Transposition of the great vessels history and symptoms
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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]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [5]
Overview
History
- 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.
Symptoms
- The parallel circulation in TGA results in a significant hypoxemic status that is observed clinically by central cyanosis contributed by
- Limited inter circulatory mixing
- 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.
- 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 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 fingers and toes.
- The bluish discoloration of the skin and mucous membranes is therefore the basic pattern of clinical presentation in transposition.
- Its onset and severity depend on anatomical and functional variants that influence the degree of mixing between the two circulations.
- 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
- 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.