Meet Paz: Paraguay’s HLHS pioneer

Before Maria de la Paz was born, her parents Violeta Gustale and Orlando Cazal learned their unborn daughter had a complex congenital heart disease called hypoplastic left heart syndrome (HLHS).

With HLHS, the left side of the newborn’s heart is so underdeveloped that it cannot pump enough oxygenated blood throughout the body. The standard treatment is a series of three corrective surgeries to re-route blood flow through the heart. The first surgery is performed at birth, the second at six months and the third about two or three years later.

Violeta and Orlando were presented with four options:

  • Travel from their home in Paraguay to Boston Children’s Hospital for treatment. The Boston Children’s Heart Center had a 95 percent success rate for the surgeries Paz would need (this was in 2011; today, that rate is 97 percent).
  • Go to São Paulo, Brazil, where the best hospitals had a 75 percent success rate for the same surgeries.
  • Go to Buenos Aires, Argentina, where surgical success rates hovered around 50 percent.
  • Stay in Paraguay and have the surgeries there. Paz could be the country’s first success story, and the hospital would cover all medical expenses.

They made their decision right away. Their daughter would be born at Brigham and Women’s Hospital and treated at Boston Children’s.

“We went online and found another family in Brazil who had a child with the same condition. We emailed them, and they wrote back immediately, saying, ‘We’re in Boston right now!’”

The Brazilian family shared the story with Dr. Pedro del Nido, chief of Cardiac Surgery, and the preparations began.

Two months after Paz was diagnosed, the Cazal Gustales were in Boston, living close to the hospital and preparing for delivery at Brigham and Women’s.

They stayed in the Yawkey Family Inn, part of the hospital’s Patient Family Housing Program, for eight weeks with weekly prenatal appointments. Their care team included Dr. Wayne Tworetzky, director of the Fetal Cardiology Program; Terra Lafranchi, NP, Fetal Cardiology coordinator; and Dr. Sunil Ghelani, a fellow in cardiac imaging.

“It felt like home there,” says Violeta. “My husband and I continued to work from our computers.” (Violeta is a lawyer and her husband works for a construction equipment company.) “We never quit!”

Paz’s first open heart surgery 

Maria de la Paz was born on Sept. 7, 2011, and had her first open-heart surgery was six days later. “The surgeon [Dr. Christopher Baird] explained everything. He used a white board to draw pictures and explain, ‘This is a normal heart, this is Paz’s heart, this is what we’re going to do.’ It was very comforting to be part of the recovery team,” recalls Violeta.

Forever friends
While in the hospital, the Cazal Gustales met a family from Uruguay whose daughter was born the day before Paz. The little girl did not have HLHS, but she did have cardiac disease that affected the left side of her heart and needed surgery.
“Our families are forever friends,” says Violeta. “We call each other on the girls’ birthdays, and just had a reunion this summer (winter in Boston)!”

The new mom was pleasantly surprised to learn she could visit Paz as much as she wanted. In Paraguay, she says, parents and visitors are allowed to spend one or two hours a day with an infant after cardiac surgery.

Orlando adds, “Every single person that we met at Boston Children’s was wonderful and friendly: the doctors, the nurses, the people in charge of the Yawkey Family Inn and the Center for Families.”

Paz stayed one week in the ICU and left the hospital three weeks later. Her family stayed in Boston for about a month and a half after the surgery for a grand total of almost three months.

Homecoming and HLHS follow-up

Paz, Pilar, and Stefano
Paz with her sister Pilar and cousin Stefano

Violeta and Orlando had met with Paz’s pediatrician, Dr Antonio Arbo, before they left for Boston, and he kept in contact with her Boston doctors throughout the family’s stay. When the family returned home, the doctor was prepared to care for Paz, armed with her full history and follow-up care plan. Paz’s local care team also included cardiologist Dr. Renee Szwako and echocardiographer Dr. Leticia Gutierrez.

The first three to six months after a child’s first HLHS surgery are a crucial time for healthy development. Parents must be vigilant about monitoring feedings, weight and any sign of failure to thrive.

“We had a binder,” says Violeta, referring to the handbook created by the Heart Center. “But mostly, we went back to normal life. The only thing was, I’m from a big family, and we couldn’t go to any events for a while to protect her from any illness that might complicate the recovery.”

When Paz was six months old, the family returned to Boston for her second surgery, which Violeta describes as “fast and easy.” Paz had her final surgery when she was three years old.

Today, Paz is a curious, happy, healthy young girl who loves drawing, writing and taking ballet classes. She enjoys playing with her younger sister Pilar and her five cousins.

Paz is the only person in her entire country who has had all three HLHS surgeries. Her doctors in Paraguay are still connected with her doctors at Boston Children’s.

“I feel like an ambassador for Boston Children’s here in Paraguay!” says Violeta. “I want people to know you can go back to your home country and to a normal life with the best results.”

For more information about fetal cardiology and resources for expectant parents, visit bostonchildrens.org/fetalheart.

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