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AN UNCOMMON CASE OF HEART FAILURE

Author : Pramono Sigit


Selasa, 23 Maret 2010 13:58:23

Dept. Cardiology and Vascular Medicine

Abstract
Introduction and Objective:
Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart disease, and isolated
ccTGA is even more rare. This case presentation try to discuss an uncommon case of heart failure in adult caused
by Congenitaly Corrected Transposition of the Great Arteries (ccTGA) which is survive until 58 year old.
Case Illustration:
A 58 year old man, presenting with chief complaint of shortness of breath. He has dyspneu d effort, orthopneu
and paroxysmal nocturnal dyspneu. He was known to have valve abnormalities since 22 year old. From physical
examination there was a single and loud second heart sound and a grade 3/6 pansystolic murmur was audible at
the apex, radiating to the axilla. The basic lung sound was vesicular with rales on the third lower part of both lung
field. The chest x ray shows pulmonayr vein congestion, ECG basic rhytm is AF with rapid ventricular response and
there was an abnormal septal activation with QS complex in V1 and absent initial Q wave in V5-6. The
Echocardiography study found situs solitus, with AV and VA discordant, and severe tricuspid regurgitation, that
appropriate with the diagnosis of ccTGA.
Discussion:
Congenitally corrected transposition of the great arteries usually asymptomatic in childhood and may not present
for diagnosis until adolescence or adulthood. Patient without associated defects asymptomatic and seems to be
good at least until the fifth decade of life. Congestive heart failure appeared for the first time in the fifth and sixth
decades, even in the absence of associated defects. Afterload reduction with ACE inhibitors or angiotensin II
receptor blockers may be less successful than when used for a morphological left ventricle. While data are lacking
to support the use of beta blockers to improve ventricular function in ccTGA. Severe tricuspid insufficiency in this
patient should be treated earlier.
Summary:
This case presented a 58 year old man with heart failure caused by ccTGA, that diagnosed late in adulthood. His
long life duration is because the absence of associated defects. His heart failure was treated using ARB and
diuretics. Tricuspid replacement surgery cannot performed because severely depressed right (systemic)
ventricular function.

INTRODUCTION
In the United States, there are approximately 1 million adults with congenital heart disease, with 20,000 new
patients reaching adolescence each year. 1

Congenitally corrected transposition of the great arteries is a rare form of congenital heart disease characterized
by atrioventricular and ventriculoarterial discordance and is associated with a variety of intracardiac defects, it

occurs in less than 1% of all forms of congenital heart disease.2 Data from several sources suggest a prevalence of
0.03 per 1,000 live births.3
Congenitally corrected transposition of the great arteries usually asymptomatic in childhood and may not present
for diagnosis until adolescence or adulthood.4 However only few patients with this anomaly survive past 50 years
of age because of associated congenital intracardiac anomalies such as pulmonary stenosis and ventricular septal
defect, or systemic AV valve (anatomical tricuspid regurgitation and subsequent development of systemic
(morphological right) ventricular dysfunction or heart block. 5
Only 110% of individuals with ccTGA have no associated defects. Their life expectancy is limited by the onset of
systemic (morphologically right) ventricular failure in their 40s or 50s.6

OBJECTIVES
To present an uncommon case of heart failure in adult caused by Congenitaly Corrected Transposition of the Great
Arteries (cc TGA) which is survive until 58 years.
CASE ILLUSTRATION
Mrs. LBS, 58 years old, presented to emergency department (EMG) of National Cardiovascular Center Harapan Kita
(NCCHK) with chief complaint of shortness of breath that getting worse since 2 days before admission. The
shortness of breath started 6 months before admission and was usually felt when he was laying flat and when he
was doing an activity which was heavier than usual activity. He was also often being woken up from sleep by some
feeling of drowning. Other complain he has were palpitation and fatique. He denied any chest pain nor history of
chest pain except some uncomfortable feeling in the chest when he has shortness of breath.
The patient has shortness of breath age 22, that only felt when he was doing hard work. He got himself checked at
the hospital and the doctor told him that he has heart valve abnormalities.
He denied any history of fever with joint pain, surgery or any history of congenital heart disease or during his
childhood. His family history contained no reports of heart disease (congenital or acquired), nor any sudden death
at a young age.
The major risk factor for coronary heart disease in this patient were the age (≥ 45 years old), and a positive
history of smoking (1-2 cigarettes/ day for 20 year; quited smoking for 1 year)
In the EMG his BP was 101/78 mmHg, heart beat was iregular at a rate of 164 bpm, pulse 90 times/minutes, with
respiratory rate of 26 x/min, without febris. She didnt look anemic nor icteric, the jugular venous pressure was
elevated (5+2 mmH2O), the first heart sound was normal, but the second heart sound was single and loud. A
grade 3/6 pansystolic murmur was audible at the apex, radiating to the axilla. The basic respiratory sound was
vesicular with rales on the third lower part of both lung field. Abdominal examination found no abnormalities, All
extremities were warm, without edema, and pulsations on both dorsal pedals arteries were normally present.
Electrocardiography record revealed atrial fibrilation with rapid ventricular rate of 164 bpm, QRS axis was
45˚, QRS duration was 0,16 s with QS complex in V1, rS complex in V2-4, and no initial q wave in V5, 6, I and
AVL, there were ST elevations in V2-V3 and Lead II,III, AVF, with upright T wave with inverted T wave. ST
depressions were found in lead V5,6, I, AVL. Serial ECG shows no change on ST segment and T wave.

Anteroposterior chest x-ray show enlarged cardiac size (The Cardio Thoracic Ratio was 66%). The aortic and
pulmonal segment were normal. The cardiac apex enlarge to the lateral and downward. Redistribution of
pulmonary vessel blood was observed as cranialization and any infiltrat was not found.

Laboratory result on admission showed hyponatremia (128mmol/L) and slight hypochloremia (94 mmol/L). The
hemoglobin to hematocryte ratio was less than 1/3. This two findings suggestive for hemodilution caused by fluid

retention in heart failure. Laboratory test performed at the adult ward 2 days later showed borderline impaired
glucose tolerance (fasting glucose level: 84mg/dL, glucose level 2 hours after ingestion of 75 g glucose: 142 mg/dL)
and subclinical hypothyroid (TSH 5,11 (0,47-4,64) FT4 1,27 (0,93-1,7)). Other laboratory result were normal.
Laboratory result on admission ( 16-02-2010)
Hemoglobin 13,2 g/dL Thrombotest 29
Hematocryte 37 % INR 1,60
Ureum 45 mg/dL Sodium 128 mmol/L
Creatinine 1,2 mg/dL Potassium 4,2 mmol/L
Leukocyte 8500 mg/dL Chloride 94 mmol/L
Random blood glucose 155 mg/dL Magnesium 1.9 mmol/L
Calcium total 2,7 mmol/L
The echocardiography performed on February 17th 2010 showed that the heart was situs solitus with AV
discordance and VA discordance. The Right Ventricular as the systemic ventricle was dilated, and its contractility
was decreased, Right Ventricular Ejection Fraction: 24%. There was no segmental wall motion abnormality. The
aorta located at anterior and to the left to pulmonary artery. There was no aortic stenosis, pulmonic stenosis, VSD,
ASD, or PDA. The aortic valve has 3 cuspices with normal function. The branch of pulmonary artery was normal.
The mitral valve was mildly regurgitate. There was severe tricuspid regurgitation, and the tricuspid valve gradient
was 80 mmHg. The Tricuspid valve morphology was normal. This finding was apropriate with Congenitaly
Corrected Transposition of the Great Arteries (cc TGA).
The patient was admitted to the adult ward at February 17th 2010. The diagnosis was Chronic Heart Failure
functional class III et causa Congenitally Corrected TGA He was given Diovan 1 x 40 mg; Digoxin 1 x 0,125 mg;
Simarc 1 x 2 mg; Lasix 1 x 40mg; at February 19th 2010 he was discharged.

LITERATURE REVIEW
Congenitally Corrected Transposition of the Great Arteries (cc TGA) of the heart is a rare form of congenital heart
disease that was first described by Von Rokitansky in 1875. The atrioventricular dissociation means that the
morphological right atrium discharges blood into the morphological left ventricle, while the morphological left
atrium drains into the morphological right ventricle. Thus, the left ventricle supplies the pulmonary circulation
while the right supports the systemic circulation.7
Prevalence:
Congenitally Corrected Transposition of the Great Arteries (ccTGA) occurs in less than 1% of all forms of congenital
heart disease. 2 Data from several sources suggest a prevalence of 0.03 per 1,000 live births.3
The international literature contains very few cases of adult patients with congenitally corrected transposition of
the great arteries without associated defects or surgical intervention. Specifically, only about 10 cases of patients
aged over 65 years have been reported.7 According to Roffi M, de Marchi SF, and Seiler C, only 110% of
individuals with ccTGA have no associated defects.
The oldest patient was a woman aged 84 years in whom the condition was discovered as a chance post mortem
finding.7
Etiology:
Population-based studies continue to document the possible importance of environmental factors in the etiology
of this condition. Still, the familial occurrence and recent molecular biology investigations suggest the importance
of genetic influence. It would seem wise therefore to continue to counsel a multifactorial etiology with a

recurrence risk in first-degree relatives of approximately 2% 4


Embriology:
Morphogenetically, the primitive cardiac tube, anchored at one end by the sinus venosus and at the other end by
the truncus arteriosus, loops to the left (l-looped) and not to the right (d-looped) as in the normal heart. This
abnormal cardiac looping brings the morphologic left ventricle to the right and the morphologic right ventricle to
the left.3
Figure 14-13 Diagram of congenitally corrected TGA (L-TGA). There is an inversion of ventricular chambers with
their corresponding atrioventricular valves. The great arteries are transposed, but functional correction results,
with oxygenated blood going to the aorta. Unfortunately, a high percentage of the patients with L-TGA have
associated defects, some of which may cause cyanosis. AO, aorta; LA, left atrium; LV, left ventricle; PA, pulmonary
artery; RA, right atrium; RV, right ventricle.
Pathology:
Theoretically, no functional abnormalities exist, but, unfortunately, most cases are complicated by associated
intracardiac defects, AV conduction disturbances, and arrhythmias. Ventricular Septal Defects (VSD) occurs in 80%
of all cases. Pulmonary Stenosis (PS), both valvular and subvalvular, occurs in 50% of patients and is usually
associated with VSD. Systemic AV valve (tricuspid valve) regurgitation occurs in 30% of patients. Occasionally,
complex associated defects are present with hypoplastic ventricle, AV valve abnormalities, or multiple VSDs and
both varying and progressive degrees of AV block and paroxysmal supraventricular tachycardia (SVT) frequently
occur.8
However, even in the patient with no associated abnormalities, it is increasingly apparent that natural history and
hemodynamics will be far from normal.3
Clinical Features
During the first months of life, most patients with associated defects become symptomatic with cyanosis resulting
from VSD and PS or CHF resulting from a large VSD. Exertional dyspnea and easy fatigability may develop with
regurgitation of the systemic AV valve (tricuspid valve).8 In childhood symptomatology, its timing and severity in
general reflect the associated lesions.3 Patients with congenitally corrected transposition of the great arteries
(CCTGA) may not present for diagnosis until adolescence or adulthood.4 Beauchesne et al report that in 66% of
the patients involved in their cohort research, reported in 2002, the initial diagnosis of CTGA was first made in
adulthood; 17% of them were 60 years or older at the time of diagnosis.9
The clinical course of corrected transposition of the great vessels depends on the presence and severity of the
associated defects.7 Patient without associated defects asymptomatic 3 and seems to be good at least until the
fifth decade of life.10 In research conducted by Presbitero et al Complete atrioventricular block was congenital in
three patients (17%) of the population and was acquired in further four patients (22%) at ages 15, 30, 35, and 44
years.10 Significant left atrioventricular valve regurgitation appeared only in the third decade in 12% and became
progressively severe in the following decades; it was established in four of six patients who reached the sixth
decade.10 Supraventricular arrhythmia began to appear in the fifth decade in three of six patients, but affected
five of the six who reached 51-60 years (two with atrial fibrillation and three with paroxysmal supraventricular
tachycardia).10
Congestive heart failure appeared for the first time in the fifth and sixth decades.10 Even in the absence of such
anomalies the question remains whether the anatomical right ventricle is capable of maintaining an adequate
cardiac output over a long period.7
The largest single-institution study to date by Connelly et al has reported clinical findings in 52 adult patients with
CCTGA. Thirteen (25%) died at a mean age of 38 years. The most common causes of death were progressive heart
failure and sudden death, accounting for 70% of the mortality in this series.4

Electrocardiography

1. The absence of Q waves in V5 and V6 or the presence of Q waves in V4R or V1 is characteristic of the condition.
This is because the direction of ventricular septal depolarization is from the embryonic LV to RV.8
2. Varying degrees of AV block are common. First-degree AV block is present in about 50% of patients. Seconddegree AV block may progress to complete heart block.8
3. Atrial arrhythmias and Wolff-Parkinson-White (WPW) preexcitation are occasionally present.8
4. Atrial or ventricular hypertrophy, or both, may be present in complicated cases. 8
X-ray Studies 8
1. A straight, left upper cardiac border, formed by the ascending aorta, is a characteristic finding. 8
2. Cardiomegaly and increased pulmonary vascular markings are present when the condition is associated with
VSD. 8
3. Pulmonary venous congestion and left atrial enlargement may be seen with severe left-sided AV valve
regurgitation. 8
4. Positional abnormalities (e.g., dextrocardia, mesocardia) may be present. 8

Echocardiography
1. The parasternal long-axis view is obtained from a more vertical and leftward scan than with a normal heart. The
aorta, which arises from the posterior ventricle, is not in fibrous continuity with the AV valve. 8
2. In the parasternal short-axis scan, a double circle is imaged instead of the normal circle and sausage pattern.
The posterior circle is the PA without demonstrable coronary arteries. The aorta is usually anterior to and left of
the PA. The LV, which has two well-defined papillary muscles, is seen anteriorly and on the right and is connected
to the characteristic fish mouth appearance of the mitral valve. 8
3. In the apical and subcostal four-chamber views, the LA is connected to the tricuspid valve (which has a more
apical attachment to the ventricular septum than the other) and the RA is connected to the mitral valve. The
anterior artery (aorta) arises from the left-sided morphologic RV, and the posterior artery with bifurcation (PA)
arises from the right-sided morphologic LV. 8
4. The situs solitus of the atria is confirmed by the drainage of systemic veins (i.e., inferior and superior venae
cavae) to the right-sided atrium and the drainage of pulmonary veins to the left-sided atrium. 8
5. The following associated abnormalities should be looked for, and their functional significance should be
assessed by Doppler and color flow studies: type and severity of PS, size and location of VSD, straddling of the AV
valve, and so forth. 8
MRI
The major role of MRI in cc-TGA patients is to evaluate the systemic right ventricular volume and ejection fraction.
It does so better than echocardiography can at present. For claustrophobic or pacemaker patients, a high-quality
radionuclide angiogram with volume estimates serves as a substitute. MRI can evaluate other issues as well
including conduit function and AV valve regurgitation.11
Cardiac Cathetherization
This is rarely required for diagnosis but may be indicated before surgical repair to demonstrate the coronary artery
anatomy, as well as ventricular end-diastolic and pulmonary artery pressures.11
Medical Therapy.
ACE inhibitor or beta-blocker therapy for patients with systemic ventricular dysfunction may be intuitive, but the
role of such agents has not yet been demonstrated. 11 The problem is at the current time, there is a very small
evidence base from which to draw recommendations for the treatment of heart failure in adults with CHD. In
those with more complex anatomy, for example a systemic ventricle that is not a left ventricle, the rationale for
applying adult heart failure treatment guidelines is much less clear.12 Eventhough, one study of a group of adult
patients with a variety of congenital heart defects showed neurohumoral activation similar to that seen in an adult
heart failure population with acquired heart disease and three recent small uncontrolled series have reported a
possible benefit of β-blockers in adults with CHD and systemic right ventricular dysfunction8 Besides that,

the appropriate use of diuretics along with afterload reduction and beta-blockade may be required.13
Patients with corrected transposition of the great arteries without associated anomalies should be protected from
bacterial endocarditis. Infection occurs on the left atrioventricular valve despite apparent normal function10 and
prophylaxis against subacute bacterial endocarditis (SBE) should be observed when indications arise.8 Patients
with congenitally corrected transposition also very often exhibit atrial tachyarrhythmias, such as atrial fibrillation,
atrial flutter, or supraventricular tachycardia. Such condition should be treated.10
Tricuspid Valve Repair/Replacement.
Prieto et al made a survival analysis from 40 patients with ccTGA and found that those with tricuspid insufficiency
were 14.8 times more likely to die than those without tricuspid insufficiency.14 By multivariable analysis, tricuspid
insufficiency was the only independent risk factor for death among the factors considered in this analysis. 10

Therefore, if significant regurgitation exists, it is preferable to have tricuspid valve repair. Systemic AV valve
regurgitation plays a significant role in worsening systemic ventricular function.11 Similar to the approach for
mitral regurgitation and left ventricular systolic dysfunction, the tricuspid valve should be considered for repair or
replacement when ventricular function worsens in the face of moderately severe to severe regurgitation, rather
than waiting for a decline in functional status. Late referrals for tricuspid valve replacement may portend a poor
surgical outcome.4 Beauchesne et al. demonstrated that low preoperative RVEF was the only marker for poor
survival.9 Therefore, tricuspid valve replacement must be considered early when there is severe regurgitation with
either RV systolic dysfunction and/or heart failure symptoms.4 However valve repair is usually unsuccessful in the
setting of Ebstein-like anatomy of the valve.11
Recent studies have focused on the impact of pulmonary artery banding on systemic right ventricular function and
AV valve regurgitation. In a series in which banding was used for left ventricular conditioning prior to arterial
switching, it was suggested that pulmonary artery banding may improve systemic ventricular function and AV
valve regurgitation through a mechanism involving septal shift13 This approach appropriate with the study
conducted by Acar et al that interventions that increase right ventricular volume or decrease left ventricular
pressure are likely to induce tricuspid regurgitation, while those that decrease right ventricular volume or increase
left ventricular pressure are likely to improve tricuspid valve function.15
Double-Switch Procedure.
There are two major approaches to surgical management of L-TGA: classic repair and anatomic repair. 8 Classic
repair leaves the anatomic RV as the systemic ventricle, in which competent tricuspid valve (or left AV valve) and
good RV function are required. Anatomic repair makes the anatomic LV the systemic ventricle8 Long-term followup of conventional surgical approaches is disappointing and has led to novel surgical approaches aimed at
restoring normal AV and VA connections.13 The procedure has been successfully performed in children. It should
be considered for patients with severe tricuspid regurgitation and systemic ventricular dysfunction.Its purpose is
to relocate the left ventricle into the systemic circulation and the right ventricle into the pulmonary circulation,
achieving physiological correction. An atrial switch procedure (Mustard or Senning), together with either an
arterial switch procedure (when pulmonary stenosis is not present) or a Rastelli-type repair, the so-called Ilbawi
procedure (left ventricle tunneled to aorta and right ventricletopulmonary artery valved conduit when VSD and
pulmonary stenosis are present), can be performed after adequate left ventricular retraining, leaving the
regurgitant tricuspid valve and failing right ventricle on the pulmonary side. Early results are promising, but final
assessment of the evidence requires further long-term follow-up.11
Cardiac Transplantation.
Patients with deteriorating systemic (right) ventricular function should be treated aggressively with medical
therapy but may need to be considered for transplantation.11

Pacemaker implantation
Pacemaker implantation is usual when complete AV block is present. The optimal pacing modality is DDD. Active
fixation electrodes are required because of the lack of apical trabeculation in the morphological left ventricle.
Transvenous pacing should be avoided if there are intracardiac shunts because paradoxical emboli may occur.
Epicardial leads are preferred under these circumstances.11
DISCUSSION
In this case, the patient was asymptomatic and started to develop symptoms like exertional dyspnea and easy
fatigability since he was 22 years old, which is later known caused by systemic AV valve (tricuspid valve)
regurgitation. From age 22 until 6 months before admission, he never has any new complain nor take any
medication. The long asymptomatic period suggests that the patients has no severe associated cardiac defects.
The physical examination in this patient reveal a loud and single S2 at the upper left sternal border, In Congenitally
Corrected Transposition of the Great Arteries (cc TGA) single S2 is due to the inaudible P2 component since the
pulmonary artery located further from chest wall (and stethoscope).16 A grade 3/6 pansystolic murmur in this
patient later confirmed by echo study, originating from severe tricuspid (systemic AV valve regurgitation) .

Figure chest x ray of a patient with ccTGA

Mr. LBS (10/02/10) Mr. LBS (18/08/09)

The characteristic finding in the chest x- ray of cc TGA patients; a straight, left upper cardiac border formed by the
ascending aorta8, is not seen in this patient. In the 18/02/09 chest x ray the pulmonic segment was unseen, even
the ascendent aorta shape was not straight. In the 10/02/10 chest x ray the pulmonic segment could be seen,
probably because there was rotation to right when the x- ray was taken. There was sign of venous congestion,
present as cephalization of the pulmonary vessel, which suggests an elevated systemic ventricle filling pressure.
Together with evidence of cardiac enlargement (CTR 66%) this data suggests systemic ventricle failure.
The ECG showed characteristic pattern in which the direction of ventricular septal activation wave was different
from normal, because direction of ventricular septal depolarization in cc TGA is from the embryonic LV to RV. This
manifest as the negative initial deflection in V1 and the absence of Q wave in V5, and V6.8
The ECG from case report about a 70 years old woman by Matsakas et al in Greek (below), shows the almost
similar pattern to our patient with QS complex in V1, V2 and rS complex in V3 that resemble an old anteroseptal
myocardial infarction.7

Abnormality in ECG indeed, become some main reason of referral to the cardiologist. In a study doing by
Presbitero et al; the ECG abnormalities becoming reasons for the initial referral to a cardiologist in 7 from 16
patients. Three of them have an abnormal electrocardiogram (Q wave in V1-V3) one of which was interpreted for
years as an anterior myocardial infarction. The other 4 patients have complete heart block, three of whom
probably had it from birth 10
Patients with congenitally corrected transposition very often exhibit atrial tachyarrhythmias, such as atrial
fibrillation, atrial flutter, or supraventricular tachycardia. If there are no associated defects, the appearance of
these arrhythmias is delayed until the fifth decade of life, much later than in patients with other abnormalities. In
every case they are potentially damaging and should be treated.10 According to Presbitero et al patients with

atrial fibrillation or flutter anticoagulants must be used early because of the embolic risks and cardioversion
attempted early.10 In this patient digoxin 1 x 0,125 mg was used to control the rate of the atrial fibrilation, and
Simarc 1 x 2 mg was used to reduced the embolic risk
First degree AV block and second degree AV block was difficult to measure because of the atrial fibrilation there
was no sign third degree AV block.
The heart failure in this patient mainly manifest as pulmonary venous congestion, with dyspneu deffort,
orthopneu and paroxysmal nocturnal dyspneu as the chief complaint. According to Shaddy and Webb, when
pulmonary venous congestion does occur in ACHD patients, it does so primarily in patients without a systemic left
ventricle like congenitally corrected TGA (ccTGA)12
The echocardiography study which confirm the diagnosis of ccTGA with associated severe Tricuspid regurgitation
also shows that the systolic RV (systemic ventricle) function was severely reduced (ejection fraction 24%).
Although not enough evidence to draw recommendations for the treatment of heart failure in this population, in a
lot of case report, Angiotensin Converting Enzyme inhibitor (ACE-inhibitor) was used to treat the heart failure in
patient withv ccTGA. Matzakas et al treat a seventy year old woman with ccTGA using ACE inhibitor in their case
report in Greek.7 Presbitero et al in his research about cc TGA have a policy to give amiodarone, ACE inhibitors,
and diuretics with careful monitoring of potassium concentrations10 This practice is also supported by the result
of study of a group of adult patients with a variety of congenital heart defects that showed neurohumoral
activation similar to that seen in an adult heart failure population with acquired heart disease 12
However in ACC/AHA 2008 Guidelines for Adults With CHD it was stated that afterload reduction with ACE
inhibitors or angiotensin II receptor blockers may be less successful than when used for a morphological left
ventricle. This patient was given an Angiotensin II- Receptor Blockers (ARB) using Diovan 40 mg once a day to treat
his heart failure. ARB and ACE-inhibitor both inhibit the Renin-Angiotensin-Aldosterone System (RAAS). The overall
data from two major trials Val-HeFT and VALIANT show that ARBs has become a reasonable alternative for use in
heart failure. However ACE inhibitor remain the logical first line therapy because of the vast experience with these
agents in heart failure.17
Shaddy and Webb in their work about applying heart failure guidelines to adult congenital heart disease patients,
emphasized that all patients with heart failure and fluid overload should be appropriately diuresed,12 so
Furosemide use in this patient is appropriate.
Beta blockers have been conclusively demonstrated to reduce morbidity and mortality in chronic heart failure due
to acquired heart disease. However, appealing though it may be to extrapolate these data to patients with CHD,
there are major differences in the populations studied. Much of the heart failure literature deals with patients
above 60 years of age. Additionally, the most common cause of heart failure in the adult trials was ischemic heart
disease, although a significant percentage of patients in many of these studies had nonischemic cardiomyopathy.
ACHD patients with heart failure are generally younger, do not have coronary disease and have a lower annual
mortality rate.12
In other hand, beta blocker use is suported by three recent small uncontrolled series that reported a possible
benefit of β-blockers in adults with CHD and systemic right ventricular dysfunction12
In ACC/AHA 2008 Guidelines for Adults With CHD it was stated that data are lacking to support the use of beta
blockers to improve ventricular function in ccTGA, and caution must be used with dosage because of the
propensity for complete AV block.18 This patient was not given beta blockers in his medical therapy.
Severe tricuspid insufficiency in this patient should be treated earlier.4 Although it is not clear whether the severe
insufficiency of the systemic atrioventricular valve is the cause of the ventricular dysfunction or the result7 , the
valve should be considered for repair or replacement when ventricular function worsens in the face of moderately
severe to severe regurgitation, rather than waiting for a decline in functional status.4 More clearly the ACC/AHA
stated that operative intervention should be performed before the ejection fraction is less than 45%.18
According to Webb, Smallhorn, Therrien, and Redington, Double-Switch Procedure should be considered for
patients with severe tricuspid regurgitation and systemic ventricular dysfunction.11 But, in the setting of this
patient which is already 58 year old, with functional class II-III when he was discharged, we should consider the
risk and benefit of surgery procedure, because anatomic repair in adults is associated with a higher mortality. 18
Summary: This case presented a 58 year old man with heart failure caused by ccTGA, that diagnosed late in
adulthood. His long life duration is because the absence of associated defects. His heart failure was treated using

ARB and diuretics. Tricuspid replacement surgery cannot performed because severely depressed right (systemic)
ventricular function.

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