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{{Transposition of the great vessels}}
{{Transposition of the great vessels}}


{{CMG}}; '''Associate Editor(s)-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, M.B.B.S.]] [mailto:psingh@perfuse.org]; {{CZ}}; [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]; '''Assistant Editor(s)-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@perfuse.org]
{{CMG}}; '''Associate Editor(s)-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, M.B.B.S.]] [mailto:psingh13579@gmail.com]; {{CZ}}; [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]; '''Assistant Editor(s)-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@elon.edu]


==Overview==
==Overview==
Patients with transposition of the great vessels should be differentiated from other cardiac and non-cardiac causes of cyanosis.


==Differentiating Transposition of the great vessels from other Diseases==
==Differentiating Transposition of the great vessels from other Diseases==
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* Abnormal hemoglobin like methemoglobin, polycythemia
* Abnormal hemoglobin like methemoglobin, polycythemia
* Peripheral cyanosis for e.g. sepsis, hypoglycemia, dehydration, and hypoadrenalism.
* Peripheral cyanosis for e.g. sepsis, hypoglycemia, dehydration, and hypoadrenalism.
Transposition of the great arteries should be differentiated from other [[cyanosis|cyanotic]] [[congenital heart disease]]s found in the pediatrics population. These [[disorders]] described in the table below:
{| class="wikitable"
|+
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Disorders
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Etiology
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Clinical Presentation
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory Findings
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Electrocardiogram Findings
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Echocardiography Findings
! align="center" style="background:#4479BA; color: #FFFFFF;" + |X-Ray Findings
|-
! style="padding: 5px 5px; background: #DCDCDC; " align="left"|'''[[Tetralogy of Fallot]]''' <ref name="MorrisFelner1975">{{cite journal|last1=Morris|first1=Douglas C.|last2=Felner|first2=Joel M.|last3=Schlant|first3=Robert C.|last4=Franch|first4=Robert H.|title=Echocardiographic diagnosis of tetralogy of Fallot|journal=The American Journal of Cardiology|volume=36|issue=7|year=1975|pages=908–913|issn=00029149|doi=10.1016/0002-9149(75)90081-8}}</ref><ref name="pmid1428277">{{cite journal |vauthors=Kothari SS |title=Mechanism of cyanotic spells in tetralogy of Fallot--the missing link? |journal=Int. J. Cardiol. |volume=37 |issue=1 |pages=1–5 |date=October 1992 |pmid=1428277 |doi=10.1016/0167-5273(92)90125-m |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |Multifactorial


* [[Polymorphism (biology)|Genetic polymorphisms]]
* Maternal [[rubella]]
* Poor prenatal nutrition
* Maternal [[alcohol]] use
* Maternal [[diabetes]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[Cyanosis]] on exertion
* [[Exertional dyspnea]]
* [[Palpitation|Palpitations]]
* [[Fatigue]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* CBC: [[Anemia]] or [[polycythemia]].
* [[Coagulation]] profile.
* Arterial blood gas: Low [[oxygen saturation]] and [[acidosis]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
*[[Right ventricular hypertrophy]]
*[[Right bundle branch block]]
*[[Tachycardia]]
* Rate of [[QRS complex|QRS]] change predicts [[Ventricular arrhythmias|ventricular arrhythmia]]
|[[Echocardiography]] may show:
* Residual [[Ventricular septal defect|VSD]] or [[Atrial septal defect|ASD]]
* RV outflow tract obstruction
* Abnormal valvular anatomy
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* The '''boot-shaped''' [[heart]] appearance
* Normal [[heart]] size
* [[Pulmonary]] vascular marking may be normal or decreased
|-
! style="padding: 5px 5px; background: #DCDCDC; " align="left"|'''[[Total anomalous pulmonary venous connection|Total Anomalous Pulmonary Venous Connection]]''' <ref name="ZhangZhang2016">{{cite journal|last1=Zhang|first1=Ziming|last2=Zhang|first2=Li|last3=Xie|first3=Feng|last4=Wang|first4=Bing|last5=Sun|first5=Zhengxing|last6=Kong|first6=Shuangshuang|last7=Wang|first7=Xinfang|last8=Dong|first8=Nianguo|last9=Wang|first9=Guohua|last10=Lv|first10=Qing|last11=Li|first11=Yuman|last12=Li|first12=Ling|last13=Xie|first13=Mingxing|title=Echocardiographic diagnosis of anomalous pulmonary venous connections|journal=Medicine|volume=95|issue=44|year=2016|pages=e5389|issn=0025-7974|doi=10.1097/MD.0000000000005389}}</ref><ref name="pmid389559">{{cite journal |vauthors=Chen JT |title=Radiologic demonstration of anomalous pulmonary venous connection and its clinical significance |journal=CRC Crit Rev Diagn Imaging |volume=11 |issue=4 |pages=383–422 |date=October 1979 |pmid=389559 |doi= |url=}}</ref><ref name="GathmanNadas1970">{{cite journal|last1=Gathman|first1=Gary E.|last2=Nadas|first2=Alexander S.|title=Total Anomalous Pulmonary Venous Connection|journal=Circulation|volume=42|issue=1|year=1970|pages=143–154|issn=0009-7322|doi=10.1161/01.CIR.42.1.143}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |Multifactorial
*[[Polymorphism (biology)|Genetic polymorphisms]]
* Maternal [[rubella]]
* Poor prenatal nutrition
* Maternal [[alcohol]] use
* Maternal [[diabetes]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[Tachypnea]]
* [[Palpitation|Palpitations]]
* [[Cyanosis]]
* [[Failure to thrive]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Arterial blood gas: Low [[oxygen saturation]] and [[acidosis]]
* [[Coagulation]] profile.
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[Right ventricular hypertrophy]] with a qR pattern
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
*[[Right ventricular hypertrophy]]
*Right ventricular loading
*Paradoxical septal motion
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Prominence of the [[pulmonary]] arteries
* Mild enlargement of [[heart]]
* The classic '''snowman''' sign is seen in supracardiac subtype
|-
! style="padding: 5px 5px; background: #DCDCDC; " align="left"|'''[[Tricuspid atresia|Tricuspid Atresia]]''' <ref name="BeppuNimura1978">{{cite journal|last1=Beppu|first1=S|last2=Nimura|first2=Y|last3=Tamai|first3=M|last4=Nagata|first4=S|last5=Matsuo|first5=H|last6=Kawashima|first6=Y|last7=Kozuka|first7=T|last8=Sakakibara|first8=H|title=Two-dimensional echocardiography in diagnosing tricuspid atresia. Differentiation from other hypoplastic right heart syndromes and common atrioventricular canal.|journal=Heart|volume=40|issue=10|year=1978|pages=1174–1183|issn=1355-6037|doi=10.1136/hrt.40.10.1174}}</ref><ref name="pmid7049815">{{cite journal |vauthors=Thiene G, Anderson RH |title=The clinical morphology of tricuspid atresia. Atresia of the right atrioventricular valve |journal=G Ital Cardiol |volume=11 |issue=12 |pages=1845–59 |date=1981 |pmid=7049815 |doi= |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |Multifactorial
*[[Polymorphism (biology)|Genetic polymorphisms]]
* Maternal [[rubella]]
* Poor prenatal nutrition
* Maternal [[alcohol]] use
* Maternal [[diabetes]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Respiratory difficulties  as nasal flaring or muscle retractions
* [[Cyanosis]]
* Growth retradation
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[Arterial blood gas|Arterial blood gases]]
* [[Complete blood count|CBC]]: [[Polycythemia]]
* [[Coagulation]] profile
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Tall [[P wave|P waves]] indicate atrial enlargement.
* First-degree [[atrioventricular block]].
* Frontal plane [[QRS complex|QRS]] axis may be leftward.
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |Echocardiography may show
* Defect size
* Pulmonary [[blood flow]]
*[[Ventricular function]]
* Valve abnormalities
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Smooth convexity of right [[heart]] border is absent
* Lower part of right [[heart]] border is limited to the [[spine]]
* Prominency of the [[pulmonary artery]] depends on their [[blood]] flow
* [[Cardiac]] size may be normal or enlarged
|-
! style="padding: 5px 5px; background: #DCDCDC; " align="left"|'''[[Transposition of the great vessels|Transposition of the Great Arteries]]''' <ref name="MahleGonzalez2013">{{cite journal|last1=Mahle|first1=William T.|last2=Gonzalez|first2=Javier H.|last3=Kreeger|first3=Joseph|last4=Marx|first4=Gerald|last5=Duldani|first5=Gul|last6=Silverman|first6=Norman H.|title=Echocardiography of transposition of the great arteries|journal=Cardiology in the Young|volume=22|issue=6|year=2013|pages=664–670|issn=1047-9511|doi=10.1017/S1047951112001503}}</ref><ref name="Warnes2006">{{cite journal|last1=Warnes|first1=Carole A.|title=Transposition of the Great Arteries|journal=Circulation|volume=114|issue=24|year=2006|pages=2699–2709|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.105.592352}}</ref>
|Multifactorial
*[[Polymorphism (biology)|Genetic polymorphisms]]
* Poor prenatal nutrition
* Maternal [[alcohol]] use
* Maternal [[diabetes]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Prominent [[cyanosis]] within hours of birth
*[[Congestive heart failure]]
*
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
*[[Arterial blood gas|Arterial blood gases]]: [[Hypoxemia]]
* Hyperoxia test
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[Right ventricular hypertrophy]]
* [[Right axis deviation]]
* Varying degrees of [[Atrioventricular block|AV block]]
* [[Q waves]]
|[[Echocardiography]] may show:
* Relationship between [[great vessels]]
* Associated anatomic lesions
* [[Coronary artery]] origin and branches
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* The classic '''egg on string''' appearance
* [[Pulmonary]] vascular marking may be normal or increased
|}
==References==
==References==
{{reflist|2}}
{{reflist|2}}
[[Category:Cardiology]]
[[Category:Congenital heart disease]]
[[Category:Genetic Disease]]
[[Category:Disease]]


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Latest revision as of 03:56, 11 April 2020


Transposition of the great vessels Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [2]; Cafer Zorkun, M.D., Ph.D. [3]; Keri Shafer, M.D. [4]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [5]

Overview

Patients with transposition of the great vessels should be differentiated from other cardiac and non-cardiac causes of cyanosis.

Differentiating Transposition of the great vessels from other Diseases

Patients with transposition of the great vessels should be differentiated from other cardiac and non-cardiac causes of cyanosis-

Cardiac causes (starts with 't')-

  • Tetralogy of Fallot
  • Truncus arteriosus
  • Total anomalous pulmonary venous connection
  • Tricuspid valve abnormalities

Other less common causes are- pulmonary atresia, hypoplastic left heart syndrome, anomalous systemic venous connection.

Non-cardiac causes

  • Pulmonary diseases - Structural abnormalities of the lung, V/P (ventilation-perfusion mismatch), airway obstruction, pneumothorax, and hypoventilation.
  • Abnormal hemoglobin like methemoglobin, polycythemia
  • Peripheral cyanosis for e.g. sepsis, hypoglycemia, dehydration, and hypoadrenalism.

Transposition of the great arteries should be differentiated from other cyanotic congenital heart diseases found in the pediatrics population. These disorders described in the table below:

Disorders Etiology Clinical Presentation Laboratory Findings Electrocardiogram Findings Echocardiography Findings X-Ray Findings
Tetralogy of Fallot [1][2] Multifactorial Echocardiography may show:
  • Residual VSD or ASD
  • RV outflow tract obstruction
  • Abnormal valvular anatomy
  • The boot-shaped heart appearance
  • Normal heart size
  • Pulmonary vascular marking may be normal or decreased
Total Anomalous Pulmonary Venous Connection [3][4][5] Multifactorial
  • Prominence of the pulmonary arteries
  • Mild enlargement of heart
  • The classic snowman sign is seen in supracardiac subtype
Tricuspid Atresia [6][7] Multifactorial
  • Respiratory difficulties as nasal flaring or muscle retractions
  • Cyanosis
  • Growth retradation
  • Tall P waves indicate atrial enlargement.
  • Frontal plane QRS axis may be leftward.
Echocardiography may show
Transposition of the Great Arteries [8][9] Multifactorial Echocardiography may show:
  • The classic egg on string appearance
  • Pulmonary vascular marking may be normal or increased

References

  1. Morris, Douglas C.; Felner, Joel M.; Schlant, Robert C.; Franch, Robert H. (1975). "Echocardiographic diagnosis of tetralogy of Fallot". The American Journal of Cardiology. 36 (7): 908–913. doi:10.1016/0002-9149(75)90081-8. ISSN 0002-9149.
  2. Kothari SS (October 1992). "Mechanism of cyanotic spells in tetralogy of Fallot--the missing link?". Int. J. Cardiol. 37 (1): 1–5. doi:10.1016/0167-5273(92)90125-m. PMID 1428277.
  3. Zhang, Ziming; Zhang, Li; Xie, Feng; Wang, Bing; Sun, Zhengxing; Kong, Shuangshuang; Wang, Xinfang; Dong, Nianguo; Wang, Guohua; Lv, Qing; Li, Yuman; Li, Ling; Xie, Mingxing (2016). "Echocardiographic diagnosis of anomalous pulmonary venous connections". Medicine. 95 (44): e5389. doi:10.1097/MD.0000000000005389. ISSN 0025-7974.
  4. Chen JT (October 1979). "Radiologic demonstration of anomalous pulmonary venous connection and its clinical significance". CRC Crit Rev Diagn Imaging. 11 (4): 383–422. PMID 389559.
  5. Gathman, Gary E.; Nadas, Alexander S. (1970). "Total Anomalous Pulmonary Venous Connection". Circulation. 42 (1): 143–154. doi:10.1161/01.CIR.42.1.143. ISSN 0009-7322.
  6. Beppu, S; Nimura, Y; Tamai, M; Nagata, S; Matsuo, H; Kawashima, Y; Kozuka, T; Sakakibara, H (1978). "Two-dimensional echocardiography in diagnosing tricuspid atresia. Differentiation from other hypoplastic right heart syndromes and common atrioventricular canal". Heart. 40 (10): 1174–1183. doi:10.1136/hrt.40.10.1174. ISSN 1355-6037.
  7. Thiene G, Anderson RH (1981). "The clinical morphology of tricuspid atresia. Atresia of the right atrioventricular valve". G Ital Cardiol. 11 (12): 1845–59. PMID 7049815.
  8. Mahle, William T.; Gonzalez, Javier H.; Kreeger, Joseph; Marx, Gerald; Duldani, Gul; Silverman, Norman H. (2013). "Echocardiography of transposition of the great arteries". Cardiology in the Young. 22 (6): 664–670. doi:10.1017/S1047951112001503. ISSN 1047-9511.
  9. Warnes, Carole A. (2006). "Transposition of the Great Arteries". Circulation. 114 (24): 2699–2709. doi:10.1161/CIRCULATIONAHA.105.592352. ISSN 0009-7322.

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