Danny’s journey to a biventricular heart

Danny, born with heterotaxy syndrome, peeks out from a tree.The first hint that something wasn’t quite right with Danny Sanchez-Garcia’s heart came at his mom’s six-month prenatal visit.

“There was a little blip on the ultrasound, but then it was gone on the next one, so they didn’t think it was anything and I didn’t worry any more about it,” says Danny’s mom, Cynthia.

Cynthia was overjoyed when Danny was born at her local hospital seeming perfectly healthy. But as the hospital staff monitored Danny overnight, they noticed his oxygen level was lower than normal and decided to run more tests. His doctors believed the tests pointed to a congenital heart defect called tetralogy of Fallot with pulmonary atresia.

“They transported him overnight to Boston Children’s Hospital,” says Cynthia. “I felt like I was on a roller coaster, especially as a first-time mom.”

More tests and a new diagnosis

Once Danny arrived at Boston Children’s, his story took another unexpected twist. Dr. Roger Breitbart, chief of Inpatient Cardiology, explained to Cynthia that Danny’s initial diagnosis was not correct. Upon more in-depth testing, Breitbart and his colleagues found that Danny had heterotaxy syndrome with double outlet right ventricle, pulmonary atresia and viscero-atrial situs inversus (abnormal connections of the large veins that return high-oxygen and low-oxygen blood to the heart). Heterotaxy is a rare, complex condition where the heart or other organs are not formed correctly or are in the wrong position in the body. Many babies with heterotaxy have congenital heart problems, as Danny did. He would need surgery right away.Danny, who has heterodoxy syndrome, smiles after surgery.

Breitbart believed Danny was a good candidate for a biventricular repair. In some cases, this involves enlarging a second ventricle (pumping chamber) that is too small at birth. In other cases, like Danny’s, there are two normal-sized ventricles, but abnormal vein and artery connections prevent them from working independently. Danny’s biventricular repair would involve complex rerouting of these connections.

“At many heart centers, they would take a single-ventricle approach rather than biventricular repair,” says Breitbart. “But we’re focused on biventricular repair here because we believe there are several long-term advantages over a single-ventricle circulation. And based on Danny’s particular anatomy, we were confident that biventricular repair would be possible.”

Danny’s surgeon, Dr. Christopher Baird, director of the Congenital Heart Valve Program at Boston Children’s, explained to Cynthia that he would have to perform two operations. First, he would place a shunt to ensure Danny would have stable oxygen levels in his blood, and then, when Danny grew bigger, he would undertake the full heart repair.

“It was a lot to take in, since Danny’s heart problems were so complex,” says Cynthia. “He had his first surgery when he was just 5 days old.”

Danny went home a few weeks after his first surgery. He started to gain weight and was happy and thriving. His second surgery was scheduled for about a month before his first birthday.

Another unexpected turn

During a pre-op appointment a few days before the surgery, Danny’s story took another unexpected turn. He was in clinic, having some routine tests, when he suddenly went into cardiac arrest.

“The whole room flooded with doctors and they started giving him CPR,” says Cynthia. “It was really scary. If he hadn’t been in the hospital that day, he probably wouldn’t have survived. They saved his life.”

Breitbart says the cardiac arrest was related to his underlying heart disease and not the biventricular repair, since he had not yet had that part of the surgery. “A blood clot had formed and blocked the shunt in his heart, which is a very narrow passageway, so no oxygen was getting to his lungs. We may never know exactly why it happened at that particular time, but he was extremely lucky to be at the hospital.”

Danny was placed on extracorporeal membrane oxygenation (ECMO), an advanced life support machine that takes over the function of the heart and lungs, and then had an emergency catheterization to re-open the blocked shunt.

Because of his cardiac arrest, the surgery had to be postponed so Danny’s heart could recover. When he was cleared for surgery about two weeks later, Baird completed the biventricular repair, giving Danny two independently working ventricles with correct vein and artery connections.

A truck-loving 3-year-old

Danny recovered quickly after surgery and has done well since then. He just turned 3 and is looking forward to starting preschool soon. He loves trucks, singing along with Little Einsteins, hanging out with his grandpa and being outside.

“Watching him run around, you’d never guess he was born with a heart condition,” says Cynthia. “He’s like any active normal 3-year-old boy — he loves bothering the dog and has already figured out how to unlock the doors.”

Danny, who has heterotaxy syndrome, eats a cracker on his front porch.

For now, Danny continues to see Breitbart about once a year to make sure all of the passageways in his heart are clear. He sees his local cardiologist for more routine visits. Eventually, he’ll need one final surgery to replace an artificial conduit that connects his right ventricle to his lungs with an adult-sized one. The timing of the surgery will depend on his growth and how well the current conduit holds up.

“Danny had a very complicated operation and had a fantastic result,” says Breitbart.  “He’s doing beautifully.”

Learn more about Boston Children’s Complex Biventricular Repair Program.