Dakota’s story: Advances in medical management of pediatric heart failure

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The Longe family (Left to right: Brooke, Roger, Erica, and Dakota)

Over the past few decades, more and more children with congenital heart defects have been receiving life-saving surgery soon after birth. As surgical techniques improve, some children who would have died within the first few weeks of life are able to survive — but many still have residual heart failure. Transplant is often the optimal therapy for such patients, but the number of hearts available to transplant has not changed much over the last several years.

“The number of patients listed for transplant is much greater than the number of donor hearts,” says Dr. Elizabeth Blume, director of the Heart Center’s Heart Failure program. “Due to this limitation, we’ve dedicated an entire service to optimizing care for children living with heart failure.”

In recent years, the Heart Failure team has made significant strides in slowing the progress of heart failure in children. In some cases, disease progression has been slowed enough for the patient to be taken off of the heart transplant list for being “too well.”

Each Monday for the next four weeks, Thriving is highlighting a very special Heart Center patient who was once listed for transplant, but was removed from the list thanks to successful medical management of her heart disease.

Dakota’s story

Dakota Longe was just three weeks old when she had her first “episode.”

“She looked really pale, and the area around her mouth turned purple,” her mom, Erica Duggento, recalls. “Then I saw that she had a rash all over her chest and back, and I immediately called 911.”

This story is part of a four part series on patients who were taken off the heart transplant list thanks to medical management. Read the entire series here.

Dakota was soon transferred to a larger facility, and in less than 24 hours, she was airlifted to Boston Children’s Hospital.

“We’re from Vermont, and when you hear you need to go to Boston … that sets off alarms in your head,” says Erica.

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Dakota and her twin sister, Brooke

Dakota’s life-threatening symptoms were mysterious, even to the Heart Center’s expert cardiologists.

Dr. TP Singh suspected she was suffering from coronary artery spasms and carefully devised a treatment plan around a drug  called diltiazen. Diltiazem, a calcium channel blocker used to lower blood pressure, is rarely given to very young children. Dr. Singh calculated the proper dosage for an infant and prescribed the appropriate regimen for Dakota. While waiting for the effects of the medicine to kick in, he suggested Dakota be placed on the heart transplant list.

“When we first met the transplant team,” says Erica, “I didn’t want to go through with it. I wanted to know more first: What was really going on with my daughter? There were still so many questions.”

Dr. Christina VanderPluym explained that while doctors still didn’t know exactly what was happening to Dakota, the team had a plan to get her on the right track. Her words helped to reassure Erica during that difficult time.

Understanding transplant might be the only option after all other measures had been tried, the Longes listed Dakota for transplant. But the diltiazem regimen — in combination with other cardiac medications — worked.

Dakota’s episodes went away, and she was taken off of the transplant list shortly after her first birthday in August 2014.

Today, Dakota is a happy, energetic two-year-old who “hates to sit still and loves being outside,” especially when she’s playing with twin sister Brooke.

Though she still sees a cardiologist twice a year for follow-up (once in Boston and once at the University of Vermont Medical Center), Dakota has no signs of any residual cardiac problems. She is still on her diltiazem regimen of one liquid dose every 8 hours. At some point, she will be switched to the oral pill. Eventually she may be able to try discontinuing the medication altogether, but Singh wants to wait until Dakota can tell someone if she’s having symptoms.

Learn more about the Boston Children’s Heart Transplant Program.