Girl faces quadruple threat—a viral illness, heart failure, pulmonary hypertension, and a rare heart defect—and prevails
When Marbella and Jadiel Rodriguez brought their baby girl, Zoraya, home from the hospital, they didn’t know that a congenital heart defect was lurking inside her chest. It hid by mimicking symptoms of a viral illness—a croupy cough, fast breathing, and a poor appetite.
“For eight months, we thought everything was normal. She had little symptoms here and there that were easily explained away,” says Jadiel.
Marbella knew something was wrong when Zoraya got to the point where she could hardly keep a little liquid down without throwing up. “It was a red flag, but everyone said she was fine,” she says.
Then Zoraya got a cough that wouldn’t go away. Her parents took her to their local emergency department near Boise, Idaho. She was diagnosed with pneumonia, which her mom now suspects was pulmonary hypertension—high blood pressure in the lungs—a condition that can accompany heart failure.
“The doctor asked, ‘Has anyone ever said she had a heart murmur?’ He said it was probably nothing, but they did an echocardiogram to be safe. That’s when they pronounced she had a heart defect, and things blew up from there,” Marbella says.
A pediatric cardiologist from St. Luke’s System in Idaho, a partner hospital for the Betty Irene Moore Children’s Heart Center at Stanford Medicine Children’s Health, quickly referred them to Stanford Children’s for advanced care, and Zoraya was airlifted that day.
“At Stanford, we found out just how severely sick she was and how dangerous her heart defect could be,” Jadiel says.
Receiving care from a large team of experts
Zoraya was on the verge of cardiac arrest the day she arrived. A team of specialists quickly assembled. Epinephrine and a defibrillator were on standby for fast action. Little did the family know that Zoraya was beginning a 70-day stay in the Cardiovascular Intensive Care Unit (CVICU) at Stanford Children’s.
Not only was Zoraya in advanced heart failure with severe pulmonary hypertension, but also she was fighting two viruses: the human parainfluenza virus, which causes a common respiratory infection in babies and young children, and rhinovirus, which causes the common cold. Her care required close collaboration among several experts who met daily to discuss next steps, including pediatric cardiologists, pulmonary hypertension specialists on the Center for Advanced Lung (CEAL) Therapies team, and heart surgeons.
“Her pulmonary arteries grew abnormally, in that her right pulmonary artery branch arose from the aorta in a condition known as discontinuous pulmonary arteries. It’s a rare birth defect, and it can be easily missed,” says Rachel Hopper, MD, a pediatric cardiologist and associate director of the nationally leading Pulmonary Vascular Disease Program and co-director of CEAL. “The abnormal blood flow caused pulmonary hypertension, which had likely been gradually getting worse with time.”
Zoraya was in a tricky spot of needing heart surgery and cardiac catheterization but being too sick upon arrival to receive it. Her congenital heart defect wasn’t causing the heart murmur; it was her severe pulmonary hypertension and her dilated heart that were making one of her heart valves leak.
“Our teams are used to taking care of very sick kids, especially those with difficult abnormalities, and we have outstanding experience in heart and pulmonary care,” Dr. Hopper says.
“During rounds, we received input from everyone, and everyone’s opinion counted,” Jadiel says. “It was amazing to be a part of my daughter’s care.”
Receiving four heart surgeries in one month
Two weeks after she arrived, Zoraya received the first of four heart surgeries—placing a pulmonary artery band to control blood flow to her overtaxed left lung. The surgery was a stopgap measure to help her gain enough strength to endure the bigger open-heart surgery that she needed to correct her heart defect.
“Our options were limited. She showed up extremely ill and with an acute viral infection, and that’s not a good time for surgery,” says Elisabeth Martin, MD, an exceptionally skilled Stanford Children’s cardiothoracic surgeon and the surgical director of the Pulmonary Artery Reconstruction Program, the Center for Advanced Lung (CEAL) Therapies, and the Pediatric Lung and Heart-Lung Transplant Program.
While the band surgery went well, Zoraya remained acutely ill. Medicines could not control her heart failure, and she was cyanotic—turning blue from too little oxygen. The team needed to perform open-heart surgery right away to save her life.
“It was a game-time decision. Her lungs were as good as they were going to get after the viral infection. It was time to move forward,” Dr. Martin says.
Most babies with discontinuous pulmonary arteries have surgery soon after birth, so her doctors were surprised that Zoraya made it to eight months. Her left lung was damaged from months of receiving extra blood from the aorta, and the infection made everything worse. Even though her open-heart surgery—a hemitruncus repair—went as planned to reposition her right pulmonary artery branch in the right position, her lungs remained sick and unable to process enough oxygen.
Zoraya was placed on extracorporeal membrane oxygenation (ECMO)—an advanced heart-lung bypass machine that helps keep children alive when their heart and/or lungs are failing.
“She had four surgeries in one month,” Dr. Martin says. “I’m grateful to be at Stanford Children’s, where we have the support, technology, and brainpower to find a path forward even when so much goes wrong at once.”
Receiving extra support along the way
Zoraya’s time in the CVICU was a roller coaster of emotions for her family. Despite their constant worry, they believed in the team’s skills and appreciated the extra support they received from nurses, respiratory therapists, and social workers so they could simply focus on their daughter. Zoraya’s care was led by Rebecca Kameny, MD, an exceptional pediatric critical care medicine doctor and the co-director of CEAL.
“You could tell the team was locked in. They had their flow and organization, and they all kind of melded together to care for her,” Marbella says.
Despite the added ECMO procedure, three more weeks on the ventilator, and two more weeks on a continuous positive airway pressure (CPAP) machine, her family felt hope. “Even though she was on so many machines, I was happy. I knew she was back,” Marbella adds.
Zoraya was sent home on oxygen and a special medicine pump for her pulmonary hypertension. She is not expected to need another heart surgery in the future.
Coping with the trauma
It has been over a year since her hospital stay, but Marbella and Jadiel are still a bit shell-shocked. If Zoraya gets a cold, they get nervous, but they are learning to trust that she’s OK. “We were worried she wouldn’t be able to recover, but she is recovering,” Jadiel says.
Zoraya’s providers at Stanford Children’s collaborated closely with providers near home. Because Dr. Hopper is licensed in several Western states, she still leads Zoraya’s pulmonary hypertension care. Nurse practitioners with the Pulmonary Vascular Disease Program also coordinated her special pulmonary hypertension medications and supported her parents via telehealth with home administration by always being available to answer questions.
“Her family is wonderful and they are great partners,” says Esther Liu, FNP-C, pulmonary hypertension nurse practitioner. “When families live out of state, it’s important to have open communication with local cardiologists and to meet with the family regularly to ensure children receive the best care.”
Meeting milestones and loving life
Zoraya recently turned 2 years old. While she’s still on one medication for pulmonary hypertension, she is off the medication via pump and off oxygen support.
“She’s running everywhere and climbing on everything. She has caught up completely on all her milestones—faster than we thought,” Marbella says.
When Zoraya started feeling better, her sassy, sweet personality really popped. She’s super-social and loves making people laugh. One of her favorite activities is snuggling with her big sister and hero, Xiomara.
“Zoraya has made tremendous progress. She’s a thriving toddler now. We are optimistic that she will someday outgrow her pulmonary hypertension,” Dr. Hopper says.
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