Vol 2 Issue 5	September/October 2007
Inside this issue
The Right Ventricular Outflow Tract in Tetralogy of Fallot
ARORA and Life in the Heart Center
Feeding Difficulties and the Infant with Single Ventricle Physiology
Spotlight on Shirley Gadi, Patient Care Tech
Spotlight on Tracey Marek, Radiologic Technician
New Nursing Director Named for CVICU
New Staff for the Heart Center

The Right Ventricular Outflow Tract in Tetralogy of Fallot

Robert D.B. "Jake" Jaquiss, M.D.

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Robert D.B. "Jake" Jaquiss, M.D.; Chief, Pediatric and Congenital Cardiothoracic Surgery, Arkansas Children’s Hospital; Professor, Department of Surgery, University of Arkansas for Medical Sciences College of Medicine

One of the most common serious congenital heart conditions is tetralogy of Fallot, named for the French physician, Etienne Fallot, who provided one of the earliest descriptions of its features in 1888. There are four cardinal features of the malformation (as indicated by the word tetralogy which derives from the Greek word tetra – meaning four) including:
            (1) an aortic valve that overrides the ventricular septum;
            (2) an unusually thick right ventricle;
            (3) a hole in the ventricular septum; and
            (4) an obstructed pathway between the right ventricle and the lung artery. 

It is the two latter features, the ventricular septal defect and the obstruction of right ventricular outflow, which together result in reduced blood flow to the lungs with resultant transfer of oxygen-poor (blue) blood to the body. The net result is the characteristic blue skin coloration seen in tetralogy patients (hence the descriptive name “blue babies” to describe children with tetralogy). The surgical treatment of tetralogy is therefore undertaken with two goals: to close the ventricular septal defect, which is fairly straightforward, and to relieve the right ventricular outflow tract obstruction, which is less straightforward and is the subject of this article.

Components of the RVOT

Figure 1: Removal of thickened muscle

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In order to understand the treatment of right ventricular outflow tract (RVOT) obstruction, it is first necessary to be familiar with the components of the RVOT, of which there are three. As blood exits a normal right ventricle, it passes first through a relatively short muscular tube known as the infundibulum. Next, blood passes through the pulmonary valve that is itself a tubular structure with three leaflets or curtains that float together as the heart relaxes and serve to prevent blood from flowing backwards into the right ventricle. The third and last component of the RVOT is the main lung artery which conveniently is thought of as a tube, and which splits into a right and left lung artery a short distance above the pulmonary valve.  Obstruction of the RVOT in patients with tetralogy may occur at one, two or all three of the component parts of the pathway of blood, and must be addressed wherever it occurs.

Figure 2: Removal of muscle and addition of enlarging patch

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At the level of the infundibulum, a cross-section of the blood pathway would look much like a doughnut, with the substance of the doughnut being composed of ventricular muscle and the blood flowing through the center.  If there is obstruction in the infundibulum, it may be relieved by the removal of some of the muscle in the wall of the doughnut (Figure 1), in effect enlarging the center of the doughnut.  This approach is limited by the outer diameter of the doughnut:  if the outer diameter is too small, removing all of the muscle will still leave obstruction.  In such cases the treatment is to cut the doughnut and place an enlarging patch on the outer wall, resulting in an enlarged blood pathway and relief of the obstruction (Figure 2).

Figure 3: Enlargement exclusively by patch

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At the level of the pulmonary artery, the issue is even simpler than in the infundibulum.  The wall of the artery has minimal thickness, so there is no doughnut wall to be removed.  All enlargement of the pulmonary artery is therefore accomplished by means of the patch technique (Figure 3).

Figure 4: Normal and abnormal pulmonary valves where abnormal valve has simple leaflet fusion

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The most complex of the three levels of the RVOT is the pulmonary valve, because obstruction at the valve level may take several forms. In order to understand the function of the valve, it may be helpful to think of the pulmonary valve as being made up of three curtains (leaflets) suspended from a circular curtain rod. In some cases, the leaflets (curtains) of the valve may be fused together, causing obstruction by failing to separate as blood is transiting the valve. In this case, splitting the leaflets apart with a scalpel (or even bluntly with an instrument known as a dilator) may relieve the obstruction (Figure 4).  In other cases, however, the valve obstruction may be a result of an abnormally small diameter of the valve and the valve diameter may have to be enlarged with a patch, as shown in Figure 5, in the same way that the pulmonary artery and some times the infundibulum is enlarged.  In those circumstances in which a patch is necessary to enlarge the valve diameter, most of the time the artery and the infundibulum are small as well, so that a single patch may be used to enlarge all three levels of the RVOT.  Such a patch is frequently termed a “trans-annular patch”, reflecting the fact that the approximately circular “curtain rod” in the pulmonary valve is known as an annulus.

More on the trans-annular patch
Because the use of a trans-annular patch effectively relieves obstruction at all three levels of the RVOT in tetralogy of Fallot, it would be reasonable to wonder why this approach is not employed always in all patients undergoing surgery for tetralogy.  The reason it is not always used relates to the fact that enlarging the annulus with a patch leaves a portion of the valve “curtainless”, which in turn makes the valve incompletely effective at preventing the backflow of blood. The backflow or regurgitation of blood across the pulmonary valve reduces the efficiency of the right ventricle; some portion of the blood that is ejected comes back, diminishing the net forward flow. Over time, the ventricle accommodates for the backflow by enlarging so that it can eject more blood to restore a normal net forward flow. In the short term, however, the ventricle has no good mechanism to compensate for a leaky valve which may result in a prolonged and difficult convalescence from surgery. In the long term, the increased size of the ventricle may result in irregular heart rhythms and/or an overall decline in cardiac efficiency. In some cases, the impact of the leaking pulmonary valve may be such that implantation of an artificial valve becomes necessary, although this is typically more than 10 years after the original surgery.

Figure 5

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The recognition of the early and late deleterious effects of trans-annular patches and leaking pulmonary valves is relatively recent. Nonetheless, a variety of innovations has resulted. As a fundamental principle, most surgeons have adopted a strategy of attempting to avoid enlarging the annulus of the pulmonary valve, even at the risk of leaving a mild degree of obstruction (this is in contrast to the historical approach of complete obstruction removal). Unfortunately, this approach is not applicable to children with extremely undersized pulmonary valves for whom a transannular patch is unavoidable. An approach that has been taken in these children is to attempt to cover the patch with an artificial curtain, which is often termed a monocusp, as illustrated in Figure 5. The initial experience with this technique was disappointing, in part because the techniques for construction of the monocusp were not well-worked out and in part because the materials used for the monocusp were not very durable.  More recently, there has been much more encouraging experience as a result of better surgical design and construction, and as a result of more durable materials being employed.

The RVOT continues to be the area of greatest interest and focus in children with tetralogy of Fallot, as our understanding of the early and late consequences of techniques to enlarge this area evolves. The simple concept of enlarging a narrowed area has, like most apparently simple problems in pediatric cardiovascular medicine, proved to be much more complex than initially thought. Nonetheless, the combination of better surgical techniques, better materials for reconstruction, better imaging techniques to study the problem with, and even the very real prospect of being able to “grow” a new pulmonary valve from a patient’s own tissue has led to the certainty that the results of today will be better than the results of yesterday, and that the results of tomorrow will be better still.

ARORA and Life in the Heart Center

William Fiser , M.D.

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William Fiser, M.D.; Research Director, Pediatric and Congenital Cardiothoracic Surgery, Arkansas Children’s Hospital; Assistant Professor of Surgery, University of Arkansas for Medical Sciences College of Medicine.

ARORA, the Arkansas Regional Organ Recovery Agency, serves all three transplant centers in Little Rock and hospitals throughout the majority of the state for organ and tissue donor services. I serve as the medical director of ARORA, a faculty member for the University of Arkansas for Medical Sciences College of Medicine, as well as a medical staff member at ACH. ARORA is an independent non-profit agency which is certified by CMS, UNOS and the FDA. ARORA’s mission is to make every effort to provide organs and tissues for life-saving and life-enhancing transplantation. This is accomplished through continuous hospital involvement, which includes hospital training, and through community involvement by providing public education. The staff will strive to be ethical and professional, thus providing care, dignity, honor and respect to all families, donors and recipients.
As you can see from the mission, ARORA has many facets, of which public and hospital education are paramount. ARORA has a staff of 46 with offices in Little Rock and Fayetteville. There are also ARORA coordinators stationed at six of the state’s larger hospitals, three in northwest Arkansas and three in Little Rock.  One of the in-house coordinators is stationed at Arkansas Children’s Hospital. As medical director of ARORA, I supervise a clinical staff of 12 critical care R.N.s, eight surgical technicians and more than eight family counselors. ARORA has contractual relationships with all of the hospitals in the ARORA service area to permit us to be part of the health care team.

How ARORA works with ACH
ACH is one of the largest pediatric heart transplant centers in the nation and is a CMS Center of Excellence. Because it is such a large center and children come in so many sizes, hearts donated to children at ACH often come from outside the ARORA service area. To recover a heart, we actually have gone as far as the central valley of California, which is at the limit of our time constraint. A heart must be transplanted within four to six hours after it is removed from the donor. To do this, ARORA works with the ACH transplant coordinators to set up a fly-out. This requires a team from ACH including a transplant surgeon, an ARORA coordinator and a fast airplane. ARORA’s role in the fly-out recoveries is to coordinate timing, transportation and surgical recovery.
Hearts for transplant can come from ACH, as well as other hospitals in the state.  ARORA has worked very hard over the past five years to increase donation rates in the state, which have doubled since 2002. Unfortunately, the organ waiting list far exceeds the supply of organs. Of the 96,000 people on the transplant waiting list in the United States, only about 2 percent are children. Despite the smaller waiting list, children are 1.5 times as likely to die waiting for an organ as adults. Because organs are often not available when needed, ACH has become a leader in using mechanical cardiac support to buy time until a heart becomes available.

Lissa Madigan, R.N.

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In-house coordinator’s role
Lissa Madigan, R.N., is the full-time ARORA coordinator at ACH. Lissa has a 25-year history of nursing in cardiology and the Burn Center at ACH. She also has a master’s in marriage and family therapy from Harding University. She is a licensed Arkansas nurse, a licensed professional counselor and a licensed marriage and family therapist. She draws on all of these in her role as in-house coordinator for ARORA. 
Lissa interacts with families as part of the health care team during the family’s darkest hour. This is when their child has been determined to have such severe brain injury that recovery is not possible. She uses her counseling skills to provide support to the grieving family and to provide information to allow them to make a decision about donation.  Most referrals for organ donations come from the intensive care units. When these occur, she is part of the health care team including the physician, social worker, chaplain and child life representative. At ACH, a collaborative approach including the physician and ARORA is used for requesting a donation. The timing of this request is very important and should not come before the family accepts the loss of their child.  In October of this year, ACH will receive for the second time the National Medal of Honor for Organ Donation. This is something we should all be proud of, knowing the benefit it has for patients and families waiting on organs, as well as those families who have made the decision to donate. We believe that if the family is given the information about donation in the proper context and timing, 90 percent of families will chose to donate to save another life or spare another family the grief they are experiencing. ARORA has a very good aftercare program for donor families. From interacting with these families, we’ve found that donation is universally a good experience that comes from a tragedy.
Lissa educates hospital staff about organ and tissue donation issues, including updates and new procedures. The most recent addition is on donation after cardiac death. Jerril Green, M.D., associate medical director and a pediatric intensivist at ACH, as well as an associate professor of Pediatrics and Critical Care Medicine at UAMS, has written a new policy on this topic in the past year.

The most important message you can take away from this brief article is the importance of organ and tissue donation. Think about it. Talk about it with your family. Express your feelings about it. Save a life!

Feeding Difficulties and the Infant with Single Ventricle Physiology

Ashley Harry, A.P.N.

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Ashley Harry, A.P.N.; Heart Center, Arkansas Children’s Hospital.

Patients with hypoplastic left heart syndrome and other single ventricle lesions are surgically palliated with the Norwood procedure shortly after birth. The right ventricle functions as the systemic ventricle, and either a Blalock-Taussig shunt or a right ventricle to pulmonary artery conduit (Sano Modification) provides pulmonary blood flow. Patients with hypoplastic left heart syndrome develop comorbid gastrointestinal conditions, such as feeding difficulties and reflux requiring surgical correction. One study revealed that after a Norwood procedure, swallowing dysfunction presented in 48 percent of patients and aspiration occurred in 24 percent. The Norwood procedure, as well as other operations on the aortic arch, involves mobilization of the recurrent laryngeal nerve. Operative damage to the nerve is one potential source of postoperative swallowing dysfunction, but is uncommon and only occurred in 9 percent of the patients described from the previous study.

Feeding intolerance can be a significant source of morbidity after a Norwood procedure. Feeding difficulties can prolong hospital stay and have been implicated in interstage death between hospital discharge and second-stage palliation. Establishing adequate oral intake is generally the last clinical hurdle to overcome prior to hospital discharge and is a major determinant of hospital length of stay. Systematic evaluation of swallowing function allows appropriate tailoring of feeding regimens or leads to possible placement of gastrostomy tubes.

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After surgery …
After cardiac surgery, nutrition facilitates growth and assists in post-operative recovery. Total parentral nutrition (TPN) administered intravenously is started in the post-operative period to allow adequate bowel rest post cardiac surgery requiring bypass. As bowel activity increases, TPN is weaned off and transpyloric feeds are started. Transpyloric feeds bypass the stomach and are a safe option to provide nutrition while a patient is still intubated. While transpyloric feeds are relatively safe, the volume required to provide sufficient nutrition, around 110 kcal/kg/day, can pose an increased risk for reflux. Single ventricle patients are at a greater risk for developing aspiration related to reflux due to their hemodynamic instability during post-operative recovery. The need for oral and nasopharyngeal tubes as well as an increase in sedation are some of the factors that contribute to this increased risk. Because of this increased risk, transitioning to feeding by mouth is a collaborative process with the cardiovascular intensive care team and occupational therapist.

An occupational therapist is instrumental in the assessment of evaluating a patient’s motor skills and readiness to attempt oral feeding. Oral stimulation is initially performed, and when appropriate, the therapist attempts to feed by mouth. Assessments are made of the patient’s ability to coordinate sucking and swallowing, stamina, signs and symptoms of gastroesophegeal reflux and signs and symptoms of aspiration. If a patient is unable to demonstrate feeding tolerance, an upper GI as well as a swallow study may be conducted.
 
Upper gastrointestinal tract radiography is an examination of the pharynx, esophagus, stomach and first part of the small intestine that uses a special form of X-ray and a contrast material known as barium. The X-ray makes it possible to see internal organs in motion. Coating the gastrointestinal tract with barium allows the radiologist to assess the anatomy and function of the pharynx, esophagus, stomach and the duodenum. 

Finding a feeding strategy
A swallow study is a X-ray procedure used to test patients experiencing difficulties with feeding and or swallowing. During the exam, a speech pathologist will feed the patient formula combined with barium. The therapist along with the radiologist will then monitor the X-ray to assess the movement of formula through the mouth and into the esophagus while the patient is sucking and swallowing. If a patient demonstrates signs of aspiration, thickening agents may be added and the study continued. When the test is complete, the therapist will give recommendations on feeding strategies based on the patient’s performance.

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If a patient has abnormal gastrointestinal anatomy, vocal cord dysfunction, aspiration on swallow study, gastroesophogeal reflux or is at greater risk for developing aspiration related to marginal cardiopulmonary status, a nissen fundiplication with gastrostomy tube is advised. A nissen fundiplication is a procedure that wraps the stomach around the esophagus. This tightens the valve between the stomach and esophagus and prevents reflux of gastric contents into the esophagus. Gastrostomy tubes are placed directly into the stomach and provide a safe route to administer nutrition. Gastrostomy tubes may be temporary or permanent.

Feeding patients with hypoplastic left heart syndrome or other single ventricle physiology can be challenging. The best outcome for managing feeding difficulties requires a multidisciplinary approach. The goal of feeding patients with single ventricles is to provide the safest route to administer nutrition. When the goal is obtained, appropriate weight gain is achieved, thus better preparing these tenuous patients for their second-stage palliation.

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Spotlight on Shirley Gadi, Patient Care Tech

Shirley Gadi, Patient Care Tech

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What is your role at ACH, and how long have you worked here?
I have been a patient care technician (PCT) for 13 years.

Why is your job rewarding?
Being in the hospital can be stressful for the patients and their families. I am able to interact with them, whether they are worried, scared or just concerned. Being able to go into the patients’ rooms and put smiles on their faces by singing, dancing or just speaking kind words to them is fulfilling and rewarding to me.

How did you become interested in pediatric cardiology or cardiovascular surgery?
When CVICU moved to the 4th floor, I was pulled from my unit as a PCT. It was a change in environment for me, and I wanted to learn more about pediatric cardiology. When I inquired more about the unit, I learned that there was an opening for a PCT. I asked for a transfer to CVICU and have been working there since.

What do you want people to know about the Heart Center at Arkansas Children's Hospital?
The Heart Center at ACH is a “miracle center.” Patients from all walks of life are seen here – each with their own unique personalities and individual needs. At the Heart Center, the staff work together as a team to bring about miracles everyday.

What do you enjoy most about working with children?
Knowing that when they are afraid, I am able to care for them, while at the same time being able to make them laugh.

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What has been your most memorable moment working in the Heart Center at Arkansas Children’s Hospital?
I have many memorable moments since I began working in the Heart Center. Recently, there was a patient here for a clinic visit. After his appointment, he came to the unit looking for “Miss Shirley”. He was late, and I had already completed the day and left. The coordinator called but was unable to reach me. I returned the next day and was informed he was searching for his friend, Miss Shirley, and he told the nurses, “I will spend the night just to see my friend, Miss Shirley, when she returns.” This patient was admitted four years ago and still remembers me. That is a blessing.

What is your greatest professional achievement?
My greatest professional achievement is being able to build my knowledge base for 13 years and becoming the best PCT I can be!

Spotlight on Tracey Marek, Radiologic Technician

Tracey Marek, Radiologic Technician

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What is your role at ACH, and how long have you worked here?
I have worked at ACH for nine years as a radiologic technician in the cath lab.

Why is your job rewarding?
I love to interact with the kids. I love to help make our kids laugh and smile while our nurses are giving them their sedation for the procedures. The babies are hard to resist.

How did you become interested in pediatric cardiology or cardiovascular surgery?
I always knew I wanted to work around children, but I did not know in what capacity. After completing X-ray school, I knew the cardiac cath lab is where I wanted to work for a living. This is the only cath lab I have worked in, and I am very proud to be a member of this staff. A lot of us have worked together for a longtime and we are each other’s family.

What do you want people to know about the Heart Center at Arkansas Children's Hospital?
ACH has so much to offer. There is always something new going on, especially in cardiology. There are many procedures that we can do in the cath lab that would have meant a surgery years ago. For example, PDA and ASD closures. There are sad situations, but it is the small miracles that inspire one to continue on here.

What do you enjoy most about working with children?
These kids are so resilient. Even though we understand more fully the nature of their disease, they go on and make us smile. Just when you think you are making a small difference in their lives, they are actually the ones making a bright spot in your heart.

What has been your most memorable moment working in the Heart Center at Arkansas Children’s Hospital?
This is a hard question. There are so many. Once I had the opportunity to watch a heart surgery. The patient was probably a couple of months old. I stood by the anesthesiologist. I am just amazed at how the surgeons can see those tiny vessels.

What is your greatest professional achievement?
The ability to use my professional training in a situation that I find rewarding.

New Nursing Director Named for CVICU

Trenda Ray, M.N.Sc., A.P.N.

The Cardiovascular Intensive Care Unit (CVICU) at ACH is under new nursing leadership with the recent appointment of Trenda Ray, M.N.Sc., A.P.N., as its nursing director. Ray has been a member of the ACH Team since 1997. Her career path has progressed from providing direct patient care as a nurse in NICU to an Advanced Practice Nurse (APN) role in the CVICU, where she provided clinical management and treatment for critically ill post-operative heart patients. 
            Most recently, as APN for the Cardiothoracic Surgical Service, Trenda has provided pre-operative evaluation and education to patients and families presenting for cardiac surgery. She has also participated in the ACH Nursing Research Committee and Evidence Base Practice Subcommittee and provided a support role for numerous research projects in the Heart Center. She has served as editor of Heart to Heart, a newsletter published by the hospital and as database manger for Cardioaccess database for the Society of Cardiothoracic Surgeons.
            Ray is enrolled in the UAMS Doctor of Philosophy in Nursing program with anticipated graduation in the spring of 2009.  She transitioned from her current APN role and officially began as nursing director of CVICU in early September.

New Staff for the Heart Center

Nischal K. Gautam, M.D.

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Himesh Vyas, M.D.

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Nischal K. Gautam, M.D.
The Heart Center is glad to have Nischal K. Gautam, M.D., who joined the cardiac anesthesia team after completing his fellowship training at Arkansas Children’s Hospital (ACH). He also completed his residency at the University of Arkansas for Medical Sciences (UAMS) and spent time at Maulana Azad Medical College in New Delhi, India. Dr. Gautam is a great addition to the team in the Heart Center. We also look forward to observing his cricket skills while he plays for the Little Rock Cricket Club!

Himesh Vyas, M.D.
The Heart Center also welcomes Himesh Vyas, M.D., an assistant professor of pediatric cardiology at UAMS and a pediatric cardiologist at ACH. He completed medical school and pediatric residency training at B.J. Medical College in Pune, India. After completing a second pediatric residency at Michigan State University, he went on to complete a pediatric cardiology fellowship at the Mayo Clinic in Rochester, Minn. In July, Dr. Vyas joined ACH and UAMS, where his primary interests are echocardiography, fetal echocardiography and cardiac MRI. He is a member of both the American Heart Association and the American Society of Echocardiography; he is certified by the American Board of Pediatrics. Dr. Vyas has presented his research at numerous national meetings.