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 .
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 .