Difference between revisions of "Transposition of the great vessels history and symptoms"

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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: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]
+
{{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  ==
 
==History  ==
  
 
* '''Cyanosis''' can seen soon after the birth, due to the low oxygen saturation of the blood.   
 
* '''Cyanosis''' can seen soon after the birth, 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.  
+
*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 ==
  
* The parallel circulation in TGA results in a significant [[Hypoxemia|hypoxemic]] status that is observed clinically by [[central cyanosis]] contributed by
+
* 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>
** Limited inter circulatory mixing
+
*Most patients present with signs and symptoms during the neonatal period (first 30 days of life).  
** Associated left ventricular outflow tract obstruction or pulmonary obstructive disease (reduces the blood flow to the pulmonary vascular bed)
+
 
* However, if no obstructive lesions are present, and there is a large [[ventricular septal defect]] that allows for satisfactory mixing between the two circulations, [[cyanosis]] may go undetected and only be perceived during episodes of [[crying]] or [[agitation]].
+
The following are the typical clinical manifestations of TGA:
* In these cases, signs of [[congestive heart failure]] prevail due to excessive ventricular workload.
 
* [[Tachypnea]], [[tachycardia]], [[diaphoresis]], [[poor weight gain]], a [[gallop rhythm]], and eventually [[hepatomegaly]] can be then detected later on during [[infancy]].
 
* A D-TGA baby will exhibit in-drawing 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 [[Fatigue|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 [[syncope]] episodes and develop [[clubbing]] of the [[Finger|fingers]] and toes.
 
  
* The bluish discoloration of the [[skin]] and mucous membranes is therefore the basic pattern of clinical presentation in transposition.  
+
* [[Cyanosis]]
* Its onset and severity depend on anatomical and functional variants that influence the degree of mixing between the two circulations.
+
** 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
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** 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:
 
* Other non-specific symptoms include:
** [[Heart murmurs]] associated with left outflow tract obstruction, persistent arterial duct or due to a septal defect may be heard, but they are not a constant finding
+
**[[Diaphoresis]]
* 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.
+
**[[Poor weight gain]]
 +
**A [[gallop rhythm]]
 +
**[[Hepatomegaly]]
  
 
==References==
 
==References==

Revision as of 17:14, 19 February 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]; 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:

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.




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