Presentation
Stenosis of valves or vessels may also be present. When no other heart defects are present it is called 'simple' d-TGA; when other defects are present it is called 'complex' d-TGA. [en.wikipedia.org]
If the anomaly is complicated by right ventricular or tricuspid valve dysfunction, patients can present with exertional dyspnea and easy fatigability. [radiopaedia.org]
Background Transposition of the great arteries (TGA) is the most common cyanotic congenital heart lesion that presents in neonates. [emedicine.com]
If no obstructive lesions are present and there is a large VSD, cyanosis may go undetected and only be perceived during episodes of crying or agitation. In these cases, signs of congestive heart failure prevail. [orpha.net]
Entire Body System
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Falling
Patients with a large ventricular septal defect, a patent ductus arteriosus, or both may have an early predilection for congestive heart failure, as pulmonary vascular resistance falls with increasing age. [emedicine.com]
Sierra M, Machado C Magnetic resonance imaging in patients with implantable cardiac devices Rev Cardiovasc Med. 2008 Fall;9(4):232-8 19. [crcd.eu]
During pregnancy the fall in systemic vascular resistance and rise in cardiac output exacerbates any right-to-left shunting, worsening pre-existing cyanosis and hypoxia. [clinicaladvisor.com]
[Celermajer] Ebstein W. (1866) Ueber einen sehr seltenen Fall von Insufficienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung. Arch Anat physiol;33:238. [Ebstein] Mulder, B. J. M. (2002). [textbookofcardiology.org]
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Hypothermia
Anaesthetic considerations related to TGA with brain abscess are following: maintenance of intravascular volume and preload and avoidance of precursors to acidosis such as hypothermia, hypercarbia, and hypotension. [medcraveonline.com]
This can also be precipitated by hypoxia, hypercarbia, acidosis or hypothermia during general anesthesia. [5] Also general anesthetics reduce SVR, which can lead to a dominant right to left shunt and cyanosis. [aeronline.org]
Gastrointestinal
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Failure to Thrive
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. [en.wikipedia.org]
Workup
X-Ray
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Pneumoperitoneum
Pneumoperitoneum was limited at 15 cm H 2 O. He experienced an uneventful post-surgical course, with complete resolution of his nausea, vomiting, and right upper quadrant pain at one-month follow-up. [oatext.com]
Blocks
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Right Bundle Branch Block
Electrocardiography findings were sinus rhythm with first degree heart block, right bundle branch block and right ventricular hypertrophy. [aeronline.org]
Hypertrophy
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Biventricular Hypertrophy
The main electrocardiographic features are a rightward deviation of the QRS complex axis associated with right ventricular hypertrophy; biventricular hypertrophy is evident in conditions that lead to a left ventricular overload. [ojrd.biomedcentral.com]
Treatment
Management and treatment Palliative treatment with prostaglandin E1 and balloon atrial septostomy are usually required soon after birth. Surgical correction is performed at a later stage. [orpha.net]
Daubeney, offers essential guidance on the anatomical issues, clinical presentation, diagnosis, and treatment options available to practitioners today. [books.google.de]
Prepare for clinical challenges and save time in addressing them thanks to expert advice on treatment options from international contributors. [books.google.com]
If the diagnosis is made in a standard hospital or other clinical facility, the baby will be transferred to a children's hospital, if such facilities are available, for specialized paediatric treatment and equipment. [en.wikipedia.org]
Prognosis
Prognosis With the advent of newer and improved surgical techniques and improved post-operative intensive care, the long-term survival is approximately 90% at 15 years of age. [orpha.net]
Treatment [ edit ] Simple l-TGA has a very good prognosis, with many individuals being asymptomatic and not requiring surgical correction. [en.wikipedia.org]
Treatment and prognosis Early surgical treatment was aimed only at repairing the associated anomalies (i.e. VSD, pulmonary stenosis or atresia and tricuspid valve abnormalities). [radiopaedia.org]
Prognosis The prognosis depends on the specific anatomic substrate and type of surgical therapy used (arterial switch operation, atrial switch operation, or Rastelli procedure). [emedicine.com]
Broadly speaking, prognosis in congenital heart lesions can be grouped into those lesions with a good outcome (normal or near normal prognosis), those with an intermediate outcome (residual haemodynamic abnormalities and therefore a more guarded prognosis [academic.oup.com]
Etiology
Etiology The exact etiology remains unknown. Some associated risk factors (gestational diabetes mellitus, maternal exposure to rodenticides and herbicides, and maternal use of antiepileptic drugs) have been proposed. [orpha.net]
Etiology The etiology for transposition of the great arteries is unknown and is presumed to be multifactorial. [emedicine.com]
Furthermore, it adds support to the concept that progressive stenosis of a bicuspid aortic valve is frequently the etiology of isolated aortic stenosis in adults. [profiles.stanford.edu]
The exact etiology of these episodes is unclear, although there have been a number of proposed mechanisms, including increased infundibular contractility, peripheral vasodilatation, hyperventilation, and stimulation of right ventricular mechanoreceptors [textbookofcardiology.org]
Epidemiology
[…] of the great vessels Classification (Orphanet): - Rare developmental defect during embryogenesis - Rare genetic disease - Rare surgical cardiac disease Classification (ICD10): - Congenital malformations, deformations and chromosomal abnormalities - Epidemiological [csbg.cnb.csic.es]
Summary Epidemiology The incidence is estimated at 1 in 3,500-5,000 live births, with a male-to-female ratio of 1.5 to 3.2:1. Clinical description In 50% of cases, the VA discordance is an isolated finding. [orpha.net]
Patient Population: Prevalence and Epidemiology Knee // Shoulder & Elbow // Hip // Spine // Foot & Ankle // Hand & Wrist Fragility Fractures: Diagnosis and Treatment Shoulder & Elbow The Characteristics of Surgeons Performing Total Shoulder Arthroplasty [mdedge.com]
Epidemiology United States data Despite its overall low prevalence, transposition of the great arteries is the most common etiology for cyanotic congenital heart disease in the newborn. [1] This lesion presents in 5-7% of all patients with congenital [emedicine.com]
Relevant External Links for GDF1 Genetic Association Database (GAD) GDF1 Human Genome Epidemiology (HuGE) Navigator GDF1 Atlas of Genetics and Cytogenetics in Oncology and Haematology: GDF1 No data available for Genatlas for GDF1 Gene Expression of growth [genecards.org]
Pathophysiology
Pathophysiology in Dextro-TGA in extra-uterine life - In normal cardiac anatomy, the aorta is positioned posterior and to the right of the main pulmonary artery. Aorta being positioned anterior and slightly rightward of the pulmonary artery. [wikidoc.org]
Management of anesthesia in these patients is a combination of understanding neuroanesthesia techniques and pathophysiologic TOF. [kink.onesearch.id]
Preoperatively, an understanding of the patient’s underlying pathophysiology, coupled with knowledge of the effects of anesthesia on the anomalous myocardium, is crucial. [medcraveonline.com]
Such patients have complex pathophysiology, which can lead to unstable haemodynamics, arrhythmias and cardiac arrest under anesthesia. [aeronline.org]
Pathophysiology The pulmonary and systemic circulations function in parallel, rather than in series. [emedicine.com]
Prevention
Antibiotics may be administered preventatively. [en.wikipedia.org]
Reference Parker SE, Mai CT, Canfield MA, et al. for the National Birth Defects Prevention Network. Updated national birth prevalence estimates for selected birth defects in the United States, 2004-2006. [cdc.gov]
Anesthesia and Analgesia 78: 1190-1192. [ Crossref ] Clemmeson L, Knudsen L (2013) Ultrasound guidance during placement of central venous catheter can prevent arrhythmias. [oatext.com]
We used titrated doses of epidural lignocaine 2% to prevent precipitous hypotension and did not require vasopressors in the intraoperative period. [aeronline.org]
Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the [emedicine.com]