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Cardiovascular Report - Three-stage surgery for congenital heart defect

Cardiovascular Report Winter 2012

Three-stage surgery for congenital heart defect

Date: December 26, 2011

Luca Vricella
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Nationally, says Luca Vricella, the survival rate for children who have undergone all three stages of surgery is about 80 percent, depending on the congenital anomaly.

At 12 weeks of pregnancy, Jenni Wilson had a fetal echocardiography, which indicated that her baby had a congenital heart defect. Further testing revealed that the baby had tricuspid atresia and would be born with three heart chambers instead of four. The right ventricle was missing. While such a prenatal diagnosis can be very difficult for prospective parents, it also can be lifesaving.

“Prenatal diagnosis allows us to help families wrap their mind around the diagnosis, make pregnancy decisions and plan for delivery and care, including therapeutic strategies in the neonatal intensive care unit,” says Priya Sekar of the Hopkins Fetal Cardiology Program.

Wilson’s daughter, Elliana, was born at Johns Hopkins. Since her congenital heart disease was known ahead of time, the NICU staff was ready to care for her immediately, including administering IV prostaglandin to keep her ductus arteriosus open. Cardiac surgeon Luca Vricella was prepared to operate a few days later.

Children such as Elliana who are born with single ventricle malformations require a three-step surgical approach, according to Vricella.

“The first operation is a palliative one. It’s the same as the blue baby operation pioneered at Johns Hopkins in 1944, in which we put in a Blalock-Taussig shunt to create a conduit to ensure reliable blood flow to the lungs,” says Vricella. “At the same time, we ligate the connection that had been kept open with prostaglandin. The baby can go home within one to three weeks, depending on any other health issues.”

For the next six to eight months, the child must take a small dose of aspirin each day to prevent clots in the shunt, and be carefully monitored since the shunt doesn’t grow to accommodate the increased blood flow demands as the child grows. When oxygen saturation declines to less than 80 percent, it is time for the second stage of the surgery, in which surgeons perform a Glenn shunt, connecting the superior vena cava to the right pulmonary artery.

“Our goal is to provide a reliable source of blood to the lungs that will grow along with the child,” says Vricella. “From the time the Glenn shunt is performed until the third surgery is needed, children are usually very stable and they develop beautifully.”

Eventually, though, the child begins to have exercise limitations because the percentage of blood coming from the lower part of the body increases as the child grows. “In the third procedure, we put in a large prosthetic conduit (TCPC or Fontan connection) to re-route the blood flow from the inferior vena cava to the pulmonary artery, which feeds the heart from the lower body and supports the child’s growth,” Vricella says.  Regarding the timing of the third operation, Hopkins surgeons prefer to operate between age 3 and 5 years. “There’s no downside to waiting,” says Vricella.

Elliana has had the first two procedures and, according to her mom, Jenni Wilson, “She is doing amazingly well. You would think she’s a completely normal child.”

Vricella says patients must be followed throughout their lives for signs of problems with their pulmonary arteries, arrhythmias, leaky valves and other issues.

Because of its long experience and multidisciplinary team of specialists, Hopkins is known as a center of excellence in caring for children and adults with congenital heart malformations.