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Understanding a life-threatening heart defect

Transposition of the great arteries

By d'mya jonson

Types of TGA

VS

Dextro-Transposition of the great arteries

Levo-Transposition of the great arteries

  • Drinking alcohol or taking certain medicines during pregnancy
  • Poorly controlled diabetes during pregnancy
  • Smoking during pregnancy
  • History of German measles or another infection by a virus during pregnancy
  • Family history of congenital heart defects
Risk Factors:

Transpostion of the Great Arteries is present at birth, which makes it a congenital heart defect

Etiology/Origion

Transposition of the Great Arteries also known as (TGA) is a rare, but serious critical heart defect. The two main arteries, aorta and pulmonary artery are characterized by being in incorrect position by being reversed. TGA is so rare that it affects about 7% of babies born with a heart defect.

INtroduction to Transposition of the great arteries

Dextro-transposition of the great arteries

The most common form of TGA. The aorta is to the right of the pulmonary artery. In D-TGA deoxygenated blood from the body flows into the right side of the heart and is pumped back out of the body though the aorta. While the oxygenated blood from the lungs flow into the left side of the heart and is pumped back to the lungs through the pulmonary artery. This type reduces the amount of oxygenated blood to the body, and always needs to be repaired during the first year of life.

Levo-transposition of the grea arteries

A less common form also known as congenitally correct tga. The aorta is to the left of the pulmonary artery. While the left ventrical is on the right side and the right ventrical is on the left side. The right ventrical pumps blood to the body, which is normally the job of the left ventrical. This overworks the right ventrical and can weaken it. This type in the absence of other heart defects, may not require repair early in life.

  • Cyanosis
  • Weak pulse
  • Fast or labored breathing
  • Rapid Heart rate
  • Lack of appetite
  • Poor weight gain
  • Pounding heart
  • Difficulty feeding

Signs & Symptoms

TGA Clincal Findings

  • Echocardiogram An ultrasound of the heart that shows the abnormal connections of the great arteries, and how blood flows through the heart. In TGA, the great arteries run parallel to each other, instead of intertwining as in a normal heart.
  • Chest X-ray Shows the heart and lungs, and can indicate an egg-shaped heart
  • Electrocardiogram (ECG) Records the electrical activity of the heart, and can show right axis deviation and right ventricular hypertrophy.
  • Newborn pulse oximetry screening A simple bedside test that can identify some infants with TGA before they show symptoms.
Lab findings:

TGA Clinical Lab Findings

  • Parallel great arteries: The most significant feature of TGA, the great arteries run parallel to each other instead of crisscrossing.
  • Abnormal aorta: The aorta may be I-shaped or have an abnormal right convexity
  • Branching pattern: The branching pattern of the great vessels, especially the MPA, is often easily demonstrated
  • Two vessels in the three-vessel tracheal view: instead of the normal three vessels, only two are visible

Sonographic findings

The aorta is shown originating from the RV in the echo image. .

Sonographic images

Echocardiography in D-TGA. Five-chamber echocardiographic image shows the PA originating from the LV.

Sonographic images

Colour Doppler in outflow tract showing parallel relationship of outflow tracts.

Outflow tract view showing aorta (Ao) originating from right ventricle (RV), MPA origination LV, parallel relation of MPA and aorta (Ao), ventricular septal defect

Radiographic appearance of D-TGA, shows cardiomegaly, with narrowing of the superior mediastinum owing to the parallel position of the great arteries. There is increased pulmonary flow. The chest radiographic appearances resembles an egg on a string (line, oval).

Diagnosic images : Radiographic

D-TGA appearance at CT. Axial oblique reformatted maximum intensity projection image from cardiac CT. In a patient with D-TGA shows the LV connected to the main PA and the RV connected to the aorta (Ao).

DIAGNOSTIC IMAGES

D-TGA appearance at MRI shows the aorta located anterior to and to the right of the main PA. MR image in the same patient shows parallel orientation of the aorta and main PA.

DIAGNOSTIC IMAGES : MRI

Medical Management before surgery include:

Prognosis

Intravenous (IV) medication

Help the heart and lungs work better

Prostaglandin E1

Medication that helps blood flow through the heart

Supplemental oxygen

Or ventilator helps do the work of breathing

Prognosis

Balloon Atrial Septostomy: is a surgery some infants get a few hours after birth. This minimum invasive procedure uses a catheter to access the heart and create or enlarge a hole between the heart's upper pumping chambers. This procedure allows more oxygenated blood to get to the body. Infants who have BAS still need TGA repair surgery later.

The Mustard procedure redirects blood returning to the heart from the body to the lungs. This corrects circulation by diverting both vena cavae to the left atrium, while allowing the pulmonary return to enter the right atrium.

The Mustard procedure is a surgical technique that corrects transposition of the great arteries (TGA), a birth defect that swaps the main arteries leading from the heart to the lungs and body

Prognosis

TGA repair surgery is an open heart procedure called an arterial switch operation. A surgeon reconstructs the heart

Prognosis

Attaching the pulmonary artery to the right ventricleAttaching the aorta to the left ventricularAttaching the coronary arteries to the aorta

The Outcome

The outcome of people with Transposition of the Great Arteries (TGA) depends on whether they have corrective surgery and the type of surgery they receive: SurgeryMost infants who have surgery to correct TGA survive infancy and grow up normally. The 20-year survival rate for treated patients is close to 90%. No surgeryIf corrective surgery is not performed, the life expectancy is only months. Type of surgeryThe type of surgery received can impact the patient's long-term outcome. For example, the Mustard procedure can lead to late complications that cause a gradual decline in the patient's condition. In contrast, the Arterial Switch Operation (ASO) is associated with high survival rates in the short, medium, and long term.

Complications

Despite successful surgery ,complications can arise

Pulmonary Hypertension

Heart failure

Enlargement of aorta

Arrythimas- may require pacemaker or defibrillator

Quality of life

Patients with Transposition of the Great Arteries often report a good quality of life with proper medical care. Many participate in normal activities, but some may face limitations due to residual heart problem

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Canan, A., Ashwath, R., Agarwal, P., Francois, C., & Rajiah, P. (2021). Transposition of the great arteries | radiology reference article | radiopaedia.org. https://radiopaedia.org/articles/transposition-of-the-great-arteriesGrewal, D. S., Khanna, V., Saxena, S., & Chamoli, S. C. (2016, October). Sonographic diagnosis of transposition of great arteries in mid trimester: Our experience. Medical journal, Armed Forces India. https://pmc.ncbi.nlm.nih.gov/articles/PMC5099430/ Hopkins Medicine, J. (2024, April 30). Transposition of the great arteries (TGA). Johns Hopkins Medicine. https://www.hopkinsmedicine.org/health/conditions-and-diseases/transposition-of-the-great-arteries-tga Mayo clinic. (2023, April 4). Transposition of the great arteries. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/transposition-of-the-great-arteries/symptoms-causes/syc-20350589 Williams , W. G. (1998). Mustard operation - operative techniques in thoracic and cardiovascular surgery. https://www.optechtcs.com/article/S1522-2942(07)70092-2/fulltext

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