulation is recommended for patients with blood clots.   For patients who fail to respond to medical therapy the only option is lung transplantion.
www.phassociation.org
If you would like to see someone at the Cleveland Clinic I would recommend Dr. Wiederman, the head of the department.  Additional information can be found at: http://www4.clevelandclinic.org/staff/GetStaff.Asp?StaffId=1019
I hope you find this information useful.  Information provided in the heart forum is for general purposes only.  Only your physician can provide specific diagnoses and therapies.  Please feel free to write back with additional questions.
If you would like to make an appointment at the Cleveland Clinic Heart Center, please call 1-800-CCF-CARE or inquire online by using the Heart Center website at www.ccf.org/heartcenter.  The Heart Center website contains a directory of the cardiology staff that can be used to select the physician best suited to address your cardiac problem.