I recently attended an advisory committee meeting near Washington, DC about our current understanding of a disease called pulmonary arterial hypertension (PAH). This is a disease that I have mentioned before in these articles. New medications and treatment plans have allowed us to focus on this disease process when evaluating someone with unexplained shortness of breath. Unfortunately, doctors still are unclear about how this disease progresses with time depending on the cause. The current classification does not always fit and the tests needed to help follow patients are often invasive.
I have been trying to get the local medical community to recognize this disease process when a patient is being worked up for shortness of breath. However, the data continues to show that the time from symptoms to diagnosis is about 3 years.
PAH is a disease of the blood vessels of the lungs. The blood vessels narrow because of some underlying cause and there are some patients who get this because of genetic issues. This narrowing of the blood vessels creates an elevated pressure that the right side of the heart, which is the smaller and weaker side, must pump against. Eventually the heart fails and this is a dire consequence of the disease. The treatments are designed to reduce this pressure and preserve right heart function.
Most patients need more than one type of specialist to help with the diagnosis and treatment. My hope is that, locally, we can continue to expand our knowledge and services.
