Presentation
Stenosis of valves or vessels may also be present. When no other heart defects are present it is called 'simple' l-TGA; when other defects are present it is called 'complex' l-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]
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
- Weakness
The exceptions are if your heart function is too weak, if you have serious lung problems, and/or if you have low oxygen levels in your body. [achaheart.org]
Hypertension in upper extremity's and weak/ delayed pulse in lower extremities. With age, intercostal arteries enlarge due to collateral circulation; arteries erode ribs, notched appearance on CXR. [brainscape.com]
Infants with d-TGA or other conditions causing cyanosis can have symptoms such as: Problems breathing Pounding heart Weak pulse Ashen or bluish skin color Poor feeding Because the infant might be bluish in color and have trouble breathing, d-TGA is usually [cdc.gov]
[…] infantile coarctation lower extremity cyanosis in infants (often at birth) what is adult coarctation of the aorta associated with bicuspid aortic valve other heart problems presentation of the adult coarctation of the aorta HTN in upper extremities and weak [flashcardbook.com]
[…] pulses on physical exam Adult type : stenosis is distal to ligamentum arteriosum (postductal) -Associated with notching of the ribs (due to collateral circulation) -Most commonly associated with bicuspid aortic valve -Hypertension in upper extremities -Weak [freezingblue.com]
- Hypoxemia
[…] right shunt (VSD, ASD, PDA) -causes increased pulmonary flow -causes remodeling of pulmonary vasculature -causes pulmonary arterial HTN -RVH to compensate -shunt becomes right to left presentation of eisenmenger syndrome late cyanosis clubbing (d/t hypoxemia [flashcardbook.com]
Pulmonary hypertension owing to lung diseases or hypoxemia. [textbookofcardiology.org]
The team should do everything possible to avoid hypoxemia, hypercarbia, and fluid overload to reduce the chance of acute right heart failure. [oatext.com]
One or all of these lesions can be present in concert with dextro-transposition of the great arteries, and the degree of arterial hypoxemia depends on the degree of anatomic mixing. [emedicine.com]
- Crying
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]
[…] by the unilateral hypoplasia/agenesis of the depressor anguli oris muscle, resulting in an asymmetric crying facies in neonatal period/ infancy (drooping of one corner of the mouth during crying) while eye closure, nasolabial fold and forehead wrinkling [mendelian.co]
[…] shaped heart (b/c of RV hypertrophy) explain tet spells in tetralogy of fallot pulmonary stenosis forces right to left flow across VSD—> -RVH -early cyanotic tet spells exacerbations of right to left flow shunt in tet of fallot (tet spells) causes 1. crying [flashcardbook.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. The exact aetiology remains unknown. [ojrd.biomedcentral.com]
- Poor Feeding
Infants with d-TGA or other conditions causing cyanosis can have symptoms such as: Problems breathing Pounding heart Weak pulse Ashen or bluish skin color Poor feeding Because the infant might be bluish in color and have trouble breathing, d-TGA is usually [cdc.gov]
Other symptoms may include lethargy, poor feeding, sweating with feeds, increased respiratory effort or poor weight gain. [cookchildrens.org]
- Swelling
The sternum is not closed immediately which allows extra space in the thoracic cavity, preventing excess pressure on the heart, which swells considerably following the surgery; the sternum and incision are closed after a few days, when swelling is sufficiently [en.wikipedia.org]
F., Stanford, W., Wexler, L., Abbott, W., Port, S. 2000 ; 215 : 85-88 Unilateral upper extremity swelling and pain. American College of Radiology. ACR Appropriateness Criteria. Radiology Polak, J. F., Levin, D. C., Bettmann, M. A., Gomes, A. [profiles.stanford.edu]
Respiratoric
- Dyspnea
Simple l-TGA does not immediately produce any visually identifiable symptoms, but since each ventricle is intended to handle different blood pressures, the right ventricle may eventually hypertrophy due to increased pressure and produce symptoms such as dyspnea [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]
Patients with a moderate or large atrial septal defect, a large ventricular septal defect, a patent ductus arteriosus, or a combination of these tend to have less severe cyanosis, but symptoms and signs of heart failure (eg, tachypnea, dyspnea, tachycardia [merckmanuals.com]
[…] approach of obstetrician, cardiologist, anaesthetist and neonatologist plays an important role in successful pregnancy outcome. [4] Case Report A 22-year-old primigravida, married for 2 years, was referred at 19.5 weeks of gestation with complaints of dyspnea [jpgo.org]
Gastrointestinal
- 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]
Cardiovascular
- Hypotension
Episodes of hypotension and decrease in saturation less than 80% were managed by bolus doses of Phenylephrine. [medcraveonline.com]
We used titrated doses of epidural lignocaine 2% to prevent precipitous hypotension and did not require vasopressors in the intraoperative period. [aeronline.org]
Early and late side effects may be observed, namely apnoeas, bradycardia, systemic hypotension, fluid-electrolyte disturbances, fever and cutaneous flushing. [ojrd.biomedcentral.com]
- Vascular Disease
Patients who have some route for oxygenated blood to enter from left to right side of heart like VSD and pulmonary vascular disease may reach adulthood without corrective surgery due to Eisenmenger physiology. [jpgo.org]
This is a practical, noninvasive, well tolerated method for both diagnostic screening and follow-up evaluation of patients with peripheral vascular disease. [profiles.stanford.edu]
Early detection of pulmonary vascular disease in pulmonary arterial hypertension: time to move forward. European Heart Journal, 32(20), 2489-2498. [Lau] Mulder, B J M. (2010). [textbookofcardiology.org]
- Weak Pulse
Infants with d-TGA or other conditions causing cyanosis can have symptoms such as: Problems breathing Pounding heart Weak pulse Ashen or bluish skin color Poor feeding Because the infant might be bluish in color and have trouble breathing, d-TGA is usually [cdc.gov]
[…] on physical exam Adult type : stenosis is distal to ligamentum arteriosum (postductal) -Associated with notching of the ribs (due to collateral circulation) -Most commonly associated with bicuspid aortic valve -Hypertension in upper extremities -Weak [freezingblue.com]
- Gallop Rhythm
Tachypnoea, tachycardia, diaphoresis, poor weight gain, a gallop rhythm, and eventually hepatomegaly can be then detected later on during infancy. [ojrd.biomedcentral.com]
Skin
- Flushing
Diuretics aid in flushing excess fluid from the body, thereby easing strain on the heart. Analgesics normally are not used pre-operatively, but they may be used in certain cases. They are occasionally used partially for their sedative effects. [en.wikipedia.org]
Early and late side effects may be observed, namely apnoeas, bradycardia, systemic hypotension, fluid-electrolyte disturbances, fever and cutaneous flushing. [ojrd.biomedcentral.com]
Myocardial infarctions have decreased from 2.4% (1967-71) to 0.44% (1973), an improvement related to careful catheter flushing, guidewire insertion technique and careful pressure monitoring within the coronary arteries. [profiles.stanford.edu]
- Sweating
Other symptoms may include lethargy, poor feeding, sweating with feeds, increased respiratory effort or poor weight gain. [cookchildrens.org]
Workup
Hypertrophy
- Ventricular Hypertrophy
Electrocardiography findings were sinus rhythm with first degree heart block, right bundle branch block and right ventricular hypertrophy. [aeronline.org]
Boot shaped heart Right ventricular hypertrophy seen in Tetralogy of Fallot Cause of Tetralogy of Fallot Anteriosuperior displacement of infundibular septum Left--> Right shunts Septal defects (VSD, ASD) and patent ductus arteriosus (PDA) TORCHeS: Patent [studystack.com]
ECG shows right ventricular hypertrophy but may be normal for a neonate. [merckmanuals.com]
This lesion consists of severe pulmonary stenosis, a non-restrictive ventricular septal defect, right ventricular hypertrophy and an overriding aorta. [clinicaladvisor.com]
- 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]
However, many cases of simple l-TGA are "accidentally" diagnosed in adulthood, during diagnosis or treatment of other conditions. [en.wikipedia.org]
Primary brain abscess treatment is to reduce the mass effect and eliminate germs. Management of brain abscess therapy can be divided into surgical and conservative treatment. [kink.onesearch.id]
Prognosis
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]
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]
CN VIII deafness Syphilis TORCHeS: Hydrops fetalis Parvovirus B19 (or syphilis) Continuous "machine-like" murmur Patent ductus arteriosus #1 heart defect and cause VSD - caused by fetal alcohol syndrome Tetralogy of Fallot defects Pulmonary stenosis (prognosis [studystack.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]
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]
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]
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]
Ferencz C, Rubin JD, Loffredo CA, Epidemiology of Congenital Heart Disease: The Baltimore-Washington Infant Study, 1981–1989. Mount Kisco, NY: Futura Publishing Company; 1993. 3. Warnes CA. Transposition of great arteries. [jpgo.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]
Pathophysiology The exact pathophysiology depends on the location and severity of the defect. [textbookofcardiology.org]
Society for Cardiovascular Pathology Symposium 2009; Anatomical and pathophysiological classification of congenital heart disease. Cardiovascular Pathology, 2010; 19: 259–274. 16. [crcd.eu]
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]
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]
European Heart Journal, 18, 1210–1219 PubMed CAS Google Scholar Pulmonary Embolism Prevention (PEP) Trial Collaborative Group (2000) Prevention of pulmonary embolism and deep vein thrombosis with low dose aspirin: the Pulmonary Embolism Prevention (PEP [link.springer.com]
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]
These antibiotics are used to prevent serious heart infections called endocarditis. The following are some English-language resources that may be useful. Please note that THE MANUAL is not responsible for the content of these resources. [merckmanuals.com]