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 [1] [2] [3] [4]. 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 [5] [6]. 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 [2] [3]. Moreover, rare cases with an intact ventricular septum have been described [3]. 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 [3] [5] [6] [7]. Infants are often unresponsive and extremely tired after minor tasks, which are followed by marked increases in heart and respiration rate [7]. Arrhythmias, pulmonary hypertension and the formation of aneurysms are known complications that can be life-threatening [5].

• Poor Feeding

  feeding and lethargy are additional features. [symptoma.com]

  feeding from becoming tired easily Bluish color of the skin and lips Clubbing (thickening of the nail beds) of toes and fingers (late sign) Failure to gain weight and grow Pale coloring Sweating Swollen legs or abdomen Trouble breathing Treatment requires [nlm.nih.gov]

  Tachypnea or breathlessness, poor feeding and slow weight gain are also common. Tachycardia and/or heart murmur may also be observed. [orpha.net]

  Within a few days of birth, a newborn with DORV usually will show these signs: Breathing fast or hard Pale or bluish skin Sweating Poor feeding with little weight gain Sleepiness Double Outlet Right Ventricle Causes DORV is due to an error in the way [nortonchildrens.com]

  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]

• Fatigue

  […] 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]

  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]

  Infants may also have other signs and symptoms, such as heart murmurs (i.e., unusual heart sounds), sweating, and fatigue. [news-medical.net]

• Fishing

  Management: Careful prenatal and postnatal search for associated anomalies and karyotype (including FISH for chromosome 22q11 deletion) are indicated. Delivery should be performed where immediate pediatric cardiac consultation is available. [w1.med.cmu.ac.th]

  Fluorescence in situ hybridization (FISH) analysis has shown deletions in the 22q11.2 region in certain individuals with TOF, DORV, transposition of the great arteries, and VSD associated with other congenital heart disease (CHD). [1, 2, 3] As a matter [emedicine.com]

• Feeding Difficulties

  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]

• Tachypnea

  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]

  A 20-year-old male patient with palpitations, tachypnea, and tiredness with weak exercise capacity and back pain was diagnosed with heart murmur in different degrees; slight cyanosis, cachexia, and developmental retardation were found clinically. [ncbi.nlm.nih.gov]

  Tachypnea or breathlessness, poor feeding and slow weight gain are also common. Tachycardia and/or heart murmur may also be observed. [orpha.net]

  These can include an abnormal heart sound during a heartbeat (heart murmur), rapid breathing (tachypnea), low blood pressure (hypotension), low levels of oxygen in the blood (hypoxemia), and a blue or purple tint to the skin caused by a shortage of oxygen [icdlist.com]

  Large left-to-right shunts cause excess pulmonary blood flow and left ventricular volume overload, which may lead to signs of heart failure (eg, tachypnea, poor feeding, diaphoresis). During infancy, these symptoms often result in failure to thrive. [merckmanuals.com]

• Rales

  In addition, abnormal pulmonary signs (eg, rales, rhonchi, wheezing) and peripheral signs of cyanosis and capillary refill should be sought. [emedicine.medscape.com]

  Rale was auscultated on bilateral chest, the blood pressure was 102/65 mmHg. The symptom improved soon after 10 mg furosemide was given intravenously and with the head of bed elevated. 2 % lidocaine 3 mL was injected into epidural for testing. [springerplus.springeropen.com]

• 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]

  Large left-to-right shunts cause excess pulmonary blood flow and left ventricular volume overload, which may lead to signs of heart failure (eg, tachypnea, poor feeding, diaphoresis). During infancy, these symptoms often result in failure to thrive. [merckmanuals.com]

  Your child may have symptoms like these: Cyanosis Trouble feeding Poor weight gain or slow growth (failure to thrive) Fast breathing or working hard to breathe Sweating more than normal Diagnosing Double-Outlet Right Ventricle Usually, doctors can diagnose [seattlechildrens.org]

• Heart Disease

  Double outlet right ventricle (DORV) is an uncommon congenital heart disease with a poor prognosis. [ncbi.nlm.nih.gov]

  This type of congenital heart disease results in a wide variety of anatomic arrangements of the great arteries and VSD. Diagnosis of a DORV is made primarily using echocardiography. [link.springer.com]

• Cyanosis

  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]

  Herein, a newborn with cyanosis and murmur diagnosed as double outlet right ventricle with intact ventricular septum is reported. [ncbi.nlm.nih.gov]

  So, we pumped phenylephrine along with epidural anesthesia in case of critical cyanosis following significant blood pressure decrease. In the process of anesthesia, dyspnea, cough and cyanosis attacked the patient for two times. [springerplus.springeropen.com]

  She denied any previous history of cyanosis; however, she had multiple hospital admissions due to recurrent chest infections for which she was on occasional diuretics. No past history suggestive of palpitations or any syncope could be elicited. [joacc.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 [7]. 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 [2] [8]. 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 [1] [8]. 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 [2].

• 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]

  […] ventricular septal defect (VSD) and pulmonary stenosis (PS): ECG shows right axis deviation. [johnsonfrancis.org]

  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]

  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]

• Oxygen Saturation Decreased

  At the same time, the oxygen saturation decreased quickly. The symptoms were ameliorated soon by treating as heart failure. But the symptoms reappeared after oxytocin administration. [springerplus.springeropen.com]

Diagnosis and Treatment of Double Outlet Right Ventricle. News-Medical, viewed 02 November 2022, https://www.news-medical.net/health/Diagnosis-and-Treatment-of-Double-Outlet-Right-Ventricle.aspx. [news-medical.net]

We feel that biventricular repair of DORV-AVSD is the preferred treatment option, although further studies are needed to define longterm outcomes. [experts.umich.edu]

Preoperative pulmonary hypertension (PH) is considered an important risk factor for early death during the surgical treatment of DORV. The aim of this study was to report our experience on surgical treatment of DORV complicated by PH. [ncbi.nlm.nih.gov]

Treatment requires surgery to close the hole in the heart and direct blood from the left ventricle into the aorta. [stlukes-stl.com]

These patients have a high rate of coarctation of the aorta leading to early congestive heart failure, and their overall prognosis is poor. [ncbi.nlm.nih.gov]

Prognosis Without treatment, prognosis is poor due to severe cyanosis, congestive heart failure or pulmonary hypertension. With biventricular repair, patients have, on average, a normal life expectancy, with possible risk or re-operation. [orpha.net]

Prognosis: Poor when combined with other anomalies, but better for isolated DOVR with surgical correction, with the survival rate possibly being as high as more than 80%. [w1.med.cmu.ac.th]

Our study confirms a better prognosis with an intention-to-treat survival rate of 86% and a much lower post-surgical mortality rate of 14% in our cohort. [degruyter.com]

Etiology To date, no predictive factor or specific causal agent has been identified in the development and pathogenesis of DORV. However, like other conotruncal defects of the heart, it is speculated that DORV may have a neural crest origin. [news-medical.net]

Etiology DORV results from a failure in the fetal development of the cono-truncus, which is the primary outlet of the heart during embryonic development. The cono-truncal anomalies result in neural crest and second heart field dysfunction. [orpha.net]

Diagnosis is by physical examination findings and imaging, usually echocardiography, but this defect may be relatively subtle and the etiology of the pulmonary hypertension may initially be obscure. [merckmanuals.com]

Double outlet right ventricle comprises about 1-1.5% of all congenital heart disease. [4] Etiology No specific causal agent or predictive event has been identified. [emedicine.medscape.com]

We queried the PubMed database using key words "double outlet right ventricle" and "DORV" for case reports, epidemiologic analyses and animal studies with this cardiac anomaly. [ncbi.nlm.nih.gov]

[citation needed] Epidemiology[edit] DORV affects between 1% and 3% of people born with congenital heart defects.[1] Chromosomal abnormalities were reported in about 40% of reported cases in the medical literature.[1] References[edit] ^ a b Obler D, Juraszek [en.wikipedia.org]

Both epidemiologic analyses of congenital heart disease and case reports were used. [doi.org]

Summary Epidemiology Double outlet right ventricle (DORV) accounts for about 2-3% of all congenital heart defects, with a birth prevalence rate of 1/ 10,000. [orpha.net]

The pathophysiology of double-outlet right ventricle and its anesthetic implications for tracheoesophageal fistula repair are discussed. [ncbi.nlm.nih.gov]

(Click picture to show/hide bloodflows) Pathophysiology The hemodynamics involved with double outlet right ventricle are dependent on the anatomy of the great vessels and associated defects. [vhlab.umn.edu]

The pathophysiology depends on the degree pulmonary stenosis. With pulmonary stenosis, the pulmonary blood flow is decreased with variable degrees of cyanosis (TOF type). [emedicine.medscape.com]

The cardiovascular pathophysiology is determined predominantly by the degree of PS, which generally increases over time in early infancy. [thoracickey.com]

Pathophysiologic description and classification is accomplished by relating the location of the VSD to the arrangement of the great vessels. Each combination results in a physiologic behavior similar to that of other CHDs. [emedicine.com]

Prevention There are no known guidelines to prevent this health problem. [winchesterhospital.org]

[…] blood clots Antibiotics before certain medical and dental procedures to prevent infection Most children with DORV go on to lead normal and active lives. [saintlukeskc.org]

Prevention Can I prevent DORV? There is no way to prevent DORV. Scientists are still trying to understand its cause. Outlook / Prognosis What is the outlook for a baby with DORV? Without surgery, a baby with DORV will eventually develop: Cyanosis. [my.clevelandclinic.org]

Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. [nemours.org]

1. Zidere V, Pushparajah K, Allan LD, Simpson JM. Three-dimensional fetal echocardiography for prediction of postnatal surgical approach in double outlet right ventricle: a pilot study. Ultrasound Obstet Gynecol. 2013;42(4):421-425.
2. Shi K, Yang Z, Chen J, Zhang G, Xu H, Guo Y. Assessment of Double Outlet Right Ventricle Associated with Multiple Malformations in Pediatric Patients Using Retrospective ECG-Gated Dual-Source Computed Tomography. Dettman RW, ed. PLoS ONE. 2015;10(6):e0130987.
3. Li S, Ma K, Hu S, et al. Surgical outcomes of 380 patients with double outlet right ventricle who underwent biventricular repair. J Thorac Cardiovasc Surg. 2014;148(3):817-824.
4. Porter RS, Kaplan JL. Merck Manual of Diagnosis and Therapy. 19th Edition. Merck Sharp & Dohme Corp. Whitehouse Station, N.J; 2011.
5. Lin CH, Huddleston C, Balzer DT. Transcatheter Ventricular Septal Defect (VSD) Creation for Restrictive VSD in Double-Outlet Right Ventricle. Pediatr Cardiol. 2013;34(3):743-747.
6. Özgür S, Ceylan Ö, Doğan V, Örün UA. Double outlet right ventricle with intact ventricular septum. Turk Kardiyol Dern Ars. 2014;42(2):190-193.
7. Baig SA, Saeed Z, Fatima A, Chagani H, Aziz S. Double outlet right ventricle, atrioventricular canal defect with severe pulmonary artery stenosis: a rare cyanotic congenital heart disease. J Pak Med Assoc. 2014;64(8):980-982.
8. Gelehrter S, Owens ST, Russell MW, van der Velde ME, Gomez-Fifer C. Accuracy of the fetal echocardiogram in double-outlet right ventricle. Congenit Heart Dis. 2007;2(1):32-37.