Miguel Marques Garcia – Atrioventricular Septal Defect Patient Story

At just 23 weeks old, doctors diagnosed Miguel Marques Garcia of São José dos Campos, Brazil, with atrioventricular septal defect (AVSD).

AVSD is a set of heart malformations. They include holes between the heart's upper and lower chambers and malformed valves that control the blood flow between them.

Miguel would need surgery to correct his AVSD when he was between three and six months old.

But, even before he was born, doctors found Miguel might have other heart problems.

A few weeks before his birth, Miguel’s parents, Pablo Garcia and Eloá Marques, learned he might have coarctation of the aorta.

Coarctation is the narrowing of one or more sections of the aorta, a key blood vessel in the heart. The narrowing causes the heart to work harder to compensate. This would present a much more urgent problem.

Heart Surgery at Four Days Old

Miguel's parents scheduled a C-section, and Eloá gave birth on September 1, 2016. Doctors confirmed Miguel did have coarctation of the aorta and they needed to repair it as soon as possible.

“It was difficult to have to run with my baby to the ICU and leave my wife alone,” says Mr. Garcia.

Miguel had the surgery to repair his aorta when he was four days old.

Then, when Miguel was three months old, his parents learned that he would need the coarctation repaired again.

Since he was now old enough to have the AVSD repaired, his doctors planned to do both surgeries at the same time.

“But the night before the surgeries,” says Mr. Garcia, “they told us they couldn’t fix the AVSD. The left ventricle of his heart was too small.”

Surgeons repaired Miguel’s coarctation and put a banding on the pulmonary aorta to help improve his heart function.

More Bad News for Miguel and His Parents

Things went from bad to worse when Miguel’s parents learned he also had grade IV pulmonary hypertension. Because of this, Miguel likely wouldn’t be able to handle another surgery to fix his AVSD.

After 15 days in the hospital, the family went home on September 16, 2016.

“Ever since Miguel was born, all the cardiologists had been speaking to us about Dr. da Silva,” says Ms. Marques. “Everyone said ‘The best person to do Miguel’s surgery is Dr. da Silva, but he’s no longer in Brazil.’”

“It was a really difficult recovery for Miguel,” says Mr. Garcia.

After two days at home, the cardiologist visited Miguel. He sent Miguel back to the ICU, where doctors stabilized him and sent him home again.

Doctors found stenosis (narrowing) in Miguel’s pulmonary veins, and he had fevers every day.

In February 2017, the family saw another cardiologist, described by Mr. Garcia as “the top guy in South America.”

The cardiologist “was very negative,” says Ms. Marques.

“He said he would do a catheterization (a less invasive procedure that would treat the stenosis) just to do something. But still, the prognosis was not very good.”

“We were so destroyed in the car going home from this appointment,” said Mr. Garcia. “And then as soon as we got home, I got a call from Dr. da Silva.”

“It was a light for us. It was a sun!” says Ms. Marques.

Things moved quickly then.

The Journey from Brazil to Children’s Hospital of Pittsburgh

Within three days, Dr. da Silva had:

• Received videos of Miguel’s heart.
• Consulted with colleague Jacqueline Kreutzer, MD, chief of the Division of Pediatric Cardiology Division of Pediatric Cardiology.
• Called Mr. Garcia back to give him the good news.

“Dr. da Silva said come to Children’s, because he has a lot of resources here. He said the team is fantastic. The hospital is wonderful, and that he will be here to follow the case after the surgery,” says Mr. Garcia.

“It took us just 10 days to get everything ready to come to Pittsburgh,” says Ms. Marques. “Neal (Neal Smalley, from Children’s International Services Team) was really helpful and gave us everything we need.”

“Dr. da Silva also called many times, to tell us how to do everything,” says Mr. Garcia.

The family flew to Pittsburgh on February 13. Miguel would have surgery four days later.

Six Procedures, One Surgery

“I was able to review data from when Miguel was born,” says Dr. da Silva. “At birth, his left ventricle was normal. It got smaller as a result of an obstruction under the aortal valve. And also because of the banding that was put in.”

“Brazil has more than its share of the world’s top cardiac surgeons,” says Mr. Garcia. “The best surgeons in Brazil worked on Miguel. Dr. da Silva told us without the banding and other things they'd done, he wouldn’t have been able to operate on Miguel.”

“The whole time, Dr. da Silva was telling us why Miguel will make it,” says Mr. Garcia. “Giving us data, saying this, this, and this — not just saying ‘everything will be okay.’”

In a surgery that took just under three hours, Dr. da Silva:

• Removed the obstruction in Miguel’s left ventricle and enlarged the ventricle.
• Removed the pulmonary banding.
• Repaired the tricuspid valve and the mitral valve.
• Closed both the ventricular septal defect and the atrial septal defect.

“Six procedures in the same surgery!” says Ms. Marques. “We told many cardiologists about this, and they all told us that this is an amazing thing.”

After the surgery, “Dr. da Silva came to us and said that it was a success,” says Ms. Marques. “Now Miguel is smiling all the time. He’s like a new guy!”

“And now he is a Pittsburgh Steelers (football) fan,” adds Mr. Garcia. 

Dr. da Silva called every day to follow up with Miguel.

“I think he’s doing very well,” he says. “His cardiologist sent us a smiling photo of him. We’re happy with the resources we have and the teamwork we did. And happy to give happiness to Miguel and Miguel’s family. Health and happiness.”

Visit the Heart Institute at UPMC Children's Hospital of Pittsburgh to learn more about our care.