Attorney Brad Tennis' busy day in Washington, D.C., started out normally with a trip on the Metro, but his afternoon commute quickly took a turn. It was August 2018, yet it wasn't the heat that suddenly made Tennis feel flushed and faint. He managed to exit the train at the next stop, but lost consciousness and awoke requiring medical care.
At a local emergency room, Tennis was discovered to have heart rhythm irregularities and an abnormal electrocardiogram. Two months, several ER visits, many fainting spells and medical tests later, Tennis was diagnosed with a rare heart condition called arrhythmogenic right ventricular cardiomyopathy, or ARVC.
ARVC is a genetic disease that weakens heart muscle cells mostly in the right ventricle, causing cells to die and be replaced with fatty or fibrous tissue that prevents the heart from beating correctly. The condition is chronic and often requires antiarrhythmic or beta blocker medications as well as surgical interventions.
Shortly after his diagnosis, Tennis began treatment and had an implantable cardioverter defibrillator placed in his heart, which could shock the heart back to a normal rhythm, preventing fainting and arrhythmias.
"Over time, I kept having more and more ventricular tachycardias and other heart events that my internal defibrillator had to correct," says Tennis. "We tried several surgeries to control these arrhythmias, but they weren't successful. The surgeries and medications began to exacerbate heart failure, so it was determined that a heart transplant was the long-term outcome."
Tennis began the testing and workups needed for a heart transplant at Johns Hopkins Medicine in February of 2024 under the care of Nisha Aggarwal Gilotra, M.D., an expert in treating patients with heart failure. Unfortunately, Tennis experienced yet another severe arrhythmia, this time requiring hospitalization at The Johns Hopkins Hospital until a donor heart became available. Facing the unknown, Tennis and his partner quickly married in a courthouse ceremony the morning before his hospitalization.
"Mr. Tennis followed a pretty typical course of symptoms, in which patients with ARVC have a primary clinical manifestation of ventricular arrhythmias, which can cause damage and cardiac arrest," says Gilotra, an associate professor of medicine at the Johns Hopkins University School of Medicine. "Thankfully, we've made progress in better identifying patients and administering treatment quickly, so they're living longer, but they then develop the secondary consequence of progressive heart failure and the need for a heart transplant."
While Tennis awaited his new heart, his medical team worked around the clock to keep his body functioning and ready for transplant surgery. One of his doctors at Johns Hopkins Medicine was Steve Keller, M.D., Ph.D., a pulmonologist and critical care medicine physician, who oversaw Tennis' pre- and postoperative care in the cardiac surgical intensive care unit. Keller has extensive experience administering lifesaving organ support through machines such as extracorporeal membrane oxygenation, or ECMO.
While on ECMO support, Tennis was able to remain active, walking around the intensive care unit despite his worsening heart function, in the hope of preserving muscle strength and stamina prior to his procedure. Keller has long studied the optimal approach to using ECMO to provide circulatory support for patients with severe cardiac conditions through research supported by the National Institutes of Health (NIH).
Thankfully, Tennis was successfully maintained on ECMO support until a suitable donor heart became available for transplant. Immediately after transplant, Tennis suffered from dysfunction of the donor heart that again required ECMO support. Over the following several days, his new heart continued to improve until he was successfully weaned off ECMO for good.
"The care we provided Mr. Tennis, and his positive outcome, is a direct consequence of us being able to develop new tools to save people's lives through NIH and federally funded research," says Keller, an assistant professor of medicine at the Johns Hopkins University School of Medicine. "Support from the NIH has allowed us to perform the challenging basic and translational work related to technologies such as ECMO that makes it possible for us to transform care and enable patients to survive what was a previously untreatable disease."
"It takes cross-disciplinary care and collaboration to care for patients like Mr. Tennis who require intense interventions such as heart transplants," says Ahmet Kilic, M.D., professor of clinical cardiac surgery at the Johns Hopkins University School of Medicine. Kilic performed Tennis' heart transplant procedure. "The ability to provide for patients across multiple divisions is instrumental not only for their well-being but also for advancing clinical care and research in cardiac surgery and lifesaving organ support."
Johns Hopkins Medicine has long researched ARVC and provided care to patients with the condition through the Johns Hopkins Precision Medicine Center of Excellence for ARVC. Genetic counselor and researcher Cynthia James, Ph.D., Sc.M., has helped lead a number of studies at the center to improve understanding of ARVC and the condition's clinical management. She says that NIH-funded research has been the bedrock of ARVC research for decades, and it is crucial in saving the lives of patients like Tennis.
"This is an exciting time for us at the center, as we are moving rapidly to gene-specific diagnosis and management of ARVC," says James. "In the field of rare diseases, we need the backing of federal funding and international collaboration to have large enough cohorts of patients to study and move our understanding of these diseases forward and to make advances in taking care of patients."
A little more than a year post-transplant, Tennis has a new lease on life with his husband and two children.
"My overarching message is that of gratefulness to everyone at Johns Hopkins. I'm grateful that the systems are in place for this kind of treatment and research to be possible," says Tennis. "Medicine is such an enormous undertaking that requires so many people, both medical and nonmedical, cooperating and sacrificing to make it work - it's truly incredible."
