Vol 1 Issue 2	March 2006
Inside this issue
Anesthesia for Congenital Heart Surgery: An Overview
Fetal Echocardiography
Coarctation of the Aorta: Still Easy to Miss
A Child Life Specialist: More than Bubbles, Blocks and Barbies
Congenital Heart Awareness Day
Spotlight on Rosie Webb
Spotlight on Becky Looney

Pediatric Cardiac Anesthesia: An Overview

Sana Ullah, M.D., Ch.B.; Pediatric Anesthesiologist; Arkansas Children's Hospital; Assistant Professor of Anesthesiology; Pediatric and Congenital Cardiothoracic Anesthesiology

There is probably no other surgical specialty where a successful outcome depends so much on good teamwork than congenital cardiac surgery. Around the time of surgery, the core of this team consists of surgeons, anesthesiologists, perfusionists and critical care physicians. The role of an anesthesiologist is generally perceived as keeping the patient asleep and pain-free while surgery is taking place. In pediatric heart surgery, the anesthesiologist assumes an even more important role in facilitating surgery, monitoring, and optimizing vital organ function – particularly during the time the patient is on the heart-lung machine. It is widely acknowledged that in the current era it is possible to do a technically excellent operation due to advances in diagnosis and microsurgery technology. The focus has now shifted in trying to minimize damage to vital organs other than the heart – primarily the brain, kidneys and lungs. It can be very disheartening to perform a technically excellent operation and then have the patient develop severe brain, renal or lung dysfunction post-operatively. Fortunately, this is a rare occurrence. The anesthesiologist works in close co-operation with all the other members of the surgical team to ensure the best possible outcome for the patient.

In the Children’s Heart Center at Arkansas Children's Hospital, there are four full-time pediatric cardiac anesthesiologists who take care of patients with congenital heart disease whether they are having cardiac or non-cardiac surgery. The anesthesiologist will meet with the patient and parents usually the day before surgery to conduct a detailed interview and physical examination. One of the most interesting aspects of congenital heart disease (CHD) is that the age of the patients will vary from newborn babies to patients in their twenties or thirties. Because of better outcomes, more patients with CHD are surviving to adulthood and this has led to a separate population of “adults with CHD” with their own set of unique problems. CHD often occurs in combination with abnormalities of other organ systems, and the preoperative interview allows the anesthesiologist to detect these problems and plan for them. A detailed anesthetic plan is then presented, the main points of which are:

1. Preoperative Fasting: Solid food and formula milk are generally stopped six hours before surgery. Breast milk can be given up to four hours and clear liquids may be taken up to two hours before surgery. The “nil by mouth” guidelines have been liberalized over the past few years as this is more comfortable for patients and minimizes the chance of dehydration. Patients who are admitted to the hospital on the day before surgery (these are generally cyanotic patients) may have intravenous fluids started the night before surgery to ensure adequate hydration, because cyanosis generally causes polycythemia (increased red blood cell production), which increases the viscosity (sludginess) of the blood. This can lead to decreased organ perfusion and increases the risk of thrombosis.

2. Premed: Patients who are going to have surgery on cardiopulmonary bypass (the heart-lung machine) are given a steroid called dexamethasone the night before surgery. An additional dose is given at anesthetic induction. This helps to reduce the general inflammatory response to cardiac surgery. Patients are also given a sedative premed thirty minutes before surgery to reduce anxiety and facilitate anesthetic induction. In many cases, parents also look like they might benefit from some sedation! Children are very astute at picking up emotional cues from their parents and parental anxiety is easily transmitted to the patient who is about to go to the operating room. Even though this can be a very stressful time for the family, it is important to have calm and relaxed parents when the patient is being taken to the operating room.  

3. Anesthetic Management: Patients without an IV are anesthetized by breathing the anesthetic gases from a mask placed over the mouth and nose. This takes a few minutes, and once unconsciousness is achieved an IV and other monitoring lines are placed. A breathing tube is placed through the nose or mouth depending on the age of the patient. An arterial line and a central line is inserted, and in most cases an echo probe is placed in the esophagus to assess the repair after the patient is taken off the heart-lung machine. Most surgeries last four to six hours, but may be longer in the case of re-operations. The patient is then transferred to the Intensive Care Unit (ICU), and depending on various patient factors may be taken off the respirator within a few hours. Some patients may require a longer period of artificial respiration. These are generally smaller babies and more complex operations. All patients are given a morphine infusion for pain control and sedation. Blood products may be given for excessive bleeding or abnormal clotting, but our aim is to minimize exposure to all blood products. The ICU is in many ways an extension of the OR and all the body’s vital organs are closely monitored. Physicians with specialized training in intensive care generally take over the care once the patients are transferred to the ICU. Parents are kept updated regularly and are encouraged to be with their children for as long as they wish.

4. Cardiopulmonary Bypass (CPB): This is also known as the heart-lung machine. Most cardiac surgeries require the patient to be placed on CPB. This consists of an artificial lung to add oxygen and remove carbon dioxide, and a pump to move blood around the rest of the body while the surgeon is operating on the heart itself. CPB has been around for almost 50 years and although considered very safe can be the source of some problems encountered during heart surgery. These include fluid overload, a whole body inflammatory response, injury to the nervous system and other vital organs and bleeding. Many recent innovations and advances have been designed to minimize the complications of CPB. These include new techniques to monitor brain oxygenation, specific drugs to minimize the inflammatory response and reduce bleeding and techniques to remove excess fluid from the body at the end of surgery (known as “modified ultrafiltration” or MUF). Some of these issues will be covered in future issues of this newsletter.

Cardiac surgery can be a very stressful time for the patients and their parents. The anesthesiologists at the Children’s Heart Center are part of a whole team focused on achieving the best possible outcomes for all patients who require their services.

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Fetal Echocardiography

Renee Bornemeier, M.D.; Physician, Pediatric Cardiology; Arkansas Children's Hospital; Associate Professor of Pediatric Medicine; University of Arkansas for Medical Sciences College of Medicine

As you might be able to tell from looking at this Web site or our newsletter, our mission is to provide excellent care to our patients with heart problems and their families. But did you know that you don’t even have to be born to be one of our heart patients? Each year many babies are diagnosed prior to birth with cardiac defects by undergoing a fetal echocardiogram.
  
What is a fetal echocardiogram?
A fetal echocardiogram is a highly specialized type of prenatal ultrasound. It is performed to evaluate the structure of the heart of the unborn child. This type of study is usually performed between 20 to 25weeks of gestation. Obstetricians and family practice physicians are all trained to get the typical “four-chamber view” of the heart. However, a fetal echocardiogram is much more comprehensive than this. It evaluates the position of the abdominal organs and the heart, as well as, the anatomy of the heart itself.  The traditional four-chamber view of the heart is usually the best place to start. The heart rate, chamber sizes, cardiac valves, cardiac septae, venous returns and the great arteries can all be evaluated. Many additional views are also utilized to look at the cardiac structures from multiple planes and perspectives.

Who should receive a fetal echocardiogram?
Women with certain types of medical conditions carry a higher risk for having a baby with a congenital cardiac defect. These women may benefit from having a fetal echocardiogram. Type 1 diabetes, autoimmune disorders, exposure to certain drugs or medications during the stage of organ development early in pregnancy, some maternal viral infections acquired during pregnancy and certain genetic conditions can all increase the likelihood of a congenital cardiac defect. Parents with a congenital cardiac defect or a close family relative with a cardiac defect is also another indication for a fetal echocardiogram.

Fetal conditions may be identified that would warrant a fetal echocardiogram.  Mid-line defects, abnormal karotypes, two vessel umbilical cords, fetal hydrops (fluid collections within the baby), cardiac arrhythmias and concerns that the heart appears abnormal are all very good indications for a fetal cardiac evaluation.

What are the limitations of this test?
This type of testing is very useful and in most cases can assess the cardiac anatomy accurately. However, there are some forms of congenital cardiac defects that may not be able to be identified until after a baby is born. The flow of blood through a baby’s heart prior to birth is different than the flow through the heart after birth. Changes occur in all babies at the time of birth allowing for a transition between intrauterine life and extrauterine life.

Coarctation of the aorta, total anomalous pulmonary venous return, coronary anomalies and some types of septal and valvar defects may be difficult or impossible to identify prior to birth.

Also be aware, the evaluation of the anatomy is only as good as the images.  Some women do not make as good high resolution ultrasound images as others; and therefore, the anatomy may not be able to be as clearly defined.  Imaging later in gestation also limits the imaging plane and quality of the images. As gestation progresses and the fetus grows, the fetal positions become less varied and the ribs begin to calcify – all limiting the imaging of the fetal heart.

What happens if my unborn baby is identified as having a cardiac defect?
Within the University of Arkansas for Medical Sciences (UAMS)/Arkansas Children’s Hospital (ACH) system, families identified as having a baby with a cardiac defect receive comprehensive care. There is a joint clinic at Freeway Medical Center where the UAMS high risk obstetricians, pediatric cardiologists, genetic counselors and ultrasound technicians work together to provide these families with a comprehensive care and delivery plan. The families receive counseling by the pediatric cardiologist. The cardiac diagnosis is explained. A diagrammatic picture of the heart is drawn to help the families understand the cardiac problem. A discussion is also held with the families regarding what to expect at the time of delivery and transfer to Arkansas Children’s Hospital.  An overview of what might be expected for the global picture of this type of defect is also given to the families. 

Deliveries that need to be at a tertiary care center are usually arranged at UAMS. The UAMS neonatologists manage the infants at both UAMS and ACH. The neonatologists receive the baby at the time of delivery and stabilize the baby. The ACH transport team is notified and the infant is transferred to ACH where a complete evaluation is performed.  Once the mother has had time to stabilize post-delivery, she can come to ACH to be with the baby. The families will meet many people involved in the care of their child – surgeons, other cardiologists, intensivists, nurse practitioners, nurses, support staff and technicians – to name a few. While infants are in the ICU, parents have a waiting room with chairs that convert to beds, locker and showers.  This enables the family to be close to their infant. Support services are available to the families including financial counseling, social work, child life and lactation support.

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Coarctation of the Aorta: Still Easy to Miss

Paul Seib, M.D.; Medical Director of Cardiac Catherization Lab; Arkansas Children's Hospital; Associate Professor of Pediatrics; Section of Pediatric Cardiology; University of Arkansas for Medical Sciences College of Medicine

Synonyms: LV outflow tract obstruction, aortic arch obstruction, interrupted aortic arch

Background: Congenital heart disease occurs in eight of every 1,000 live births and coarctation of the aorta accounts for five to eight percent of all congenital heart defects. Coarctation of the aorta may occur as an isolated defect or in association with a variety of other lesions, most commonly bicuspid aortic valve and ventricular septal defect. The association with bicuspid aortic valve has been estimated to range from 13 to 85 percent. Even in the present era, the diagnosis of coarctation of the aorta is often delayed until the patient develops congestive heart failure or hypertension. This overview will focus on the presentation, clinical features and therapeutic options associated with coarctation of the aorta.

Mortality/Morbidity: Autopsy studies suggest that unoperated coarctation of the aorta carries a 90 percent mortality by age 50 with a mean age of death of 35 years. Mortality is often determined by patient age, patient size and the presence or absence of associated major cardiovascular anomalies. Associated problems that may contribute to death or morbidity include hypertension, intracranial hemorrhage, aortic rupture or dissection, endocarditis and congestive heart failure.

Race: No definitive racial differences in the occurrence of coarctation of the aorta have been documented; though some authors have suggested that coarctation of the aorta occurs less commonly in Asian people. 

Sex: There is a marked male predominance of coarctation of the thoracic aorta in a 2:1 male to female ratio. This is not true for abdominal coarctation of the aorta where females predominant in this very rare lesion. Approximately one abdominal coarctation of the aorta occurs for every 1,000 coarctations of the thoracic aorta. 

Age: In general, patients with coarctation of the aorta may present early in life with congestive heart failure or later in life with hypertension. Studies continue to document that coarctation of the aorta is often missed in the first year of life and the median age of referral to a pediatric cardiologist in one study was 5 years of age.  Among 2,192 patients reported to the Pediatric Cardiac Care Consortium for the years 1985 to 1993, 1,337 were infants, 824 were children and 31 were adults. 

History: The history in patients with coarctation is variable but may be related to those patients who present early, often with congestive heart failure and those who present later, most often with hypertension.

Early presentation: Young patients may present in the first three weeks of life with poor feeding, rapid breathing, lethargy and progress to overt congestive heart failure and shock. These patients may have appeared well prior to hospital discharge with their deterioration coincident with closure of the patent ductus arteriosus. Development of symptoms is often accelerated by the presence of associated major cardiac anomalies such as ventricular septal defect.  Such patients historically have carried the highest risk of operation. Symptoms may be subtle at first and patients may make repeated trips to the physician before finally presenting in critical condition. 

Late presentation: Patients often present after the first month of life with hypertension or a murmur.  Such patients have often not developed overt congestive heart failure because of the presence of arterial vessels that bypass the obstruction. Such patients may often be diagnosed after hypertension is noted as an incidental finding during evaluation of other problems such as trauma or a more routine illness. Other presenting symptoms may include headaches, chest pain, fatigue or even life-threatening intracranial hemorrhage. True lower extremity pain with exercise is rare. Many patients are asymptomatic except for their incidentally noticed hypertension.

Physical: As with the history, the physical examination may be conveniently divided into those patients presenting early with heart failure and those who present later with hypertension.

Early presentation: Infants may present with tachycardia, rapid and increased work of breathing, and even may present moribund with shock. Keys to the diagnosis include blood pressure discrepancies between the upper and lower extremities as well as reduced or absent lower extremity pulses to palpation.  In the case of aberrant origin of the right subclavian artery from the aorta distal to the obstruction, such differences may not be present though there will be reduced lower extremity pulses compared to the carotid pulses.

Differential cyanosis (pink upper extremities with cyanotic lower extremities) may occur when lower body blood flow is provided by right to left flow across a patent ductus arteriosus. This may be documented by pre- and post-ductal pulse oximetry measurements as well as by inspection. Reversed differential cyanosis (upper body cyanosis with normal lower body saturation) may occur with transposition of the great arteries, patent ductus arteriosus and pulmonary hypertension resulting in right to left ductal shunting.

In the case of the low cardiac output and ventricular dysfunction, pulses may be diffusely diminished and blood pressure gradients may seem minimal. 

The murmur associated with coarctation of the aorta may be non-specific but is usually a systolic murmur in the left infraclavicular area as well as under the left scapula. Additional murmurs due to the presence of associated abnormalities, such as a ventricular septal defect or aortic valve stenosis, may also be detected.  An ejection click may signify the presence of a bicuspid aortic valve. A gallop rhythm may signify ventricular dysfunction. 

Late presentation: Older infants and children may be referred for evaluation of hypertension or a murmur.  Hypertension in a fussy infant may be attributed to agitation and thus it is important to compare four extremity blood pressures. Occasionally, the left arm pressure may be lower than the right arm pressure if the origin of the left subclavian artery is involved in the coarctation.  Careful simultaneous palpation of upper and lower extremity pulses may help confirm suspected coarctation. 

A murmur in the left infraclavicular area as well as under the left scapula may be systolic but may also sound continuous in the presence of multiple collateral vessels or occasionally in severe coarctation.  An ejection click may be present when there is an associated bicuspid aortic valve as well as a murmur of aortic stenosis or insufficiency.  Similarly, a murmur of mitral stenosis or LV outflow tract obstruction may also occur. A gallop rhythm may occur in the presence of a hypertrophic, non-compliant left ventricle.

Other abnormalities on physical exam may include abnormalities of the blood vessels in the retina and a prominent suprasternal notch pulsation. A thrill may be present in the suprasternal notch or on the precordium in the presence of significant aortic valve stenosis.  In the rare case of abdominal coarctation, an abdominal bruit may be noted.  

Causes: A number of theories have been proposed to explain the occurrence of coarctation of the aorta.  These include postnatal ductal constriction as well as a theory that alterations in intrauterine blood flow result in altered flow through the aortic arch and result in the substrate for coarctation.  The coarctation becomes manifest when the ductus closes. 

Like most forms of congenital heart disease, multifactorial influences seem to affect the occurrence and severity of coarctation. These include genetic abnormalities such as XO Turner Syndrome in which 15 to 20 percent of the patients have coarctation of the aorta.  There are reported familial patterns of inheritance of coarctation and other left heart obstructive lesions.  An increase in seasonal occurrence of coarctation of the aorta has been noted in September and November. 

Differential Diagnosis:

• Other LV outflow tract obstructive lesions – aortic stenosis, subaortic stenosis, supravalve aortic stenosis, Shone’s complex
• Mitral stenosis – mitral regurgitation
• Interrupted aortic arch
• Truncus arteriosus with interrupted aortic arch
• Hypoplastic left heart syndrome
• Aortic arch hypoplasia with complex congenital heart disease
• Sepsis
• Metabolic disease
• Myocarditis
• Dilated cardiomyopathy

Laboratory Studies:

• Pre- and post-ductal pulse oximetry
• Neonatal patients presenting in shock
• Septic work-up to include blood, urine, and CSF cultures
• Electrolytes, BUN, creatinine, and glucose
• Arterial blood gas and serum lactate
• Older patient with hypertension
  
  
  • Urinalysis, electrolytes, BUN, creatinine, glucose

• Chest X-ray
• Early onset – may reveal heart enlargement, pulmonary edema and other signs of congestive heart failure
• Late onset – cardiomegaly, arch indentation in the area of coarctation, rib notching
• Echocardiography – delineates intracardiac anatomy and allows for the assessment of associated significant intracardiac anomalies, also the suprasternal notch view allows evaluation of the aortic arch to assess the transverse aortic arch, the isthmus and the severity of coarctation. Doppler echocardiography allows measurement of the gradient at the area of coarctation.
• CT scan and MRI – useful in the older patient with more limited echocardiographic views or the post-operative patient for assessment of residual arch obstruction or arch hypoplasia.

Other Tests:

• Electrocardiograms
• Early onset in the neonate or infant may reveal right ventricular hypertrophy rather than left ventricular hypertrophy. May also reveal evidence of ischemia.
• Late onset usually reveals left ventricular hypertrophy and may show signs of left ventricular ischemia or strain.

• Cardiac catheterization
  • Allows evaluation of the severity of coarctation and arch anatomy including hypoplasia of the transverse arch or isthmus.
  • Helps to confirm the diagnosis when the echocardiogram is not completely clear.
  • Allows evaluation of intracardiac anatomy and the contribution of associated lesions to the overall hemodynamic disturbance.
  • May allow intervention in the form of balloon aortoplasty in native or recurrent coarctation.

Histological Findings: Coarctation of the aorta results from marked ridge-like thickening of the media of the aortic wall opposite the insertion of the patent ductus arteriosus or ligamentum arteriosum. The intima in this area may initially be thin but may thicken over time. This ridge or shelf becomes obstructive when the patent ductus involutes and when ductal tissue in the wall of the aorta involutes.

• Early presentation
• Supportive care
• Treatment of congestive heart failure with diuretics and inotropic drugs
• Infusion of prostaglandin E1 (.05 to .15 mcg/kg/min) to open the ductus arteriosus
• Ventilatory assistance in cases of markedly increased work of breathing
• Foley catheter insertion to follow renal perfusion and urine output
• Arterial blood gases to follow acidosis
• Umbilical artery catheter may be placed in the neonate to assess the response to prostaglandin infusion with regard to improving lower body blood flow
• Stabilization makes the patient a better candidate for surgical intervention
• Late presentation
• Treatment of hypertension
• Evaluation of associated abnormalities such as aortic stenosis, subaortic stenosis or mitral valve disease
• Evaluate adequacy of collaterals in order to assess the safety of operative intervention
• Preoperative evaluation of intracranial vessels to exclude aneurysms.

Surgical Care: Surgical correction of coarctation of the aorta may be performed in isolation or as part of the repair of associated complex anomalies. For isolated repair, three techniques have evolved over time including end-to-end anastomosis, patch aortoplasty and left subclavian flap aortoplasty.  These techniques may occasionally be combined or modified to fit the individual patient’s complex anatomy.  For instance, a reverse left subclavian flap aortoplasty may be utilized in which the left subclavian artery is turned as a patch back towards the left carotid artery to enlarge an area of transverse arch hypoplasia.  In addition, a so-called “extended” end-to-end repair may be utilized in which the segment of descending aorta is beveled and brought up to the underside of the transverse arch to enlarge areas of transverse arch hypoplasia.  The exact technique employed varies depending on patient’s age at presentation, size, associated abnormalities and arch anatomy.

Early presentation coarctation: The neonate or infant who presents in extremis with congestive heart failure requires urgent resuscitation.  In infants less than two weeks of age in whom the patent ductus arteriosus may have only recently closed, a trial of prostaglandin E1 infusion is indicated to try to reopen the patent ductus.  Restoring lower body perfusion by opening the ductus allows improvement in urine output, clearing of metabolic acidosis and makes the patient a more stable and better candidate for eventual surgical repair. The availability of prostaglandin E1 has greatly lowered the risk of morbidity and mortality associated with coarctation repair in the neonatal period. 

After a period of stabilization and assessment of associated malformations if present, the patient may undergo elective operation for repair of coarctation.  In a review of 1,337 patients undergoing repair of coarctation in infancy: subclavian flap aortoplasty was performed in 763 (57 percent); resection in end-to-end anastomosis was used in 406 patients (30 percent); and patch aortoplasty in 133 (9.9 percent). Twenty additional patients underwent placement of an interposition graft or bypass graft.  Mortality risk in this series was highest in neonates operated on during the first week of life while there were only eight deaths among 279 infants operated on between three months and one year of age. Operative mortality was also higher for smaller infants, particularly those less than three kg, and when there were associated cardiac anomalies.  The presence of a ventricular septal defect either with or without minor associated anomalies increased the risk of death from 0.9 percent when no anomalies were present to 6.8 percent when a VSD was present.  Complex associated anomalies such as single ventricle or transposition of the great arteries greatly increased mortality to 16.6 percent. Similarly, neonates who required an operative procedure prior to coarctation repair had an operative mortality of 45 percent. Urgent operation may need to be undertaken if the patent ductus cannot be opened and the patient continues to have evidence of poor urine output and acidosis. 

In the presence of a hemodynamically significant VSD, placement of a pulmonary artery band at the time of left thoracotomy for coarctation repair may be considered.  In the case of severe transverse arch hypoplasia resulting in significant residual obstruction following coarctation repair, an ascending to descending aortic conduit may be placed.  This is generally a less favorable approach as it may obligate the patient to subsequent surgeries for conduit replacement.  In some centers, associated defects may be addressed at the time of coarctation repair by proceeding with median sternotomy and VSD closure after completion of the coarctation repair.

Late presentation coarctation: Repair of coarctation in the asymptomatic child or adolescent is usually undertaken on an elective basis after assessment of associated anomalies and appropriate pre-operative evaluation. Timing of intervention in the otherwise asymptomatic patient has been debated over the years. Rationale for delaying surgery until 3 to 5 years of age has been based on the size of the aorta in childhood relative to the anticipated adult size. Since significant obstruction occurs when the diameter of the aorta is reduced by 50 percent or more, delaying surgery until 3 to 5 years of age allows the aorta to be greater than half the anticipated adult size at the time of operation; thus, theoretically reducing the risk of significant residual obstruction in the event that the surgical repair site does not grow over time. Improvements in operative technique have led most cardiologists to recommend surgery at the time of diagnosis, especially if the patient is hypertensive. In patients with only mild obstructive, especially those diagnosed in the first year of life, surgery may be safely delayed and the patient followed for worsening obstruction or the development of hypertension prior to recommending surgery. 

Pseudocoarctation of the aorta refers to abnormal tortuousity of the aorta that does not result in significant obstruction, does not result in hypertension and is also noted for the lack of development of collateral vessels. This abnormality does not require surgical intervention.

Surgical technique: Surgical repair of coarctation of the aorta is accomplished in children and adolescents with generally good results and very low mortality. Of 824 patients undergoing coarctation repair in childhood, 431 (52 percent) underwent end-to-end anastomosis while patch aortoplasty was performed in 214 patients (26 percent) and only 109 patients (13 percent) underwent subclavian flap aortoplasty. There were only six deaths out of 824 patients for a total mortality of 0.73 percent. There has been a trend towards less frequent use of patch aortoplasty because of concerns regarding the development of aortic aneurysm at the site of repair.

Recurrent coarctation: In the current era, recurrent coarctation of the aorta is usually treated by balloon aortoplasty. Re-operation is occasionally undertaken, especially in the presence of residual obstruction in the transverse arch in the setting of unusual arch anatomy. Balloon aortoplasty for recurrent coarctation has been shown to be highly effective in relieving residual obstruction both acutely and in the intermediate term. More recently, endovascular stents have been placed for treatment of recurrent coarctation in older children and adults with good success, though long-term follow-up is lacking. 

Balloon aortoplasty of native coarctation: Initial experience with balloon aortoplasty for native coarctation was significantly less successful than surgical intervention. This led some cardiologists to reserve balloon aortoplasty exclusively for recurrent coarctation. With improvements in balloon technology allowing the use of smaller balloons with less risk of trauma to the femoral vessels, some centers preferentially perform balloon dilation of native coarctation of the aorta as initial therapy. Surgery is reserved for those patients who have a poor response to balloon dilation or who have recurrent coarctation after initial balloon dilation.  Long-term results with this technique are lacking and surgical intervention remains the gold standard by which the approach of initial balloon dilation must be judged. 

Consultations Regarding Non-Cardiac Problems: In the infant presenting early with congestive heart failure, management in the ICU for stabilization may be required prior to operative intervention.  Premature infants may require the assistance of a neonatologist in the management of associated diseases related to prematurity such as hyaline membrane disease. Genetics consultation may be indicated if Turner Syndrome or other genetic conditions are suspected.  Acute renal failure may occur pre- or post-operatively and require the assistance of a nephrologist, who may also assist with management of hypertension.

Older patients may be initially referred to other consultants prior to the diagnosis of coarctation.  For instance, referral to a hypertension clinic may lead to the diagnosis of coarctation of the aorta by the nephrologist.  Patients being evaluated for suspected genetic syndromes may be referred to the cardiologist for evaluation because of previously undiagnosed coarctation. Complications of coarctation of the aorta, such as intracranial hemorrhage or endocarditis may require the consultation of a neurosurgeon or infectious disease specialist respectively.

Diet: Persistent hypertension has been shown to increase the incidence of coronary artery disease.  Patients who have undergone repair of coarctation of the aorta must be followed for hypertension and a healthy diet low in fat should be recommended. Cholesterol should be measured and even pharmacologic intervention should be undertaken as indicated with a goal of total cholesterol less than 200 grams per deciliter. Patients with persistent hypertension may require varying degrees of salt restriction. Dietary counseling and avoidance of obesity and smoking are particularly important.

Activity: Patients with coarctation who have not yet undergone surgical repair and are hypertensive should have limitation of heavy isometric exercises to a degree commensurate with their degree of hypertension.  In general, it is felt that the duration of hypertension after coarctation repair is related in part to the duration of hypertension prior to diagnosis and repair of coarctation of the aorta.  Patients who undergo repair of coarctation in infancy most often remain normotensive in the absence of significant residual arch obstruction and require no specific restrictions or limitations to activity.  It should be remembered that with growth, recurrent coarctation may occur and that some patients may be normotensive at rest but have significant upper extremity hypertension provokable with exercise. Such patients, especially those who wish to participate in competitive athletics, should undergo exercise stress testing prior to clearance for participation in competitive athletics. 

Patients who undergo repair later in life and who have had a significant period of pre-operative hypertension are at particular risk to have sustained post-operative hypertension that may be permanent.  Such patients should be restricted from heavy isometric exercise and other activities restricted commensurate with their degree of hypertension and blood pressure control. Exercise testing to assess blood pressure response to exercise is particularly useful in this group of patients as a means of delineating reasonable exercise restrictions.

Medical Treatment: Congestive heart failure: The occasional infant may present with significant ventricular dysfunction, which very often improves or resolves after repair of coarctation and relief of obstruction. Occasionally, the patient will have sustained significant injury to the left ventricle resulting in a chronic cardiomyopathy. Such patients are best treated with standard management, which might include digitalis, diuretics and afterload reduction. A careful evaluation should also be made to exclude residual arch obstruction or heart failure due to other associated anomalies such as aortic valve stenosis or insufficiency, subaortic stenosis, mitral stenosis or regurgitation or coronary anomalies.

Hypertension: Antihypertensive therapy is very commonly needed both in the immediate post-op period and on a chronic basis. Some centers place patients on antihypertensive therapy pre-operatively, usually with beta-blockers, in the hope of reducing the severity of post-operative hypertension after repair of coarctation.

Acute post-operative hypertension may be treated with standard therapy such as afterload reducing agents, beta-blockers, or angiotensin converting enzyme inhibitors. Sodium nitroprusside may be used, but is frequently inadequate in the early post-operative period to control hypertension, even at high doses. The goal of antihypertensive therapy should be to keep the systolic blood pressure less than the 95th percentile for age if possible. Beta-blocker therapy is best instituted with an Esmolol infusion in the early post-operative period with the infusion titrated to effect.  Patients who have a satisfactory response may be converted to oral beta-blocker therapy with other drugs such as Atenolol if hypertension persists. Angiotensin converting enzyme inhibitors may be used for chronic therapy either alone or in combination with diuretics and/or beta-blockers. 

Early Post-Operative Recovery: Post-operative recovery should focus on control of blood pressure, slow advancement of the diet, manipulation of antihypertensive medications, progressive ambulation and a focus on pulmonary therapy to improve left lung atelectasis, which is so common after left thoracotomy. 

Outpatient Care: Early outpatient follow-up care should focus on wound healing, resolution of lung atelectasis and adjustment of antihypertensive medications.  Many patients may be weaned off antihypertensive therapy over months or years following repair of coarctation. Other patients may require some form of on-going antihypertensive therapy. A post-operative assessment of blood pressure response to exercise should be employed prior to complete discontinuation of antihypertensive therapy. A search for late complications of coarctation repair including recurrent coarctation and aneurysm formation is also indicated.

Prevention: While the occurrence of coarctation of the aorta cannot be prevented per se, early detection can be accomplished by fetal echocardiography. The diagnosis of coarctation of the aorta can be difficult by fetal echocardiography but might be considered when there is unexplained enlargement of the fetal right ventricle, difficulty in identifying the true aortic arch, identifying an unusual aortic arch contour or when associated anomalies are identified that might be associated with coarctation of the aorta such as other forms of left heart obstruction. A fetal echocardiogram is indicated in every woman who has delivered a previous child with congenital heart disease, especially left heart obstructive lesions.

Postnatally, early detection of coarctation of the aorta is important to avoid prolonged hypertension or other complications. Careful evaluation of the lower extremity pulses in the newborn nursery and at subsequent well child check-ups is imperative.

Complications: Coarctation of the aorta is a life-long disease with complications that may not be evident until many years following initial apparent successful repair. 

Recurrent coarctation: Recurrence of coarctation is associated with smaller patient size and younger age at operation, and in the presence of associated transverse arch or isthmic hypoplasia.  Ductal tissue in the wall of the aorta may involute and contribute to recurrent coarctation as does scarring at the repair site. Some surgeons feel that utilizing interrupted sutures in the anterior portion of the anastomotic suture line improves aortic growth and reduces the risk of re-coarctation.  Sometimes the surgical repair site is unobstructed but obstruction develops at the transverse arch or isthmus related to failure of these areas to grow proportionally to the rest of the arch. Such obstruction may not be detected for many years after initial repair.

Some patients who have undergone initial repair with a left subclavian flap aortoplasty may have a tortuousity to the repair site which does not result in obstruction until they go through the period of rapid growth in adolescence.

Aortic aneurysm: Aneurysm of the aorta can occur in unrepaired coarctation of the aorta and has been described in patients with Turner Syndrome and coarctation of the aorta.  In addition, endocarditis can result in aortic arch aneurysm (mycotic aneurysm) usually distal to the site of obstruction. Patch repair of coarctation of the aorta has appeared to result in an increased incidence of aortic aneurysm, particularly if the shelf of coarctation tissue is excised. The aneurysm usually occurs opposite the site of the patch and can be entirely asymptomatic.  Hoarseness, due to stretch of the recurrent laryngeal nerve associated with aortic aneurysm, has been described. Chest X-ray may detect the aortic arch aneurysm but magnetic resonance imaging is more useful in delineating the size and extent of the aneurysm.

Persistent hypertension: Hypertension may persist even after successful repair of coarctation of the aorta and is, in general, related to the duration and severity of pre-operative hypertension.  This is probably related to alterations in the renin angiotensin system and baroreceptors. As with other forms of uncontrolled hypertension, patients may be at risk for premature coronary artery disease, ventricular dysfunction and rupture of cerebral aneurysms. 

Cerebral aneurysm: Berry aneurysms of the circle of Willis or other vessels are thought to occur in as many as 10 percent of patients with coarctation of the aorta and may be multiple.  Aneurysm size tends to increase with age as does the likelihood of rupture.  Uncontrolled hypertension promotes growth of the aneurysms and increases the risk of rupture. Most patients are asymptomatic until rupture occurs, though some aneurysms may leak prior to rupture resulting in warning symptoms of headache, photophobia, weakness or other symptoms.  Rupture of a cerebral aneurysm is associated with high mortality and should prompt repair of both the aneurysm and the coarctation.

Paralysis: Though rare, paralysis can occur due to spinal cord ischemia resulting from compromised blood supply to the anterior spinal artery. This risk is increased when there are reduced arterial collaterals, with prolonged aortic cross-clamp time, with intraoperative sacrifice of intercostal arteries, as well as other factors.  Paralysis is uncommon in the presence of a well developed collateral supply and this emphasizes the importance of assessing collateral arterial flow prior to surgical intervention.  Methods to prevent cord ischemia include hypothermia, utilization of cardiopulmonary bypass or the insertion of a bypass graft with partial aortic clamping. 

Cardiomyopathy: Cardiomyopathy is most often present in the infant who presents with critical coarctation, especially if there are additional levels of left heart obstruction such as aortic stenosis or subaortic stenosis. Some patients may have changes of endocardial fibroelastosis that result in a chronic dilated cardiomyopathy requiring medical management or in rare cases cardiac transplantation.  Hypertrophic cardiomyopathic changes may also occur, which may predispose the patient to subendocardial ischemia, arrhythmias or congestive heart failure related to diastolic dysfunction.

Chylothorax: An extensive dissection may result in disruption of the thoracic duct leading to chylothorax which is recognized when feedings are instituted post-operatively. Persistent chylous pleural effusions may necessitate chronic chest tube drainage. Some patients respond to therapy with fat restriction or total parental nutrition. Refractory cases may require pleurodesis or thoracic duct ligation. 

Post-coartectomy syndrome: Restoring pulsatile blood flow to the mesenteric arteries may result in a mesenteric arteritis in which the arteries become distended and may rupture. Reflex arteriolar vasoconstriction occurs as part of autoregulation of blood flow and can result in ischemia. Clinical manifestations may range from mild abdominal discomfort to acute abdomen with severe abdominal distention, vomiting, ileus and progression to intestinal wall hemorrhage or perforation. The development of this syndrome is thought to be related to early refeeding after coarctation repair. Thus, feedings are normally delayed for 48 hours after surgery and nasogastric tube decompression is continued during that time until feedings are slowly begun and advanced as tolerated. Severe cases may require exploratory laparotomy for treatment of bowel necrosis or perforation.

Prognosis: Coarctation of the aorta is a life-long disease with a prognosis that is guarded.  Relief of obstruction, control of hypertension, follow-up for recurrent obstruction and follow-up of associated anomalies is imperative. SBE prophylaxis should be continued indefinitely even in the absence of associated abnormalities. 

Patients without residual obstruction who are normotensive both at rest and with exercise should lead normally active lives without restriction. Most such patients should be able to obtain health insurance and life insurance. Patients with persistent hypertension, untreated residual obstruction or other complications have a variable prognosis related to the severity of these problems.

Education: Patients, in addition to their families, should be educated that coarctation of the aorta requires life-long follow-up to detect recurrent coarctation or late complications. They should be educated about a healthy lifestyle to include the avoidance of smoking and obesity, the value of aerobic exercise, the appropriate control of blood pressure and reduction of other factors that influence cardiovascular disease.

Special concerns: After successful repair of coarctation of the aorta, pregnancy should be well tolerated.  Prenatal care should include careful monitoring of blood pressure as hypervolemia of pregnancy may contribute to worsening hypertension, especially in patients with some degree of residual obstruction or pre-existing hypertension. The presence of other associated lesions such as aortic valve disease may further complicate obstetrical management.  There are anecdotal cases of aortic rupture during pregnancy. 

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A Child Life Specialist:  More than Bubbles, Blocks and Barbies

Mandy Staggs, CCLS; Child Life and Education Department; Arkansas Children's Hospital

Throughout the five years I have been a Certified Child Life Specialist in the Heart Center at Arkansas Children's Hospital (ACH), I cannot begin to count the number of times I have been asked the question, “What is a Child Life Specialist?”  The answer to this question can take many forms based on the time I have to explain, but to put it simply, “a Child Life Specialist is a part of the team that helps a child and family cope with being in the hospital, thereby reducing anxiety associated with the hospital stay.”

Helping a child or family cope with being in the hospital begins when a new patient comes into the hospital. Explaining the unfamiliar and allowing the patient/family to explore the hospital environment is key to promote positive coping to the hospitalization.  This is achieved through explanations of surgical procedures and medical equipment in language the patient/family can understand. This allows for questions and misconceptions to be addressed. Also, medical play can be introduced to the patient. An example of medical play is a play session in which real medical equipment can be used to perform procedures on dolls. Medical play can be used to explain procedures as well as the medical equipment present after surgery. This can assist with reducing anxiety and allow the patient to gain understanding of the hospital experience.
 
Playrooms are another tool that can assist in coping with a hospital stay. There are many playrooms located throughout ACH, including specialized playrooms on units. The Heart Center has its own playroom specifically for the cardiac patients. There are many toys, activities, books, movies and video games available to accommodate all ages from newborns to teens.

Another tool that assists with coping is the TAILS (Therapeutic Animal Interventions Lift Spirits) program. TAILS is animal-assisted therapy, in which certified therapy dogs visit patients in their rooms, providing a therapeutic interaction to help reduce pain or anxiety associated with diagnosis or hospitalization.

There is also scrapbooking time each week for families to make memories to take home. The family provides the scrapbook, but the pages and other supplies are supplied for the families who wish to participate.

There is also the Hospital School Program where teachers can assist with any schooling needs. This helps the patients stay caught up with any school work that may be missed because of the hospitalization. 

All of the various aspects of the Child Life role will be available during a hospitalization in the Heart Center. Beginning in pre-op clinic, I will provide teaching for surgery by explaining the various medical equipment the patient and family will encounter, and provide a tour of the unit. I am available to assist with distraction for medical procedures such as IV starts, blood draws, vital signs, etc.  In addition, toys, activities, emotional support, sibling support and play opportunities will be provided.  I will continue to follow the patient and family throughout the hospital stay in order to promote a positive hospital experience.

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Congenital Heart Defects Awareness Day

Alysanne Crimes; Mom to ACH patient, Brendan, 13, complete congenital heart block/pacemaker, repaired ASD, and Lauren, 18

Congenital Heart Defects (CHD) Awareness Day began in November 1999. Jeanne Imperati, a member of The Congenital Heart Information Network (TCHIN), encouraged members of the online support groups, visitors to the site and interested health professionals to write to their state governors, requesting that February 14, 2000 be proclaimed as A Day for Hearts: Congenital Heart Defect Awareness Day™. Within several weeks of her initial announcement, Mrs. Imperati and members of our online community received proclamations from forty-four states!  I am proud to say that Arkansas was one of these states and proclamations have been approved every year since.  In 2006, Congenital Heart Defects Awareness Day was expanded to a full week and renamed Congenital Heart Defects Awareness Week.  It was celebrated from February 7-14, 2006.

The purpose of CHD Awareness Week is stated best in the proclamation: to encourage all citizens to increase awareness, education and services for CHDs, which each year affect thousands of babies. Raising public awareness about CHD will help lift the barrier of ignorance, helping both the families and individuals who currently are affected by CHD, and those whose lives may be affected in the future. TCHIN members have always been concerned about the number of children who go undiagnosed at birth and the lack of newborn screening.  Members are also concerned that even though CHD is the most frequently occurring birth defect and leading cause of birth defect deaths worldwide, a disproportionately small amount of funding is available for CHD research and support. 

The first CHD Awareness Day in Arkansas was in 2000 and it was organized on extremely short notice. The Governor's Office notified me that our proclamation had been approved. The ceremony would be held in two days and a list of attendees was needed the following day.  I scrambled to assemble a group together and participants at our first ceremony included four “heart kids” and their families.  We were especially proud that KATV, Channel 7, here in Little Rock agreed to do a short piece.  Donny McGuire, our adult CHD patient was interviewed along with Sawyer Evans and mother, Kerri, and also Kim Wilkins, mother of Brittany Wilkins.  They discussed what it is like living with CHD and Kim Wilkins emphasized the importance of organ donation. 

The following year saw participation from the Cardiology Department of Arkansas Children's Hospital. Connie Jones, RNP, Dr. Chris Erickson, and Kathy Ainley, RNP attended, along with Dr. Mary Aitken from general pediatrics.  The American Heart Association was also represented.  We were also pleased to welcome more families and children affected by congenital heart disease.

By far, our best-attended event was in 2002, the same year Dr. Drummond-Webb became the chief of cardiovascular surgery at ACH.  I sent out the invitation to hospital employees and Dr. Drummond-Webb, I understand, cleared the surgery schedule so that his operating room staff could attend.  We had the largest group ever, including even more family members.

CHD Awareness Week was proclaimed this year on February 15 in the Governor's Reception Room at the State Capitol.  I was most pleased that ACH chief of cardiothoracic surgery, Dr. Jaquiss was able to attend, along with Deeann Martin, RNP, Dr. Michael Schmitz, anesthesiology and Mark Charette, principal sales and clinical consultant from Medtronic. The “heart kids” included Brendan Crymes, Xavier Taylor and Lindsey Taylor and their families.

I wish to express my gratitude to the cardiology staff at ACH for their support and encouragement over the years for this project.  For more information on TCHIN or Congenital Heart Defects Awareness Week, visit the Web site at www.tchin.org.  It is a great resource for parents as well as health care professionals.

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Spotlight on Rosie Webb

What is your role at ACH and how long have you worked here?
I am an instrument tech. My role is to count, check, assemble and sterilize instruments to make sure everything is ready and sterile for the cases. I have worked at ACH for two and half months.

Why is your job rewarding?
Being part of the team, helping the children, praying for their recovery and the staff in the cardiovascular operating room (CVOR) unit make my job rewarding. Also, knowing my co-workers feel I am an important part of the team.

How did you become interested in pediatric cardiology or cardiovascular surgery?
I worked at the Heart Hospital in cardiovascular surgery when I came to ACH for my interview. I say that it was not just a job. It is about relationships – a loving, caring, helpful team. It is important to have co-workers like everyone in the CVOR; this makes it a joyous place to work.

What do you want people to know about the Heart Center at Arkansas Children's Hospital?
That this is the best place to work. The people are so wonderful, not just in the CVOR, but also the cardiovascular intensive care unit and everyone that makes up the team. When you have a wonderful team you have a wonderful place to work. I have worked with instruments for 30 years, and this place is the greatest

What do you enjoy most about working with children?
I have always been concerned about children, and have prayed for their safety. I got my answer. I am here and I enjoy being here. I love children, and will do anything to help them. My heart goes out to them.

What has been your most memorable moment working in the Heart Center at Arkansas Children's Hospital?
The first impression… the way everyone received me and appreciated me as part of the team. I couldn’t believe the way they welcomed me as a part of the team. I know the welcome was a true welcome, and I am glad I came.

What is your greatest professional achievement?
To be the best interior decorator I can be!

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Spotlight on Becky Looney

What is your role at ACH and how long have you worked here?
I’m a registered diagnostic cardiac sonographer, or more simply, an echo tech.  The first time, I worked here for 16 years (1980-1996). The second time, five years (2000-present)

Why is your job rewarding?
I receive a great deal of satisfaction knowing that I have a small part in the care of children with heart disease as well as assisting their families. This position has never been a “job” to me; it’s been more of a calling.  I’ve learned so many things and am so fortunate to have this opportunity. It’s always been about how I can use the skill God has given me in assisting the physicians in delivering better care to the patients and showing compassion to our families.   

How did you become interested in pediatric cardiology or cardiovascular surgery?  As a college student, I worked part time at UAMS in the Department of Pediatrics as a secretary. I helped with Cardiology Clinic appointments, copied charts as the Pediatric Cardiology Clinic moved from UAMS to ACH and then I began to transcribe clinic dictations. I learned about the clinical histories of many of the patients. I had a true desire to learn and do more for these patients and their families.  Dr. J. B. Norton gave me the opportunity to do just that. I was trained in EKG, stress testing, holter application and echocardiography by Drs. Norton, Dungan, Readinger and Kiel in a small room across the hall from the cafeteria that was affectionately named “The Heart Station”.  I’m thankful they took a chance with me.

What do you want people to know about the Heart Center at Arkansas Children's Hospital?  The Heart Center is a team effort to care for sick children and their families.   It takes all of us, working diligently, as “experts” in our fields to provide a positive outcome for our patients and their families. When the outcome is positive, we rejoice with the family. When the outcome is heart breaking, we grieve with the family.  We work as team but our work never stops at the bedside.

What do you enjoy most about working with children?
I love their smiles, laughs, hugs and most of all, when they come back as young adults and say “thank you for what you did for me and for helping my family through a hard time.”  I think God smiles when that happens and what better reward can you receive? 

What has been your most memorable moment working in the Heart Center at Arkansas Children's Hospital?
There are so many that it’s impossible to name just one. I’ve had the opportunity to work with and for some of the most intelligent, caring and devoted people who have kept their focus on the patients. Thank you for teaching me and tolerating my questions.  Please be patient, I still have so much to learn.

What is your greatest professional achievement?
Obtaining my credentials, Becky Looney, RDCS.

Staff Testimonial about Becky Looney
“I wanted to thank you for featuring Becky Looney in the Heart Center newsletter. As so many of us know, she has been the backbone of the non-diagnostic testing in cardiology since 1981!! She started the Heart Station and was the only Heart Station employee when it began with Dr. Norton's help in 1981. Then a few years later, she got the help of another tech and then in 1986, I came along as the Heart Station secretary.  We have been together ever since.  Without her, the non-diagnostic testing would not be where it is today. Her care and love of the patients and there families is endless as we can all attest.   She keeps scrapbooks and scrapbooks full of cards, graduation announcement, wedding announcements and yes, even birth announcements from the patients she has seen throughout the years. This itself should speak volumes about the care, love and hope that she has given the kids with congenital heart disease throughout the years.”

--Lori Heil, registered diagnostic cardiac sonographer

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