Emile Bacha, MD, Chief of the Division of Cardiac, Thoracic, and Vascular Surgery at NewYork-Presbyterian/
Jack Foley is one of these cases. When his mother was five months pregnant, she found out that Jack had HLHS. Being from the New York area, his parents were urged to meet with Dr. Bacha to discuss what they could expect once Jack was born and to plan for his future surgical needs that would give him the best chance of being able to live a full life.
Below Dr. Bacha discusses HLHS, the surgical approach he took to correct Jack’s heart defect, and how Jack’s recovery has inspired him in his career.
What is hypoplastic left heart syndrome (HLHS)?
When a baby is born with HLHS, it basically means that they’re missing the left side of the heart, which is critically important as the left side pumps oxygen-rich blood to the body. When the baby begins to grow in the womb, two small openings develop between the right and left side of the heart start– the foramen ovale and ductus arteriosus. In healthy babies, these openings close within the first few days of life.
In babies with HLHS, when the foramen ovale and ductus arteriosus close, the right side of the heart cannot get oxygen-rich blood to the rest of the body, causing the babies to have trouble breathing, a weak pulse, ashen skin color, and a pounding heart. This is fatal without both medical and surgical intervention.
Illustration showing hypoplastic left heart syndrome
It is essential that we prevent these holes, or connections, from closing, so we immediately place babies with HLHS on medications to keep them open. We also have to perform surgery swiftly, within the first few days of life, to bypass the malformed or missing left side of the heart and reconstruct the heart in such a way as to allow it to pump blood to the rest of the body. This operation is called the Norwood procedure and is the first of a series of 3 operations that have to occur perfectly for a good survival. These operations are called the Norwood, performed at birth, the Glenn, performed at around 6 months of age, and the Fontan, performed at around 2-3 years of age.
When a family comes to you with an infant with HLHS, how do you approach the case?
HLHS is typically diagnosed in utero, so families will come to me before the baby is even born to seek my help. I sit with them and have an honest conversation about the seriousness of the condition and what it will take to correct this severe malformation. However, I also like to give them hope and explain that, at NewYork-Presbyterian, we have pioneered advanced, minimally invasive techniques for correcting congenital heart defects. The mortality for HLHS procedures globally is around 20-30%, but our mortality is in the range of 5%.
Can you elaborate on the surgeries you performed on Jack so that he would be able to lead a normal life?
Infants with HLHS typically undergo a three-step procedure that has been used since the early 1980s and allows us to create normal blood flow into and out of the heart. At NYP, we typically perform between 60 and 70 of these surgeries a year.
In Jack’s case, the Norwood procedure took place four days after his birth. The Glenn procedure took place when Jack was 4.5 months old. And the Fontan procedure took place when Jack was two-and-a-half years old. Essentially, these surgeries reconstructed the right side of his heart to work as a left-sided heart, allowing oxygen-poor blood from his lower body to go directly to his lungs and for high-oxygen blood to go into his heart.
Baby Jack after the Glenn procedure
What was so special about Jack and his recovery from HLHS?
Jack did remarkably well. Typically, these surgeries require lengthy hospitalizations afterward. But after his first surgery, I was so impressed that Jack was able to go home after only a week-and-a-half, partially due to the strength of his heart and his character and fighting spirit even as an infant. And he continued to recover just as well in the subsequent procedures. The care team at NewYork-Presbyterian took to calling him “Super Jack” with how well he recovered.
Dr. Bacha with Jack at age three
Even a decade later, Jack is thriving. The hearts of HLHS patients who undergo these surgeries typically wear out quicker than a normal heart; however, we’re not seeing any signs of Jack’s heart wearing out. His heart muscle continues to be strong, as does his courage and determination. He has no restrictions – he can play contact sports and live a normal life. That includes spending a lot of his time doing what he loves most – playing ice hockey with his teammates.
What is your relationship like now with Jack and his family?
When I see Jack doing so well, it makes me even more energized to help the next baby with a congenital heart defect. Staying in contact with my patients’ is so important to me as it gives my career a deeper meaning. I am still very close with Jack and his family. Going through a series of very serious surgeries at a young age has an impact not only on the child but on the parents as well. His parents have been wonderful at keeping me engaged with Jack’s life. We are connected on social media, and they give me updates there. I hope to get to see him play in a hockey game soon.
Jack now at age 12
What enables you to excel in the treatment of congenital heart defects like HLHS?
We have an incredible multidisciplinary team of experienced and dedicated doctors and other healthcare professionals who treat a variety of children with congenital heart conditions with high levels of success. The NewYork-Presbyterian Pediatric Heart Center’s combined pediatric program is one of the largest and most well-known pediatric cardiology and cardiac surgery centers in the United States. We are also home to the first infant cardiac intensive care unit (CICU) dedicated exclusively to the care of newborns and infants with congenital heart disease.
We invest an enormous amount in resources and training physicians, surgeons, nurses, anesthesiologists, pulmonologists, and others caring for these tiny patients. We are all deeply committed and passionate about providing the best care and outcomes possible for our patients and our success rates are a result of that. My vision and goal, above all, is to continue learning and improving the ways we treat these conditions so that all children, even those with the most complex heart problems, can live full, active lives.
