The Heart Center - Arkansas Children's Hospital

Our hearts have four chambers inside (Figure 1). Under normal conditions, the blood from the body comes back to the heart into the right atrium. This blood is relatively blue (deoxygenated blood). This blue blood goes to the right ventricle, which pumps the blood to the lungs through the pulmonary artery. In the lungs, the blood becomes red blood (oxygenated blood) and returns to left atrium and then to the left ventricle. The left ventricle pumps this red blood to the whole body through the aorta.

The aorta and the pulmonary artery, the two major arteries carrying blood away from the heart, are often called the “great arteries.” However, in the patient with DORV, both the aorta and the pulmonary artery arise from the right ventricle (Figure 2). Usually, the patient with DORV has a ventricular septal defect (VSD), which is the hole between the ventricles. According to the position of VSD, DORV is classified into four groups:
I. In the patient with DORV with a subaortic VSD (Figures 2, 3), the red blood from the left ventricle goes to the aorta easily. In the absence of pulmonary stenosis, the patient will develop congestive heart failure but in the presence of pulmonary stenosis, the patient will become cyanotic.
II. In the patient with DORV with a subpulmonic VSD (Figures 4, 5), the red blood from the lungs moves through the left ventricle, and then easily through the pulmonary artery. The blue blood comes back from lungs and moves to the body easily again. This type of patient usually develops cyanosis, regardless of pulmonary stenosis.

III. In the patient with DORV with a doubly committed VSD (Figure 6), the red and blue blood mixes. In the absence of pulmonary stenosis, the patient usually develops congestive heart failure as described earlier, while those with pulmonary stenosis generally develop cyanosis.

IV. In the patient with DORV with a non-committed VSD (Figure 7), the red and blue blood also mixes, and in the absence of pulmonary stenosis, the patient usually develops congestive heart failure. Again, in the presence of pulmonary stenosis, the patient become cyanotic.

In the case of DORV, additional intracardiac anomalies may occur, including ventricular septal defect, pulmonary valve stenosis, pulmonary valve atresia, pulmonary artery stenosis, subpulmonary stenosis, aortic stenosis, coarctation of the aorta, hypoplasia of the aortic arch, interrupted aortic arch, hypoplasia of one ventricle and common atrioventricular valve. In the presence of a ventricular septal defect and pulmonary valve stenosis, different treatment is required. The ventricular septal defect is the most common additional anomaly except for patent duct arteriosus and atrial septal defect. Since the patient with DORV has a variety of other cardiac anomalies, treatment for DORV is chosen depending on the natures and degrees of other anomalies.

Signs and Symptoms
The babies with DORV usually are diagnosed in the neonatal period due to cyanosis, for example, blue color of skin, lip, and nails or congestive heart failure evidenced by tachypnea, respiratory distress, poor feeding and poor weight gain. Cyanosis develops in the presence of poor oxygenation of the red blood cells. Occasionally, the baby with transposition receives a large amount of pulmonary blood flow and will not demonstrate prominent cyanosis.
Congestive heart failure develops since the organ has to bring more than the usual amount of blood to the lungs, making the lungs “heavy.” This requires more work on the part of both the heart and lungs. These infants require more energy for their heart and lungs to work effectively, which interferes with their ability to gain weight and grow appropriately.

Diagnosing
Definitive diagnosis is made by echocardiography. Some patients recently have been diagnosed before birth by fetal echocardiography.

Treatment
If there is no shunting inside the heart, the baby with this disease is not able to survive. Usually, the baby has a hole between atriums. However, some babies do not have a hole, and they will die soon after birth. In order to obtain adequate mixing inside heart, the hole between the right and left atrium is enlarged with a balloon.

In the presence of two ventricles, usually two ventricular repairs will be required in early infancy. However, in the presence of severe additional anomaly, the repair will be delayed. When the patient is missing two adequate-sized ventricles, the patient will get palliative surgery of systemic to pulmonary shunt placement or pulmonary artery banding. The patient will undergo Fontan surgery in the future.

I. Biventricular Repair

Figure 8

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Figure 9

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a. Suboartic VSD
Ventricular septal defect closure will be performed in order to bring the left ventricle blood to the aortic valve (Figures 8, 9).
b. Subpulmonary VSD
Ventricular septal defect closure will be performed in order to bring the left ventricle blood to the pulmonary valve. Additionally, arterial switch operation will be performed (Figures 10,11,12). (Arterial switch operations previously were discussed in the July issue of Heart to Heart in the section on transposition of the great arteries.)

Figure 12

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Figure 11

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Figure 10

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c. Doubly committed VSD
When the left ventricle blood flow will be re-routed to the right ventricle and aortic valve, biventricular repair (two ventricle repairs) will be performed in order to bring the left ventricle blood to the aortic valve (Figure 13).
d. Non-committed VSD
When the left ventricular blood flow will be re-routed to the right ventricle and aortic valve, biventricular repair will be performed in order to bring the left ventricle blood to the aortic valve (Figure 14).

Figure 13

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Figure 14

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The patient usually has subpulmonary stenosis, and relief of this will be performed simultaneously. Occasionally a conduit, or tube graft, will need to be placed between the right ventricle and the pulmonary artery (Figure 15, 16).

Additional anomalies ranging from ventricular septal defects to atrial septal defects and arch anomalies are repaired as part of the operation. Risk factors for a biventricular repair include small left ventricles, abnormal mitral valves and coarctation of the aortas or hypoplastic aortic arches.

Figure 16

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Figure 15

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II. Single Ventricle Repair
In the presence of excessive pulmonary blood flow, pulmonary artery banding will be performed in the neonate, followed by a bidrectional cavopulmonary anastomosis as a second-stage surgery. Eventually, this patient will have a Fontan operation. The Fontan surgery will be discussed in the following issue of Heart to Heart.

Treatment Results
In the major cardiovascular centers, the surgical mortality of biventricular repair or Fontan type repair is less than 5 percent. Patients with the above mentioned risk factors have a higher mortality rate after a biventricular repair.

Rev. Kenneth Myers; Pastoral Care Chaplain, Arkansas Children’s Hospital

The role of the chaplain in this dynamic place of healing and wellness is to be a constant spiritual presence. I personally and professionally strive to be a beacon or lighthouse for all whom I encounter, encouraging and promoting a high quality of life by embracing wellness and healing.

The Heart Center is rightly named because the heart is the center of life. Our goals and endeavors are to meet the patient and family at the very point of their need. In the spiritual aspect of all these things there are many intangibles which only the creator himself can reveal by his instruments and vessels. Each individual that makes up the team serves a special role or part of Family Centered Care. Every individual also is that instrument for healing and a vessel for wellness. I am not only a part of the psychosocial team for the Heart Center, but I am truly interwoven in the care team.

The healing is really about the whole individual heart, mind, body and soul. The chaplain in the Pastoral Care Department is visible, present and constant to help all patients and their families from any faith background with religious and spiritual needs. The Chaplain can call your local church or pastor, set up a visit from someone of like faith or background such as a minister from your denomination, a Roman Catholic priest, a Jewish rabbi, a Muslim imam, etc. A chaplain also can listen to your concerns as well as meet with you for prayer.

My role as a chaplain for the Heart Center also includes creating an environment of good morale and positive presence. A chaplain is always available 24 hours a day, seven days a week, all year round. A chaplain can be reached by calling the Pastoral Care Department office. By using the psychosocial resources such as Social Work, Child Life, Family Services and Interpreters, there are many overflowing wells of resources that can be provided for the patient, families and staff.

The team approach to support, comfort and care is very effective through compassion. The chaplain has the opportunity to meet the patient and family at their very point of need. When the patient and family come in for a major surgery or clinic visit, there may be some concerns; many times, however, knowing that someone has taken into consideration their peace of mind means so much to them. The family visit that comes with being in the Heart Center led by the chaplain assures them that they are not alone or forgotten, reminding them how others care for them through intercession.

The chaplain’s intervention with the patient and family really is all about the compassion of wellness and healing. There has to be a center of focus for the patient, as well as for the family and medical team. Encouragement also is a large part of the big picture when healing and wellness is the focus. Everybody wants to be rewarded, cheered for and acknowledged when they have done something good. Each patient and family loves to feel the success of working hard to achieve such reward or acknowledgement of accomplishing goals of healing and wellness.

The Pastoral Care Department also provides the Prayer Blanket Ministry and Basket of Hope. Prayer Blanket Ministry is a prayer ministry designed to mobilize people of faith in the state of Arkansas to pray for the needs of patients and families at Arkansas Children’s Hospital. When a child is ill or injured, many lives are impacted. Stress and struggles can become overwhelming. For those who believe in the power of prayer, what better way to support those in need than to cover them with a “blanket” of prayer? The Pastoral Care office will compile the information and send a list of requests to those who have agreed to partner with us in this ministry. The list is sent each Wednesday morning by e-mail. Throughout the state of Arkansas, there are presently more than 63 organizations or churches distributing the list to its members for prayer.

The Basket of Hope program and the Pastoral Care Department have joined together to provide baskets to children in certain areas of the hospital, including CVICU. The goal is to give encouragement and support during their trying time. The baskets are delivered to the patient and family on Sundays, Tuesdays and Thursdays, right to the family waiting room or the unit front desk. The basket is filled with inspirational items to nourish the child and their parents physically, mentally, emotionally and spiritually. Each child’s name is placed on a prayer list. The baskets also consist of age appropriate bibles, gender specific hats, inspirational books and devotionals for the child and their parents, children’s videos, cassette or CD players, even make-up for teen girls, music CDs or cassettes, toys, games and puzzles, journals and food items.

The chaplain’s presence is one which is spiritual – though prayerfulness, meditation and devotion. I witness and give testimony to many great miracles, wonders and signs of many talented, gifted and blessed people.

Michael Schmitz, M.D., ;Chief, Pediatric and Congenital Cardiothoracic Anesthesiology, Vice Chief, Pediatric and Congenital Cardiothoracic Surgery, Arkansas Children’s Hospital; Professor of Anesthesiology and Pediatrics, Assistant Professor of Surgery, University of Arkansas for Medical Sciences College of Medicine

Heparin hinders blood from forming clots. It does this by enhancing the ability of the body’s own natural anticoagulant blood protein, antithrombin. Antithrombin binds to thrombin, making thrombin inactive while the two proteins are bound together. Thrombin is the enzyme responsible for creating fibrin strands, or the fibers that make up the framework of the blood clot. When heparin binds to antithrombin, it causes antithrombin to hang on more tightly to thrombin so that overall, the thrombin in the blood is much more inactive. When plenty of antithrombin and heparin are around, little or no clot can form from blood. Lower levels of heparin can weaken, but not fully eliminate the ability of the blood to clot, an important property when blood clotting ability is necessary for wound healing but too much blood clotting ability would be risky for clots forming on something like an artificial heart valve. Heparin has been a mainstay to prevent blood from clotting when using ECMO, cardiopulmonary bypass and artificial hearts, as well as to prevent clots from forming on artificial surfaces inside the body such as artificial heart valves and blood shunts (small, soft, plastic tubes used to allow blood to travel from one blood vessel to another).

Although the majority of patients who receive heparin do fine without any complications, some patients can have a drop in the number of platelets in their blood. This is called thrombocytopenia (platelet counts of less than 150,000 x 109/L). Platelets are small disc-shaped blood cells that are important in stopping bleeding by plugging holes in damaged blood vessels. Bleeding occurs more readily and is harder to stop if there are not enough platelets in the blood. To make matters more complicated, this heparin-induced thrombocytopenia (HIT) comes in two types: Type I and Type II.

Type I HIT is fairly common and occurs in approximately 10 percent of patients who are given heparin for a few days or more. But most of the time, their drop in platelet count is mild, rarely falling below 100,000 x 109/L, then rising to normal values after several more days. This return to normal occurs even if heparin continues to be given. This type is not mediated by the immune system.

The rest of this article will focus on Type II HIT, which is immune-mediated and potentially harmful. It occurs when a platelet becomes “activated,” meaning the platelet has become “sticky” and releases it chemicals. One of these chemicals is a molecule called platelet factor 4 or PF4. Some PF4 sticks to the platelet and heparin sticks to PF4. The trouble occurs when the immune system in some patients sees the PF4-heparin complex on the platelet as a “foreign” substance. Antibodies are then made by the immune system and bind to the PF4-heparin molecules on the surface of the platelets. This causes more platelets to release PF4, more immune response and eventually clumps platelets sticking to each other, forming microparticles. These microparticles activate other elements of the clotting system so that clots may form and impair blood flow to parts of the body. Platelets are used up faster than they are produced. Bleeding may result from severely low platelet counts. Ironically, abnormal blood clotting (potentially causing strokes, heart attacks, blood clots to the lungs or other organs, or deep venous thrombi or DVTs) may occur at the same time as bleeding complications due to low platelets.

The chances of a patient developing Type II HIT are small, but not insignificant. A person who has never been given heparin before can develop Type II HIT in the first five to 14 days after starting it. It can occur earlier if someone has received heparin before and occasionally, it can occur later than 14 days. About 8 percent of adults receiving heparin will develop HIT antibodies, but only 1 to 5 percent will go on to develop Type II HIT.

Type II HIT can be diagnosed by measuring a low platelet count along with significant PF4 antibodies in the blood. Platelet counts are monitored when a patient is on heparin therapy. Clinically, it may be suspected when someone is receiving heparin and they develop signs or symptoms of blood clots to any part of the body. Such signs may include bruising or blackened skin areas, especially in fingers and toes or symptoms such as shortness of breath, chest pain, changes in heart rate or dizziness or anxiety.

For any patient suspected of having Type II HIT, the first step is to discontinue heparin. There are other anticoagulation medicines that directly inhibit thrombin, the enzyme that converts fibrinogen to fibrin (which is the main structural element of blood clots). FDA-approved direct thrombin inhibitors for the treatment of HIT are lepirudin, argatroban, and bivalirudin. Fondaparinux (Arixtra) is a drug that works by inhibiting the clotting enzyme factor Xa. It may be used instead of a direct thrombin inhibitor. Warfarin (Coumadin) that inhibits vitamin K, causing a reduction in the production of thrombin and other clotting enzymes (factors VII, IX and X), may be used in combination with a direct thrombin inhibitor or fondaparinux after the platelet count has normalized. (Warfarin can make clotting complications worse if used during a Type II HIT episode as it inhibits the production of the body’s own anticoagulant protein C).

If someone has had Type II HIT, it is important that his or her doctor knows about it. The troublesome antibody to PF4 usually goes away after three months or so. Heparin might be considered for short-term use if the PF4 antibody screen is negative. Often, however, a direct thrombin inhibitor might be used for open-heart surgery using cardiopulmonary bypass (heart-lung machine). Another direct thrombin inhibitor, desirudin (IprivaskTM) can be used instead of heparin for injection into the fatty area under the skin to prevent blood clots from forming in veins (deep venous thrombi or DVTs) while a patient spends several days in bed. Other than using other medicines instead of heparin, ways to reduce Type II HIT include:

1. Using warfarin early to replace heparin if long-term anticoagulation is needed (except during Type II HIT).
2. Using low molecular weight heparin (LMWH), as it is less likely to cause HIT.
3. Using pork-derived heparin rather than beef-derived heparin.
4. Avoiding any unnecessary heparin flushes to intravascular lines.
5. Checking platelet counts regularly for patients receiving heparin

Heparin is a low-risk, very beneficial and commonly used anticoagulation medication. A big advantage for heparin is that it has an antidote medicine (called protamine) that allows doctors to quickly inactivate heparin and restore the blood’s ability to clot. Such quick antidote medicines do not exist for medicines that may be used instead of heparin. But doctors need to be aware of the potential for someone to develop Type II HIT, as it can have some risky complications. Several alternatives exist for those patients who cannot receive heparin but who require either short-term or long-term anticoagulation.

What is your role at ACH and how long have you worked here?
I am a Heart Station technician. My primary responsibility is coordinating the Cardiopulmonary Stress Lab, plus ECGs, holters, event monitors and regional clinics. I have been at Children’s for 11 years. I started in the CVICU in January 1996 and transferred to the Heart Station in August 2000.

Why is your job rewarding?
I love working with the kids, and we do have a great staff here in the Heart Station, as well as in the Cardiology Clinic, and our support staff are also great.

How did you become interested in pediatric cardiology or cardiovascular surgery? Actually, pediatric cardiology found me … I cannot remember which job I applied for in 1996, but it wasn’t for the CVICU. Sandra Meredith, the nurse manager at the time, saw my application in someone else’s stack, read it and decided she wanted to talk to me. I met with Sandra, Tammy Webb and the area manager of the CVICU at the time, toured the unit and was offered the job on the spot and started two weeks later. So, I am a true believer of the saying, “The right job will find you.”

What do you want people to know about the Heart Center at Arkansas Children’s Hospital?
I think one of the best things about the Heart Center is the staff. Several of the staff members have been here much longer than I have. They are all wonderful, dedicated and experienced. We also have some of the best doctors and nurses in the whole hospital.

What do you enjoy most about working with children?
Children can be so resilient to anything. Most of the patients that I have worked with are just amazing. It is difficult to put it into words. Many times, they put the smiles on your face, even when they are the sick ones.

What has been your most memorable moment working in the Heart Center at Arkansas Children’s Hospital?
There are so many of them. But I guess the most memorable one was the first patient that I allowed myself to get attached to, and then he passed away. I still remember his name and face today. It was almost to the point where I thought I couldn’t handle it and questioned if I could continue to do my job. I went to the funeral and it was there that his mom put things into perspective. She actually thanked me for everything that I did for her son and for putting “smiles” on his face, even when he didn’t really want to smile. She said to keep doing what I was doing because it really does mean a lot to the patients and their families. So, here I am, 10 years later.

What is your greatest professional achievement?
That’s a tough one. The experiences that I have gained here in Cardiology, and I guess working full-time and putting myself through massage school and building a massage business, part-time only. I have no immediate plans for leaving cardiology anytime soon.

Dana Snapp; Administrative Assistant for Congenital & Cardiothoracic Surgery

What is your role at ACH and how long have you worked here?
I am an administrative assistant in the Pediatric Cardiothoracic Surgery office. I started working at ACH in 1991 and have spent my entire employment of 15 years in this same office.

Why is your job rewarding?
I love knowing that so many children are helped by our Heart Center. It makes me very proud to say I work here.

How did you become interested in pediatric cardiology or cardiovascular surgery?
To be honest, I didn’t know too much about pediatric cardiology or cardiac surgery. My sister worked in the Pediatric Cardiology office, and she would talk about all the new things she was learning and how much she enjoyed it. When a position came open in the Cardiac Surgery office, I thought I would probably enjoy it …and I do.

What do you want people to know about the Heart Center at Arkansas Children’s Hospital?
I think the Heart Center is an amazing place, and I am so glad we have it for the children in need. The Heart Center is comprised of several individual departments and each plays a vital part in the care of the patient. We have a very professional and caring staff that truly loves taking care of children.

What has been your most memorable moment working in the Heart Center at Arkansas Children’s Hospital?
My most memorable moment was when the first DeBakey VAD was implanted here at the Heart Center. It was a very exciting time for all of us. I knew this could possibly help save the lives of many children.

What is your greatest professional achievement?
My greatest professional achievement is knowing that I have worked in a job for 15 years that I truly enjoy. I have the satisfaction of knowing that I love where I work and am happy being here.

Cardiology Staff Waddle Through the Hospital Bringing Halloween Fun

Penguins on Parade

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Halloween Surprise

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In the true spirit of teamwork, several staff members from the Cardiology clinic dressed up as penguins for Halloween. They all lined up from tallest to shortest with Jim Fasules, M.D., leading the way and paraded through the halls of the hospital traveling to the third floor playroom, the administrative offices and CVICU. They surprised ACH President and CEO Jonathan Bates, M.D., by surrounding him at his conference table in

his office. Later, a member of the public relations department was so impressed with their efforts that she presented them each with a Victory Vision “Presentation” pin, a merit honor within the hospital.