The Heart Center - Arkansas Children's Hospital

Angela Green , PhD (c), APN; Research Nurse; Arkansas Children's Hospital; Clinical Assistent Professor; UAMS College of Nursing

As survival has improved for children with even the most complex congenital heart defects (CHD), quality of life has become an increasing concern for parents and professionals. Despite this, there are few published research studies focusing on quality of life (QOL) in children with CHD. Drawing conclusions from the existing research is challenging for a number of reasons including inconsistent definitions of QOL, inconsistent methods of measuring QOL, and reliance on parent informants. While there is no universally accepted definition of QOL for children with chronic medical conditions, parents and professionals agree that QOL is multi-dimensional and includes at least psychosocial and physical aspects.

Acknowledging these limitations in the research, we still can use that research to inform practice and future research studies. Studies generally report that children with CHD have positive perceptions of their QOL and that it is similar to that of children without CHD. There is some evidence that children with complex CHD, such as single ventricle, may have lower QOL than children with less complicated heart disease. Even in children with favorable descriptions of their QOL, issues have been identified that impact QOL. One of these issues is that children often feel different from their peers both in terms of physical appearance and ability to participate in recreational activities. Troubling differences in physical appearance include sternotomy or thoracotomy scars, small stature, cyanosis, and/or edema. Participation in activities may be restricted by parents and/or the health care team. Further, some children with CHD, even if allowed to participate, may be unable to keep up with their peers. Additional issues that may impact QOL include symptoms, need for hospitalization, and absences from school.

Parents and professionals can work together to help children with heart problems have the best QOL possible. One of the most important ways is to keep the child as healthy as possible so he or she can participate fully in school, family, and recreational activities. This includes administering medication and scheduling follow up as recommended by the child’s health care team. Most children with CHD do go to school and participate in at least some age-appropriate activities. School is important for both academic and social reasons. Therefore, efforts to minimize absences from school are crucial. In terms of decision making about recreational activities, parents should seek the advice of their health care team in early childhood and then seek approved activities that match the child’s interests. Parents can also help children by providing opportunities to socialize with peers beginning at an early age. They can also help children see that everyone is different and teach the child to view potentially troubling differences, such as a sternotomy scar, as a trait that differs, just like eye or hair color. Finally, the medical team and the family can work to minimize the impact of the treatment regimen on the child’s and family’s activities.

More research is needed in this area, particularly research that seeks the children’s own perceptions of their QOL at an early age. This is crucial because early identification of issues that negatively impact QOL can provide the basis for interventions to maximize QOL for children with CHD. At Arkansas Children’s Hospital, we are currently studying the QOL of school-aged children after cardiac transplantation and hope to identify the key factors that they believe impact their QOL. Similar studies are needed with children with CHD at all stages of repair. This is particularly true for complicated lesions such as hypoplastic left heart syndrome or other forms of single ventricle.

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Krol, Y. et al. (2003). Health related quality of life in children with congenital heart disease. Psychology and Health, 18 (2), 251-260.

Magnetic resonance imaging and multi-detector computed tomography for congenital heart disease

S. Bruce Greenberg, M.D.; Professor of Radiology; University of Arkansas for Medical Sciences and Arkansas Children's Hospital

Magnetic resonance imaging and multi-detector computed tomography are playing an increasing role in the care of children and adults with congenital heart disease. Black blood double inversion imaging and gadolinium enhanced magnetic resonance angiography are used to evaluate morphology. Gadolinium enhanced magnetic resonance angiography results in images similar to conventional digital angiography but does not require invasive catheterization or ionizing radiation. The 3D data set allows for multiplanar reformations and 3D volume rendering from any viewpoint. These images are useful for coarctation of the aorta, vascular rings, pulmonary artery stenosis, and pulmonary vein anomalies.

Magnetic resonance gradient cine imaging of the heart is the gold standard for cardiac function. This is especially important following surgery for tetralogy of Fallot to determine right ventricle function and quantify pulmonary regurgitation. Flow analysis is a magnetic resonance technique used to measure blood flow and velocity similar to Doppler echocardiography, but is not hampered by window limitations caused by bone or air. The technique is useful for evaluation of pulmonary or aortic regurgitation, coarctation, and flow mapping of Fontan palliations.

Multi-detector computed tomography has revolutionized computed tomography in recent years. Prior to thedevelopment of multi-detector computed tomography, computed tomography was too slow to image the moving heart. The current generation of multi-detector computed tomography scanners allow for high spatial resolution images of the heart and great vessels. Cardiac gating can be used to create a data set allowing for cardiac function measurement.

Eudice E. Fontenot, M.D.; Associate Professor of Pediatrics (Cardiology); UAMS; Arkansas Children's Hospital

The physician, nursing and x-ray technician team in the Pediatric Cardiac Catheterization laboratories at Arkansas Children’s Hospital perform approximately 500 procedures per year. Cardiac catheterization is a special, invasive test whereby a long narrow tube known as a catheter is inserted into an artery or vein and passed into the heart. With the aid of fluoroscopy, the catheter can be manipulated into the cardiac chambers and vessels where oxygen levels and pressures can be measured. Additionally, an angiogram can be performed during the procedure. An angiogram involves injecting contrast material or dye through the catheter into the heart chambers and vessels. The dye, which can be seen with the cath lab x-ray equipment, is filmed as it is pumped through the heart chambers and vessels. A permanent record of the angiograms is made and these records can then be reviewed at any time by members of the heart team to help make decisions regarding patient care.

Pediatric cardiac catheterization has evolved over the last few decades to be used for more than just establishing a diagnosis or gathering information. There is now a new subspecialty within pediatric cardiology known as interventional cardiology. Cardiac catheterization can now be used to treat many conditions that previously would have required surgery or even some that were untreatable. The first recorded reports of catheter techniques used to treat congenital heart disease date back to the early 1950’s.

In 1953, Dr. Rubio-Alvarez and his team reported using a catheter designed to work in the urinary tract to perform a pulmonary valvotomy. In 1966, Drs. Rashkind and Miller published a paper in the Journal of the American Heart Association describing creation of a hole between the upper chambers of the heart without surgery. Their technique had been developed for palliation in infants born with transposition of the great vessels. This is an abnormality where the two large arteries that arise from the heart are switched so that the red blood goes back to the lungs and the blue blood goes back to the body. These infants do not survive unless there is someplace inside their heart where the red and blue blood can mix. Prior to 1966, if babies with transposition were not born with a communication in the heart to allow the blood to mix, the only way to make this communication or hole was with a difficult and complicated operation. Dr. Rashkind and his colleagues showed that one could safely make a hole in the upper chambers of the heart without surgery. As you might expect, this procedure revolutionized the care of infants with transposition and started the field in cardiology we know today as interventional cardiology.

An atrial septal defect is a hole in the wall that separates the upper chambers of the heart. This defect is a relatively common congenital cardiac condition representing about 6-10% of all cardiac abnormalities that are encountered. Atrial septal defects are two times more common in females than in males. There are different types of atrial septal defects, but the most common type, the secundum defects, are the type that are potentially closed in the cardiac catheterization laboratory.

In 1976, Dr. Terry King, a pediatric cardiologist at the Oschner Clinic in New Orleans along with Dr. Noel Mills, a heart surgeon at the Oschner Clinic, was the first to successfully close an atrial septal defect in a human without surgery. The patient was a 17 year old young lady who did very well and according to a recent communication from Dr. King is still doing well today. Drs. King and Mills went on to do four additional patients after this first 17 year old patient. This was the first report of a catheter based technique to repair a congenital heart defect. Dr. King and colleagues were the first to show that non-operative closure of an atrial septal defect could be an attractive alternative to open heart surgery in selected patients.

The team at Arkansas Children’s Hospital has been performing transcatheter closures of atrial septal defects now for several years. The first device used was known as the “Angel Wings” device. This device was being used as part of a research study controlled by the Food and drug Administration. Once that trial was closed, the “Angel Wings” device was no longer used here at Arkansas Children’s Hospital. Recently, a new generation device, the Amplatzer Atrial Septal Occluder has gained FDA approval for closure of atrial septal defects in children. The approval of this new medical device for use in children marked another milestone in pediatric interventional cardiology. The new device has two opposing discs that are made of a wire mesh. The wire material is nitinol, a nickel and titanium alloy. Nitonol has a wonderful memory property that makes the device particularly suitable for use in children. The device can be compressed into a very small catheter, then when extruded, the device will “remember” its original shape and reform to its original design. A soft Dacron fabric is woven into the discs. When in place across the atrial septum, the discs will occlude the atrial defect and the Dacron fabric helps to trap red blood cells and platelets to help completely close the atrial defect. The device is available in varying sizes, from very small to very large.

Children with secundum type atrial septal defects and who are candidates for device closure are scheduled for an outpatient elective cardiac catheterization procedure. The patients are taken to the cardiac catheterization laboratory where they are put to sleep by the pediatric cardiac anesthesiologist. Once asleep, a special echocardiogram known as a transesophageal echocardiogram is performed. A detailed look at the atrial septal defect is performed along with measurement of the defect and the surrounding tissues. This echocardiogram is reviewed by the team and if the defect is felt to be suitable for device closure, the catheterization procedure continues. A catheter is then passed from the large vein in the groin into the heart. The catheter is directed across the atrial defect into the left upper heart chamber and the defect is then sized. An appropriate sized device is then chosen and deployed across the defect. One disc is opened on the left side of the atrial defect and the other disc is opened on the right side of the defect. When released, the discs squeeze together and close the defect. The Amplatzer device, in addition to being made of nitinol, has a unique property in that the device is easily retrievable prior to its release. Once the operator is satisfied with the device position, the device is released. All of the catheters are removed and a large bandage is placed over the groin. The patient is awaked from the anesthesia and is recovered in the hospital overnight. The next morning, the patient has a chest x-ray and echocardiogram performed and is then allowed to go home.

After six weeks, the device becomes covered with the lining of the inside of the heart and the patient is allowed to resume normal activity. The device is designed to stay in the heart forever. Patients who have had their atrial defects closed with this device are seen in the cardiology clinic once per year or once every other year after the device has been placed.

In addition to closing atrial septal defects, the pediatric interventional cardiology team at Arkansas Children’s Hospital performs many other interventional procedures such as balloon dilation of aortic and pulmonic valves, balloon dilation and stent enlargement of narrowed vessels, along with closing unwanted communications between the arteries and veins. The field of interventional cardiology has grown in the past 50 years to now routinely offer children with certain cardiac defects a non-surgical alternative to treat these defects. Hopefully, as technology continues to improve, we will be able to offer catheter based therapy to more and more children.

Bryan Watkins, M.D.; Assistant Professor of Anesthesiology; Section of Pediatric Cardiac Anesthesia; Arkansas Children's Hospital

The Heart Center at Arkansas Children’s Hospital is unique among most pediatric hospitals in that it has four anesthesiologists who care exclusively for children with congenital heart disease who are undergoing cardiac and noncardiac procedures. When you meet the anesthesiologist who will care for your child you know that he will be putting your child to sleep for the surgery or procedure. What you probably do not realize is that after your child is asleep, the anesthesiologist will be responsible for placing invasive monitoring lines into your child which can be used to measure continuous blood pressure, pressures within the heart, blood gases and other laboratory values, and provide more reliable venous access for administering fluid, cardiac drugs, and blood products.

Invasive monitoring: In addition to the more standard monitors, which consist of EKG, non-invasive blood pressure, pulse oximetry, end tidal CO2, and temperature, many children with congenital heart disease require additional monitoring such as a catheter placed into an artery and a catheter placed into a major vein entering the heart. The arterial line is usually placed in either the radial artery in the wrist or the femoral artery in the groin. The arterial line enables the anesthesiologist to measure blood pressure continuously and allows for blood gas analysis as well as the measurement of other laboratory values during the operation. A central venous catheter placed into a major vein either in the internal jugular vein in the neck or the femoral vein in the groin enables the anesthesiologist to measure the pressure in the right atrium of the heart which helps to guide him in the amount of fluid or blood the patient may need during the operation. The central venous line also provides a more reliable route for administering fluid, additional drugs and infusions, and blood products.

Placement of invasive monitors: Adults undergoing cardiac surgery often get these same invasive lines. Typically they are placed by anesthesiologists who use external landmarks on the body of the patient to guide them in finding the blood vessels. Once the needle is under the skin the anesthesiologist is relying on his or her knowledge of anatomy to determine that they are in the right place. Generally, this technique works very well in adults who have much larger blood vessels and a greater margin for error than our pediatric patients. For the past five years anesthesiologists at the Children’s Heart Center have been using a real-time 2-D ultrasound device about the size of a laptop computer that allows them to place these invasive monitors under direct visualization. A small ultrasound probe is placed in a sterile sleeve and then placed on the skin over the vessel they wish to cannulate. The anesthesiologist can actually see the blood vessel underneath the skin and watch his needle and catheter go into the blood vessel, taking all the guess work out of the procedure. This technique is used routinely on all patients with congenital heart disease who need invasive monitoring, some weighing as little as two kilograms (a little over four pounds).

Previously published complication rates in children undergoing invasive line placement without ultrasound guidance range from 2.5 to 22%. Newborns and infants have a higher degree of anatomical variation in the position of their blood vessels and represent a high risk population for invasive line placement. Complications can range from a small insignificant bruise under the skin to more serious complications which can result in cancellation of the surgery or lead to postoperative complications. Studies comparing the use of ultrasound to the landmark technique in the pediatric population specifically have not been done, but analysis of studies combing adult and pediatric patients indicate a reduction in failed placement by 68% and a reduction in complications by 78%. These reductions may be even higher in the more challenging pediatric population.

Richard T. Fiser, M.D.; Associate Professor, Pediatric Critical Care and Cardiology; University of Arkansas for Medical Sciences; Medical Director, ECMO Program; Arkansas Children's Hospital

In 1989, Arkansas Children’s Hospital joined a small but growing number of pediatric centers offering the supportive technique of extracorporeal membrane oxygenation (ECMO) for acute, reversible respiratory and cardiac failure in neonates and children. By that time, ECMO was becoming a more commonly used modality to support the acutely failing lungs or heart, particularly in neonates. In that same year, the Extracorporeal Life Support Organization (ELSO) was chartered to oversee and supervise a registry of patients supported with ECMO, to help standardize approaches to care, and to stimulate research into respiratory failure and its therapies. Arkansas Children’s Hospital was one of the original members of ELSO. Now, 17 years later, the ECMO program at ACH is one of the nation’s busiest, having supported almost 650 patients with a variety of diseases causing acute cardiorespiratory failure.

Essentially, ECMO is a cardiopulmonary bypass circuit, in some ways similar to that used in the operating room during open-heart surgery, that is modified for long-term support. Surgically inserted tubes drain deoxygenated blood out of the patient’s right atrium. The blood is pumped through a circuit that includes a membrane oxygenator, or “artificial lung,” and returned fully oxygenated to the body. The pump can circulate blood through the system and back to the body at a rate equal to or greater than the patient’s normal cardiac output. A photograph of the ECMO circuit and equipment is shown below. ECMO support is not without substantial risks, most of which relate to bleeding. Since blood that is exposed for long periods of time to plastic surfaces will clot, successful use of ECMO requires that the patient receive anticoagulants, or blood thinners, to keep the blood from clotting in the circuit. Although the dosing of blood thinner is controlled and monitored very carefully, life-threatening bleeding, including hemorrhaging into the brain, can sometimes occur in patients supported with ECMO. Patients can remain supported with the ECMO circuit for hours to weeks. In some instances, children with certain types of heart conditions may require ECMO for stabilization prior to more definitive surgical interventions, with ECMO support perhaps lasting from a few hours to a few days. Neonates and older children with respiratory failure severe enough to require ECMO may be on the circuit for 1-2 weeks while the lungs heal enough to be supported with a conventional mechanical ventilator. Sometimes ECMO is used to “bridge” children waiting for a heart transplant in order to support their circulation while waiting on a donor heart. In these circumstances, the patient may remain on ECMO for weeks while waiting on a donor heart. However, the risk of serious complications of ECMO certainly increases with longer lengths of time on the ECMO circuit.

ECMO is a hugely labor-intensive undertaking and is the epitome of a “team effort,” requiring dedicated ECMO Specialists as well as physicians, including cardiovascular surgeons, neonatologists, cardiologists, intensivists, and pediatric surgeons. At ACH, our ECMO Specialists are very experienced ICU nurses and respiratory therapists who undergo extensive training to become ECMO Specialists. The ECMO Coordinator at ACH is Lorrie Baker, R.N., who provides the program with exceptional leadership and experience, having been involved in ECMO since the program’s inception. It would be impossible to have a top-notch ECMO program without additional broad commitment and support from the hospital, including blood bank, radiology, pharmacy, laboratory, social work, and pastoral care staff. ECMO represents an enormous mobilization of people and resources to support one critically ill child or adult.

Outcomes of patients supported with ECMO at ACH during 2005 are shown in the table below, as are comparative survival data from all ECMO centers internationally reporting to the ELSO Registry. In all categories, the percentage of patients surviving to hospital discharge at ACH met or exceeded survival rates reported to the ELSO Registry.

*Reason for ECMO Support*	*Number of patients in 2005*	*% Survival to Discharge*	*Average % Survival in ELSO Registry*
Neonatal respiratory failure	9	80%	65%

Pediatric respiratory failure	7	57%	55%

Cardiac support	12	63%	38%

In 2005, 2 patients received ECPR, or ECMO used as rescue during CPR for a cardiac arrest. Both of these patients survived to leave the hospital. Three neonates with respiratory failure from meconium aspiration syndrome were supported with ECMO with 100% survival to hospital discharge. In the PICU, 4 children with acute respiratory distress syndrome (ARDS) received ECMO with a 75% survival rate to hospital discharge, compared to the international ELSO Registry average of 57% survival to discharge for ARDS patients.

Arkansas Children’s Hospital is one of only three institutions in the United States to offer air transport of critically ill patients on an ECMO circuit. The map below demonstrates the range of the ACH Mobile ECMO program from 1989 – 2005. This year, through a relationship with JetMed Corporation, we are hoping to expand the range of our mobile ECMO transport capabilities. The accompanying photograph depicts Carl Chipman, R.N. and Wes Ware, RRT preparing the mobile ECMO equipment to bring a child from Austin, TX back to Arkansas Children’s Hospital.

For 2006-2007, we hope to accomplish three primary initiatives. First, through increased utilization of a heparin-bonded ECMO circuit, we hope to lessen the degree of anticoagulation required to support a patient with ECMO. It is our hope that this change will result in less bleeding risk and less utilization of blood products while on ECMO. Secondly, we hope to expand our capabilities in the arena of “rapid deployment” ECMO, particularly as it relates to the ability to rapidly place a child in cardiac arrest on an ECMO circuit for circulatory support. Although the ACH ECMO program has had substantial success with such patients in the past, we hope to implement changes that will further improve our capabilities in this area. Finally, we hope to expand our ability to offer mobile ECMO as a service to critically ill children and adults throughout the country through our relationship with the JetMed Corporation and its Lear jet.

Trenda Ray, MNSc, APN.; Advanced Practice Nurse for Pediatric & Congenital Cardiothoracic Surgery; Arkansas Children's Hospital

Karyn Haynes has been selected as the new Nursing Director of the Heart Center. Karyn began working at ACH in 1995 as a Student Nurse Assistant and then transitioned to the Heart Center in 1996 as a staff nurse. Karyn worked to become a level IV RN in the Heart Center while also serving as a preceptor to nurses. She has served on the management team for the Heart Center for more than two years and previously as the Interim Assistant Director and Assistant Director.

“We are so privileged to be included in those moments with someone's child, and I still treasure those visits from patients whom I cared for years ago,” says Karen. When asked what she wants others to know about the Heart Center Karyn said, “It takes a special person to give of yourself every day as our nurses and staff have shown. We have a special group of people who work here.”

The Assistant Nursing Director position has also been filled with Eve DeMontmollin. Eve began working at ACH in 1988 as a Student Nurse Assistant and later worked as a new graduate nurse in PICU in 1989. She has worked in the Heart Center for the last 16 years, progressing to an RN IV and most recently as the CV East Nurse Coordinator. During her time as CV East coordinator, Eve helped institute discharge guidelines which improved discharge time by 50 percent. Since serving as Interim Assistant Director, Eve has been reminded time and again of the incredibly talented staff who provide an amazing service to our most precious patients.

Both members of our new management team hope to continue the Care, Love and Hope that is so obviously practiced in the Arkansas Children's Hospital Heart Center everyday.

What is your role at ACH and how long have you worked here?Nurse Manager of Cardiac Surgery

Why is your job rewarding?
Playing a small part in improvement of children with heart defects

How did you become interested in pediatric cardiology or cardiovascular surgery?
Through experience with adult cardiac patients at the VA, I was offered the opportunity to come to ACH when the pediatric heart program started.

What do you want people to know about the Heart Center at Arkansas Children's Hospital?
That it has been active and going strong since 1981

What has been your most memorable moment working in the Heart Center at Arkansas
Children's Hospital?
The ABC series was fun, but my most memorable experience for me in my career was touching a beating heart for the first time.

What is your greatest professional achievement?
Being involved with the first Heart Transplant in Arkansas- though it was performed at Baptist Hospital, our Children’s Heart Team retrieved the donor heart.

Why is your job rewarding?
Knowing what we do in the CVICU changes peoples lives in such a big way.

How did you become interested in pediatric cardiology or cardiovascular surgery?
When I was in nursing school, I worked at another hospital, where I occasionally worked with pediatric patients. I knew I really loved working with kids. When I found out about the job in CVICU, I knew that’s where I wanted to work.

What do you want people to know about the Heart Center at Arkansas Children's Hospital?
I want them to know about our innovative technologies we use and the quality of our staff.

What do you enjoy most about working with children?
Their resiliency as well as seeing them smile and laugh.

What has been your most memorable moment working in the Heart Center at Arkansas
Children's Hospital?
Seeing a patient go from pre-transplant to receiving a heart and being discharged.