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{{Transposition of the great vessels}}
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{{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]; [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@elon.edu]


==Overview==
==Overview==
The clinical features of D-TGA are solely dependent on the degree of mixing between the parallel circuits. Most patients present with signs and symptoms during the [[neonatal]] period. Symptoms of D-TGA present with [[cyanosis]], [[tachypnea]] and [[murmurs]]. Patients with L-TGA present with symptoms of [[heart failure]] until later in life when the [[right ventricle]] can no longer compensate increased [[after load]].
==History  ==
* '''Cyanosis''' can seen soon after the birth, due to the low oxygen saturation of the blood. 
*Screening ultrasounds do not routinely reveal TGA in-utero.
*Peripheral areas such as around the mouth and lips, fingertips, and toes are affected first because they are furthest from the heart, and since the circulated blood is not fully oxygenated to begin with, very little oxygen reaches the peripheral arteries.
== Symptoms ==
== Symptoms ==


'''Cyanosis''' will appear soon, due to the low oxygen saturation of the blood. Peripheral areas such as around the mouth and lips, fingertips, and toes are affected first because they are furthest from the heart, and since the circulated blood is not fully oxygenated to begin with, very little oxygen reaches the peripheral arteries.  
* The clinical features of D-TGA are solely dependent on the degree of mixing between the parallel circuits.<ref name="pmid17159076">{{cite journal |vauthors=Warnes CA |title=Transposition of the great arteries |journal=Circulation |volume=114 |issue=24 |pages=2699–709 |date=December 2006 |pmid=17159076 |doi=10.1161/CIRCULATIONAHA.105.592352 |url=}}</ref>
*Most patients present with signs and symptoms during the neonatal period (first 30 days of life).
 
The following are the typical clinical manifestations of TGA:
 
* [[Cyanosis]]
** The degree of [[cyanosis]] is dependent on the amount of mixing between the two parallel circuits.<ref name="pmid27244826">{{cite journal |vauthors=Oster ME, Aucott SW, Glidewell J, Hackell J, Kochilas L, Martin GR, Phillippi J, Pinto NM, Saarinen A, Sontag M, Kemper AR |title=Lessons Learned From Newborn Screening for Critical Congenital Heart Defects |journal=Pediatrics |volume=137 |issue=5 |pages= |date=May 2016 |pmid=27244826 |pmc=5227333 |doi=10.1542/peds.2015-4573 |url=}}</ref>
** Factors affecting intracardiac mixing include the size and presence of an [[ASD]] or [[VSD]].
** [[Cyanosis]] is not affected by exertion or supplemental [[oxygen]].
* Tachypnea
** Patients usually have a respiratory rate higher than 60 breaths per minute but without retractions, grunting, or flaring and appear comfortable.
* Murmurs
** Murmurs are not typically present unless a small [[Ventricular septal defect|VSD]] or [[pulmonic stenosis]] exists.<ref name="pmid2808986">{{cite journal |vauthors=Van Praagh R, Geva T, Kreutzer J |title=Ventricular septal defects: how shall we describe, name and classify them? |journal=J. Am. Coll. Cardiol. |volume=14 |issue=5 |pages=1298–9 |date=November 1989 |pmid=2808986 |doi=10.1016/0735-1097(89)90431-2 |url=}}</ref>
** A murmur resulting from a [[Ventricular septal defect|VSD]] will be pansystolic and prominent at the lower left sternal border.
** [[Pulmonic stenosis]] causes a [[systolic ejection murmur]] at the upper left sternal border.
 
* Patients with L-TGA are typically unaffected until later in life when the right ventricle can no longer compensate for the increased afterload of the systemic circulation. These patients present with signs and symptoms of heart failure.
* Other non-specific symptoms include:
**[[Diaphoresis]]
**[[Poor weight gain]]
**A [[gallop rhythm]]
**[[Hepatomegaly]]
 
<br />
{| class="wikitable"
|+
! colspan="2" |TGA manifestations depending upon intercirculatory mixing and the associated anatomic lesions
|-
|Transposition of the great arteries with intact ventricular septum
|
* Prominent and progressive cyanosis within the first 24 hours of life is the usual finding in infants if no significant mixing at the atrial level is evident.
|-
|Transposition of the great arteries with large ventricular septal defect
|
* Infants may not initially manifest symptoms of heart disease.
* Mild cyanosis is often noted during agitation or crying.
* Signs of congestive heart failure ([[tachypnea]], [[tachycardia]], [[diaphoresis]], and failure to gain weight) may become evident over the first 3-6 weeks as pulmonary blood flow increases.
|-
|Transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction
|
* Infants often present with extreme cyanosis at birth, proportional to the degree of left ventricular (pulmonary) outflow tract obstruction.
* The clinical history may be similar to that of an infant with [[Tetralogy of Fallot|tetralogy of Fallot.]]
|-
|Transposition of the great arteries with ventricular septal defect and pulmonary vascular obstructive disease
|
* The pulmonary vascular obstructive disease can prevent this rare subgroup of patients from developing symptoms of congestive heart failure, despite a large ventricular septal defect.
* Most often, patients present with progressive [[cyanosis]], despite an early successful palliative procedure.
|}


A d-TGA baby will exhibit indrawing beneath the ribcage and rapid breathing; this is likely a homeostatic reflex of the autonomic nervous system in response to hypoxic hypoxia. The infant will be easily fatigued and may experience weakness, particularly during feeding or playing; this interruption to feeding combined with hypoxia can cause failure to thrive. If d-TGA is not diagnosed and corrected early on, the infant may eventually experience syncopic episodes and develop clubbing of the fingers and toes.
== 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines<ref name="pmid30121240">{{cite journal| author=Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM | display-authors=etal| title=2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. | journal=J Am Coll Cardiol | year= 2019 | volume= 73 | issue= 12 | pages= 1494-1563 | pmid=30121240 | doi=10.1016/j.jacc.2018.08.1028 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30121240  }}</ref> ==


=== Diagnostic Recommendations for d-TGA With Atrial Switch ===
{| class="wikitable"
|-
| colspan="1" style="text-align:center; background:LightGreen" |[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class I]]
|-
| bgcolor="LightGreen" |'''1.'''Ambulatory monitoring for bradycardia or sinus node dysfunction is recommended for adults with d-TGA with atrial switch, especially if treated with beta blockers or other rate-slowing agents. ''(Level of Evidence: C-EO)''
|-
| bgcolor="LightGreen" | '''2.'''Adults with d-TGA with atrial switch repair should undergo annual imaging with either echocardiography or CMR to evaluate for common long-term complications of the atrial switch. ''(Level of Evidence: C-EO)''
|}
{| class="wikitable"
|-
| colspan="1" style="text-align:center; background:LemonChiffon" |[[ACC AHA guidelines classification scheme#Classification of Recommendations|Class IIa]]
|-
| bgcolor="LemonChiffon" | '''1.''' Assessment for a communication through the interatrial baffle or venous stenosis is reasonable for adults with d-TGA with atrial switch, particularly if transvenous pacemaker/ICD implantation is considered or leads are already present.''(Level of Evidence: C-LD)''
|}
==References==
==References==
{{reflist|2}}
{{reflist|2}}
 
[[Category:Cardiology]]
==Acknowledgements and Initial Contributors to Page==
[[Category:Congenital heart disease]]
Leida Perez, M.D.
[[Category:Pediatrics]]
[[Category:Embryology]]
[[Category:Disease]]




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{{WH}}
{{WS}}
{{WS}}

Latest revision as of 08:37, 16 December 2022

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]; Kristin Feeney, B.S. [5]

Overview

The clinical features of D-TGA are solely dependent on the degree of mixing between the parallel circuits. Most patients present with signs and symptoms during the neonatal period. Symptoms of D-TGA present with cyanosis, tachypnea and murmurs. Patients with L-TGA present with symptoms of heart failure until later in life when the right ventricle can no longer compensate increased after load.

History

  • Cyanosis can seen soon after the birth, due to the low oxygen saturation of the blood.
  • Screening ultrasounds do not routinely reveal TGA in-utero.
  • Peripheral areas such as around the mouth and lips, fingertips, and toes are affected first because they are furthest from the heart, and since the circulated blood is not fully oxygenated to begin with, very little oxygen reaches the peripheral arteries.

Symptoms

  • The clinical features of D-TGA are solely dependent on the degree of mixing between the parallel circuits.[1]
  • Most patients present with signs and symptoms during the neonatal period (first 30 days of life).

The following are the typical clinical manifestations of TGA:

  • Cyanosis
    • The degree of cyanosis is dependent on the amount of mixing between the two parallel circuits.[2]
    • Factors affecting intracardiac mixing include the size and presence of an ASD or VSD.
    • Cyanosis is not affected by exertion or supplemental oxygen.
  • Tachypnea
    • Patients usually have a respiratory rate higher than 60 breaths per minute but without retractions, grunting, or flaring and appear comfortable.
  • Murmurs
  • Patients with L-TGA are typically unaffected until later in life when the right ventricle can no longer compensate for the increased afterload of the systemic circulation. These patients present with signs and symptoms of heart failure.
  • Other non-specific symptoms include:


TGA manifestations depending upon intercirculatory mixing and the associated anatomic lesions
Transposition of the great arteries with intact ventricular septum
  • Prominent and progressive cyanosis within the first 24 hours of life is the usual finding in infants if no significant mixing at the atrial level is evident.
Transposition of the great arteries with large ventricular septal defect
  • Infants may not initially manifest symptoms of heart disease.
  • Mild cyanosis is often noted during agitation or crying.
  • Signs of congestive heart failure (tachypnea, tachycardia, diaphoresis, and failure to gain weight) may become evident over the first 3-6 weeks as pulmonary blood flow increases.
Transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction
  • Infants often present with extreme cyanosis at birth, proportional to the degree of left ventricular (pulmonary) outflow tract obstruction.
  • The clinical history may be similar to that of an infant with tetralogy of Fallot.
Transposition of the great arteries with ventricular septal defect and pulmonary vascular obstructive disease
  • The pulmonary vascular obstructive disease can prevent this rare subgroup of patients from developing symptoms of congestive heart failure, despite a large ventricular septal defect.
  • Most often, patients present with progressive cyanosis, despite an early successful palliative procedure.

2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines[4]

Diagnostic Recommendations for d-TGA With Atrial Switch

Class I
1.Ambulatory monitoring for bradycardia or sinus node dysfunction is recommended for adults with d-TGA with atrial switch, especially if treated with beta blockers or other rate-slowing agents. (Level of Evidence: C-EO)
2.Adults with d-TGA with atrial switch repair should undergo annual imaging with either echocardiography or CMR to evaluate for common long-term complications of the atrial switch. (Level of Evidence: C-EO)
Class IIa
1. Assessment for a communication through the interatrial baffle or venous stenosis is reasonable for adults with d-TGA with atrial switch, particularly if transvenous pacemaker/ICD implantation is considered or leads are already present.(Level of Evidence: C-LD)

References

  1. ↑ Warnes CA (December 2006). "Transposition of the great arteries". Circulation. 114 (24): 2699–709. doi:10.1161/CIRCULATIONAHA.105.592352. PMID 17159076.
  2. ↑ Oster ME, Aucott SW, Glidewell J, Hackell J, Kochilas L, Martin GR, Phillippi J, Pinto NM, Saarinen A, Sontag M, Kemper AR (May 2016). "Lessons Learned From Newborn Screening for Critical Congenital Heart Defects". Pediatrics. 137 (5). doi:10.1542/peds.2015-4573. PMC 5227333. PMID 27244826.
  3. ↑ Van Praagh R, Geva T, Kreutzer J (November 1989). "Ventricular septal defects: how shall we describe, name and classify them?". J. Am. Coll. Cardiol. 14 (5): 1298–9. doi:10.1016/0735-1097(89)90431-2. PMID 2808986.
  4. ↑ Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM; et al. (2019). "2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines". J Am Coll Cardiol. 73 (12): 1494–1563. doi:10.1016/j.jacc.2018.08.1028. PMID 30121240.


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