Left Ventricular Noncompaction Cardiomyopathy: Ananya's Story
Patient Story Highlights
- Ananya, a 6-year-old girl from South India, was diagnosed early in life with a rare form of cardiomyopathy, left ventricular noncompaction cardiomyopathy (LVNC).
- During a family visit to Florida, Ananya's heart condition suddenly worsened, leading her to be admitted to Johns Hopkins All Children's Hospital.
- The hospital's Heart Institute placed Ananya on extracorporeal membrane oxygenation (ECMO) and then implanted a left ventricular assist device (LVAD) as they awaited a donor heart for a transplant.
- After nearly five weeks on the national transplant waiting list, Ananya received a new heart through a successful transplant surgery.
- Ananya quickly recovered from the surgery and will continue to receive comprehensive follow-up care at Johns Hopkins All Children's Hospital.
- Ananya's mother, Swarna, expressed immense gratitude for the care her daughter received at the hospital, saying, "It is the very gift of life playing out before me. There is nothing more precious."
Two contrasting memories will live forever in the heart of one little girl’s mother.
Swarna will never forget the way her daughter, 6-year-old Ananya, arrived at Johns Hopkins All Children’s Hospital.
And she will also never forget the way she left.
The year had started off in a hopeful fashion.
The family, visiting from South India, was enjoying a stay with a relative in Oldsmar. Swarna was looking into obtaining a nutritionist’s license while here, and Ananya was active and happy, soaking up the Florida sunshine with her brother.
Ananya had been diagnosed early in life with a rare form of cardiomyopathy. Left ventricular noncompaction cardiomyopathy (LVNC) is a disease of the heart muscle where the inside wall is spongy instead of smooth, making it harder for the heart to pump blood to the body.
Ananya’s heart condition had been stable for many years. But on Easter morning, she suddenly became very sick.
“Her face was puffy, she was vomiting. We thought she might have food poisoning, so I gave her medicine for that,” Swarna says.
But her illness grew more severe. Within hours, she developed a high fever. A pediatrician sent the family to a Tampa emergency center.
Ananya was hospitalized in Tampa for four days, but her condition did not improve.
As it turned out, this little girl was in the final stages of heart failure.
There didn’t appear to be many options for Ananya — a critically ill child with a failing heart in a country that was foreign to her.
But from across the Bay — a glimmer of hope.
Heart specialists at Johns Hopkins All Children’s Hospital had learned about Ananya’s case and had agreed to have the child transported there for care.
The hospital had been rebuilding and strengthening its Heart Institute over the past year, acquiring highly skilled pediatric heart surgeons and a comprehensive support team of cardiac specialists from across the country.
The new heart team hoped and believed it could help this little girl.
But the path to wellness would not be an easy one.
To sustain her life, Ananya was quickly placed on extracorporeal membrane oxygenation (ECMO), a machine that serves as a sort of bypass for the heart and lungs.
It was becoming clear that in order to survive, Ananya had one option — a heart transplant.
The Johns Hopkins All Children's Heart Institute has experience with pediatric heart transplants.
But to carry out a life-saving heart transplant — you must first have a heart.
Would there be a new donor heart for Ananya in time to save her life?
Within 48 hours of her arrival, the Heart Institute team removed Ananya from the ECMO machine. Instead, they surgically implanted a left ventricular assist device (LVAD) into her chest, a mechanical pump that could help distribute oxygenated blood to her body in a way that her failing heart could not.
This temporary LVAD would buy some time — perhaps a number of weeks — as they waited to see if a donor heart could be found for the girl.
Ananya was placed on the national transplant waiting list. As the days and weeks ticked by, the heart transplant team did all within their power to ensure that Ananya stayed strong as they managed the multitude of details and arrangements necessary to receive a donor heart, should one become available.
“It’s not a simple thing to secure a donor heart,” says Alfred Asante-Korang, M.D., a pediatric cardiologist with the Heart Institute. “Your position on the waiting list is related to how critical you are.”
After nearly five weeks of waiting — joy came in the morning.
Grace arrived in the form of two bespectacled heart doctors who tiptoed into Ananya’s hospital room with big news.
“I was still sleeping,” Swarna says. “They said, ‘Momma, we have good news for you! We have a new heart for your daughter. Tonight, she will get a new heart!’”
It’s one of the perks of the trade. To deliver this kind of news to a parent.
"The care here left me speechless. Speechless, because I never could have conceived of a hospital like this, of a team of people like this. I'm so grateful my baby was under the care of such a hospital. It is the very gift of life playing out before me. There is nothing more precious."Swarna
It is difficult to overstate the perfect symphony of planning, precision and people involved in a heart transplant.
In the wee hours of May 4, Ananya was wheeled into the OR as the transplant team prepared for the long surgery ahead. The team included two transplant surgeons. Also, an anesthesiologist, a certified anesthetist, a surgical physician assistant and nurses. The transplant cardiologist was in attendance, as well as the transplant coordinator, who had meticulously arranged for the entire process.
Simultaneously, a surgeon and a nurse had chartered a fast flight out of state to inspect the donor heart, with the mission of bringing it back in pristine condition to the waiting transplant team.
With a “thumbs up” from the procurement team that the heart was perfect for Ananya, the surgeon and his team in the OR would complete the removal of the temporary LVAD, and then remove Ananya’s failing heart — precisely in time to replace it with her new one.
More Than Surgery
Ananya’s heart transplant surgery went exceedingly well. She would recover from the surgical procedure quickly, surprising her mother with her strength — both of heart and spirit.
“It seemed in no time, she was alert, playing video games, and chatting with the doctors,” Swarna says.
But a successful heart transplant involves more than the surgery itself. The comprehensive follow-up care Ananya will continue to receive at Johns Hopkins All Children’s Hospital is critical to how she will fare over time.
“You need to have all the resources in place,” Asante-Korang says. “Not just pediatric surgery, but a critical care team, pediatric nephrology, pediatric GI specialists, pediatric ENT. You need an immunology lab skilled in reading transplant biopsies. It’s a very complex network of highly-skilled professionals all coming together to care for kids like Ananya.”
It is a level of care that has left her mother filled with gratitude.
“The care here left me speechless. Speechless,” Swarna says. “Because I never could have conceived of a hospital like this, of a team of people like this. I’m so grateful my baby was under the care of such a hospital.”
Swarna has many memories of her child’s medical journey, and there are many still to be made.
But the two strongest memories, the two in deep contrast to one another, will remain with her always. The agonizing memory of her gravely ill child arriving at Johns Hopkins All Children’s Hospital on a ventilator, clinging to life — and the memory of 76 days later, the day Ananya was discharged — walking on her own, offering hugs, waves and smiles for the care team she had grown to love.
Her little girl — with the strong, beating heart — saying goodbye.
“It is the very gift of life playing out before me,” Swarna says.
“There is nothing more precious.”