Double outlet right ventricle is a congenital heart disease in which the pulmonary trunk and the aorta both arise from the right ventricle, with several anatomical variations and additional cardiac malformations. Hypoxia and other accompanying manifestations may be life-threatening. Echocardiography and other imaging studies of the heart and major vessels are used to confirm double outlet right ventricle.
In double outlet right ventricle (DORV), the pulmonary trunk and a significant portion (> 50%) of the aorta (or sometimes the entire vessel) stem from the right ventricle, resulting in the transfer of primarily deoxygenated blood into systemic circulation    . The severity of manifestations significantly depends on the presence of various accompanying defects, most notable being atrial septal defect (ASD), stenosis of the pulmonary valve, hypoplasia (or hypertrophy) of the left ventricle, mitral atresia, mitral stenosis, and coarctation of the aorta  . In virtually all cases, however, a ventricular septal defect (VSD), arising as a compensatory mechanism of the heart, almost universally develops, but its location (subaortic, subpulmonary, non-committed or doubly-committed) may determine the amount of oxygenated blood that reaches the right ventricle from the left heart  . Moreover, rare cases with an intact ventricular septum have been described . Regardless, one of the most important clinical presentations of this rare congenital heart disease (CHD) is the onset of cyanosis during the first few months of life, often characterized by hypoxic spells, whereas tachycardia, tachypnea, sweating, deprivation of growth and inability to gain weight due to poor feeding and lethargy are additional features    . Infants are often unresponsive and extremely tired after minor tasks, which are followed by marked increases in heart and respiration rate . Arrhythmias, pulmonary hypertension and the formation of aneurysms are known complications that can be life-threatening .
Entire Body System
Abnormal heartbeat, a heart murmur, sweating and fatigue are other possible symptoms. What are double outlet right ventricle care options? In most cases, double outlet right ventricle can be repaired with one or more surgical procedures. [nicklauschildrens.org]
[…] babies include: rapid breathing rapid heartbeat sweating disinterest in feeding or tiring while feeding poor weight gain blue color of the skin, lips and nail beds ( cyanosis ) heart murmur (detected by doctor) In older children, symptoms may include: fatigue [childrenshospital.org]
[…] include: A heart murmur (an extra, unusual heart sound) Breathing problems, including difficulty breathing, shortness of breath, or rapid breathing Difficulty eating and/or gaining weight Blueness of the lips, nails, and/or skin (cyanosis) Sweating Fatigue [my.clevelandclinic.org]
Signs and symptoms of severe defects in newborns include Rapid breathing Cyanosis - a bluish tint to the skin, lips, and fingernails Fatigue Poor blood circulation Many congenital heart defects cause few or no signs and symptoms. [icdlist.com]
Other sypmtoms include fatigue, difficulty breathing, and cyanosis. These symptoms are not specific for RVOT. [wikidoc.org]
Much of the most rigorous genetic and developmental study of normal cardiac development and of the genesis of DORV has come from recent animal studies. 56 There are, however, two important limitations of the animal studies that must be considered in the [doi.org]
Failure to Thrive
These infants may also have difficulties breathing as well as poor feeding and a general failure to thrive. In severe cases, infants may have congestive heart failure, which requires immediate and aggressive therapy. [news-medical.net]
Your child may have symptoms like these: Bluish skin, lips or nailbeds (cyanosis) or pale skin Trouble feeding Failure to thrive Fast breathing or working hard to breathe Sweating more than normal Diagnosing Double Outlet Right Ventricle To diagnose this [seattlechildrens.org]
Symptoms include feeding difficulties and as a consequence poor weight gain, failure to thrive. Other sypmtoms include fatigue, difficulty breathing, and cyanosis. These symptoms are not specific for RVOT. [wikidoc.org]
Feeding tolerance, weight gain, breathing problems, and a general failure to thrive should be elucidated. [emedicine.medscape.com]
Double outlet right ventricle (DORV) is an uncommon congenital heart disease with a poor prognosis. [ncbi.nlm.nih.gov]
Herein, a newborn with cyanosis and murmur diagnosed as double outlet right ventricle with intact ventricular septum is reported. [ncbi.nlm.nih.gov]
Physical examination can reveal tachycardia, a heart murmur, tachypnea and signs of central cyanosis, after which echocardiography, the gold standard in detecting CHD, should be employed. [symptoma.com]
The appearance of cyanosis during the neonatal period or infancy in the absence of an obvious cause must raise clinical suspicion to a CHD. This illustrates the importance of a detailed patient history and a proper physical examination. Firstly, the onset of symptoms and their course must be determined with the parents, as patients are mainly too young to provide adequate information. Family history is also an important component of the workup, as preexisting congenital malformations (but also other congenital diseases) within the family seem to increase the risk for many CHDs, including DORV. In addition, maternal use of antidepressants, such as selective serotonin reuptake inhibitors (SSRIs) during pregnancy, was also confirmed as a risk factor . Physical examination can reveal tachycardia, a heart murmur, tachypnea and signs of central cyanosis, after which echocardiography, the gold standard in detecting CHD, should be employed  . Echocardiography is highly useful due to the fact that it can detect CHD both prenatally and postnatally. Supplementary to traditional transthoracic 2D cross-sectional cardiac ultrasonography, limited by the experience of the physician and a very narrow field of observation, the introduction of 3D echocardiography has greatly improved the diagnostic accuracy, and is performed whenever possible and feasible  . However, digital subtraction angiography (DSA), an invasive procedure carrying potential for catheter-related complications, and electrocardiographically (ECG)-gated dual-source computed tomography (DSCT), used in the pediatric population without arrhythmias, have been postulated as most reliable methods when it comes to detection of DORV .
Right Axis Deviation
ECG revealed gross right axis deviation and right ventricular hypertrophy. Echocardiogram showed features of double outlet right ventricle. [ncbi.nlm.nih.gov]
As a result of pressure and volume overload, there are signs of RV hypertrophy and there is a right axis deviation. There are a lot of ECG variations depending on the variety of DORV the presence of associated defects. [wikidoc.org]
Electrocardiogram • NSR • PR Prolongation • Atrial electrical abnormality • Counter clock loop • Clockwise QRS • RVH • Right axis deviation 20. Clinical classification • The More Common Types • A. [slideshare.net]
The electrocardiogram is notable for right axis deviation and a right ventricular hypertrophy pattern, with rR′, qR, or rsR′ pattern; these findings are not, however, sufficiently specific to be diagnostic. [thoracickey.com]
ECG The RV will be hypertrophied with right axis deviation and possible RAE. CXR Mild or no pulmonary stenosis will cause increase pulmonary blood flow resulting in cardiomegaly with increase pulmonary vascular markings. [pedcard.rush.edu]
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