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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 The Ross procedure is among the most inventive and ingenious cardiac surgical operations. It was described in 1967 by Donald Ross in London as a solution for patients requiring replacement of a malfunctioning or malformed aortic valve. The operation, which is less commonly known as the autograph procedure, involves the use of the patient’s own pulmonary valve as a substitute for the aortic valve. The pulmonary valve is then itself replaced by a human valve (known as an allograft or a homograft) from a tissue bank. The underlying principle of the operation is that the patient is left with a normally functional, living valve in the aortic position. This valve is capable of growth and self-repair to account for the accumulated micro-trauma associated with millions of cycles of valve opening and closing and does not require the use of anti-coagulant medications to prevent clot formation on the valve. The homograft valve in the pulmonary position is non-living and is therefore susceptible to requiring replacement because the accumulated wear-related damage. The pace of such damage is remarkably slow for homograft valves in the pulmonary position, however, presumably because the blood pressure (and thus the valve-damaging force) on the right side of the heart is relatively low. Because of considerations of growth and its applicability in sizes far smaller than commercially available artificial heart valves, the Ross procedure has found great application in pediatric cardiac patients. The operation is particularly useful in patients in whom the area just beneath the diseased aortic valve, known as the left ventricular outflow tract, is pathologically small. In such circumstances, the left ventricular outflow tract may be enlarged and the aortic valve replaced in a combination operation known as the Ross-Konno procedure. Another appealing aspect of the Ross procedure is that potent anti-coagulant medications, as would be required in patients with a mechanical heart valve, which are fraught with hazard in growing and active children, can be completely avoided after the Ross procedure. The procedure has also been used in patients who may wish to become pregnant (and therefore wish to avoid the increased risk of birth defects in children whose mothers were taking certain anti-coagulant medications during pregnancy). Ross procedures have also been performed in active and athletic adult patients who simply wish to avoid alteration in lifestyle associated with anticoagulant medications. In addition to its many advantages, the Ross procedure also has certain disadvantages that have tended to limit its universal application across all patient groups. The first of these is that it is a relatively technically challenging operation, which not all cardiac surgeons may be trained in or feel comfortable performing. In large volume cardiac surgical centers with appropriately experienced surgeons, the operative risk for the procedure is very low and approximates that of “simple” aortic valve replacement, but this is not likely true in centers with less experience. Furthermore, the concept that the valve in the pulmonary position is at risk for requiring replacement raises the issue of creating a potential problem where there was not one before. This concept occasionally has been framed as “creating a two-valve problem out of a one-valve problem.” Another concern that has been raised about the Ross procedure is that the autograft valve (formerly the pulmonary valve) seems to enlarge disproportionately and even begin to leak in certain patient populations. In rare instances, the autograft valve has even failed and required replacement, negating any initial advantage of the autograft over its competitors. Certain types of aortic valve dysfunction – those associated with rheumatic heart disease or Marfan syndrome, for example – represent conditions not typically felt suitable for the Ross procedure because of the risk of recurrence of the original aortic valve abnormality in the autograft. After considering the various advantages and disadvantages of the Ross procedure, certain technical considerations must be borne in mind by the surgeon in planning and performing the operation. The first and most obvious of these is that the pulmonary valve may not be suitable for transfer. This assessment of suitability is made intra-operatively and consists of surgical evaluation of the structure and size of the pulmonary valve. Occasionally, the valve may be congenitally malformed or may have been damaged in a previous operation, rendering it unfit for use as an autograft. Another relatively unfavorable finding is massive disproportion between the size of the proposed autograft and the left ventricular outflow tract, although this is generally manageable by various reconstructive and tailoring maneuvers. There are several techniques used for implanting the autograft that differ chiefly in the question of whether any of the native aortic wall above the valve is left in place, as the autograft is sewn in. The merits of the different approaches continue to be debated without any clear consensus. The appearance of the base of the heart as the autograft is excised and transferred as is the location of the coronary arteries which must be removed from the original aortic root and reimplanted in the autograft in the most commonly performed version of the operation. A novel modification was recently proposed by Dr. Ross Ungerleider, which involves placing the autograft within a cloth (Dacron) cylinder as it is implanted. The cylinder then prevents the autograft from expanding, which in turn should prevent the valve from developing leakage as described above, and the preliminary results with this approach have been very encouraging. Since its introduction in 1967, the Ross procedure has found wide acceptance, particularly among congenital heart surgeons. For young patients, active patients, potentially pregnant patients, and patients who are not yet finished growing, the procedure often provides the best option if aortic valve replacement is required. The operative risks have generally been very low, and have diminished further as wider experience has been gained and certain technical modifications applied. Furthermore, although the concern about creating two-valve disease has certainly been validated over time, the observed risk of reoperations to replace the homograft in the pulmonary position has been exceptionally low, and has been preferred by many young adult patients to the sentence of lifelong exposure to anticoagulant medications inherent in the alternative choice of mechanical aortic valve replacement. If not yet quite routine, the Ross procedure has certainly become a standard option for aortic valve replacement for appropriate candidates, and is invaluable especially in our youngest and smallest patients.
Looking Back on a Holiday Season of Care, Love and Hope As winter sets in, patients at Arkansas Children’s Hospital have just endured another holiday season in our care. Every year, we see several patients who are unable to leave the hospital during this important time. Family members and friends may wonder how these patients have coped and how ACH has helped them. When a child is hospitalized, parents often experience feelings of frustration, sadness, anxiety or helplessness. Parents and families worry about what will happen to their child, even though they may not show it in ways we expect. Many feel unprepared to discuss their fears, feelings and questions with their child, their other children or other family members. Having a child hospitalized in an intensive care setting often challenges parents’ core beliefs around the safety of their children. For parents of newborns, this can also be a time of grief for the experiences that they have anticipated for several months. Parents’ relationships with the medical team may take priority, while other important relationships and activities may be disrupted. These experiences are multiplied during the holidays when families face the added strain of being away from their loved ones as well as the daunting task of creating a new tradition outside of what is familiar. Additionally, families feel stressed by their personal relationships, finances, expectations, time and physical demands. The social workers at Arkansas Children’s Hospital recognize that the holidays can be especially difficult and stressful for patients and families. During the holiday season, ACH social workers continue to advocate and provide support, while maintaining a critical role in the communication between the medical team, patients and their families. Social workers help families handle the stress of hospitalization during the holidays by providing emotional support and acknowledging the hardships they experience. Furthermore, social workers assist with connecting families in need to community resources whose donations help families have holiday celebrations. Social workers also help inform patients and families about the various holiday programs and support that occurs through the hospital; these can range from carolers or a holiday meal, to stockings at the bedside on Christmas morning. Together, the social workers at ACH work to extend care, love, and hope to patients and their families during the holiday season.
Two Conditions Make Happy Infant a Challenging Patient
Jujuana Story didn’t know what to expect when she learned the baby she was carrying would be born with hypoplastic left heart syndrome. For one thing, she’d never heard of the condition before. Her physician referred Jujuana to Arkansas Children’s Hospital (ACH) in Little Rock, where a team of physicians, nurses, social workers and child life specialists explained what was wrong with her soon-to-born baby boy and how they could help. “They made it so I could understand everything,” Story said. “I wasn’t confused because they told me to ask questions.” She learned from the team that her baby’s heart didn’t develop properly on the left side. In a healthy heart, the right chamber pumps blood to the lungs so it can receive oxygen, while the left pumps the oxygenated blood to the rest of the body. Having hypoplastic left heart syndrome meant the right side of her baby’s heart would have to work extra hard to pump blood to both the lungs and away from them to his little body. Jujuana delivered her baby at full term and named him Dalton. Soon after the labor, a transport team arrived at the hospital and carried Dalton via helicopter to ACH. Jujuana and her husband, Roscoe, followed in their car as soon as she was discharged. “I was scared to even hold him the first time after he was born,” Story said. “He was so little and had so many monitors. But they had everything set up smoothly. By the time he went into surgery, we knew exactly what to expect.”
Before Dalton could even have his newborn screening tests, he needed emergency surgery to help his heart work better. “Unfortunately, this is a complicated surgery that means we had to stop his heart,” said Dalton’s cardiologist, Brian Eble, MD. He also is an assistant professor of Pediatrics in the Section of Pediatric Cardiology at the University of Arkansas for Medical Sciences College of Medicine. “That meant he had to have a lot of blood transfusions.” Though his surgery was successful, Dalton’s plight would soon become even more complicated. He soon had another procedure to make his heart more efficient. Then, his team learned something new about Dalton. Because his newborn screenings used blood from the transfusions he received from donors, they were inaccurate. During further testing prior to his second surgery, doctors found out he also had sickle cell anemia. This condition worsens when the blood has less oxygen. Patients with hypoplastic left heart syndrome tend to have lower oxygen levels in their blood because of mixing of blue and red blood inside the single pumping chamber. “So Dalton has two completely separate and complicated diseases,” Eble said. “Unfortunately, they react to each other.” The team at ACH began to pore over textbooks and call physicians across the country for advice because they’d never seen a case like Dalton’s. It turned out nobody else had, either. “We talked to a lot of people and came up with a lot of opinions,” Eble said. “Then we brought our team together with the Storys and came up with a plan.” That plan includes Dalton coming to ACH about once a month to have a double volume exchange transfusion, which pulls all of the blood with sickle cells out of his body and replenishes it with healthy, fully oxygenated donor blood. Dalton has this procedure performed in the Cardiovascular Intensive Care Unit because of the team’s familiarity with children with heart disease, with the assistance of staff from the Hematology unit, which specializes in patients with blood disorders. After his birth last May, Dalton stayed at ACH in the Cardiovascular Intensive Care Unit for a little over two months. Now his family makes the four-hour drive to Little Rock from their Ripley, Miss. home at least once a month. “We do this because we have always wanted to give our little boy a chance to live,” Jujuana Story said. The hospital’s emphasis on involving the family in medical treatment planning has been helpful for the Storys. The entire medical team is dedicated to ensuring parents understand what is happening to their child and what can be expected. “I would never pretend to understand what these parents go through,” Eble said. “But I can imagine what I think it would be like and how I would want to be involved and find out about my child’s care. We want to put ourselves in their shoes.” Eble also said that parents often feel powerless when their children are sick and hurting, and equipping them with good information gives them an opportunity to regain control.
“We want them to feel included and empowered,” he said. Today, Dalton is a happy infant who is starting to blow bubbles and enjoys being spoiled by his older brother, Jamal. Though he has more major surgeries on the horizon, he feels good most of the time, his mother said. He smiles and giggles. He’s even begun to stand. “The reason people know about his condition is because we tell them,” Jujuana said. “He acts like a happy baby. When we go to church, people can’t understand how he can be going through what he is because he seems healthy.” Dalton’s experience has been positive in large part because of his parents’ attentiveness and involvement, Eble said. “Dalton is a complicated kid to care for, and they’ve been true champions for him every step of the way,” Eble said. “Miraculously, his mom has even been a champion for other mothers who are going through similar surgeries with children who only have one of Dalton’s diseases.”
What is your role at ACH, and
how long have you worked here? Why is your job rewarding? What do you want people to know
about the Heart Center at Arkansas Children’s What do you enjoy most about
working with children? What has been your most memorable
moment working in the Heart Center at Arkansas Children’s
Hospital? What is your greatest professional
achievement?
Spotlight on Lindsay Titsworth, RN, Pediatric Cardiovascular Surgical Nurse
What is your role at ACH, and
how long have you worked here? Why is your job rewarding? How did you become interested
in Pediatric Cardiovascular Surgery? What do you want people
to know about the Cardiovascular Operating Room at
Arkansas Children’s Hospital? What do you enjoy most about
working with children? What has been your most
memorable moment working in the CVOR at Arkansas
Children’s Hospital?
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