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DGPK guideline Double Outlet Right Ventricle (DORV) H. Bertram, MHH, Hannover J. Weil, UKE, Hamburg J. Sachweh, UKE, Hamburg DGPK guideline committee Guideline DORV Definition 100 % + 100 % 100 % + > 50 % Double Outlet Right Ventricle (DORV) represents a spectrum of congenitally malformed hearts in which the circumference of both arterial valves, or the greater part of both circumferences, are supported by the right ventricle prevalence: • 1,3 % of cardiac defects (PAN) • 1,1 / 10.000 live births Guideline DORV Definition > 150 % rule Mahle WT et al. Cardiol Young 2008; 18(Suppl. 3): 39–51 Guideline DORV Definition Double Outlet Right Ventricle (DORV) represents a spectrum of congenitally malformed hearts in which the circumference of both arterial valves, or the greater part of both circumferences, are supported by the right ventricle • same ventriculo-arterial connection, but variations in - infundibular morphology - arterial interrelationship - coronary arterial anatomy • any arrangement of the atrial appendages, or situs • any atrioventricular connection • multiple combinations of associated malformations Guideline DORV spatial relationship of the semilunar cusps in hearts with DORV Guideline DORV ‚Interventricular communication‘ vs ‚VSD‘ Mahle WT et al. Cardiol Young 2008; 18(Suppl. 3): 39–51 Guideline DORV Diagnostics Goal: displaying cardiac anatomy with emphasis on potential surgical biventricular repair (feasibility of tunneling the interventricular communication to one or other arterial trunk) • position, size, interrelationship, course of the great arteries • morphology and size of the interventicular communication / the VSD in relation to diameter of the aortic valve • location and severity of a subpulmonary or subaortic obstruction • morphology and size of both ventricles and AV-valves Methods: • Echocardiography • Angiography (Cath./MRT/CT) Guideline DORV Classification of the interventricular communication / VSD according to its location in relation to the great arteries • subaortic (65%) • subpulmonary (20-25%) • doubly committed (3%) • non committed (7%) Guideline DORV subaortic VSD subaortic VSD • size in relation to the diameter of the aorta • distance between VSD and aortic valve • presence and severity of a subpulmonary obstruction • subcostal coronal and sagittal planes; parasternal long axis Guideline DORV subaortic VSD parasternal long axis Ao Ao LV LA Guideline DORV subaortic VSD Subcostal TEE RV Ao PA Guideline DORV subaortic VSD subaortic VSD with severe subpulmonary obstruction RV RV Ao Ao PA Guideline DORV doubly committed VSD • size and distance of the VSD to the aorta / pulmonary artery • presence and severity of a subpulmonary obstruction • subcostal coronal and sagittal planes Guideline DORV subpulmonary VSD • size in relation to the diameter of the pulmonary artery • presence and severity of a subpulmonary obstruction • subcostal coronal and parasternal long axis planes Guideline DORV subpulmonary VSD RV PA RV PA Guideline DORV subpulmonary VSD RV PA Guideline DORV subpulmonary VSD Taussig-Bing malformation: • DORV with subpulmonary VSD • semilunar valves side-by-side • no subpulmonary obstruction • semilunar valves and AV-valves separated by conal septum Guideline DORV non-committed VSD • location and size; distance to semilunar valves • presence and severity of a subpulmonary obstruction • subcostal coronal / 4 C views LV Ao Guideline DORV non-committed VSD RV PA Ao RV LV Guideline DORV non-committed VSD Guideline DORV Double Outlet Right Ventricle Malposition of the great arteries, which arise completely (100% + 100%) or with the greater part of their circumference (100% + > 50%) from the right ventricle interventricular communication in relation to the great arteries concommittant malformations clinical symptoms clinical subtype surgical strategy subaortic VSD ‚doubly committed‘ subpulmonary VSD valvular / subvalvular PS valvular / subvalvular PS aortic coarctation (in ~ 50 %) pulmonary hyperperfusion; congestive heart failure reduced lung perfusion; mild severe cyanosis severe cyanosis; congestive heart failure VSD - type TOF - type biventricular repair 1-6 mo (VSDclosure) biventricular repair 4-12 mo (VSD closure + relief of RVOTO) TGA - type biventricular repair < 1 mo (VSD-closure + arterial switch) ‚non committed‘ AVSD / heterotaxy subpulmonary obstruction • right atrial isomerism • TAPVD • l-SVC • subpulmonary obstruction clinical symptoms determined by concommittant malformations complex DORV biventricular repair 2-6 years (complex intracardiac tunneling +/- VSD incision / arterial switch) or definitive univentricular palliation Guideline DORV medical treatment • PG E in duct dependent pts with severe subpulmonary obstruction • diuretics, ß-blockers, … in pts with pulmonary hypercirculation and congestive heart failure catheter intervention • TOF-type: balloon valvuloplasty; ductal stent; RVOT stent • TGA-type: BAS surgical palliation • TOF-type: modified BT-shunt if primary repair is not suitable or considered ‚high risk‘ • PAB in ncVSD to delay complex intraventricular repair Guideline DORV Surgical Repair VSD - type • VSD closure in the age of 1 to 6 months - some pts need enlargement of the VSD (> 4/5 aortic annulus); cave: AV-Block TOF - type • VSD closure and relief of subpulmonary obstruction in the age of 4 to 12 months +/- muscular and transjunctional incision or patch enlargement Guideline DORV Surgical Repair TGA - type • neonatal corrective surgery with arterial switch, VSD closure +/- resection of aortic coarctation / aortic arch reconstruction +/- resection of subaortic infundibulum cave: coronary artery anomalies • alternatively ‚Rastelli - type repair‘: baffling of the left ventricle to both arterial valves and placement of a conduit from RV to the pulmonary trunk Subpulmonary VSD with valvular/subvalvular pulm. stenosis • ‚Kawashima-OP‘ • ‚Rastelli-OP‘ • ‚REV-procedure‘ (Reparation a l‘ Etage Ventriculaire) • ‚Aortic translocation‘ – ‚Nikaidoh-procedure‘ Guideline DORV Surgical Repair Complex DORV • biventricular repair aged 2-6 years: complex intraventricular baffling (LV Ao/PA) +/- VSD enlargement +/- arterial switch • definitive functionally univentricular palliation Guideline DORV Surgical Repair Implications of the 200 % rule (‚true‘ DORV) Percent of Great Vessels Arising from the RV DORV 200 % > 150 % technical difficulty ‚VSD – type‘ no yes simple ‚Fallot – type‘ no yes average ‚TGA – type‘ no yes important DORV-AVSD yes yes major DORV ncVSD yes yes major Modified from: F. Lacour-Gayet: Intracardiac Repair of Double Outlet Right Ventricle Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2008;11:39-43 Guideline DORV prognosis • biventricular repair achievable in most pts • increased operative risk determined by concommittant malformations: • aortic arch obstructions • AV-valve anomalies • coronary arterial anomalies • LV hypoplasia • multiple VSDs Guideline DORV Residual lesions requiring reinterventions after surgical repair • Depending on different morphology and type of previous surgical repair • TOF – type: - pulmonary valve incompetence - residual subpulmonary obstruction • RV-PA-conduit: definitive reoperation for conduit replacement (stenosis, incompetence, size-mismatch in growing children) • complex intracardiac baffling: subaortic obstruction biventrcular surgical repair has a much higher rate of reintervention than a strategy of functionally univentricular palliation Guideline DORV Follow-up • Life-long follow-up by pediatric cardiologists and subsequently specialists for adult congenital heart disease is mandatory