Caring for Kids with Congenital Heart Disease

Submitted by Dr. Daphne Hsu, Pediatric Cardiology at White Plains Hospital

One in 100 babies is born with congenital heart disease, an abnormality of the heart that occurs as it develops. Until recently, heart defects were a grim diagnosis for newborns and their families. But in the past two decades, thanks to surgical advances, 98% of those born with heart defects will now survive to adulthood.

However, even those statistics should be a cause of concern. Serious congenital heart defects are often – but not always – diagnosed before or soon after a child is born. Signs that parents should watch out for include:

• Cyanosis: pale gray or blue lips, tongue or fingernails
• Shortness of breath during feedings, which can contribute to less-than-standard weight
• Continued shortness of breath/labored breathing
• Swelling in the legs, stomach, or around the eyes
There are also less serious congenital heart defects, which may not be diagnosed until the child grows older. Those include:
• Being short of breath/easily tiring during physical activity, where before there were no such symptoms
• Fainting
• Selling in the hands, ankles or feet

If any of these symptoms are present, contact your healthcare provider.

As noted, congenital heart disease can reveal itself as a child gets older – or may not show up until adulthood. There are some 20 congenital heart conditions, each with their own characteristics; some of the most common are:

• A ventricular septal defect, which account for about 20% of all congenital heart defects, according to the American Heart Association. These are small- to large-sized holes between the lower chambers of the heart; the larger the hole, the greater the chance is that the infant will develop congestive heart failure. Treatments can include a relatively simple surgery where the holes are sewn shut.

• An atrial septal defect, where an abnormal hole exists between the upper two chambers of the heart, or atria; such conditions are usually asymptomatic. A cardiac catheterization is usually the recommended treatment.

• A tetralogy of Fallot, consisting of an abnormal position or orientation of the ventricular septum, with a hole present between the two lower heart chambers. All cases of TOF require surgical repair at some point, usually when the child is around six months old.
Not that surgery in childhood is not a permanent fix: Valves can narrow; connections can fail; sutures can weaken. And as the heart matures, issues such as arrythmias or heart failure may develop.

Whether a child or adult, the following can help:
• Heart-healthy lifestyle. The same advice for warding off adult-onset heart disease — eat a healthy diet, exercise regularly, and monitor your weight and blood pressure — applies even more to patients managing a congenital condition. Their hearts are already under stress, so anything raising their risk for hypertension, coronary artery disease, or similar conditions is something to avoid.

• Starting a family. Women with congenital heart disease often worry that they will pass their condition on to their babies. While that risk is about five times higher, it’s still a low risk in the broad sense. Early detection can further dispel anxiety. If an anatomy scan in utero detects an issue, patient education on the plan of action begins immediately.

• Upkeep and maintenance. When surgical heart repairs need a refresh or some upkeep, those procedures can almost always be done in a hospital’s cardiac catheterization lab. This minimally invasive approach requires a one-night stay, with far less recovery time than open-heart surgery. Stents may be placed to open up arteries that have been narrowed, other devices can be deployed to close residual holes, and transcatheter valves can be implanted. All of these are minor adjustments that can relieve stress on the heart and help keep it healthy for years to come.

White Plains Hospital has recently opened a Pediatric Specialty Center, where a range of pediatric cardiac and gastrointestinal specialists from the Children's Hospital at Montefiore (CHAM) are available.

Dr. Daphne Hsu is Director of the Montefiore Einstein Adult Congenital Heart Disease Program and a Professor of Pediatrics and Medicine at the Albert Einstein College of Medicine. For an appointment, call 914-849-KIDS (5437).