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The local cardiology team that took on Miranda Jones’ congenital heart condition after she was admitted to a Memphis, Tenn., hospital had run out of options, leaving the teenager at the most literal of dead ends.
Her heart had arrested twice while in surgery, and her time on life support equipment was expected to be short when doctors reached out to the Heart Institute at Children’s Hospital of Pittsburgh of UPMC.
Children’s Hospital dispatched its specialized transport team to bring Miranda to Pittsburgh, but upon arrival, her situation was found to be even more dire, when doctors discovered that she had suffered a massive stroke. The notion of any kind of heart surgery seemed out of the question.
Yet nine months later, Miranda began her senior year of high school, much the same as any 17-year-old, thanks to her own strong spirit, pioneering capabilities developed by Children’s Heart Failure and Recovery team, and a nearly full recovery from the stroke, which together made her a candidate for a successful heart transplant.
Miranda had a form of hypertrophic cardiomyopathy (HCM), an inherited and disabling disease in which part of the heart muscle thickens and stiffens without any obvious cause. HCM can lead to serious and life-threatening arrhythmias, and often goes undiagnosed because many patients have only mild symptoms or none at all. Its asymptomatic nature makes HCM a common cause of sudden cardiac arrest in young people, including athletes.
The Jones family had endured more than its share of experiences with this congenital condition. A brother, Damian, who Miranda never knew, died in 1994 at the age of 14 following a basketball game. In 2008, Miranda’s mother, Marilyn, died from congestive heart failure at the age of 50. A month later, the disease robbed older sister Jennifer, then 18, of a college music scholarship when she collapsed during her first season of summer band camp. Even following surgery, Jennifer still suffers consequences of the condition, requiring medications and use of a defibrillator.
Miranda had HCM with obstruction (HOCM). The wall between the heart’s two bottom chambers was enlarged, restricting blood flow to the lungs, which reduced oxygenation and caused shortness of breath, fatigue and loss of stamina. Since 14, Miranda had been on medicine to promote blood flow. Still, she would sometimes get winded on her short walk to school. Breathing while sleeping was easier if she was propped up in bed – until the frightful night before Halloween in 2013, when she awoke gasping for breath, prompting the call to 911.
Local doctors tried surgically to promote blood flow through her constricted heart. Her father, Erwin, recalled being told her heart stopped twice in the operating room. After the procedure, doctors informed the family “they were going to try to make her as comfortable as possible, because they didn’t know if she was going to make it,” he said.
Sedated, Miranda was placed on extracorporeal membrane oxygenation (ECMO), a heart and lung bypass machine, to temporarily support circulation and blood oxygenation while the doctors contacted other hospitals around the country in search of help. The Heart Institute at Children’s Hospital answered the call, and within hours its transport team was on the scene.
“We anticipated that we might transfer her to a total artificial heart,” said Peter Wearden, MD, PhD, surgical director, Pediatric Heart and Lung Transplantation. “However, a CT scan obtained on her arrival showed that she had suffered a stroke on one side of her brain.”
The stroke meant Miranda was no longer a candidate for transplant. Dr. Wearden also knew that Miranda could not be sustained for long on ECMO in a drug-induced coma. He recommended instead using a ventricular assist device (VAD), a type of miniature pump, in an unconventional way to support the heart and allow her lungs to handle blood oxygenation.
Typically, a VAD connects via tubing to either of the heart’s lower chambers, called ventricles, promoting blood flow when the heart muscles cannot perform as needed. However, Dr. Wearden’s proposal was to use a VAD to route oxygenated blood from the left atrium directly to the aorta, essentially bypassing the seriously compromised left ventricle.
Tired of losing people to this heart condition, Miranda’s father encouraged Dr. Wearden to do what he could “to save my baby.” Two weeks after arriving at Children’s she was removed from ECMO and placed on the VAD. Remarkably, when she awoke following the procedure, she had experienced a nearly full neurologic recovery. Sister Jennifer recalled that one of Miranda’s first questions was, “When can I walk?”
Before long, Miranda was walking while still on the VAD – initially with help from a physical therapist and then on her own. Given her positive response, she was quickly put back on the transplant candidate list. In February of 2014, Miranda received her new heart, and following months of physical and occupational therapy she went home to Memphis.
For Miranda, life is no longer compromised by obstructions of the heart. Her future is as wide open as the skies.
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