Case Study 1

Complete Transposition of the Great Arteries (d-GTA)

Graduate Diploma of Clinical Physiology

(Clinical Measurements Science)
Griffith University
Nathan Campus

Mitchell Hollamby
5001226

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Introduction
A 41 year old female present to the Prince Charles Hospital for a
maximal exercise stress test (MEST) upon referral from a doctor.
Patient complained of heart palpitations with maximal exercise, in
which she often partakes. The patient has a previous history of a
complete transposition of the great arteries (TGA), or dextrotransposition of the great arteries (d-TGA), A congenital disease that
simply put, switches the pulmonary artery and the aorta, that is the
left ventricular outflow tract is connected to the pulmonary artery
and the right ventricular outflow tract is connected to the aorta
(Figure 1). This creates two separate blood flow circuits and cause
hemodynamic complications shortly after birth. Not to be confused
with Congenitally corrected TGA. Approximately 2-3/10000 live
births are found to have d-TGA and are discovered when shortly
after birth the newborn begins to express cyanosis from limited
systemic blood flow (Bianca & Ettore, 2001). Intervention is to be
immediately taken, or the loss of life is eminent (Hraska, 2005).
There is no clear defined aetiology in literature; however,
speculation states that it is concerned with the anatomical location
of the formation of the great arteries. Variations of d-TGA can be
found in table 2, this patient in consideration was born with an
intact ventricular septum (Flinn, 1961). There is a familial
component in increasing prevalence, (Bertagna, 2007) other risk
factors can be found in table 1. This case study follows the
management of d-TGA in a patient with a high exercise.
Table 1: Risk factors of d-TGA, adapted from
Webb and Co Braunwalds Heart Disease: A
Textbook of Cardiovascular Medicine.
Age of Mother: >40 upon conception
Alcoholism
Diabetes
Poor Nutrition during pregnancy/prenatal
Rubella or other viral illness during
pregnancy

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Figure 1: Illustration of the systemic and pulmonary circuits in a

normal hear and dextro-transposition of the great arteries (d-TGA).
Table 2: Clinical Variations of d-Transposition
of the Great Arteries. Adapted from Snider,
AR. 1997.
TGA with Ventricular Septal Defect (VSD)
TGA with Intact Ventricular Septum (IVS)
TGA with Left Ventricular Outflow Tract
Obstruction (LVOTO)
TGA with Pulmonary Vascular Obstruction
Disease (PVOD)
Case Presentation
Patient presented for a MEST at TPCH with referral from a doctor as
patient had mentioned noticing palpitation whilst exercising. As
patient lives in Townsville, Far North Queensland, the referring
doctor thought that a MEST would potentially allow the patient to
exercise at her maximal capacity without worry. Patient is not part of
a low socioeconomic demographic.

Patient Characteristics
- Relevant Patient Information
Age- 41y
Sex- Female
Height- 150cm
Weight- 41.5kg
Body Mass Index- 18.4kg/m2
Medications- Nil.

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Previous Medical History

Mustard Repair in 1974
Moderate-Severe dilation of Right Ventricle
Mild Systolic Dysfunction
Moderate Tricuspid Regurgitation
Presenting Signs and Symptoms
Resting Heart Rate- 67 bpm.
Resting Blood Pressure-105/65mmHg.
Resting ECG- Sinus Rhythm with and atrioventricular block grade
1 block (PR interval >220ms), indication of right ventricular
hypertrophy (high amplitudes in R wave of right ventricle leads)
and right axis deviation (negative in leads I and II), as seen in
Appendix 1.

Testing/ Outcomes
Electrocardiogram
Patients resting ECG showed normal sinus rhythm with a rate of
74bpm, with an atrioventricular block grade 1 (PR> 220), right axis
deviation, biphasic T-waves in leads V2-V4. The progressively
lengthening PR interval can be assumed from the patients hearts
surgery has caused some damaged to the AV node and has caused
some conduction issues within the pathway (Flinn, 1961).
Another interesting ECG to keep in consideration is seen in Appendix
2. The ECG taken at the age of 8 days old, indicate a normal sinus
tachycardia ECG. The drugs used to maintain the patent foramen
ovale, have shown to be successful and enabling the infant to
maintain adequate oxygen saturation to maintain life. this allows
the infants heart to beat and respire with normal ranges that of a
newborn In a case where oxygen saturation was not sufficient, heart
rate and respiration rate can increase drastically.
Maximal Exercise Stress Test
A MEST was conducted and the test met the requirements for a
perceived maximal effort. Patient was referred for this test as she
stated to her cardiologist that she had noticed palpitations during
maximal exercise (Levin & Whittington). The patient exercises
frequently and competes in amateur triathlons and marathons. Her
physician thought it beneficial, whilst in the capital city for MEST to
be conducted.
The patient exercised according to the Bruce Protocol for 12:42,
achieving a work level of 15.5 metabolic equivalents. Normal
response to maximal exercise for blood pressure and heart rate
(BP= 105/65- 140/70)( HR= 57-187 (104%APMHR)). High effort,
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predicted by sex and age category, no symptoms reported along

with no significant ST segment changes. There were infrequent,
isolated, unifocal ventricular ectopies with exercise, and a 2*3 beat
run of bigemeny during exercise.
Echocardiogram
Echocardiogram revealed moderate to severe systemic right
ventricular dilatation and hypertrophy with moderate systolic
dysfunction. There was normal left ventricular size and function.
Mild turbulence was noted in the caval baffle with patent pulmonary
vein and Grade 1/4 systemic tricuspid valve regurgitation. MRI
results concur, stating that there was a reduced ejection fraction of
39% however a normal ranged right ventricular volume.
Holter Monitor
Earlier in the year, patient had a Holter monitor applied. Reports
show that she had one ventricular 3 beat run whilst exercising, and
isolated ventricular and atrial ectopics also. HR ranged from 38174bpm. Holter monitor report was taken into consideration when
assessing if MEST was necessary.

Management/Treatment Options
Ongoing management and treatment options predominately include
annual consultations and review of patients wellbeing. This includes
mainly echocardiography and Holter monitors. This patient does not
exhibit much AV nodal dysfunction other than first degree AV block
(Levin & Whittington). This is important to monitor to capture and
treat as early as possible if any supraventricular dysfunctions such
as atrial flutter were to manifest. Echocardiography is crucial to
determine the level of function or dysfunction within the ventricles
and assess any issues such as pulmonary hypertension or limited
systemic flow. M-mode and Simpson biplane method can be utilized
to monitor ejection fraction and chamber wall thickness and
diameter.
Upon birth, discovery of the clinical manifestation of TGA, occurs
through cyanosis of the newborn and thoracic examination with
radiographic imaging and ultrasound. This imaging may show an
egg shaped heart (Webb, Smallhorn, Therrien, & Redington).
Echocardiography can view the size, location and the aorta from the
RV and the pulmonary artery from the LV. As this patient was not
born with TGA with IVS, measures were required immediately to
maintain a patent ductus arterosis, an arterial connection from the
pulmonary artery to the proximal descending aorta. This is done
with prostaglandin E1, this drug is not without fever inducing

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qualities, it allows the cardiac cycle to maintain life by mixing of

oxygenated and deoxygenated blood. Although not needed in this
case, an atrial balloon septostomy may be performed, which
maintains the patent foramen ovale, allowing further mixing of
oxygenated blood (Levin & Whittington). Long term management
post surgical intervention will be discussed further in this report.

Discussion
d-TGA causes there to be two parallel circuits one systemic and
another pulmonary, to maintain life, surgical intervention is
required. From three procedures, only two will be considered in this
case as the third (Rastelli Procedure) requires a large patent VSD.
Others to be discussed are Atrial Baffle and the Atrial Switch.
Atrial Baffle Procedure (Mustard/Senning) vs Atrial Switch Procedure
(Jatene)
- Atrial Baffle Procedure
This intervention shunts oxygenated venous blood from the
pulmonary circuit through the tricuspid valve into the RV, then
ejected through the aorta into the systemic circuit. In unison, the
caval blood, returned from the superior and inferior vena cava, is
returned into the LV and then ejected into the pulmonary circuit,
thus creating two simultaneous circuits sustaining life (Levin &
Whittington). Mustard and Senning procedures use pericardium and
native atrial tissue respectively, to accomplishing the blood baffling.
This procedure leaves the RV to facilitate systemic pressure and flow
as seen in figure 2.

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Figure 2: Shunting of blood flow from baffling from both Mustard and
Senning procedure- image taken from Love, BA. 2008.
- Atrial Switch Procedure
The atrial switch procedure involves the transection and
reattachment of the aorta and the pulmonary artery. There is a
transection of the midportion of the ascending aorta and that of the
pulmonary trunk proximal to the bifurcation (Hong, 2012). The distal
portions of the transections are reattached on the opposing
anatomical landmark, that is, pulmonary trunk to the aorta and vice
versa. The coronary sinus is relocated form the aortic root to the
previous pulmonary trunk. This is performed with the transection of
buttons containing the coronary sinus and the remaining hole is
repaired with pericardium. During the procedure, the aortic root is
brought inferiorly to the pulmonary trunk.
This procedure allows the LV to facilitate systemic blood flow.
The means for the atrial switch, surgeon skill level and patient
maintence were not up to a standard and was first performed in
1975 (Villafane, 2014) (Bertagna, 2007), oddly enough the same
year that this patient had her Mustard procedure performed. There
is a decreased rate of mortality and morbidity seen in patients who
receive the atrial switch, however with surgeons having already
been trained in Mustard operations. Today the atrial switch is the

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preferred method, as it returns the LV to manage systemic blood

flow.

Right Ventricle as the Left Ventricle

The right ventricle is not anatomically and physiologically designed
concurrently as the LV. Therefore they are not interchangeable
(Petterson, 2007). The greatest differentiating factor is the
mechanism in which it contracts. The right is not designed to
generate a high degree of force than that of its left counter part.
The means by which the left ventricle can generate high forces and
still maintain adequate volumetric output, is that it not only do the
myocardial cells contract towards each other, they rotate about
themselves (Sengupta, 2006). That is that the superior portion of
the bullet shaped chamber rotates anticlockwise and the inferior
portion rotates clockwise, achieving a wringing effect, seen in figure
3. This allows for adequate volume to be ejected in a more rapid
fashion.

Figure 3: Simplified Left Ventricular contraction and Right Ventricular

Contraction, image taken from Petterson, 2007.
Physiological Effects on Cardiac System
- Right Ventricle Dysfunction
Dysfunction within the RV can occur due to having to generate a
force that it is not accustomed to, the systemic system. The
requirement to meet adequate ejection fraction, forces the RV to
compete with a great pressure which can lead to right ventricular
hypertrophy (Warnes, 2006). Along with this, the RV is required to
increase volume, overcoming this necessity can lead to dilation of
the chamber. Both of which have been seen in this patient.
Assessment of the chamber is critical as these morphologies can
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lead to Left sided heart failure, once a degree has been met on the
Frank Sterling Curve (Shah, 2015). The LV in this patient has
remained within normal ranges and function. This is often the case
however, can also be dysfunctional also.

- Tricuspid Regurgitation
The tricuspid valve is also not designed to withstand the forces that
are being generated into the systemic system (Martins & Castela,
2008). Through echocardiography, the tricuspid valve has been
deemed a grade regurgitation. It has been noted that due to the
changes in pressures within the two ventricles have occurred, the
septal papillary muscle may be dysfunctional leading to the septal
leaflet becoming limited in its coaptation (Webb, Smallhorn,
Therrien, & Redington). It has also been noted that an increasing
degree of TR can beget the further worsening of RV dysfunction.
- Arrhythmias
Patients with Mustard d-TGA, can be susceptible to arrhythmia
arising from within the Right Atria (Flinn, 1961). Sinus node
dysfunction occurs in 50-60% of patients, speculated from damage
to the sinus node artery during surgery. This patient had an AV block
and no sign of atrial flutter (intra-atrial re-entrant tachycardia). The
Holter monitor check up is useful in the assessment of arrhythmias
that may occur (Flinn, 1961). Sudden cardiac death has been seen
in TGA suffers, however this is more prominent in patients with VSD
(Schwerzmann, 2009). However, this patient is at a reduced risk due
to no evidence of pulmonary hypertension, severe RV dysfunction or
a VSD.

Summary
d-TGA is a somewhat common congenital disease process with
idiopathic aetiology. The cyanosis upon birth is the first telltale sign
of the presence, then with drug intervention, then further surgical
intervention, the immediate mortal threat is relieved. However, not
without consequence. Close monitoring is crucial predominately
echocardiography. However there is no great reason why patients
cannot live a normal life. Such as this patient who has two healthy
children and competes in high degree of physical activity.

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Appendix
Appendix 1: A resting ECG of a patient with d-TGA before MEST, age
41.

Appendix 2: A resting
ECG of a patient with dTGA, age 8 days.

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Reference
Bertagna, F. (2007). Transposition of the great arteries: Factors
influencing prenatal diagnosis. Prenatal Diagnosis .
Bianca, S., & Ettore, G. (2001). Sex ratio imbalance in transposition
of the great arteries and possible agricultural environmental
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Elzinga, G., Piene, H., & Jong, J. (1980). Left and Right Ventricular
Pump Function and Consequences of Having Two Pumps in
One Heart. CIRCULATION , 564-574.
Flinn, C. (1961). Cardiac rhythm after the mustard operation for
complete transposition of the great arteries. the New England
Journal of Medicine , 1635-1638.
Hong, S. (2012). Clinical Features and surgical outcomes of
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Hraska, V. (2005). Long-term outcome of surgically treated patients
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Arteries. Anesthesiology: Problem-Oriented Patient
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Love, B., Mehta, D., & Fuster, V. (2008). Evaluation and management
of the adult patient with transposition of the great arteries

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following atrial-level (Senning or Mustard) repair . Nature

clinical practisce cardiovascular medicine , 5, 454-467.
Martins, P., & Castela, E. (2008). Transposition of the great arteries.
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Schwerzmann, M. (2009). Ventricular arrhythmias and sudden death
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great arteries. European Heart Journal , 30 (15), 1873-1879.
Sengupta, P. (2006). Left Ventricular Structure and Function : Basic
Science for Cardiac Imaging. Journal of the American College
of Cardiology , 48 (10), 1988-2001.
Shah, S. (2015). Managing heart failure in transposition of the great
arteries. The Schsner journal , 290-296.
Snider, A. (1997). General echocardiography approach to the adult
with suspected congenital heart disease. The practice of
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Villafane, J. (2014). D-Transposition of the great arteries. Journal of
the american college of cardiology , 498-511.
Warnes, C. (2006). Congenital Heart Disease for the Adult
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Webb, G., Smallhorn, J., Therrien, J., & Redington, A. Congenital
Heart Disease. In Braunwald's Heart Disease: A Textbook of
Cardiovascular Medicine. Bonow

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