Lifesaving advances in pediatric cardiology

Throughout history, there have been a number of advances that have helped prolong the lifespan of infants and children with congenital heart disease. What would have been fatal as little as 25-30 years ago is now frequently treatable, with many of these young patients expected to live a normal life span.

A few of the most important historical advances include the discovery of prostaglandin, a compound which helps newborns with serious congenital heart disease survive while they await cardiac surgery. Another key advance is “staged reconstruction,” a series of three surgeries done over a baby’s first few years that gradually reconfigure the heart and circulatory system. These procedures are used to repair many types of single ventricle heart defects.

More recent advancements include the following:

  • Ventricular-assist devices benefit children with poor heart function by helping their hearts pump blood while they await heart transplantation.
  • Improved ability to image young hearts allows more accurate diagnosis and management planning through 3D echocardiography, cardiac MRI and cardiac CT scanning.
  • New devices enable doctors to close some holes in the heart (atrial septal defects and some ventricular septal defects). These devices can be placed through a catheter without requiring open-heart surgery.
  • Heart valves that can be placed through a catheter in some cases.
  • “Hybrid procedures” where some interventions are performed through a catheter while others are simultaneously performed by surgery, which has allowed interventions to be performed in some infants who could not be helped with either catheter procedures or heart surgery alone.

In addition to these advancements, increased awareness of congenital heart disease in the public has facilitated public health initiatives that have improved outcomes of children with heart disease. An example is the proliferation of AEDs in schools and at sporting events. Also, the passing of the Emerson Rose Act in 2013 mandated screening for serious congenital heart disease for all infants in South Carolina prior to discharge from the nursery.

It is difficult to predict what improvements will be most successful or when those improvements might happen. I am excited about the possibility of newer transcatheter devices that would allow more heart disease to be treated without surgery and/or with fewer potential complications.

Likewise, my colleagues and I are excited about the possibility of stem cell treatments for heart failure and possibly also some forms of congenital heart disease.

I think the biggest fairly recent improvement in treating children with heart disease has been the cooperation of a large number of major children’s hospitals internationally to allow research into best practices. An example of the benefit of this cooperation is the single-ventricle interstage monitoring program here in South Carolina. This program allows potential problems to be identified sooner than would otherwise be possible even with the very frequent cardiology visits that these patients have.

Through the project, single ventricle patients have their weights and oxygen saturations measured regularly at home, with the values reviewed by a care team in order to spot potential problems as early as possible. This program has significantly reduced the number of single ventricle patients who die between their first heart surgery and their subsequent heart surgeries.

While it is still experimental and only considered in very specific circumstances, fetal interventions are sometimes done to try to address serious heart problems prior to birth. Specifically, some unborn babies who appear to be at risk for developing hypoplastic left heart syndrome due to severe aortic valve stenosis have undergone a fetal procedure in an attempt to open the aortic valve using a balloon. This has only been done in a fairly small number of patients, and the results have varied. But as the procedure is refined, this type of fetal intervention may become more common.

Jon Lucas, MD, FAAP, FACC, is division director of Pediatric Cardiology for Prisma Health Children’s Hospital–Upstate. Prisma Health has pediatric cardiology practices in Greenville, Spartanburg, Greenwood and Anderson.

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