Children born with congenital heart defects are living longer, thanks to a better understanding of the conditions, improved surgical and interventional techniques, and dedicated nurses in all settings who make a difference in these childrens lives.
Years ago, many children died from congenital heart disease because there werent as many options, said Melissa Barry, RN, BSN, MA, staff nurse in outpatient pediatric cardiology at HackensackUMC in Hackensack, N.J. Its amazing to see the change in children.
For instance, patients may be in congestive heart failure preop and return to the cardiology clinic postop feeling well, smiling and laughing.
I have an opportunity to see amazing things happen, just watching kids come in not in the best of shape and having the ability to recover them, said Svetlana Streltsova, RN, BSN, CCRN, charge nurse in the Pediatric Cardiac Intensive Care Unit of NewYork-Presbyterian Morgan Stanley Childrens Hospital, Manhattan. Its a delightful feeling to see everything we can do for these children so they can live normal lives.
Congenital heart defects are the most common among birth defects, affecting nearly 1% of births, or 40,000 babies each year, according to the Centers for Disease Control and Prevention. Some require immediate surgery, while others go home, grow a little and then return for a surgical repair.
A National Center on Birth Defects and Developmental Disabilities study reported in 2013 in the journal Pediatrics reported that survival of babies born with critical CHDs increased from 67.4% in 1979-1993 to 82.5% in 1994-2005. A 2010 article from the same CDC agency in Circulation reported a 24.1% overall decline in mortality for patients with CHD from 1999 to 2006.
Mary Berry LeBoeuf, RNFA, APNC, MSN, coordinator of pediatric and congenital heart surgery at Barnabas Healths Childrens Hospital of New Jersey at Newark (N.J.) Beth Israel Medical Center, credited improved surgical and perfusion techniques.
We realize now some things need to be repaired earlier in life before there is unresolvable damage to the heart, lungs or other organs, LeBoeuf said, explaining that includes an arterial switch procedure for children with transposition of the great arteries. Defects are (repaired) before there are sequela.
Streltsova attributes better outcomes to neonatal screening, new technologies, better surgical techniques and strong ICU nursing care. Nurses are good in anticipating the care the patient will require and how they will tolerate ventilator support or sedation, Streltsova said.
Nurses contribute to positive outcomes, said Heather Robson, RN, of The Childrens Hospital at Montefiore in the Bronx, adding the heart plays a big role in how the brain, lungs and other organs are perfused, and the challenges patients face. Nurses are the first line of defense in taking care of a patient, Robson said.
Early diagnosis helps
After delivery, all newborns at Morgan Stanley Childrens Hospital are monitored with pulse oximetry to check for desaturation between upper and lower extremities, which could be indicative of a CHD and warrant a further workup, Streltsova said.
Barry reports more babies are being diagnosed in utero.
An ultrasound might identify a problem, and a fetal echocardiogram between 18 and 20 weeks gestation can confirm a diagnosis. Mothers can continue to be monitored at a cardiology clinic, parents may receive genetic screening, and a cardiologist will see the baby after delivery.
The nice thing is the family has a plan, said Barry, part of an interdisciplinary team to educate parents about a defect, surgery and what to expect postop. We comfort them when they need it most, and they leave our office with answers, a plan and a sense of empowerment.
Many closures are done in the cath lab, going up through a large vein in the leg, said LeBoeuf, who specifically mentioned artrial-septal defects and patent ductus arteriosis closures.
Betsy Tirado, RN, BSN, patient care coordinator in the cardiac cath lab at Montefiore, said atrial-septal defects often can be corrected with an interventional procedure. The most complex is the melody valve to replace the pulmonary valve. You are able to do an intervention on a moving heart, she said.
Some repairs can be performed with minimally invasive procedures, but many children require open-heart surgery. Postop patients may struggle with feeding, wound healing or extubation, Streltsova said. Newark Beth Israel and Montefiore offer hybrid surgical options, where children can have a catheterization and an open-heart procedure at the same time.
Long-term follow up
About 1 million adults born with a CHD live in the U.S., according to a 2013 report in Pediatrics. They require follow-up care for life because they may develop other health problems, such as dysrhythmias or infective endocarditis. Some might need medications or additional surgeries. According to the American Heart Association, certain patients may need to take antibiotics before dental procedures. Others may need to take anticoagulants. Some who develop congestive heart failure may need diuretics or digoxin, while patients who develop arrhythmias may need anti-arrhythmic medications or anticoagulants. Some may need radiofrequency ablation or surgery to interrupt abnormal electrical conduction. They also may need pacemakers or implanted defibrillators.
Forty years ago, kids with certain congenital defects, there wasnt much we could do, LeBoeuf said.
Now those children live full, active lives.
The best part of my job is having a relationship with these people, Barry said.
Debra Anscombe Wood, RN, is a freelance writer.