Strong Enough to “Skip a Beat”
On 11/1/2024, Maz passed away from a branch falling on his weak but strong heart. In the fall of last year, he was doing what most 21-year-old boys do, and I scolded him like an older sister would. Little did I know it would be my last words to him.
Maz was the first pediatric patient heart transplant at NYU Langone in 2021. At age 18, he had hypertrophic cardiomyopathy (HCM). HCM is the most common cause of death in young adults. It’s prevalent in 1 out of 500 people in the US, with up to 85% of individuals who often remain undiagnosed.
The heart is the hardest working and strongest organ in our body. It beats over 3 billion times in a person’s life and tirelessly and involuntarily pumps 2,500 gallons of blood continuously every day. The heart has four chambers, a left and right atrium, and a left and right ventricle. The right atrium pumped oxygen-poor blood from vena cava to the right ventricle. The right ventricle then pumped the blood to your lungs through pulmonary arteries to become oxygenated. The blood comes back into the heart through the pulmonary veins to the left atrium, where it is then transferred to the left ventricle. The left ventricle pumps the now oxygen-rich blood to the rest of your body. In HCM, the heart muscles, myocardium, thickens. A thick myocardium doesn’t make your heart stronger, it makes it weaker. Maz had a weak but strong heart.
One day, Maz was complaining of chest pain, shortness of breath, and heart palpitations. Maz’s heart was skipping a beat. Cardiologists quickly diagnosed him with HCM, going into heart failure, and needing a heart transplant as soon as possible. He quickly receivedr an orthotopic allotransplant. We always thought it was a blessing that he was able to find a heart replacement so quickly.
HCM is a genetic mutation that encodes sarcomeric proteins. It’s the proteins that are the basic contractile units in muscle fibers, made up of actin (thin filaments) and myosin (thick filaments). This can cause hyper contractions or impaired relaxations overproducing myosin heads and weakening the “closed” state of the proteins, destabilizing muscle formation, and alter regulatory proteins like myosin binding protein C. This ultimately results in force generating contractions which causes excessive thickening over time.
A genetic mutation that was formed during gestation caused him to slowly develop abnormal heart thickening and altered calcium handling in his heart muscles. I don’t know what exact genetic mutation he had, but two of the most common are on the MYH7 (beta myosin heavy chain 7) and the MYBPC3 (myosin binding protein C) gene. Those two genetic mutations essentially causes myocyte hypertrophy which is the overstimulation and stress on the heart. HCM thickens of the walls of the left ventricle, the main pumping chamber of the heart. Downstream, the heart becomes stiff and cannot fill chambers properly or pump enough blood to provide an adequate supply to the body.
Ironically, Maz’s heart wasn’t the reason for his passing. Over the years, we always worried about him—making sure he ate well and didn’t overexert himself, especially since he loved lifting. On November 1, 2024, he took his little sister on a hike, where a large branch fell on him. The force of the impact stopped his heart instantly.
At 2am, I drove his little sister back home. All I kept thinking about were my last words. While they came with good intent, they weren’t particularly kind. Maz may have had a weak heart physically; it was so kind that it was a strong. He would take our moms on long drives, singing in the car, taking their pictures without a single complaint, did fall activities with them, and was always patient. Since then, I took a page from his book. No last meeting was left with unkind words.
