Transposition of the great vessels history and symptoms

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Dextro-transposition of the great arteries
L-transposition of the great arteries
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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.
• 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.
  • 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 VSD or pulmonic stenosis exists.
  • A murmur resulting from a 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

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

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

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