Toddler Dances Around Heart Defect

Born with a congenital defect, Leni had open-heart surgery at only 6 weeks old. But her health issues weren’t truly resolved until the toddler required a devastating intervention.

Leni’s condition at birth, tetralogy of Fallot, meant a hole in her tiny heart allowed oxygen-rich blood to mix with oxygen-poor blood. In addition, a narrowing of her pulmonary valve and lung arteries didn’t allow enough blood to reach her lungs. This condition made her right ventricle work extra hard, but doctors alleviated the issues with early surgical repair.

While successful, the procedure caused scar tissue to develop, which later led to specific type of supraventricular tachycardia called atrial flutter. Different medications were tried and cardioversion was performed. Those steps controlled the arrhythmia to some extent until an accidental overdose occurred when the little girl was 2 and staying with family.

Keeping steady

Leni was airlifted to Children’s Mercy where doctors worked to stabilize her condition. After several days on ECMO (extracorporeal membrane oxygenation, a treatment that provides breathing and heart support), Svjetlana Tisma-Dupanovic, MD, performed an ablation on the little girl’s heart to stop the arrhythmia. Because there was no reliable sinus node function several days after the procedure was performed, the next step was to install a pacemaker to keep Leni’s heart beating fast enough. 

The pacemaker was only part of the treatment during Leni’s four-week stay at Children’s Mercy, however. Staff also oversaw her withdrawal from the medications she’d been on to keep her comfortable and manage her pain. Despite the dire circumstances, her mother Cori described the experience as “outstanding.”

“The entire staff of the PICU was great to work with,” she said. “We had most of our dealings with Laura A. Ortmann, MD, and Kelly S. Tieves, MD, and they were very supportive and comforting to our family. The numerous nurses were all outstanding. In particular, Shaq and Charlotte were always there for us as parents and, of course, for Leni as their patient.”

From bushed to busy

Before getting her pacemaker, Leni’s parents occasionally noticed their daughter struggle with a lack of energy related to her heart arrhythmia. But since its installation, she’s mostly led a normal life back in her hometown of Wichita, Kan., her mom said. “Aside from restrictions related to the technology of the pacemaker – which are surprisingly few – Leni has been cleared for normal activity.” In fact, Cori added, the device has done nothing to slow down the toddler, who enjoys twirling, dancing and singing.

Today, Leni is off all medication but remains heavily reliant on her pacemaker, which her heart uses more than 95 percent of the time to allow her to have a normal life and be active. The little girl has follow-up appointments with Dr. Tisma-Dupanovic every six months to check on the device’s and her heart’s overall function. 

“She currently experiences a slightly leaky valve in her heart that will, more than likely, require repair or replacement at some time in the future,” Cori said. Even so, she added, “The entire experience at Children’s Mercy was second to none. From the comforting atmosphere, to the friendliness of the staff, to the facilities, there’s no place like Children’s Mercy.”

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