Q&A: Advancing Care for Children With Congenital Heart Disease

Pediatric interventional cardiology has had meaningful advances over the past decade that are changing how children with congenital heart disease are treated at Cedars-Sinai.

Congenital heart defects, which arise from abnormal or incomplete formation of the heart, valves and blood vessels, are among the most common birth defects worldwide. The condition affects approximately 40,000 babies each year in the United States.

From less invasive, catheter-based procedures to improved imaging and emerging technologies, innovations are helping reduce risk, shorten recovery times and expand treatment options for the smallest patients with heart problems.

The Cedars-Sinai Newsroomrecently spoke with Stephen Nageotte, MD, MBA, an interventional cardiologist and director of the Congenital Catheterization Laboratory at Cedars-Sinai Guerin Children'sand the Smidt Heart Institute. Nageotte treats children and adults using minimally invasive procedures. He shared what's new in the field and what families need to know:

What are the most significant advances you've seen in pediatric interventional cardiology in recent years?

Over the past five years, there have been major advances in three key areas:

• Premature patent ductus arteriosus [PDA] closure. It's a congenital heart condition in which a connection between two blood vessels in an infant fails to close after birth. This can cause abnormal blood flow to the lungs and breathing difficulties, particularly in premature infants.
• Transcatheter pulmonary valve replacement, which is a minimally invasive procedure in which we replace a faulty heart valve using a catheter instead of surgery.
• PDA stenting. Sometimes babies have heart problems that limit blood flow to the lungs. We can keep a small vessel called the ductus arteriosus open by placing a tiny tube, or stent, inside it to improve blood flow.

All three procedures have evolved significantly and are allowing us to treat patients in less invasive ways, often replacing or delaying the need for surgery.

How has transcatheter PDA closure changed care for premature infants?

Transcatheter PDA closure has become an important option for premature infants with a hemodynamically significant PDA-meaning the vessel is causing strain on the heart and lungs. In fact, Evan Zahn, MD, of the Smidt Heart Institute, helped develop the device cardiologists use to close a PDA during a transcatheter procedure.

The procedure is quick and can be done at the bedside in the Neonatal Intensive Care Unit[NICU], which helps us avoid transporting fragile infants. Research also suggests many babies show respiratory improvement after closure, which may reduce complications associated with long-term ventilation.

Who is typically a candidate for the transcatheter PDA closure?

These procedures are generally for premature infants who are showing signs that the PDA is affecting their health, such as needing increased respiratory support or showing enlargement of the heart. There's no strict minimum weight requirement, and the focus is really on whether the PDA is having a meaningful impact on the baby's heart and lungs. Transcatheter PDA closure is also performed on older kids and adults.

What impact has new device technology had on how these procedures are performed?

One recent advancement is an improved delivery system for PDA closure devices. The device, known as the Amplatzer Piccolo Delivery System, recently received FDA [Food and Drug Administration] clearance to treat premature infants with PDA, including babies weighing as little as 2 pounds. Previously, the procedure required exchanging multiple catheters during the case, which could introduce instability; the newer system allows physicians to navigate and deliver the device using a single, shorter, softer catheter, potentially improving patient stability and precision.

How has transcatheter pulmonary valve replacement expanded treatment options for patients?

The development of newer self-expanding valves has significantly increased the number of patients who can receive pulmonary valve replacement through a catheter instead of surgery. These procedures are typically considered from late childhood through adulthood and are used for patients with pulmonary valve disease related to congenital heart conditions. Early outcomes have shown good durability and high success rates.

Imaging plays a big role in heart care. What improvements are making the biggest difference?

There have been significant advances in cardiac CT and MRIs. Imaging is faster, clearer and uses lower radiation doses than in the past. These tools help us better understand heart anatomy, blood flow and timing for interventions. There's also growing interest in using virtual or augmented reality to create 3D heart models for procedure planning, allowing teams to better visualize anatomy before a procedure.

What should families know as they navigate a congenital heart diagnosis?

Early detection and screening are incredibly important, ideally before birth, so families can meet the care team and understand what to expect. At Cedars-Sinai we can identify a defect while a baby is still in the womb and will continue to care for the baby at birth, during childhood, and all the way through adulthood, so the patient never needs to transfer to another institution.

Congenital heart care is lifelong and truly a team effort. Families aren't expected to understand every technical detail-our role is to guide them, answer questions and be there every step of the way. Building long-term relationships with patients and families is one of the most meaningful parts of this work.

Read more from Cedars-Sinai Stories and Insights: Making Strides in Treating the Smallest Heart Patients