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Pacemaker successfully implanted in smallest child at Packard Children's
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In November, 15-minute-old Jaya Maharaj became one of the smallest recipients of a pacemaker.
A fetal echocardiogram diagnosed Maharaj with congenital heart block midway through the pregnancy. Born 9 weeks early and weighing just more than 3.5 lb, Maharaj underwent her first surgery upon delivery at Lucile Packard Children’s Hospital. The newborn received two pacemaker leads implanted in her heart and the pacemaker was implanted in her upper abdomen.
Congenital heart block is not a common condition, according to Valerie Chock, MD, a neonatologist at Stanford University and Lucile Packard Children’s Hospital. Chock was a member of the multidisciplinary team that planned Maharaj’s birth and surgery. “It is estimated that congenital heart block occurs in one in 20,000 babies, but the actual incidence might be higher because many babies die in utero before a diagnosis can be made,” Chock said.
Mother Leanne Maharaj was referred to Lucille Packard Children’s Hospital at 28 weeks’ gestation. Weighing the risks of congenital heart block and premature delivery, the multidisciplinary team that managed the Maharaj case planned to maintain the pregnancy until 31 weeks. However, if the baby developed any HF or further decline in heart rate, the team’s plan was to perform an emergency cesarean section.
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Photo credit: Norbert von der Groeben
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“We knew [baby] Jaya was coming and we knew what was going to happen, we just needed to figure out how to best utilize our resources,” Theresa Tacy, MD, medical director of the echocardiogram lab and the director of fetal cardiology at Lucile Packard Children’s Hospital, said in an interview.
The multidisciplinary team was comprised of experts in pediatric cardiology, cardiac surgery, cardiac electrophysiology, neonatology, maternal fetal medicine, CV anesthesia, respiratory therapy and pharmacy, among others. In all, about 25 clinicians were involved. The cardiology and maternal fetal medicine teams followed the fetal heart status and the pregnancy frequently before birth and the mother was given a steroid to minimize damage to the baby’s heart.
At 31 weeks, two operating rooms were set up for the procedure. In the first room, the team would perform the cesarean section. In the second, Katsuhide Maeda, MD, a cardiothoracic surgeon at Lucile Packard Children’s Hospital, would implant the newborn’s pacemakers. Maeda said he originally planned to implant a temporary pacing lead, but opted for a permanent pacemaker when the newborn arrived healthier than expected.
Although the procedure is risky, Maeda told Cardiology Today that Maharaj would have died within 1 to 2 hours if the pacemaker was not implanted.
“When she was born, her heart rate was only 45 beats per minute. Without the pacemaker, the patient could not survive,” Maeda said.
The surgery was completed with just eight stitches and in about 1 hour.
The newborn spent 6 weeks in the NICU after surgery and was sent home on Jan. 12. Aside from battery replacement every 5 to 7 years, Maeda said Maharaj should live a normal life with the pacemaker.
This is just one example of a newborn with congenital heart block receiving a pacemaker. Typically considered a rare condition, health care professionals at Lucile Packard Children’s Hospital are seeing an increase in the number of children delivered with the condition.
“Each case is unique, and we have to adapt to the challenges … and continue to learn from each case,” Chock said. – by Casey Murphy