Jaya Maharaj, a baby girl born 9 weeks early with a congenital heart defect was fitted with a pacemaker just 15 minutes after birth. Weighing only 3.5 pounds (1.6 kg), she was delivered by cesarean section at Lucile Packard Children’s Hospital, Palo Alto, California, in November last year.

Jaya, born to parents Leanne and Kamneel Maharaj of Hayward, also in California, is thought to be the smallest patient noted in the medical literature ever to receive a pacemaker.

Her heart was the size of a walnut when the pacemaker was fitted. Her doctors said in a statement released this week that they expect her to lead a normal life.

Jaya had been diagnosed with a severe condition known as congenital heart block while she was still in her mother’s womb.

The unborn baby’s congenital heart block developed when her mother’s immune system mistakenly attacked the nerves fibres that transmit heartbeat signals in the fetal heart muscle.

This caused her fetal heart rate to fall to a dangerously low level, vastly increasing the risk of congenital heart failure as a result of fluid accumulating around her heart and lungs and preventing her body getting much needed oxygen to grow.

Another problem that can develop with this condition is that the heart can stop because the different chambers start beat out of step with each other.

Jaya’s surgery is remarkable on several counts: first her very small size, and second, because she got to be born at all. Many fetuses diagnosed with congenital heart block do not survive: research shows that up to half of those diagnosed before birth die either in the womb or shortly after birth.

And another reason, is that delivering a baby prematurely in order to fit a pacemaker is not without risk.

Doctors referred Leanne to Packard Children’s when she was 28 weeks pregnant. From that day, the hospital’s Center for Fetal and Maternal Health, which has treated a few congenital heart block cases in the past, put together a large, multidisciplinary team to plan Jaya’s birth and surgery.

As well as experts in high risk obstetrics and neonatal care, the team comprised specialists in pediatric cardiology, cardiac electrophysiology, cardiac surgery, cardiovascular anesthesia, respiratory therapy and pharmacy.

Team member and neonatologist Dr Valerie Chock spoke about weighing up the risks:

“We knew that at any time the baby’s heart could give out. We had to decide: At what point do we deliver to balance that risk against the risk of premature delivery?”

Dr Yasser El-Sayed is a high-risk obstetrician at Packard Children’s; he supervised Leanne’s care through her pregnancy.

“So much was in the balance,” said El-Sayed, “We just had to take it day by day, week by week.”

El-Sayed and colleagues checked Jaya twice a week before birth. They checked her heart rate and growth, looking to see if there was any fluid accumulating around her heart and lungs. As she was growing and not showing signs of heart failure, the team decided to wait until week 31 of the pregnancy before delivering her.

During these weeks, Leanne took steroids to limit the damage to her baby’s heart.

The delivery, on 22 November 2011, was quick. 25 clinicians attended to perform the cesarean and stabilize the newborn, and in another room, a separate surgical team performed the surgery.

For parents Leanne and Kamneel it was an emotional time. During these last weeks of the pregnancy they had hoped and prayed everything would be ok. They relied on their Hindu faith and their family:

“Praying every day gave us faith and hope that something would help,” said Kamneel.

The delivery was quick, as Leanne recalls:

“When they took her out, she was crying, and my husband started crying.”

But within minutes, after Leanne and Kamneel quickly welcomed their child into the world, the surgical prep team got to work. They checked the little girl’s heart rate, inserted a breathing tube, and intravenous catheters, readying her for surgery. They checked her oxygen level, gave her drugs to stabilize her premature lungs, and double-checked that her congenital heart condition had not affected her growth in the womb.

Although 3.5 pounds is a healthy weight for a 9-week premature baby, Jaya’s heart rate was pitifully low for a newborn. A typical newborn’s heart rate is 120 to 150 beats per minute. But Jaya’s was under 55, the threshold for which it is essential to immediately start artificial pacing.

There was no time to lose, and they hastily wheeled little Jaya next door for the pacemaker-fitting operation, which was performed by surgeon Katsuhide Maeda.

Maeda had originally planned to fit Jaya with a temporary external pacemaker that attached to her heart via wires through her chest. This had been done in small premature infants before. The advantage is a less invasive, fast initial surgery, but the disadvantage is the need within weeks to replace this with a full implanted and permanent device.

Maeda changed his mind when he saw that while Jaya’s condition was critical, she was not so sick as to need chest compressions. And although her heart rate was slow, it was stable. So he decided to implant the permanent device there and then, and spare Jaya the need for a second operation in a few weeks.

But that did not mean this procedure was without risk: the team had to proceed extremely carefully, as Maeda explained:

“Her heart muscle was so fragile that I had to be extra careful about putting stitches in. The heart muscle on such a small baby is easily torn; that’s why people usually prefer just putting in temporary pacing wires.”

It took just 8 stitches to connect two pacemaker’s leads to the baby’s tiny heart. Maeda implanted the pacemaker itself into her upper abdomen.

By the time Jaya was 2 hours old the surgery was completed and her heart was beating at the normal rate.

Jaya then had to spend six weeks in the hospital’s Neonatal Intensive Care Unit. Her heart was pumping normally, she put on weight quickly, and by the middle of January she was home.

Doctors say Jaya’s prognosis is “excellent”. She will need further surgery as she grows, to reposition the pacemaker leads, and every 5 to 7 years to fit a new battery, but apart from that, they say she should be able to lead a normal life.

Jaya is now almost three months old, and weighs 8 pounds: a thriving baby.

Her father told the press that all through the pregnancy, they never lost hope. Whenever they were worried, Jaya would kick from inside as if to say “I am here, I am alive!”

“We thought maybe she was trying to tell us that everything was OK, so we were always hopeful,” said Kamneel Maharaj.

It is fitting that he and his wife chose to name their little girl “Jaya”: it means “victory” in Hindi.

Written by Catharine Paddock PhD