My HOCM Adventure
Thanks to my big “sister” Sheryl I began playing tennis at the age of 8 or 9. A tennis player herself, she took me to the courts adjacent to Carlsbad High School and I began to bat the ball around with her using my Wilson Jack Kramer Autograph wood racquet graciously provided by my Uncle Mark.
I played only recreationally in the 20 or so years that followed, then dropped it altogether after injuring my right shoulder in 1993. After moving to the Washington DC Metro area, I picked up tennis again in 2007 thanks to my friend Darron, who encouraged me to purchase a racquet and hit with him. Playing upwards of two to three hours at a time without issue, I progressed to the point where I played in my first competitive tournament at the age of 46 in July 2008.
By that time my body was already breaking down physically because of the demands I was putting on it because of tennis. Unbeknownst to me, I had torn the labrum in my left hip a couple months earlier. Following surgery and 12 weeks of rehab, I was back on the court by the end of the year. As I was finishing up rehab, I began the first of 9 consecutive years of personal training to strengthen my body and satisfy tennis’ demands on it.
To further improve my game, I began private lessons in Rockville in August 2011. On an unusually mild 70° day, however, I felt short of breath during the warm-up. My instructor and I were both confused because it wasn’t hot and I really hadn’t been exerting myself to any degree. Before the end of the Summer, I’d been connected with a nutritionist who identified sodium intake as a substantive problem affecting my blood pressure and perhaps my performance on the tennis court. As a result, changes in my diet resulted in lowering my daily sodium intake from 6,000+ mg to 2,200 mg and losing 18 pounds over a three-month period.
However, that was not the entirety of the issue. In October, I began a series of cardiac exams that showed increased thickness in the basal septal wall due to hypertrophic obstructive cardiomyopathy (HOCM). The first measurement was 13mm, (12 mm or less is considered normal) and I was immediately prescribed diltiazem, which, according to my local cardiologist, would buy me about 10 points in my blood pressure. An electrocardiogram showed a wider than normal QRS complex, which continues to this day.
I’d had a heart murmur for years; my best recollection was an Air Force flight surgeon telling me so when I was in my late 20s. Flight medicine being what it was -- with its quack doctors, horror stories, and all -- one flight surgeon after another chalked it up to “nothing” and I pressed on.
By the Fall of 2012, I was playing my best tennis, winning almost twice as many matches as I was losing, and consequently had my highest career ratings in singles and doubles. However, success was muted slightly by an August echocardiogram that showed a septal wall thickness of 14mm and further subdued by the tearing of my right lateral meniscus in November that led to a seven-match losing streak and a large dose of frustration.
In the Summer of 2014, I switched leagues and joined a USTA-affiliated team. By the Fall, I’d been asked to captain the team and by the Winter I was taking weekly clinics run by the former team captain. A December echocardiogram showed a septal wall thickness of 16mm. While issues with shortness of breath had appeared to subside over the previous three years, they came back roaring like a lion at the first of the year. I found myself doubled over, trying to catch my breath, during weekly indoor warm-up drills. Again, each instance wasn’t like I’d been exerting myself for hours in oppressive heat and humidity prior to experiencing shortness of breath.
Continuing the drill of diltiazem and a low-sodium diet had kept my blood pressure within normal parameters and my weight steady at around 206 lbs on my 6’2½” frame for better than three years. But this regimen had little effect on the continuing problem of feeling short of breath. It was odd. Most of the incidents occurred at the beginning of a tennis clinic, never in the middle or at the end. None of the incidents occurred outside in searing heat and humidity with on-court temperatures near 130°. Nevertheless, I had been short of breath while on various bicycle rides of up to 30 miles around the local area but these episodes usually occurred at or near the beginning of the ride.
The wearing of a Holter monitor and another echocardiogram (showing a septal wall thickness of 19mm) in June 2016 demonstrated the need for on-going attention, if not concern, for the left ventricular hypertrophy of my heart. My dynamic subvalvular left ventricular outflow tract (LVOT) gradient was worsening and was especially violent after rising from a squatting position. A follow-up six months later led my local cardiologist to conclude that only two more options remained -- a cardiac MRI and a septal myectomy.
In a twist of irony, a re-injury of my right shoulder and tendonitis in my left elbow about the same time put me on my team’s disabled list for about 7½ months. In hindsight, this was fortuitous because my local cardiologist, a highly experienced and highly respected man nationally, was unaware that tennis is the #2 sport for players experiencing sudden cardiac death (SCD) due to HCM. While it has been shown that moderate exercise may benefit HCM patients, the reality was that he didn’t recommend stopping high intensity or competitive athletics such as tennis.
After finally returning to the courts in early March, I was free of injury and not suffering from any bouts of shortness of breath. After raising my level of play the next three months, I decided to enter a tennis tournament over the July 4th weekend in College Park. With temps in the mid-90s and heat indices nearing 105°, the conditions for singles and doubles were brutal at best. After being on court three days out of four, I concluded doubles play with my hitting partner, Mark, around 1 p.m. on July 4 without any heart or heat-related issues, then showered, relaxed, went out to dinner, and climbed into bed before the news.
By 12:30 a.m., it was an entirely different story. I felt completely overheated and got up because I thought I’d forgotten to turn on the air conditioning. After lying down again, my heart began to palpitate and there was wheezing and gurgling in my chest that I imagined could be heard from the next room. By 1:15 a.m. I was on my way to the ER with the help of my spouse.
There, tests revealed an increased level (0.4) of troponin proteins in the blood, indicating damage to the heart muscle. A chest x-ray showed minor damage to the heart, a result of years of the heart working exceptionally hard to push blood from the upper left ventricle to the body. The cardiac MRI was negative, meaning that I had no plaque in my heart, a condition that the leading cardiologist in the country described as “the envy of every 55-year-old male in the U.S.” However, the echocardiogram technician read the septal wall thickness as 23mm, a 20 percent increase over a year ago.
Three weeks later at a follow-up visit, my local cardiologist presented me with the inevitable conclusion -- a septal myectomy was in my near future. On his recommendation, I decided to proceed in earnest, rather than wait until after the completion of several Fall vacations. By the following Monday, I was already on the calendar for tests, consults, and surgery at the #1 hospital in the country the last three years.
By the end of August, I had completed another chest x-ray and echocardiogram (with a correct assessment of the septal wall thickness (21 mm), received a recommendation for automatic implantable cardioverter-defibrillator (AICD) surgery from a leading cardiologist in the country, and been admitted to the hospital for a successful extended septal myectomy by one of the most experienced surgeons in the country. The myectomy effectively reduced my septal wall thickness to approximately 10-11 cm. After seven days total in the hospital -- prolonged two days because of the Labor Day weekend -- I was released.
My stay was not without complication, however. Antibiotics used during my open heart surgery -- likely, cefuroxime – disrupted enough bacteria in the colon to cause Clostridium difficile, which led to a high fever (up to 103.3°), diarrhea (16 instances in a 26-hour period), and dehydration. C diff ultimately caused the delay in my AICD surgery because 10 days of antibiotics (metronidazole) were necessary to treat the colitis. Treatment began no less than 36 hours after retrieval of a stool sample because of the seemingly inordinate amount of time to complete the C diff assessment. While taking metronidazole, I experienced occasional mild nausea and a near-constant bitter taste in my mouth. Upon completion of the antibiotic regimen, stools never did return to normal and, approximately two weeks later, chronic diarrhea returned (10 instances in a 34-hour period at its worst) and I subsequently returned to my PCM for a 7-day antibiotic (vancomycin) regimen to determine if there was a relapse of C diff colitis.
Regarding the post-surgical effects of septal myectomy:
- I had considerable pain in the sternal region for about three weeks, which I treated with prescription medication (500 mg Tylenol). Upon my release from the hospital’s step-down unit, I was taking between 2,000 and 2,500 mg Tylenol daily. 28 days after my release, I stopped taking Tylenol for pain.
- I did not sleep through the night uninterrupted until five days after I was released. (My clinic’s heart-shaped pillow came in handy for turning on my side, which didn’t occur until about two weeks post-surgery, and getting in and out of bed.)
- After losing 7 lbs during my hospital stay -- during which time I was on a clear liquid diet -- my appetite returned (and finally had hunger pains) about eight days after surgery.
- My post-surgical resting heart rate (bpm) continues to be in the mid- to upper-90s. While no professional would offer any firm timeline, experiential data indicates that a return to a normal (pre-surgical) pulse (60 to 70 bpm) may occur about six months after surgery.
- My heart murmur has been eliminated but Unipolar chest leads V1, V2, V3 (posterior anterior) show taller (more elevated) S waves than the pre-surgical S waves, indicating a right bundle branch block.
- While I anticipated (hoped) that there would no longer be a need to take diltiazem after surgery, such is not the case. In fact, the surgeon upped the daily dosage from 180 mg to 240 mg. After informing my local cardiologist eight days after release that I had several dizzy spells and bouts of lightheadedness, he recommended returning to the pre-surgical dosage, after which there were no further incidents.
- I had no infection from the median sternotomy. Chest drain tube holes scabs fell off approximately three weeks after surgery.
- Even after a month post-surgery, my physical energy was not at its pre-surgical level.
- For the first month, food did not taste the same post-surgery. There was a general “flatness” to the taste of most foods.
Because my father experienced sudden cardiac death (SCD) (almost 17½ years following quadruple bypass surgery) and I subsequently inherited a mutation in one of the seven HCM genes from him, the leading cardiologist in the country concluded that I was at higher risk (3 to 4%) for SCD and recommended AICD surgery. Of particular concern to him was my desire to participate in bicycling and competitive USTA league tennis for the foreseeable future. Notable athletes experiencing SCD while on court include Boston Celtics basketball player Reggie Lewis (1993) and Loyola Marymount basketball player Hank Gathers (1990)
I completed AICD surgery at the #1 hospital in the country exactly 13 weeks from the date of my extended septal myectomy. The technician who tested the device the day after surgery said it would activate if my heart rate fell below 40 bpm or elevated above 200 bpm. He also said that the device begins recording cardiac activity at or above 171 bpm. Following an overnight stay, I was released from the hospital about 24 hours after surgery. Once home, I followed the procedures required to set up a wireless device, given to me at the hospital, that must always sit on the nightstand next to my bed. I then called back to the hospital to have AICD monitoring transferred from the hospital to my local heart clinic.
Following a similar septal myectomy post-surgical script, I was restricted from driving and vigorous activity for a month. I was also not allowed to raise my arm above my shoulder, lift anything heavier than 5 lbs, or push/pull with any kind of exertion during the same period.
My recovery time from implantation was a little over three weeks (whereas my cardiologist estimated recovery time to be four to six weeks). I stopped taking pain medication altogether about two weeks after surgery.
Five weeks after surgery, I visited my electrocardiologist who adjusted downward the AICD voltage from 2½ volts to ½ volt. This serves two purposes – to extend the battery life (estimated to be 12 years if the device is not activated) and to provide the minimum voltage necessary to shock the heart into normal sinus rhythm. While there, I learned that I’m required to schedule recurring visits with the electrocardiologist every six months. (This is in addition to the annual visits with my cardiologist.)
Most importantly, with an insurance policy now embedded just below my left clavicle, I’ve returned to the tennis court – my second home.