Congetive Heart Defect - May 08

The Visit: As part of the Compass III research, I would repeat the following tests; MRI, Echocardiogram and a Right Heart Catheterization. These tests would determine whether Dr. Torres would add the second drug (Sildenefil) to my therapy. I was eager for this visit. Since being diagnosed with PH and having been on Bosantan for 4 months now, I was feeling good and hopeful that I would feel even better if an additional medication was added to my therapy.

The Goal: To add Sildenefil to my therapy.

The Process: After completing the MRI and echocardiogram I still awaited the right heart catheterization. Dr. Warner would perform the catheterization. He would measure my pulmonary pressures as required by the Compass III but also expressed interest in doing further exploring. He performed a right and left catheterization. During the procedure the Dr. Warner indicated that he had new information about my diagnosis but I did not understand the details of the findings. The procedure would be a little longer than intended but that was fine with me as this was very positive news.

The Outcome: Even better outcome than we had anticipated . There was now a cause for the Pulmonary Hypertension. A congenital heart defect. Because I no longer had Primary Pulmonary Arterial Hypertension but rather Secondary Pulmonary Hypertension, there was now hope to cure the Pulmonary Hypertension. It would be (3-4) weeks before we'd find out if surgery, to fix the defect, was a possibility. Dr. Torres would present my case to a team of Dr.'s and collectively they would determine my future. As Chris said at that time (and continues to say) we were "cautiously optimistic" that the Dr.'s would consider performing surgery to correct the defect and hopefully by correcting the defect this would also cure the PH. After the month long wait we received confirmation that surgery was an option.

Because of the change in diagnosis I would no longer be part of the Compass III research. I no longer was considered a PRIMARY Pulmonary Hypertensionon patient.

With surgery on the horizon came other therapies and goals. I would begin Flolan, a continuos IV medication, to reduce my Pulmonary Vascular Resistant (PVR) pressures. Reason being, my PVR's were at 10 and the Surgeon preferred to do surgery when those pressures were in between the 6-8 range. Therefore, it was to dangerous to perform surgery now.


New Diagnosis: Congenital Heart Defect; Pulmonary Vein Anomaly with an Atrial Septal Defect.

First off, if you are like me and are an obsessive GOOGLE R don't expect to find a lot of information about a Pulmonary Vein Anomaly. This is a rare condition in Adults. I have found a little bit of information about the Anomaly but not specific to my condition. My mother has found one article and she's a CNP, who has access to this data.