Diseases that may lead to the development of PH

[Andrew Strunk]

I have a very rare form of PH it is called Porto-Pulmonary Hypertension. In 1998 I had a blockage in gallbladder which was not identified for several days and eventually caused Portal Vein Thrombosis or in laymans terms total blockage of the Portal Vein. Because my portal vein is blocked it causes an enlarged spleen and gastric varices both of which I have. As the pressure in the portal vein builds it causes “Portal Hypertension” and over time this back pressure due to the limited blood flow from the stomach to the liver etc resulted in me developing PH and being diagnosed with Porto Pulmonary Hypertension in August 2008. I have only ever had one Cardiologist whom I can not speak highly enough of and I respect greatly however I would be interested to know if there are any Specialist doctors over in the States as I am in Australia that specialise in treating my condition.

[Brittany Foster]

Andrew,
Thank you for sharing more about your diagnosis. I have never heard of this before but it sounds like you would benefit from seeing a pulmonary hypertension specialist. I am from the US and see doctors that specialize in this. My PH is also caused by a rare condition called central hypoventilation syndrome which basically means that for what ever reason my brain does not make the connection with my lungs to breathe. I know my doctors have talked to me about going to something like the national center for rare diseases or specialists that specialize in rare diseases. They have told me that it is a complicated case because both conditions I have a considered rare and are not studied as much which creates it’s own obstacles in getting treatment. I would suggest seeing a specialist in PH and researching doctor who specializes in rare diseases, specifically if there are any that work with the condition you have. All the best !