Tricuspid Atresia: Srishti's Story

Srishti loves cooking, painting, bicycling, swimming and reading. She is fluent in English and Kutchi, a subdialect of Hindi. You would never know that this vibrant child has had three open-heart surgeries.

The day after Srishti was born, in August of 2004, a pediatrician at her birth hospital heard a heart murmur. Echocardiography showed tricuspid atresia, a congenital heart defect in which the valve between the heart’s right chambers is blocked.

Within hours, an ambulance took the 5 pound, 1 ounce infant to The Children’s Hospital of Philadelphia.

srishtiWhen Srishti’s mother, Sejal, arrived the next day, she was overwhelmed.

“We were really frightened looking at her, because she had tubes all over her body,” she recalls. “Seeing her like that was a little bit devastating.”

But gradually, as she and her husband, Samir, spoke with nurses and doctors, their fears ebbed. Srishti’s cardiologist explained that while tricuspid atresia is a severe defect, a majority of children who receive treatment survive and thrive.

The course of treatment for Srishti, he explained, was three surgeries to change the flow of blood to bypass the underdeveloped right ventricle/right side of the heart.

The first surgery would be at approximately 6 weeks of age.

After three days, Srishti was allowed to go home. As is characteristic of children with some heart defects, she had difficulty nursing and her breathing was rapid. Her parents monitored her intake of breast milk and formula to make sure she was eating enough to gain weight — and strength.

Tricuspid atresia treatment

The first surgery was October 26. After handing their daughter to an anesthesiologist, who carried her to the operating room, the Sejal and Samir met with cardiothoracic surgeon J. William Gaynor, MD, who explained the procedure.

After the two-hour surgery, Srishti was taken to the Tabas Cardiac Intensive Care Unit. As her parents again experienced the shock of seeing their child attached to numerous tubes and machines, the explanations of the staff helped calm them.

“Every nurse and doctor was very, very helpful,” Samir recalls. “Everyone answered our questions in great detail.”

For four days, they stayed by their daughter’s side. Sejal slept in a room in a family lounge area at the Hospital and Samir at the Philadelphia Ronald McDonald House, just a block away.

In the coming months, Srishti and her entire family, including her grandparents, would return to The Children's Hospital and experience the stress of open heart surgery twice more: the hemi-Fontan/Glenn operation in December of 2004, and the Fontan operation in April of 2007.

After tricuspid atresia treatment

The procedures Srishti had were to reconfigure her circulatory system. Normally, the right side of the heart pumps deoxygenated blood to the lungs. After her surgery, Srishti’s deoxygenated blood now flows to her lungs without passing through the right side of her heart.

In a child with a normal heart, blood oxygenation levels are close to 100 percent. Srishti’s level — which were previously as low as 65 percent  rose to 89 percent as a result of the surgeries.

Srishti takes one-half of a baby aspirin each day, to thin her blood. She visits her cardiologist once a year for checkups.

“We tended to be more cautious as parents, but the doctor told us don’t stop her from doing anything any kid her age can do,” Sejal says.

“She is a very busy person,” Samir adds. “If you were to see her you wouldn’t be able to tell what she went through.”

Srishti doesn’t seem to have memories of the surgeries or her time in intensive care. She has asked to go as a doctor at Halloween. “And she said, ‘When I grow up I want to become a doctor,’” Sejal says.

Fond memories

Srishti treasures a stethoscope a Cardiac Center nurse gave her, and a baby-doll and blanket that were among the gifts left in her hospital room on her first Christmas, four days after her second surgery. Srishti woke up that day to find the table near her bed covered in presents.

“When we woke up it was all there,” her mom recalls. “Books, a teddy bear, toys. Baby dolls. A quilt. A blanket. You name it. All wrapped. It was the real Santa Claus.

“We are so appreciative of CHOP. Everything is so much about kids. It was Christmas but the kids were in pain. Although it was an emotional moment, they tried to help the kids celebrate and made it festive.”

Each year, Srishti and her family, who are Hindu, have a Christmas story-time and exchange gifts in honor of that day.

Growing up, grateful

As Srishti grows, she has begun to ask about her scar. “We told her she had a small boo-boo and assured her we will explain it when she’s older,” Samir says.

For the Sejal and Samir, like many Cardiac Center families, their daughter's diagnosis with congenital heart defect and treatment were one of the most difficult experiences of their lives. But when they explain it to her, there will be plenty of good to tell.

“The experience we had was painful,” Sejal says, “but it was excellent in terms of the people: the doctors, the nurses, everyone.”

“It’s a God-sent place for every child there,” Samir says. “Everybody was a helping hand in a time of distress. They kept us afloat.”


Originally posted: June 2010