Transposition Of The Great Arteries: Clinical Anatomi, Comorbidities And Types Of Transposisition.
TRANSPOSITION OF THE GREAT ARTERIES:
CLINICAL ANATOMI, COMORBIDITIES AND
TYPES OF TRANSPOSISITION
Sri Endah Rahayuningsih,
Department of Pediatrics Hospital Dr. Hasan Sadikin,
Padjadjaran University School of Medicine Bandung
Background. Transposition of the great arteries (TAG) is one of the cyanotic congenital heart disease
(CHD) that manifests in the newborn period. Comorbid disorders often found are ventricular septal defect
(VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA), and left ventricular outflow tract
obstruction.
Purpose.To acknowledge the relationship of the type of transposition with comorbid disorders on the
transposition of the great arteries.
The method. This was an analytical descriptive study. The population was all patients who came to the
Heart Installation Service Dr. Hasan Sadikin Hospital Bandung to do echocardiography from January
2006 until January 2011. Subjects were all patients who met the inclusion criteria. Diagnosis was
confirmed by echocardiography. TAG was divided into 2 groups based on transposition type, the
complete transposition and partial transposition. Comorbid disorders in the TAG were divided into two
groups, namely the complex and non complex.
Results. During the study, 3910 children were performed echocardiography. There were 54 TAG children
who met the inclusion criteria. The youngest age at the time of echocardiography was 4 days, while the
oldest was 13 years of age. We found 47 TAG accompanied by DSV, most common type was
perimembran, and rarest was doubly commited. We also found 16 children with pulmonary stenosis
(mostly infundibular stenosis). Complex comorbid disorders were in the form of TAG with pulmonary
stenosis, DSV, found together with other TAG comorbid disorders, namely a complete AVSD, mitral
atresia, tricuspid atresia, single ventricle, and dekstrocardia situs inversus. There is a significant
relationship with the type of transposition to the comorbid disorders (p = 0.01).
Conclusion. Complete transposition TGA is more often accompanied by complex comorbid disorders.
Key words: transposition of great arteries, complete transposition, partial transposition.
Address of correspondence
DR. dr. Sri Endah Rahayuningsih SpA (K)
Department of Pediatrics
Padjadjaran University School of Medicine
Dr RS. Hasan Sadikin Bandung
Jl. Pasteur Bandung 38 40 161
Phone / Fax. 022-2034426
Email: endah.perkani @ gmail.com
Transposition of the great arteries (TAG) is one of the cyanotic congenital heart disease (CHD)
that manifests in the newborn period. 1, + .2 These abnormalities were found in 5-7% of all congenital
heart disease, especially in males. 1
, 3
The estimated incidence is 1:3.500-5.000 TAG live births. The
TAG etiology associated with the disruption of embryology at the time of the formation of arterial
trunk. Genetic factors are thought to contribute to the TAG. Without surgical correction, 30% will die
during the first week of life and 90% at the age of one year. 5-year survival rate of postsurgical
correction is more than 80%.
Comorbid disorders that often found are ventricular septal defect (VSD), atrial septal defect
(ASD), patent ductus artiousus (PDA), and left ventricular outflow tract obstruction. 3
On TAG there were abnormal arrangement exits of the arteries, the aorta comes out of the right ventricle
located anterior to the pulmonary artery, while the pulmonary artery comes out of the left ventricle,
situated posterior to the aorta. As a result, aorta receives blood from the vena cava vein, right atrium, right
ventricle, and forwarded the blood to the systemic circulation and blood from the pulmonary veins were
drained into the left atrium, left ventricle, and forwarded to the pulmonary artery and lungs. Thus, both
systemic and pulmonary circulation are separate and life can only take place if there is communication
between the two circulation. 1.2
Until now there are some varieties of classification of the transposition of the great arteries. 4 Jaggers et
al. 5 states in complete transposition, the aorta will be out from the right ventricle while the pulmonary
artery be out of the left ventricle, as if the aorta and pulmonary artery to move past the ventricular
septum. Partial transposition takes place only if one major artery that moves through the septum, whereas
the other major arteries remain in place, thus the large artery coming out of the right ventricle (double
outlet right ventricle) or from the left ventricle (double outlet left ventricle ).
Complete and partial transpositions are often accompanied by other comorbid disorders that would serve
as decision-making about the type of operation to be selected for the correction of TAB. 5.6
The purpose of this study is to investigate the characteristics of clinical anatomy, comorbid disorders, the
type of transposition, and relationship of the transposition types with comorbid disorders on the TAG.
Method
This was a descriptive analytic study. The population was all patients who came to the Heart Installation
Service Dr. Hasan Sadikin Hospital Bandung to do echocardiography from January 2006 until January
2011. Subjects were patients who met the TAG inclusion criteria. TAG diagnose was confirmed by
echocardiography. Inclusion criteria were patients who have not been performed surgery of TAG, have
completed data, and digital files stored on echocardiogram CD at the Heart Installation Service Dr. Hasan
Sadikin Hospital Bandung. Complete data included name, gender, birth date, weight, height, date of
echocardiography, and interpret results of echocardiography. A complete digital echocardiogram file
according to the segmental analysis approaches that include 1) visceral sites, 2) atrioventricular
relationship, 3) atrioventricular valve, 4) ventricular morphology, 5) relationship of arterial ventrikulo, 6)
the location of large arteries and 7) the infundibulum, and 8) other accompanying disorders. 2.7
Echocardiography
and
Doppler
examination
performed
by
a
pediatric
consultant
cardiologist.. Echocardiography performed on children in a state of calm or sleep on the appropriate use
of echocardiography Logic General Electric Type 700 and Type General Electric Vivid 3. Examination of
two-dimensional, M-mode and M-mode with the Doppler. Echocardiography examination results were
stored in the form of digital files on CD.
TAG was divided into 2 groups based on transposition types, complete and partial. Complete
transposition, the aorta came out of the right ventricle and the pulmonary artery came out of the left
ventricle. Partial transposition was namely only if one major artery that moves through the septum,
whereas the other major arteries remain in place, so that the two major arteries coming out of the right
ventricle (double outlet right ventricle) or from the left ventricle (double outlet left ventricle). 5 Comorbid
disorders on the TAG divided into two groups, namely the complex comorbid disorders (other than PDA,
VSD, ASD, and pulmonary stenosis) and complex (PDA, VSD, ASD, and pulmonary stenosis).
Simple TAG was noted only if discovered PDA as a comorbid disorder. Clinical anatomy to see the place
of communication between the systemic circulation and pulmonary circulation of theVSD, ASD and
PDA.
Result
During the study at the Heart Installation Service Dr. Hasan Sadikin Hospital Bandung , we found 3910
children that were performed echocardiography. TAG were found in 59 children who met the inclusion
criteria, five of whom had undergone surgery, thus excluded from the study.
Table 1. Characteristics of the Data by the Type of Transposition
Characteristics
Age at diagnose
Sex
Boys
Girls
Complete Transposition
4 days–72 months
Partial Transposition
7days–156 months
11
12
21
10
The youngest age at the time of echocardiography 4 days, while the oldest 13 years of age.
Table 2. Comorbid disorders on the TAG, clinical anatomy, and pulmonary stenosis
Comorbidities
n
Clinical Anatomy
Pulmonary stenosis
DAP DSA DSV PFO
Non complex
Simple
ASD
38
3 3
12 3
VSD
Complex
Mitral atresia
Single ventricle
Tricuspid atresia
Complete AVSD
Ectopic cordis
Total anomaly pulmonary vein drainage
Dekstrocardia situs inversus
21
16
2
3
1
4
1
3
2
12
10
-
2
5
10
21
4
7
1
1
1
1
1
1
1
1
1
2
4
3
1
2
3
3
4
1
1
2
1
2
1
1
2
1
-
Description:
PDA = duct arteriousus persiten;ASD = atrial septal defect; VSD = ventricular septal defect; persistent
foramen ovale PFO =
Most TAG cases accompanied by VSD, some cases were also accompanied by other comorbid
disorders, such as PDA, ASD and / or pulmonary stenosis .Only three cases of simple TAG, the TAG was
not accompanied by VSD and the ASD only with PDA. We found some rare cases of complex TAG, one
case with ectopic cardiac TAG, two cases dekstrocardia situs inversus TAG, and three cases of TAG
with total pulmonary vein drainage anomalies. (Table 2)
We found a 13-year-old case with partial transposition, there was a TAG, double outlet right
ventricle with pulmonary stenosis and DSV.
Figure 1. DSV type in TAB
Of the 54 cases found 47 cases of TAG accompanied by VSD. Most often type was perimembran, while
the rarest was doubly commited type. (Figure 1)
Figure 2. Types of pulmonary stenosis at the TAB
Of the 54 cases of TAG, 16 children with pulmonary stenosis, tmostly infundibular type. (Figure 2)
Figure 3. Pulmonary stenosis which is accompanied by other comorbid disorders on the TAB
Pulmonary stenosis most often accompanied the VSD in the case of TAG and can be found
together with other TAG comorbid disorders, such as a complete AVSD, mitral atresia, tricuspid
atresia, single ventricle, and dekstrocardia situs inversus. (Figure 3)
Table 3. Transposition type relationship with comorbid disorders
Comorbidities
Transpositin Type
Complex Non complex Total p
n=16
N=38
Partial Transposition
5
26
31
0,01
Complex Transposition 11
12
23
* Relationship significant if P
CLINICAL ANATOMI, COMORBIDITIES AND
TYPES OF TRANSPOSISITION
Sri Endah Rahayuningsih,
Department of Pediatrics Hospital Dr. Hasan Sadikin,
Padjadjaran University School of Medicine Bandung
Background. Transposition of the great arteries (TAG) is one of the cyanotic congenital heart disease
(CHD) that manifests in the newborn period. Comorbid disorders often found are ventricular septal defect
(VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA), and left ventricular outflow tract
obstruction.
Purpose.To acknowledge the relationship of the type of transposition with comorbid disorders on the
transposition of the great arteries.
The method. This was an analytical descriptive study. The population was all patients who came to the
Heart Installation Service Dr. Hasan Sadikin Hospital Bandung to do echocardiography from January
2006 until January 2011. Subjects were all patients who met the inclusion criteria. Diagnosis was
confirmed by echocardiography. TAG was divided into 2 groups based on transposition type, the
complete transposition and partial transposition. Comorbid disorders in the TAG were divided into two
groups, namely the complex and non complex.
Results. During the study, 3910 children were performed echocardiography. There were 54 TAG children
who met the inclusion criteria. The youngest age at the time of echocardiography was 4 days, while the
oldest was 13 years of age. We found 47 TAG accompanied by DSV, most common type was
perimembran, and rarest was doubly commited. We also found 16 children with pulmonary stenosis
(mostly infundibular stenosis). Complex comorbid disorders were in the form of TAG with pulmonary
stenosis, DSV, found together with other TAG comorbid disorders, namely a complete AVSD, mitral
atresia, tricuspid atresia, single ventricle, and dekstrocardia situs inversus. There is a significant
relationship with the type of transposition to the comorbid disorders (p = 0.01).
Conclusion. Complete transposition TGA is more often accompanied by complex comorbid disorders.
Key words: transposition of great arteries, complete transposition, partial transposition.
Address of correspondence
DR. dr. Sri Endah Rahayuningsih SpA (K)
Department of Pediatrics
Padjadjaran University School of Medicine
Dr RS. Hasan Sadikin Bandung
Jl. Pasteur Bandung 38 40 161
Phone / Fax. 022-2034426
Email: endah.perkani @ gmail.com
Transposition of the great arteries (TAG) is one of the cyanotic congenital heart disease (CHD)
that manifests in the newborn period. 1, + .2 These abnormalities were found in 5-7% of all congenital
heart disease, especially in males. 1
, 3
The estimated incidence is 1:3.500-5.000 TAG live births. The
TAG etiology associated with the disruption of embryology at the time of the formation of arterial
trunk. Genetic factors are thought to contribute to the TAG. Without surgical correction, 30% will die
during the first week of life and 90% at the age of one year. 5-year survival rate of postsurgical
correction is more than 80%.
Comorbid disorders that often found are ventricular septal defect (VSD), atrial septal defect
(ASD), patent ductus artiousus (PDA), and left ventricular outflow tract obstruction. 3
On TAG there were abnormal arrangement exits of the arteries, the aorta comes out of the right ventricle
located anterior to the pulmonary artery, while the pulmonary artery comes out of the left ventricle,
situated posterior to the aorta. As a result, aorta receives blood from the vena cava vein, right atrium, right
ventricle, and forwarded the blood to the systemic circulation and blood from the pulmonary veins were
drained into the left atrium, left ventricle, and forwarded to the pulmonary artery and lungs. Thus, both
systemic and pulmonary circulation are separate and life can only take place if there is communication
between the two circulation. 1.2
Until now there are some varieties of classification of the transposition of the great arteries. 4 Jaggers et
al. 5 states in complete transposition, the aorta will be out from the right ventricle while the pulmonary
artery be out of the left ventricle, as if the aorta and pulmonary artery to move past the ventricular
septum. Partial transposition takes place only if one major artery that moves through the septum, whereas
the other major arteries remain in place, thus the large artery coming out of the right ventricle (double
outlet right ventricle) or from the left ventricle (double outlet left ventricle ).
Complete and partial transpositions are often accompanied by other comorbid disorders that would serve
as decision-making about the type of operation to be selected for the correction of TAB. 5.6
The purpose of this study is to investigate the characteristics of clinical anatomy, comorbid disorders, the
type of transposition, and relationship of the transposition types with comorbid disorders on the TAG.
Method
This was a descriptive analytic study. The population was all patients who came to the Heart Installation
Service Dr. Hasan Sadikin Hospital Bandung to do echocardiography from January 2006 until January
2011. Subjects were patients who met the TAG inclusion criteria. TAG diagnose was confirmed by
echocardiography. Inclusion criteria were patients who have not been performed surgery of TAG, have
completed data, and digital files stored on echocardiogram CD at the Heart Installation Service Dr. Hasan
Sadikin Hospital Bandung. Complete data included name, gender, birth date, weight, height, date of
echocardiography, and interpret results of echocardiography. A complete digital echocardiogram file
according to the segmental analysis approaches that include 1) visceral sites, 2) atrioventricular
relationship, 3) atrioventricular valve, 4) ventricular morphology, 5) relationship of arterial ventrikulo, 6)
the location of large arteries and 7) the infundibulum, and 8) other accompanying disorders. 2.7
Echocardiography
and
Doppler
examination
performed
by
a
pediatric
consultant
cardiologist.. Echocardiography performed on children in a state of calm or sleep on the appropriate use
of echocardiography Logic General Electric Type 700 and Type General Electric Vivid 3. Examination of
two-dimensional, M-mode and M-mode with the Doppler. Echocardiography examination results were
stored in the form of digital files on CD.
TAG was divided into 2 groups based on transposition types, complete and partial. Complete
transposition, the aorta came out of the right ventricle and the pulmonary artery came out of the left
ventricle. Partial transposition was namely only if one major artery that moves through the septum,
whereas the other major arteries remain in place, so that the two major arteries coming out of the right
ventricle (double outlet right ventricle) or from the left ventricle (double outlet left ventricle). 5 Comorbid
disorders on the TAG divided into two groups, namely the complex comorbid disorders (other than PDA,
VSD, ASD, and pulmonary stenosis) and complex (PDA, VSD, ASD, and pulmonary stenosis).
Simple TAG was noted only if discovered PDA as a comorbid disorder. Clinical anatomy to see the place
of communication between the systemic circulation and pulmonary circulation of theVSD, ASD and
PDA.
Result
During the study at the Heart Installation Service Dr. Hasan Sadikin Hospital Bandung , we found 3910
children that were performed echocardiography. TAG were found in 59 children who met the inclusion
criteria, five of whom had undergone surgery, thus excluded from the study.
Table 1. Characteristics of the Data by the Type of Transposition
Characteristics
Age at diagnose
Sex
Boys
Girls
Complete Transposition
4 days–72 months
Partial Transposition
7days–156 months
11
12
21
10
The youngest age at the time of echocardiography 4 days, while the oldest 13 years of age.
Table 2. Comorbid disorders on the TAG, clinical anatomy, and pulmonary stenosis
Comorbidities
n
Clinical Anatomy
Pulmonary stenosis
DAP DSA DSV PFO
Non complex
Simple
ASD
38
3 3
12 3
VSD
Complex
Mitral atresia
Single ventricle
Tricuspid atresia
Complete AVSD
Ectopic cordis
Total anomaly pulmonary vein drainage
Dekstrocardia situs inversus
21
16
2
3
1
4
1
3
2
12
10
-
2
5
10
21
4
7
1
1
1
1
1
1
1
1
1
2
4
3
1
2
3
3
4
1
1
2
1
2
1
1
2
1
-
Description:
PDA = duct arteriousus persiten;ASD = atrial septal defect; VSD = ventricular septal defect; persistent
foramen ovale PFO =
Most TAG cases accompanied by VSD, some cases were also accompanied by other comorbid
disorders, such as PDA, ASD and / or pulmonary stenosis .Only three cases of simple TAG, the TAG was
not accompanied by VSD and the ASD only with PDA. We found some rare cases of complex TAG, one
case with ectopic cardiac TAG, two cases dekstrocardia situs inversus TAG, and three cases of TAG
with total pulmonary vein drainage anomalies. (Table 2)
We found a 13-year-old case with partial transposition, there was a TAG, double outlet right
ventricle with pulmonary stenosis and DSV.
Figure 1. DSV type in TAB
Of the 54 cases found 47 cases of TAG accompanied by VSD. Most often type was perimembran, while
the rarest was doubly commited type. (Figure 1)
Figure 2. Types of pulmonary stenosis at the TAB
Of the 54 cases of TAG, 16 children with pulmonary stenosis, tmostly infundibular type. (Figure 2)
Figure 3. Pulmonary stenosis which is accompanied by other comorbid disorders on the TAB
Pulmonary stenosis most often accompanied the VSD in the case of TAG and can be found
together with other TAG comorbid disorders, such as a complete AVSD, mitral atresia, tricuspid
atresia, single ventricle, and dekstrocardia situs inversus. (Figure 3)
Table 3. Transposition type relationship with comorbid disorders
Comorbidities
Transpositin Type
Complex Non complex Total p
n=16
N=38
Partial Transposition
5
26
31
0,01
Complex Transposition 11
12
23
* Relationship significant if P