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dextro-looped transposition of the great arteries

ICD-10 Codes

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Description

Dextro-Looped Transposition of the Great Arteries (d-TGA)

Dextro-looped transposition of the great arteries, also known as d-TGA, is a congenital heart defect that occurs when the two main blood vessels leaving the heart are in abnormal positions. This condition is characterized by complete inversion of the great vessels, so that:

  • The aorta arises from the right ventricle instead of the left ventricle [3][6]
  • The pulmonary artery arises from the left ventricle instead of the right ventricle [4]

This means that the blood flow cycle is impaired, and the heart's ability to provide oxygen-rich blood to the body is severely limited. As a result, the body receives less oxygenated blood, leading to cyanosis (a bluish discoloration of the skin) [7].

In d-TGA, the ventricles are oriented so that the right ventricle is positioned to the right of the left ventricle, and the origin of the great vessels is reversed. This condition can lead to serious complications or death if left untreated.

Key Features:

  • Complete inversion of the great vessels
  • Aorta arises from the right ventricle
  • Pulmonary artery arises from the left ventricle
  • Impaired blood flow cycle
  • Cyanosis (bluish discoloration of the skin)
  • Requires surgical repair to correct [9][10]

References:

[3] The more common form of transposition of the great arteries, dextro-looped TGA, consists of complete inversion of the great vessels, so that the aorta incorrectly arises from the right ventricle and the pulmonary artery incorrectly arises from the left ventricle.

[4] In dextro-transposition of the great arteries (d-TGA), the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle,

[6] Uniprot Description A congenital heart defect consisting of complete inversion of the great vessels, so that the aorta incorrectly arises from the right ...

[7] Dextro-transposition of the great arteries (D-TGA) is a cyanotic condition that accounts for approximately 8% of congenital cardiac malformations and has a ...

[9] Dextro-Transposition (pronounced DECKS-tro-looped) of the Great Arteries, also known as d-TGA, is a congenital heart defect that occurs when the two main blood vessels leaving the heart are in abnormal positions.

[10] Symptoms are usually noticed during pregnancy, immediately after birth or within a few weeks of birth. Without treatment, serious complications or death can occur.

Additional Characteristics

  • congenital heart defect
  • complete inversion of the great vessels
  • aorta arises from the right ventricle
  • pulmonary artery arises from the left ventricle
  • impaired blood flow cycle
  • cyanosis (bluish discoloration of the skin)
  • requires surgical repair to correct

Signs and Symptoms

Common Signs and Symptoms

Dextro-looped transposition of the great arteries (d-TGA) is a congenital heart defect that can cause various signs and symptoms in affected individuals. Some of the common signs and symptoms include:

  • Neonatal Cyanosis: A bluish discoloration of the skin due to inadequate oxygenation of the blood, which is often present at birth [1].
  • Heart Failure: In some cases, heart failure can occur if there is an associated ventricular septal defect (VSD) [3].
  • Abnormal Heart Sound: An abnormal sound during a heartbeat, known as a heart murmur, can be heard in individuals with d-TGA [4][5].
  • Rapid Breathing: Rapid breathing or tachypnea can occur due to the body's attempt to compensate for inadequate oxygenation [4][5].
  • Low Blood Pressure: Low blood pressure or hypotension can also be present in some cases [4][5].

Long-term Complications

In addition to these immediate signs and symptoms, individuals with d-TGA may experience long-term complications, including:

  • Coronary Stenoses: Narrowing of the coronary arteries, which can lead to reduced blood flow to the heart muscle [7].
  • Distortion of Pulmonary Arteries: Abnormal development of the pulmonary arteries, which can affect lung function [7].
  • Aortic Regurgitation: Leaking of the aortic valve, which can lead to inadequate blood pressure and other complications [7].

Other Symptoms

In some cases, individuals with d-TGA may experience additional symptoms, such as:

  • Diaphoresis: Excessive sweating
  • Irritability: Restlessness or agitation
  • Failure to Gain Weight: Inadequate weight gain due to inadequate nutrition and energy expenditure [6][9]

It's essential to note that not all individuals with d-TGA will experience these symptoms, and the severity of the condition can vary widely from person to person.

Additional Symptoms

Diagnostic Tests

Diagnostic Tests for Dextro-Looped Transposition of the Great Arteries

Dextro-looped transposition of the great arteries (d-TGA) is a congenital heart defect where the two main arteries that carry blood out of the heart are reversed. Diagnosing d-TGA requires a combination of clinical evaluation, imaging studies, and laboratory tests.

Common Diagnostic Tests:

  • Echocardiogram: An echocardiogram is an ultrasound of the heart that can show incorrect positioning of the two main arteries [1]. It is often the first test used to diagnose d-TGA.
  • 2-Dimensional Echocardiography: This type of echocardiogram provides a detailed image of the heart's structure and function, which can help establish the diagnosis of d-TGA [2].
  • EKG (Electrocardiogram): An EKG is a test that measures the electrical activity of the heart. It may show abnormal heart rhythms or other signs of d-TGA [3].
  • Holter Monitor: A Holter monitor is a portable device that records the heart's electrical activity over a 24-hour period. It can help diagnose arrhythmias and other cardiac conditions associated with d-TGA [4].
  • Stress Testing: Stress testing, such as an exercise stress test or a pharmacological stress test, can be used to evaluate the heart's function under physical or chemical stress [5].

Additional Diagnostic Tests:

  • MRI (Magnetic Resonance Imaging): MRI can provide detailed images of the heart and its blood vessels, which can help diagnose d-TGA and assess cardiac function [6].
  • CT/CTA: CT scans with contrast enhancement can directly visualize abnormal great vessel anatomy and assess cardiac function [7].

Genetic Testing:

While not a primary diagnostic test for d-TGA, genetic testing may be used to identify genetic mutations associated with the condition.

It's essential to consult with a cardiologist or a pediatrician specializing in congenital heart defects to determine the best course of diagnosis and treatment for an individual case of d-TGA.

References:

[1] May 15, 2024 — The most common test is an echocardiogram, which is an ultrasound of the heart. An echocardiogram can show incorrect positioning of the two main arteries. [2] Diagnosis of transposition of the great arteries is suspected clinically, supported by chest x-ray and ECG, and established by 2-dimensional echocardiography. [3] Routine evaluation may include EKG, echocardiogram, Holter monitor and stress testing. [

Additional Diagnostic Tests

  • Genetic Testing
  • Echocardiogram
  • MRI (Magnetic Resonance Imaging)
  • 2-Dimensional Echocardiography
  • EKG (Electrocardiogram)
  • Holter Monitor
  • Stress Testing
  • CTA

Treatment

Medications for d-TGA

While surgical repair is the preferred treatment for dextro-transposition of the great arteries (d-TGA), certain medications can help alleviate symptoms and improve quality of life. According to medical literature, patients with d-TGA may benefit from:

  • Medications to help the heart pump better: These include medications that increase cardiac output and reduce symptoms of heart failure [3].
  • Diuretics: To control fluid accumulation in the body, which can be a concern for individuals with d-TGA [3].
  • Blood pressure control: Medications may be prescribed to manage high blood pressure, which is common in patients with d-TGA [3].

However, it's essential to note that these medications are not a cure for d-TGA and should be used under the guidance of a healthcare provider. The primary goal of treatment remains surgical correction of the defect.

References:

  • Medications to help the heart pump better, control fluid accumulation (diuretics) and control blood pressure may help [3].
  • Patients who've had the arterial switch operation may still require medications to manage their condition [3].

Recommended Medications

  • Diuretics
  • Medications that increase cardiac output
  • Blood pressure control

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Differential Diagnosis of Dextro-Looped Transposition of the Great Arteries

Dextro-looped transposition of the great arteries (d-TGA) is a congenital heart defect characterized by discordant connections between the ventricles and great arteries. The differential diagnosis for d-TGA involves considering other conditions that may present with similar clinical features.

Conditions to Consider:

  • Complete Transposition of the Great Arteries: This condition is similar to d-TGA, but the aorta arises from the left ventricle instead of the right.
  • Double Outlet Right Ventricle (DORV): In DORV, both the aorta and pulmonary artery arise from the right ventricle, leading to a similar presentation as d-TGA.
  • Truncus Arteriosus: This condition involves a single common arterial trunk arising from the heart, which can lead to similar clinical features as d-TGA.

Key Features to Distinguish:

  • Aortic and Pulmonary Artery Origins: In d-TGA, the aorta arises from the right ventricle, while in complete transposition, it arises from the left ventricle.
  • Ventricular Septal Defects (VSDs): The presence of VSDs can be a distinguishing feature between d-TGA and other conditions like DORV or truncus arteriosus.

Diagnostic Tests:

  • Echocardiography: This imaging test can help visualize the heart's anatomy and identify any abnormalities.
  • Cardiac Catheterization: This procedure involves inserting a catheter into the heart to measure blood pressures and oxygen levels, which can aid in diagnosing d-TGA.

References:

  • [3] Transposition of the great arteries (TGA) is a congenital conotruncal abnormality characterized by discordant connections between the ventricles and great arteries.
  • [11] In dextro-transposition of the great arteries (d-TGA), the aorta arises from the right ventricle and the pulmonary trunk from the left ventricle, resulting in 2 parallel circuits that are incompatible with life unless there is a shunt, such as a patent ductus arteriosus (PDA) or septal defect, to allow for blood mixing.
  • [13] A diagnostic dilemma: transposition of the great arteries. Cureus. 2023 May. 15(5):e38931.

Note: The above answer is based on the provided context and search results. It is essential to consult with a medical professional for an accurate diagnosis and treatment plan.

Additional Differential Diagnoses

Additional Information

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