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Double Outlet Right Ventricle (DORV) PowerPoint Presentation

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Description : Double Outlet Right Ventricle (DORV)

Tags : Double Outlet Right Ventricle, DORV, ventriculoarterial connection, univentricular hearts, heterotaxy syndrome, AV discordance

Published on : Oct 04, 2020 | Views : 3113 | Downloads : 10

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Double Outlet Right Ventricle Gail E. Wright | Katsuhide Maeda | Norman H. Silverman | Frank L. Hanley | Stephen J. Roth Textbook Reading Divisi Pediatrik Oleh: Enggar Sari K. Pembimbing: dr. Ali Nafiah Nasution, Sp.JP(K)
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Definition Double outlet right ventricle (DORV) is a type of a ventriculoarterial connection in which both great arteries arise 50% or more from the right ventricle (RV) DORV may occur in univentricular hearts, particularly in the heterotaxy syndrome, and with AV discordance This chapter focuses in DORV with concordance atrioventricular (AV) connection with two ventricle. Insidence < 1% of all congenital heart defects (approximately 0.06 cases per 1000 live birth) There is no known racial or gender predilection, and no associated genetic defect has been identified
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In the normal heart, the pulmonary valve sits up on the conus, a circular tube of muscle, and is positioned anteriorly and superiorly. In contrast, the aortic, mitral, and tricuspid valves are all attached to the central fibrous body of the heart. The conal muscle beneath the aortic valve largely resorbs, leaving the aorta positioned inferiorly and posteriorly The more conal muscle present beneath a semilunar valve, the more valve is pushed anteriorly and superiorly, and the more likely it is that the associated great artery will align with the RV Morphology
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Mophologic variability Description of DORV must include association relationship of great vessel and VSD Other anomalies: PS valvar or subvalvar (50%) ASD Secundum (25%), ASD Primum (8%) Coronary artery anomalies (10%) Low: PDA, Right aortic arch, subaortic stenosis, addition muscular VSD, left SVC, mitral valve anomalies
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Clinical Features
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Clinical Features
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“Tetralogy Type” Most common variant (40% cases), also known by “DORV with subaortic VSD with PS” PV positioned superiorly and anteriorly, aorta overrides IVS Blood from the LV passes through VSD to aorta
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Surgery indicated to relieve the obstruction to pulmonary blood flow and to septate the circulation Surgical outcomes excellent in both neonates and older infants In most patients, surgery performed electively 2-4 mo before infants becomes excessively cyanotic or develop hypercyanotic spells “Tetralogy Type”
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“Transposition type” Approximately 20% cases, include ‘Taussig-Bing anomaly” or “DORV with subpulmonic VSD” Ao pushed anteriorly and superiorly  rightward position Ao relative to PA No pulmonary-mitral continuity Streaming high O2 blood from LV  VSD  PA, most deoxygenated blood from RV  Ao
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Surgery indicated to improve systemic oxygenation and performed in neonatal period Surgical repair for establishing continuity between LV and Ao Baloon artrial septostomy for improves artrial mixing for severe hypoxemia for non-emergency surgical repair in 1st wk of life, often for newborns with d-transposition and intact IVS “Transposition type”
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“VSD Type” Approximately 15% cases, also known as “DORV with subaortic VSD without PS” Slightly more conal tissue beneath P valve than Ao valve  P valve still anterior, Ao rotated rightward Large VSD  L to R shunting  Pulmonary overcirculation History: Infants typically sign of CHF as the pulmonary vascular resistance falls between 4-8 wks Failure to thrive Surgery indicated to eliminate L ro R shunt Timing elective
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DORV with “Noncommitted VSD” Occurs <10% cases Significant conus beneath each great vessels, neither both vessels truly posterior IVS connection not directly related to either great vessel May some degree valvar dan subvalvar PS  balanced circulation or inadequate Pulmo blood flow Attention to attachment of AV valves, esp tricuspid (may interposed between VSD and GA, may impact VSD closure) Repair deffered until several years
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Other less common variants
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Essential determinants of surgical correction and timing of the operation Surgical decision making for DORV is the most complicated in congenital heart surgery Depends on: specific anatomy features, age, body size, clinical condition and surgeon’s skill Relatively poor outcomes in the early years of surgery for DORV due to lack of precise anatomic definition and recognition Relationship of the conduction system to the VSD is important, some VSD must be enlarged to create sufficient pathway for intracardiac baffling If an adequate pathway cannot be achieved, Fontan operation is required
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Types of surgery and surgical strategy Principles: Biventricular repair is performed if it is feasible An adequate LVOT must created, even if it compromises the RVOT Surgical correction also physiologic correction “VSD type”: eliminate L-R shunt  closing VSD “Transposition type”: ventricular outflow must be switched to achieve normal two-ventricle physiology “Tetralogy type”: routing blood from the LV to aorta with enlargement of RVOT
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Reintervention and Reoperation In patients with simpler forms of DORV such as VSD type and tetralogy type, freedom from reoperation is excellent Patients with complex intracardiac baffle are substantial risk of LVOT obstruction. Risk reoperation 5-50%, particularly high in those with subpulmonary VSD
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Management of the adult with DORV Depends on patients conditions, type of repair, residual lesion Exercise intolerance and decline functional status should alert cardiologist to the possibility of either residual lesion or reduction in ventricular function Arrythmia caused by progressive, abnormal hemodynamics leading to chamber dilatation and/or hypertrophy with resultant ventricular dysfunction RBBB and QRS prolongation after repair increased risk sustained VT and SCD Succesful pregnancy and delivery after biventricular repair Patients following DORV repair not require routine endocarditis prophylaxis except: prosthetic cardiac devide, prev endocarditis, etc
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Terima kasih
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