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SRUGICAL REPAIR OF TRANSPOSITION OF THE GREAT ARTERIES:
LATE COMPLICATIONS AND THE CARE REQUIRED
Context Note
This paper, Surgical Repair of Transposition of the Great Arteries: Late Complications
and the Care Required, examines the research done on patients with Transposition of the Great
Arteries (TGA) and the procedures that they had done. This importance of this is to raise
awareness of adults with it and to encourage more research on adults rather than children. TGA
is a congenital heart defect that requires complex corrective surgery. The goal of the article is to
discuss the complications that can be seen with each procedure and what this means for health
care professionals, specifically nurses. I reviewed the research and decided what would be
critical information for nurses and their care of these patients. Using my knowledge of nursing
and their assessments, I integrated nursing care into an abundance of medical information. Since
the audience is nurses, I would publish this article in a nursing journal, such as the American
Journal of Nursing as the article focuses on nursing care. Nurses all over the country have access
to this journal, both online and in paper and it is a popular place to find information.
While the patients discussed in this paper will go through many things normally seen
with entering adulthood, I decided to only focus on pregnancy for that section. I felt that this one
example got the message across that cardiac care needs to be integrated into every other care
plan. The medications mentioned are also medications that are not commonly seen in patients
unless they are cardiac. But, all nurses should know these medications because these patients will
have other issues and may not always be on a cardiac floor. Last, there is the most important
section, which is the teaching and assessment associated with nursing and its importance. Like I
said, the only things mentioned are aspects of the care that would be different, compared to a
normal person, which requires the audience to have an understanding of a nurse’s role in the care
of a patient and a nurse’s assessment.
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Response Letter
I would like to thank the people who reviewed this essay for me. I took a lot of guidance
from all three reviewers and it greatly improved the essay. All three reviewers thought that the
motive and purpose needed to be made clearer and I agreed. When re-reading my essay, I kept
this motive in mind to make sure the audience will fully understand the importance of it. I want
to raise awareness of this defect and the complications in adulthood as well as encourage more
research on the subject. The reviewers also thought that I explained some of the basics a little too
much which made it hard to picture this audience and my persona as scholarly. I agreed with this
and made sure to edit out anything that was explained in too much detail. I also consulted with a
fellow nurse who was able to pick up on some things that she felt was a basic understanding by
all nurses. All the reviewers felt that the conclusion did not review the importance of the subject
and the thesis which I made sure to edit. I want everyone who reads this to understand the
importance of the complications and the fact that because two different generations of these
patients are entering adulthood right now, it is important to know how to treat these patients.
Reviewer #2 pointed out that I did not have transition sentences in between all the
paragraphs which I agreed with and made sure to change. I think that the essay flows much better
now. Reviewer #3 also reminded me that I am taking on a scholarly persona so I went through
my essay and tried to replace some simple words with more sophisticated vocabulary. A minor
point that reviewer #1 pointed out was that I switched between writing “arterial switch
operation” and using the abbreviation throughout the essay. This made it very confusing to the
reader and I can see why it would. I made sure to use the abbreviation throughout because the
name is very similar to the other procedure’s name and I felt that this would make it clearer and
easier to understand, which in turn also improved the flow of the essay. Reviewer #1 also
suggested making the sub-headings bold to
Overall, the reviewers’ comments were extremely helpful and the changes I was able to
make made the paper much stronger than it originally was.
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Running Head: SURGICAL REPAIR OF TRANSPOSITION OF THE GREAT ARTERIES:
LATE COMPLICATIONS AND THE CARE REQUIRED
Surgical Repair of Transposition of the Great Arteries:
Late Complications and the Care Required
Grace Sroka
Northeastern University
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SURGICAL REPAIR OF TRANSPOSITION OF THE GREAT ARTERIES:
LATE COMPLICATIONS AND THE CARE REQUIRED
Abstract
This paper raises awareness and encourages more research on the complications and different
care required in adults who have had surgical repair of Transposition of the Great Arteries
(TGA). The paper reviews five scholarly articles on this subject to answer the question, “what
are the different complications associated with atrial verses arterial switch operations (ASO) and
the implications for nursing?” Both the last generation having the atrial switch operations
performed and the first generation having the ASO performed are entering adulthood, requiring
different methods of care. These patients will experience the normal parts of aging, such as
pregnancy as well as severe complications that can be life-threatening if not caught immediately,
so nurses must be constantly monitoring these patients and know the signs. There are many
things that nurses can teach these patients including the signs of complications. Nurses will also
know the physical findings in an assessment and the possible interactions with common
medications for these patients.
Keywords: TGA, transposition of the great arteries, mustard, senning, atrial switch,
arterial switch, nursing
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Surgical Repair of Transposition of the Great Arteries:
Late Complications and the Care Required
Transposition of the Great Arteries
Due to the time periods that corrective surgeries were first introduced for Transposition
of the Great Arteries (TGA), health care professionals are currently seeing more people with this
defect entering adulthood. The majority of people think of TGA as an extremely rare congenital
heart defect and they would be partly correct in assuming this. TGA only accounts for about 5%7% of congenital heart defects (Junge et al., pg. 1505). It is, however, the second most common
cyanotic heart defect (Junge et al., pg. 1505). Up until the 1960s, babies did not survive with this
defect.
The Mustard and Senning procedures changed this. The survival rate 20 years after these
atrial switch operations is about 80%-86% (Junge et al., pg. 1505). In the 1980s, these
procedures were mostly replaced with the arterial switch operation (ASO), also known as the
Jatene procedure, but they are still used when the ASO is contraindicated in the baby (Junge et
al., pg. 1505). What all of this means is that the last generation of patients who had atrial switch
operation and the first generation of patients who had the ASO are currently entering adulthood.
Because it is repaired in childhood and these patients can lead mostly normal lifestyles, there is
not a large amount of research on the adults who survived these operations. Most people, even
nurses, do not even know what the defect is. These patients make up a very high risk population
for severe complications that can easily go unnoticed and are life-threatening if not caught
immediately. Health care professionals, specifically nurses, need to know the possible
complications of both procedures, the signs of those complications, and the differences in care
needed for these patients. More research is needed along with awareness of congenital heart
defects in general, but for this article, we will focus on the complications that can result in
patients who had an atrial switch operation verses an ASO as they enter adulthood.
Atrial Switch and Arterial Switch Operations
To understand the importance of this issue, one needs to understand what TGA is and the
procedures that these patients undergo. TGA is a congenital heart defect where the aorta and
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pulmonary artery have switched positions. A simple way to describe it is that the blood should be
moving in a figure eight shape, but is instead moving in two separate circles. The oxygenated
blood is moving from the lungs to the heart and back to the lungs while the de-oxygenated blood
is moving from the body to the heart back to the body. The babies affected are almost always
“blue babies”. Commonly seen with TGA is a septal defect which prevents the baby from being
completely cyanotic. If the newborn does not have this defect, a surgeon will keep the foramen
ovale open to allow the baby to survive until he/she can have open heart surgery.
The open heart surgeries include the Mustard and Senning procedures and the ASO. In
the Mustard or Senning procedure, a baffle redirects blood coming back from the body to the left
atrium instead of the right atrium. A baffle is an artificial tunnel or wall made in the heart to
redirect the blood flow. These procedures switch what each side of the heart is used for. The
difference between the two procedures is that the Mustard procedure uses an artificial baffle
while the Senning procedure creates the baffle with atrial tissue. The ASO, the more preferred
method today, switches the aorta and pulmonary artery back into their correct position. While
both the types of operations fix TGA, they use completely different methods, which will result in
different complications later in life.
Review of the Research
As these patients age, they will struggle with more and more complications, but one must
understand that the complications from the atrial switch procedure are different from an ASO.
While there is an abundant amount of research on children post-operative with TGA, the
research on adults who survived with TGA is limited. Even though it is not the most common
congenital heart defect, health care professionals will come across it at some point in their career
and, due to the severity of the defect and procedures, it is important to understand what these
patients need and how to treat them. A few studies followed up with both atrial switch and ASO
patients to research how they were functioning later in life.
While Lange et al. found that atrial switch operation patients had good survival,
functional status, and quality of life in the first two decades post-operative, that function declines
with age. Lange et al. followed up with the patients who had an atrial switch operation from
1974-2001. 15.9% of these people needed reoperations, mainly baffle related, and 13.3% of them
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had atrial or junctional rhythm problems (Lange et al., pg. 1908). The majority of patients with
baffle-related complications had the Mustard procedure rather than the Senning procedure and
the reason is that it uses synthetic material, rather than natural atrial tissue (Lange et al., pg.
1908). Gelatt et al. observed patients for 30 years after the Mustard procedure and found that
tachyarrhythmias were extremely common, with 14% of the subjects experiencing just atrial
flutter. 11% of these patients required pacemakers and all of them needed antiarrhythmic
medications (Gelatt et al., pg. 194). While these are all major complications, the most concerning
complication is right ventricular failure (Lange et al., pg. 1909). As these patients enter
adulthood, they start to experience right ventricular deterioration, valve regurgitation, and
arrhythmias (Lange et al., pg. 1905).
Right ventricular failure is not much of a concern with the ASO because the right and left
sides of the heart are being used as they normally would be. There are not any baffle-related
complications with the ASO either, which is another reason the ASO is preferred to the atrial
switch procedures. The ASO is not complication free, however. ASO patients still suffer from
arrhythmias, valve stenosis or regurgitation, and eventual heart failure.
Junge et al. conducted a study on patients who had either an ASO or an atrial switch
operation. Their study included clinical status, cardiac function, cardiorespiratory performance,
and neurohormonal activity (Junge et al., pg. 1505). The ASO patients showed higher function
than Mustard patients in all categories and were all in New York Heart Association Class I,
compared to the Mustard patients being in class II-IV (Junge et al., pg. 1506). The New York
Heart Association Classes range from I to IV, starting with no limitations in activity going to
severely limited (AHA). Mustard patients also had higher levels of the Tei index, which is a
measurement more indicative of heart failure, and more cases of sudden death from arrhythmias
(Junge et al., pg. 1507). Lastly, in Junge et al.’s study, none of the subjects who had undergone
the ASO had a pacemaker, while 43% of Mustard patients did.
Resulting from these studies is an understanding of the complications that may come with
either an atrial switch operation or an ASO. In general, the atrial switch operations have much
more complications associated with it, the most serious being baffle-related or right ventricular
failure. The right ventricle is easy to overload as it is on the naturally weaker side of the heart.
Arrhythmias, however, are the most common complication of surgical repair of TGA, due to
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sinus node dysfunction, in all three corrective surgeries (Handler & Walker, pg. 2100).
Associated with the procedures, as well as arrhythmias, are heart muscle weakness or stiffness,
arterial narrowing, and valve issues.
What Does This Mean For Health Care Professionals?
While, after surgical repair of TGA, patients can have complications due to the defect and
procedure, they will also have the common issues that people deal with when aging. When these
patients need care for something unrelated to their heart surgery, their cardiologist will still have
to be consulted and the health care professionals need to be aware of the effects when treating
them. Nurses specifically need to be aware of this disorder and be able to maintain that
continuity of care. Communication with all providers involved in the patient’s care is vital to
their health, especially their cardiologist. A common example of this is when a patient wants to
get pregnant after having an atrial switch operation or an ASO. There are so many potential
complications with pregnancy to begin with that it is sometimes difficult to keep this in mind.
Continuity of care is crucial for these patients, as can be seen in the study to follow.
Pregnancy
It is possible for these patients to have a normal pregnancy and deliver a healthy baby,
but they need to be monitored throughout the pregnancy by their cardiologist. Tobler et al.
researched the pregnancy outcomes in women who had undergone an ASO for TGA, observing
nine women from August 2000 to August 2009, resulting in 17 pregnancies. 15% of the
pregnancies were complicated by an adverse cardiac event (Tobler et al., pg. 419). One woman
had undergone a mitral valve replacement in childhood, which led to postpartum valve
thrombosis after anticoagulants were held during delivery, resulting in another mitral valve
replacement (Tobler et al., pg. 419). A decrease in ventricular systolic function, ventricular
tachycardia, and supravalvar pulmonary stenosis were also complications seen (Tobler et al., pg.
419). No one in this study developed pulmonary edema or ischemic events during their
pregnancy. There were no maternal deaths or preterm deliveries, but fetal birth weight was low
due to the mothers’ impaired left ventricular function, arrhythmias, and beta-blocker therapy
(Tobler et al., pg. 419). One child was born with a ventricular septal defect and another had
pyloric stenosis (Tobler et al., pg. 419).
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While there are high risks of complications in the mothers during pregnancy, there are
fewer complications in women who had an ASO verses an atrial switch operation (Tobler et al.,
pg. 419). The increased oxygen demand, anemia, and hypercoagulability during pregnancy can
cause a myocardial infarction and the hormonal changes can cause aortic root dilatation (Tobler
et al., pg. 419). The many risk factors during pregnancy require constant monitoring for women
who have undergone an ASO or atrial switch operation. TGA is not believed to be influenced by
genetic factors, but there is no way to prevent it so it is important to watch for signs of a heart
defect in the newborn as well as monitoring his/her heart rhythm.
Arrhythmias
Monitoring heart rhythms is a huge component of nursing care. These patients are on
continuous telemetry. While arrhythmias such as atrial fibrillation and atrial flutter are very
common after these procedures, they can be life-threatening, especially if it turns into ventricular
tachycardia. As seen in Junge et al.’s study, sudden death is more likely to be seen in Mustard
patients and is usually caused by an arrhythmia. As soon as a Mustard patient goes into an
arrhythmia, they must be treated. Atrial flutter and atrial fibrillation can be exacerbated by so
many factors, but can also start without any trigger. Many Mustard or Senning patients have
pacemakers or are on antiarrhythmic medications. This indicates that they have rhythm issues
and might need a different treatment plan if they are continually experiencing severe
arrhythmias. A commonly seen medication to control atrial flutter is digoxin and Gelatt et al.
found that digoxin could be used prophylactically with Mustard patients.
Medications
Besides digoxin, there are many other anti-arrhythmic medications that these patients
may be taking and a possible side effect of some, such as sotalol, is ventricular tachycardia. If
these patients become ill or are being treated for anything else, this needs to be monitored
because these medications have many drug interactions. All TGA patients are also commonly
taking routine diuretics due to heart muscle weakness or stiffness or valve stenosis or
regurgitation. As with any patient on diuretics, the patients need to be monitored for dehydration
and kidney failure. Diuretics will be seen after both procedures, but digoxin or other
antiarrhythmic medications are seen more in Mustard and Senning patients. Other medications
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commonly seen with Mustard and Senning patients are beta-blockers, angiotensin-converting
enzyme inhibitors, and blood thinners. This is an extremely important reason for the continuity
of care between all health care providers. These medications have many drug interactions and are
also effected by the patient’s diet. Even though nurses do not always work with cardiac
medications, they need to recognize the interactions between these medications ordered by
doctors because they are usually the ones to catch these problems. Diet is extremely important as
well. For example, a patient’s dose of Warfarin can change depending on how much vitamin K is
included in their diet. The patient must maintain that diet so as not to cause a dramatic increase
or decrease of their prothrombin time (PT). Nurses must understand the importance of diet with
these medications and do their best to accommodate these patients’ diets.
Nurse’s Role
Other than working with the patient to keep the same diet, nurses play an important role
in the care of these patients, so it is vital that they understand the procedures, complications,
medications, and care. As seen in Tobler et al.’s study on pregnancy in TGA patients, the nurses
must be constantly monitoring for complications in both the mother and fetus. The signs of
myocardial infarction or a valve problem need to be watched for. Also seen in pregnancy is
holding anticoagulants so as to prevent hemorrhage. In a TGA patient, however, this can be
deadly. Their heart already struggles to oxygenate their body which means when anticoagulants
are held, the patient can become hypoxic suddenly or develop a clot due to the difficulty moving
their blood. Monitoring the patient and fetus is extremely important for the nurse because the
patient’s cardiologist is rarely present at birth. Nurses need to know their patient and possible
complications to monitor for.
As with all patients, a complete history is needed, and nurses will now know the correct
questions to ask. When performing an assessment, one will see many physical differences in
TGA patients. Vital signs will show a lower resting heart rate if the patient has sinus node
dysfunction and while all of these patients will have a sternal scar, the other scars around it can
tell a lot about the procedures they had done. A left thoracotomy scar would mean a coarctation
repair, a right thoracotomy scar indicates an atrial septectomy before the main operation, and a
scar in the left sub clavicular area will suggest a pacemaker (Medscape).
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As mentioned before, a common finding in Mustard or Senning patients is right
ventricular hypertrophy. If present, the electrocardiogram will show a right bundle-branch block
if there is ventricular pacing (Medscape). These patients will also have a loud, single second
heart sound will be heard. While this may indicate pulmonary hypertension in a healthy person,
it is actually normal in an atrial switch patient (Medscape).Peripheral pulses are often extremely
difficult to palpate and many of these patients will not even have a palpable pulse in one of their
arms. These are normal findings in Mustard and Senning patients, but not in ASO patients. It is
important to understand the differences in assessments to provide the best care for these patients
without complications.
For cardiac nurses, a big part of the care is walking because it is vital that cardiac patients
walk every day to improve their cardiovascular function. Unfortunately, if a person has right
ventricular failure, the nurses need to understand that walking will not improve that function
(Gelatt et al., pg. 194). This does not mean that they should be sedentary, but if a patient’s
exercise performance is deteriorating, the nurses must not push these patients more, but
understand that, if the patient had an atrial switch operation, this might be a sign of worsening
right ventricular failure. Another sign of right ventricular failure is worsening hypoxia. What
makes it so difficult to diagnose is that the symptoms are very nonspecific and it can really only
be diagnosed with volumetric testing. This is why it is crucial for nurses to note all changes, no
matter how small. As mentioned before, this is not a problem with the ASO which is why it is
important to understand the differences between these procedures.
The other common complication with Mustard and Senning procedures is the baffle
obstruction. This is usually recognized when the pacemaker lead cannot be navigated through the
superior vena cava or decreased exercise capacity (Medscape). If the obstruction is in the inferior
vena cava, however, the patient will exhibit signs of hepatomegaly or ascites due to the increased
venous pressure on the liver (Medscape). Other signs include cyanosis, full appearance of the
face, or jugular vein distention (Medscape). If recognized quickly, the problem can be fixed.
While nurses must be looking for these signs during the patient’s stay, the patients must
also take care of themselves. There is certainly a lot to teach these patients in order to maintain
their health on their own. This includes teaching Mustard and Senning patients the signs of right
ventricular failure or baffle-related complications, as well as the importance of checking their
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pacemaker and getting their PT tested routinely if on a blood thinner. It means teaching all TGA
patients about their medications, arrhythmias, and signs of valve problems. It is also important
for the patients to bring lists of all their medications and their medical history to any doctor’s
appointments. These patients mainly maintain their health on their own. There are many
medications and guidelines to follow, and patients must understand it all.
Conclusion
To conclude, TGA is a more common congenital heart defect than most people think and
health care professionals need to be more aware of it in adults. Nurses play a huge role in
catching the complications or medical mistakes that can be made with a TGA patient. There is a
large mix of atrial switch operation and ASO patients currently entering adulthood and the most
common complication seen with these patients are arrhythmias. Also seen are heart muscle
weakness or stiffness, valve stenosis or regurgitation, and decreased exercise function. Very
serious complications specifically seen in Mustard and Senning patients are right ventricular
failure, baffle-related complications, and an elevated Tei index.
These patients lead very normal lifestyles if taken care of. When a TGA patient becomes
pregnant, nurses need to closely monitor the women and her fetus. In all TGA patients, no matter
what they are admitted for, nurses need to watch for signs of complications, and know the
differences that will be seen in an assessment of an atrial switch patient verses an ASO patient.
This knowledge could increase their patient’s quality of life or save it. Continuity of care is key,
especially including the patient’s cardiologist in all decisions, and nurses are the link in that care.
In order to provide the best care, more awareness and research is needed on adults with repaired
TGA. Everyone focuses on children with it, as that is when it is most severe, but as these patients
age, they face much higher risks of complications from the surgery they had. There is a great
need for more studies to be conducted on adults with either an atrial switch procedure or an ASO
because these patients make up a very high risk population for severe complications that can be
life threatening if not caught immediately.
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