• Double outlet right ventricle (DORV) refers to a heterogeneous series of associated cardiac anomalies that involve the right ventricular outflow tract in which both of the great arteries arise entirely or predominantly from the right ventricle. (medscape.com)
  • The anatomic dysmorphology of double outlet right ventricle can vary from that of tetralogy of Fallot (TOF) on one end of the spectrum to complete transposition of the great arteries (TGA) on the other end (see the image below). (medscape.com)
  • Double outlet right ventricle (DORV) with transposition of the great arteries accounts for 26% of cases of DORV. (medscape.com)
  • The aorta (AO) is anterior and to the right of the pulmonary artery (PA), and both arteries arise from the right ventricle (RV). (medscape.com)
  • In the United States, the incidence of double outlet right ventricle is an estimated 0.09 cases per 1000 live births. (medscape.com)
  • Double outlet right ventricle comprises about 1-1.5% of all congenital heart disease. (medscape.com)
  • In 1793, Aberanthy described a heart with the origin of both great arteries from the right ventricle. (medscape.com)
  • The definition of a double outlet right ventricle (DORV) has been a point of controversy among professionals in the field of congenital heart surgery. (medscape.com)
  • [ 6 ] However, the Congenital Heart Surgery Nomenclature and Database Project defines double outlet right ventricle as a type of ventriculoarterial connection in which both great vessels arise either entirely or predominantly from the right ventricle. (medscape.com)
  • In general, from a surgical perspective, defining the lesion as double outlet right ventricle is reasonable when more than 50% of both of the great arteries arise from the right ventricle. (medscape.com)
  • All of one vessel and most of the remaining vessel typically arise from the right ventricle. (medscape.com)
  • From the morphologic standpoint, some suggest that the absence of the fibrous continuity between the arterial and atrioventricular valves is a feature of double outlet right ventricle. (medscape.com)
  • Before 1972, double outlet right ventricle (DORV) was defined as complete emergence of both great arteries from the right ventricle and no fibrous valvular continuity. (medscape.com)
  • [ 9 ] In addition, Lev et al began to classify the group of anomalies in double outlet right ventricle by the ventriculoseptal defect (VSD) location (ie, the great vessel to which the VSD was anatomically adjacent). (medscape.com)
  • Double outlet right ventricle is almost always associated with a VSD. (medscape.com)
  • Lev et al noted four possibilities of commitment of the double outlet right ventricle to the great arteries and termed them subaortic, subpulmonic, doubly committed, and noncommitted (or remote). (medscape.com)
  • [ 9 ] The location of the VSD has important implications on the physiologic manifestations of double outlet right ventricle and on surgical considerations. (medscape.com)
  • The relative anatomic anomalies identified in the spectrum of double outlet right ventricle determine the clinical presentation and the surgical approach required for repair. (medscape.com)
  • BACKGROUND: Atrioventricular septal defect (AVSD) and double outlet right ventricle (DORV) with normally related great arteries and normal ventricular sizes are associated with a good long-term prognosis after biventricular (BV) repair. (mcmaster.ca)
  • Electrocardiographic findings are rarely diagnostic for double outlet right ventricle (DORV). (medscape.com)
  • Common findings in a child with double outlet right ventricle include right ventricular hypertrophy, right axis deviation, and, occasionally, evidence of left ventricular hypertrophy. (medscape.com)
  • [ 11 ] Of the 12 infants in whom double outlet right ventricle (DORV) was diagnosed and confirmed with angiography, 11 previously received a diagnosis based on subxiphoid two-dimensional echocardiography. (medscape.com)
  • Cardiac catheterization, once the criterion standard for confirming double outlet right ventricle, is now rarely required in the evaluation or preoperative planning of this cardiac disorder. (medscape.com)
  • MRI has been used in the diagnosis of double outlet right ventricle, but it is not yet a routine or well-established diagnostic modality forthis condition. (medscape.com)
  • Regardless of the end of the clinical spectrum (tetralogy of Fallot [TOF] or transposition of the great arteries [TGA]) at which double outlet right ventricle occurs, findings on anteroposterior and lateral chest radiography depend on the degree of pulmonary (or subpulmonary) stenosis. (medscape.com)
  • Preoperative CT scanning is potentially useful for identifying coronary artery anatomy in children with TOF or Fallot type of double outlet right ventricle. (medscape.com)
  • [ 12 ] A study that assessed the incidence and diagnostic accuracy of preoperative cardiac CT scanning for identifying detailed coronary artery anatomy in 318 children with TOF or Fallot type of double outlet right ventricle found a 95% concordance between cardiac CT scanning and surgical findings, and a 96.9% diagnostic accuracy for cardiac CT scanning. (medscape.com)
  • Demir MT, Amasyall Y, Kopuz C, Aydln ME, Corumlu U. The double outlet right ventricle with additional cardiac malformations: an anatomic and echocardiographic study. (medscape.com)
  • [9] Cardiac defects can include Tetralogy of Fallot, aortic arch interruption, double outlet right ventricle with arch vessel abnormalities, and atrioventricular septal defects (AVSD). (aao.org)
  • Its posterior wall is formed by the [[ventricular septum]], which bulges into the right ventricle, so that a transverse section of the cavity presents a semilunar outline. (wikidoc.org)
  • Return to Right ventricle . (wikidoc.org)
  • From the morphological standpoint, some suggest that the absence of the fibrous continuity between the arterial and atrioventricular valves is a feature of double outlet right ventricle. (medscape.com)
  • Based on the location of ventriculoseptal defect (VSD) in relation to great arteries, double outlet right ventricle can be classified into 4 main categories: double outlet right ventricle with subaortic VSD, double outlet right ventricle with subpulmonary VSD, double outlet right ventricle with doubly committed VSD, and double outlet right ventricle with noncommitted VSD. (medscape.com)
  • In this variant of double outlet right ventricle, the pulmonary artery preferentially receives left ventricular oxygenated blood, and the desaturated blood from the right ventricle streams to the aorta (TGA type). (medscape.com)
  • The Taussig-Bing anomaly is a typical example of double outlet right ventricle with subpulmonary VSD. (medscape.com)
  • A double outlet right ventricle with a ventricular spetal defect (a hole between the two bottom chambers (ventricles) of the heart), that is considered to be closely related to the pulmonary origin. (nih.gov)
  • Essential Modifiers of Double Outlet Right Ventricle: Revisit With Endocardial Surface Images and 3-Dimensional Print Models. (nih.gov)
  • How should we diagnose and differentiate hearts with double-outlet right ventricle? (nih.gov)
  • Autologous Umbilical Cord Blood-Derived Mononuclear Cell Therapy Promotes Cardiac Proliferation and Adaptation in a Porcine Model of Right Ventricle Pressure Overload. (ouhsc.edu)
  • PHENOTYPE: Homozygotes exhibit double outlet right ventricle {SDD}, pulmonary atresia/hypolastic pulmonary artery, atrioventricular septal defect, and right aortic arch. (utsouthwestern.edu)
  • Other frequent anomalies are patent ductus arteriosus, double outlet right ventricle with atrioventricular canal, ventricular septal defect and atrial septal defect with or without cleft mitral valve. (netlify.app)
  • 17. Hagler D, Double-outlet right ventricle and double outlet left ventricle. (medresearch.in)
  • In double outlet right ventricle, both the aorta and pulmonary valve connect to the right ventricle. (msdmanuals.com)
  • Normally the pulmonary valve connects to the right ventricle, and the aorta connects to the left ventricle. (msdmanuals.com)
  • In double outlet right ventricle, both the aorta and the pulmonary artery are connected to the right ventricle and no artery attaches to the left ventricle. (msdmanuals.com)
  • The structures initially seen from this perspective include the superior vena cava, right atrium, right ventricle, pulmonary artery, and aorta. (medscape.com)
  • The right ventricle receives blood from the right atrium across the tricuspid valve, which is located in the large anterolateral (ie, sinus) portion of the right ventricle. (medscape.com)
  • The right ventricle discharges blood into the pulmonary artery across the pulmonic (semilunar) valve located in the outflow tract (infundibulum). (medscape.com)
  • The inflow tract (sinus) and outflow tract (infundibulum) of the right ventricle are widely separated. (medscape.com)
  • Of these 3 subdivisions, the conal septum is clinically significant because it can be malpositioned in patients with congenital disorders (eg, double outlet right ventricle ). (medscape.com)
  • Ventricular septal defects (VSDs) commonly occur in the area between the sinus and the outlet tract of the right ventricle. (medscape.com)
  • However, because the surface of the right ventricle is trabecular, small defects of the muscular portion of the ventricular septum may be difficult to see. (medscape.com)
  • Fetal echocardiographic parameters comparing BV repair versus single ventricle (SV) palliation were obtained, including the presence or absence of an apex-forming bLV was recorded. (mcmaster.ca)
  • In format the book appears similar to a large collection of case reports covering all types of congenital heart disease, including complex lesions such as single ventricle and atrial isomerism. (stanford.edu)
  • The Single Ventricle Home Monitoring Program at AdventHealth for Children was developed for babies with single ventricle-type congenital heart disease to help anticipate any potential issues and provide support through the vulnerable period between discharge from the hospital and the Glenn operation. (adventhealth.com)
  • The only outflow from the left ventricle (LV) is a ventricular septal defect (VSD), which diverts blood toward the RV. (medscape.com)
  • Echocardiography can be used to correctly identify the relative position of the great arteries, the degree of subsemilunar narrowing, the position of the ventricular septal defect (VSD), and the status of the mitral valve and left ventricle. (medscape.com)
  • Atrioventricular septal defect (AVSD) or atrioventricular canal defect (AVCD), also known as " common atrioventricular canal " or " endocardial cushion defect " (ECD), is characterized by a deficiency of the atrioventricular septum of the heart that creates connections between all four of its chambers. (wikipedia.org)
  • Current options and outcomes for the management of atrioventricular septal defect. (nih.gov)
  • A ventricular septal defect is virtually always present and provides the only outlet of blood flow from the left ventricle. (msdmanuals.com)
  • Chambers of either right or left ventricular type were found supporting either or both great arteries (outlet chambers), and also without a direct outlet (trabecular pouches). (nih.gov)
  • Although hearts with atrioventricular discordance (ie, congenitally corrected TGA) or univentricular atrioventricular connections (ie, double inlet left ventricle) can be correctly grouped in this spectrum of anomalies, this article focuses on only those hearts with atrioventricular concordance and two functional ventricles. (medscape.com)
  • DOLV occurs most commonly in the form of atrial situs solitus with atrioventricular (AV)concordance but is often associated with myriads of cardiac anomalies such as VSD, ASD, PDA,pulmonary stenosis, right ventricular hypoplasia, and tricuspid atresia (TA). (medresearch.in)
  • Hearts with these ventricular morphologies possessed either double inlet atrioventricular connexions, or absence of the right or left atrioventriuclar connexion. (nih.gov)
  • In double inlet ventricles, cases were observed with two valves, a common valve, one imperforate valve, and with straddling atrioventricular valves. (nih.gov)
  • Not only have new operations been developed and expanded, such as the intra/extracardiac conduit Fontan and the double root translocation for corrected transposition, but in addition, diagnostic methods-particularly cardiac CT and MRI-have been dramatically transformed. (libreriastudium.it)
  • Information on all of the patients' cardiac lesions was collected, including anomalous heart position, common atrium, major atrioventricular (AV) valve anomaly, pulmonary outflow obstruction, anomalous pulmonary venous connections, obstruction of pulmonary venous connections, and systemic outflow obstruction. (springeropen.com)
  • Double outlet left ventricle (DOLV) is an extremely rare cyanotic congenital cardiac malformationwith an incidence of less than 1 in 200,000 live births and usually manifests during the neonatalperiod. (medresearch.in)
  • Coronary sinus, normally located between the LEFT ATRIUM and LEFT VENTRICLE on the posterior surface of the heart, can serve as an anatomical reference for cardiac procedures. (bvsalud.org)
  • A tendinous band, which may be named the tendon of the conus arteriosus, extends upward from the right atrioventricular fibrous ring and connects the posterior surface of the conus arteriosus to the aorta. (wikidoc.org)
  • The transthoracic echocardiography demonstrated atrioventricular and ventriculoarterial discordance. (scirp.org)
  • In four-chamber view, atrioventricular discordance was detected with severe tricuspid regurgitation (vena contracta = 9.3 mm) and dysplasia of the mitral leaflets with severe mitral regurgitation (vena contracta = 10.3 mm) by color Doppler, Video 1. (scirp.org)
  • The left-sided atrioventricular valve, in this case, the tricuspid valve, showed apical displacement of the septal valve suggesting Ebstein's anomaly. (scirp.org)
  • Pulmonary root translocation for biventricular repair of double-outlet left ventricle with absent subpulmonic conus. (medresearch.in)
  • A straddling valve was also found in hearts with absence of one atrioventricular connexion. (nih.gov)
  • A total of 69 % of patients with a CAVV had moderate or severe regurgitation, while 27 % with a single atrioventricular valve had moderate or severe regurgitation. (springeropen.com)
  • The ventricle morphology is described in hearts in which the entire atrial inputs are committed to a single chamber in the ventricular mass, this being considered the criterion for definition of the univentricular heart. (nih.gov)
  • Double outlet from chamber of left ventricular morphology. (medresearch.in)
  • 4Dimensional XStrain echocardiographic assessment by sequential chamber analysis of Double Outlet Left Ventricular with Tricuspid Atresia. (medresearch.in)
  • 1. Bengur AR, Snider AR, Peters J, Merida-Asmus L: Two dimensional echocardiographic features of double outlet left ventricle. (medresearch.in)
  • Medial rotation from the left exposes the left ventricle apex, left pulmonary veins, and left atrium. (medscape.com)
  • The morphologic spectrum of double outlet left ventricle and its surgical significance. (medresearch.in)
  • MRI is useful to obtain additional anatomic information, such as the relationship of both ventricles. (medscape.com)
  • Double outlet ventricles: review of anatomic and imaging characteristics. (nih.gov)
  • A hole in the wall between the right and left lower heart chambers, or the ventricles. (vejthani.com)
  • 2. Manner J, Seidl W, Steiding G: Embryological observations on the formal pathogenesis of double-outlet left ventricle with a right ventricular infundibulum. (medresearch.in)
  • As discussed earlier, double outlet right ventricle (DORV) comprises a heterogeneous series of associated cardiac anomalies that involve the RV outflow tract in which both of the great arteries arise entirely or predominantly from the RV. (medscape.com)
  • Double outlet right ventricle (DORV), as depicted in the image below, is a type of ventriculoarterial connection in which both the aorta (AO) and pulmonary artery (PA) arise entirely or predominantly from the right ventricle (RV). (medscape.com)
  • Double outlet right ventricle (DORV) with transposition of the great arteries accounts for 26% of cases of DORV. (medscape.com)
  • DORV is usually associated with concordant atrioventricular (AV) connections (ie, the right atrium drains into the RV and the left atrium drains into the LV). (medscape.com)
  • Double outlet right ventricle (DORV) is a type of rare congenital heart condition. (childrenshospital.org)
  • Double outlet right ventricle (DORV) is a rare congenital heart defect, meaning it's a condition a baby is born with. (abelspeaks.org)
  • In DORV, the pulmonary artery and the aorta - the heart's two major arteries - both connect to the right ventricle. (abelspeaks.org)
  • DORV creates a problem because the right ventricle carries oxygen-poor blood, which then gets circulated in the body. (abelspeaks.org)
  • Double outlet right ventricle (DORV) is a heart disease that is present from birth (congenital). (stlukes-stl.com)
  • Double outlet right ventricle (DORV) is a type of conotruncal deformity in close relationship with complex cardiac anomalies, which are bound together by a characteristic that both great arteries emerge predominantly from the right ventricle. (rbccv.org.br)
  • The final diagnosis was DORV with situs solitus, D-loop ventricles, and L-position of the aorta {S,D,L}, left aortic arch associated with a large subaortic VSD, and pulmonary stenosis. (rbccv.org.br)
  • DORV repair, including the Rastelli procedure (VSD closure with porcine pericardium, right ventricle-pulmonary trunk conduit: 14 mm Contegra® Medtronic, Inc. Medtronic Parkway Minneapolis, United States of America) and cessation of the antegrade flow by over-and-over suture technique, was performed. (rbccv.org.br)
  • The patients had combinations of defects that included dextrocardia, transposition of great arteries, double-outlet right ventricle, atrioventricular septal defects, and caval vein abnormalities. (eur.nl)
  • Some of the heart defects involve structures within the heart itself, such as the two lower chambers of the heart (the ventricles) or the valves that control blood flow through the heart. (nih.gov)
  • Complex biventricular repair is an option for children with single ventricle heart defects to achieve two-ventricle circulation. (childrenshospital.org)
  • Discuss in detail anomalies of the cardiac chambers to include septal defects and anomalies of the right and left ventricle. (medicine.com.co)
  • Ventricular septal defects (VSDs) commonly occur in the area between the sinus and the outlet tract of the right ventricle. (medscape.com)
  • However, because the surface of the right ventricle is trabecular, small defects of the muscular portion of the ventricular septum may be difficult to see. (medscape.com)
  • Normally the pulmonary valve connects to the right ventricle, and the aorta connects to the left ventricle. (msdmanuals.com)
  • In a normal heart, the pulmonary artery connects to the right ventricle, and the aorta connects to the left ventricle. (abelspeaks.org)
  • Patients with congenital heart disease characterized by a functional single ventricle make up an increasing number of patients presenting for cardiac or noncardiac surgery. (silverchair.com)
  • Thirty patients aged 4-12 months with a functional single ventricle were randomized to receive fentanyl-midazolam or sevoflurane. (silverchair.com)
  • Herein we present the case of a female patient who had received Fontan operation due to functional single ventricle at the age of five. (j-nn.org)
  • Fontan operation is a palliative surgical procedure performed in pediatric patients who have a functional single ventricle. (j-nn.org)
  • In this report, we present a case of 19-year-old female who had received Fontan operation due to functional single ventricle at the age of five and experienced acute basilar artery occlusion that was successfully treated with mechanical thrombectomy. (j-nn.org)
  • Monckeberg's heart, however, was characterised by the presence of discordant atrioventricular connections, and double outlet right ventricle. (bmj.com)
  • Unguarded mitral orifice associated with discordant atrioventricular connection, double-outlet right ventricle, and pulmonary atresia. (uchicago.edu)
  • Although many anatomical variations may be encountered in children with double outlet right ventricle, coexistence of levo-malposed great vessels and left juxtaposed atrial appendages is uncommonly observed. (rbccv.org.br)
  • Two recently described Doppler echocardiographic modalities, the myocardial performance index and Doppler tissue imaging, can be applied to single-ventricle patients because they are independent of ventricular geometry. (silverchair.com)
  • Long-term results of the Fontan operation for double-inlet left ventricle. (uchicago.edu)
  • In four-chamber view, atrioventricular discordance was detected with severe tricuspid regurgitation (vena contracta = 9.3 mm) and dysplasia of the mitral leaflets with severe mitral regurgitation (vena contracta = 10.3 mm) by color Doppler, Video 1. (scirp.org)
  • Mitral insufficiency , also known as mitral regurgitation ( MR ), is a type of valvular heart disease in which there is backward flow of blood from the left ventricle , through the mitral valve , into the left atrium , when the heart contracts . (mdwiki.org)
  • The patient was a 3-month-old male with cyanotic congenital heart disease consisting of double outlet right ventricle, near absent intraventricular septum, and aortic arch hypoplasia, who had previously undergone a Norwood stage I palliation with 3.5 mm modified Blalock-Taussig shunt (see Figure 1 ). (hindawi.com)
  • This is a hole in the tissue wall (septum) that normally separates the right and left ventricles. (abelspeaks.org)
  • Of these 3 subdivisions, the conal septum is clinically significant because it can be malpositioned in patients with congenital disorders (eg, double outlet right ventricle ). (medscape.com)
  • The degree of blood oxygenation in the systemic as well as the pulmonary circuits is determined by degree of mixing in the systemic (ie, right) ventricle, which, in turn, depends on the degree of resistance upstream of the pulmonary valve. (medscape.com)
  • Pulmonary atresia occurs when the pulmonary valve - normally located between the right ventricle and the pulmonary artery - doesn't form properly. (childrenshospital.org)
  • The left-sided atrioventricular valve, in this case, the tricuspid valve, showed apical displacement of the septal valve suggesting Ebstein's anomaly. (scirp.org)
  • In double outlet right ventricle, both the aorta and the pulmonary artery are connected to the right ventricle and no artery attaches to the left ventricle. (msdmanuals.com)
  • The VSD allows oxygen-rich blood to pass from the left ventricle to the aorta and pulmonary artery. (abelspeaks.org)
  • A Sub Aortic Membrane, which is absent in a normal heart, causes obstruction to blood flow out of the Left Ventricle into the aorta. (sssihms.org)
  • Medial rotation from the left exposes the left ventricle apex, left pulmonary veins, and left atrium. (medscape.com)
  • During contraction of the left ventricle there is backflow (arrow) into the left atrium. (mdwiki.org)
  • The right ventricle discharges blood into the pulmonary artery across the pulmonic (semilunar) valve located in the outflow tract (infundibulum). (medscape.com)
  • The inflow tract (sinus) and outflow tract (infundibulum) of the right ventricle are widely separated. (medscape.com)
  • The structures initially seen from this perspective include the superior vena cava, right atrium, right ventricle, pulmonary artery, and aorta. (medscape.com)
  • The right ventricle receives blood from the right atrium across the tricuspid valve, which is located in the large anterolateral (ie, sinus) portion of the right ventricle. (medscape.com)
  • A preoperative echocardiogram demonstrated the above described anatomy, insignificant atrioventricular valve insufficiency, a patent modified Blalock-Taussig shunt, and patent proximal branch PA's. (hindawi.com)
  • 9. Virtual reality three-dimensional echocardiographic imaging for planning surgical atrioventricular valve repair. (nih.gov)
  • These are the valves that separate the atria (top collecting chambers) from the ventricles (bottom pumping chambers). (stlukes-stl.com)
  • The normal heart beats according to the electrical impulses from the sinoartial node to the atrioventricular node. (sssihms.org)
  • In cases of a subaortic VSD, which occurs in 60-70% of patients, the VSD is closer to the aortic valve, thus oxygenated blood from the LV is directed to the AO and desaturated blood from the right ventricle (RV) is directed primarily to the PA (see the image below). (medscape.com)
  • CASE PRESENTATION: A 80-year-old Caucasian female with a past medical history of hypertension and hyperlipidemia presented with exertional chest pain, lightheadedness, and shortness of breath with telemetry and electrocardiogram revealing persistent Mobitz type II second degree atrioventricular block. (bvsalud.org)