Heart Failure to Transplant: Diagnoses

I posted the following on my CaringBridge journal in 2014:

In 2010 I saw my primary care doctor to investigate the irregular heart beats I had been feeling.  At first she wanted to brush it off as just anxiety, but decided to do an EKG just in case.  After reading the results of the EKG she was insistent that I see a cardiologist right away because the pattern of my heartbeats was consistent with left ventricular hypertrophy.  The cardiologist did an echocardiogram and confirmed the diagnosis of Hypertrophic Cardiomyopathy (HCM).

He told me it wasn't a big deal, just mostly annoying because of the palpitations, which I could control by avoiding caffeine and chocolate.  Oh, but in rare cases some HCM patients experienced Sudden Cardiac Death.  Mostly athletes, so just avoid strenuous activity and I should be fine.  Come back once or twice a year to make sure it wasn't getting worse, and that would be it.

Fast forward to 2014, and a few days after giving birth to my third baby, I was diagnosed with pneumonia from a chest x-ray.  Antiobiotics didn't make a difference, and a few days later, I went to the ER with extreme shortness of breath worse when lying down, new swelling in my lower legs, and one instance of coughing up blood.  They performed a chest CT and then admitted me, I thought for severe pneumonia. 

They treated me with IV antibiotics and Lasix, and performed an echocardiogram. As I neared readiness for discharge two days later, a doctor informed me that the primary reason for admission wasn't the pneumonia which was actually not that bad, but the pleural effusion related to heart failure.  I was sent home with Lasix to take and instructions for a low sodium fluid restricted diet until the remaining of my leg swelling dissipated.  Then I threw it away, because I was sure everything would go back to the way it was.  I stopped watching my sodium intake and limiting fluids as well.

I had an echocardiogram a month later and because of the results of that was referred to the Heart Failure and Transplantation clinic in Santa Clara.  My heart now had thickening on the right ventricle as well as the left, and the atria were dilated.  I had a few phone appointments and did some blood work remotely.  The doctor wrote me a new prescription for Lasix, which I kept forgetting to fill.  I understood that I should take the Lasix only if the leg swelling retuned, or if I gained weight rapidly.  I was supposed to weigh myself every day, but sometimes forgot.  Toward the end of the summer, the times I did weigh myself I noted the scale inching up a bit and my waistline getting a little thicker but figured I may have been slightly overeating or something.

Then at the beginning of September, the extreme fatigue and shortness of breath/discomfort when breathing returned.  I felt sure I had pneumonia again.  On the phone with the advice nurse, I was informed that my chart showed I have pulmonary hypertension (PH).  To my recollection, that was the first time I had been given that information, so I double checked with my PCP who confirmed that my most recent echo did show PH.  A chest x-ray ruled out pneumonia, so I was referred back to my local cardiologist, Dr. Z.

The cardiologist in Santa Clara who had been following my case had left Kaiser, but before he left he had mentioned wanting me to have an exercise tolerance test to see if I needed a implantable defibrillator or pacemaker.  I had been waiting for someone to call me to set the appointment and then had almost forgotten about it. 

Dr. Z got me in touch with another cardiologist at the Heart Failure and Transplantation clinic, Dr. Weisshaar.  She put the order in for the Treadmill EKG, and answered all my questions very clearly and directly. 

The PH is secondary to Stage C, Level II or III (depending on if I'm having a good day or bad day) Heart Failure, which is a result of my cardiomyopathy.  Because of my young age, I most likely will need a heart transplant one day, but hopefully not for a long time with proper lifestyle management.

I filled the prescription for Lasix, adopted a strict low sodium diet and began weighing myself faithfully first thing in the morning and last thing before bed.  I lost four pounds of water weight in the first two days and continued to lose a pound a day for the next few days.  My thickened waistline disappeared, and my breathing eased a bit. 

However, I'm still feeling worse now than I did over the summer.  I fatigue and get winded easily, and sometimes get lightheaded.  My resting heart rate is slow, between 57 and 60 beats per minute, and my blood pressure is quite low. 

I had the Treadmill EKG on September 24.  According to Dr. Weisshaar, the results mean I don't need an implantable defibrillator or pacemaker, but because my exercise capacity is so poor, they want to do an VO2/cardiopulmonary stress test to determine why. 

On October 13 I'll be driving to Santa Clara to ride on a stationary bicycle while hooked up to an EKG and breathing into a special tube.  Best case scenario, they discover my poor exercise tolerance is just because I'm deconditioned (fancy talk for just really really out of shape).  Worst case, they determine that I might need a transplant sooner than originally thought.

My case is a little tricky to treat with medication because of my slow heart rate and low blood pressure.  Most people in heart failure have rapid heart beats and high blood pressure, so most of the drugs approved to treat heart failure work by slowing the heart rate and lowering blood pressure.