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Dorv
- 1. DOUBLE OUTLET RIGHTVENTRICLE
- 2.
- 3. INTRODUCTION 1793 -John Abernethy 1st described the condition. 1957 - Witham coined the term as DORV. 1957 - 1st repair: Kirklin
- 4. DEFINITION When onegreat artery arises nearly or wholely from RV and the other more than 50%. TOF with DORV is same as DORV with pulmonary stenosis Taussig Bing Heart: variability in origin of pulmonary artery
- 5.
- 6. EMBRYOLOGY AND ANATOMY Conus: The muscle between semilunar and AV valve. Lev’s theory of Conotruncal malseptation Van Praagh’s theory of Conal underdevelopment
- 7. VAN PRAAGH THEORYOF CONAL UNDERDEVELOPMENT “ the distal or semilunar part of infundibulum or conus arteriosus performs an arterial switch during cardiogenesis”
- 8. EXTENSION OF VANPRAAGHS THEORY
- 9. VENTRICULAR SEPTAL DEFECT The only outflow tract of the left ventricle. Mostly conoventricular. Accordingly however DORV can be classified with respect to VSD location. Subaortic Subpulmonary Doubly committed Non committed.
- 10.
- 11. CORONARY ARTERY ANATOMY Depends upon the great arteries At the transposition end of the spectrum: LMCA and LAD pass anterior to PA TOF: LMCA and LAD pass posterior to PA.
- 12. HELLEN TAUSSIG ANDRICHARD BING
- 13. THE TAUSSIG BINGHEART Applied in physiological and anatomical sense. Physiological Anatomical: Helen Taussig and Richard Bing (1949) Van praagh (1968) summarized anatomic definition
- 14. THE TAUSSIG BINGHEART
- 15. ASSOCIATED ANOMALIES Coarctationof aorta Arch hypoplasia Interrupted aortic arch
- 16. CLINICOPATHOLOGY AND HEMODYNAMICS Highly variable Large VSD with severe fixed pulmonary stenosis: cyanotic child VSD with no pulmonary stenosis: features of heart hailure with progression towards pulmonary vascular disease.
- 17. STREAMING Determined byrelationship of semilunar valves to VSD and position and presence of infundibular septum. Simple DORV: High Qp; heart failure; without cyanosis Subpulmonary VSD: Taussig Bing heart: SpaO2> SaO2. Infants present like TGA
- 18. CLINICAL FEATURES ANDEVALUATION Type of symptoms and age of appearance depends upon degree of pulmonary stenosis Echo: Size of VSD, Relation to semilunar valves Subvalvular conus AV valve abnormalities Position of great arteries Coronary artery anatomy
- 19. EVALUATION Cardiac catheterization:not in infants; indicated in older children for PVR Ventricular end diastolic pressure MAPCA Cineangiography: Assessing complex interrationships in DORV with superior- inferior ventricles and criss cross hearts
- 20. MANAGEMENT: MEDICAL ANDCATHETER BASED Decongestive medications: in cases of high Qp heart failure; followed by surgery BAS: at the transposition end of DORV: Allows improved mixing Atrial septal defect allows decompression of left side of heart: better surgical field
- 21. INDICATIONS AND TIMINGOF SURGERY Diagnosis is sufficient for surgery Timing of surgery depends upon the subset of the patients in question Simple DORV: electively by 3-6 months of age Sooner if symptoms of heart failure or failure to thrive appear DORV with pulmonary stenosis: same as for TOF Taussig Bing: during 1st month of age Operations including extracardiac conduit but excluding ASO: 3-6 years of age.
- 22. SURGICAL MANAGEMENT 1957– Kirklin and 1958 – Barratt-Boyes: 1st successful repair of DORV
- 23. ANATOMICAL DETERMINANTS OFMETHOD OF REPAIR 1. Separation of Pulmonary and tricuspid valve 2. Prominance of Conal septum 3. Presence of subpulmonic stenosis
- 24. SEPARATION OF PULMONARYAND TRICUSPID VALVES
- 25. PROMINENCE OF CONALSEPTUM Length of conal septum largely determined by the development of subaortic conus A prominent conal septum will necissitate a longer baffle pathway along its inferior margin Long conal septum associated with shorter distance between TV and PV: prone to stenosis.
- 26. PRESENCE OF SUBPULMONARYSTENOSIS At the tetralogy end of DORV spectrum Requires relief of stenosis. LV baffle pathway will further cause RVOT narrowing: Infundibular outflow patch Significant stenosis precludes the technique of ASO for Transposition end of DORV spectrum
- 27. SURGICAL TECHNIQUES SA-VSDwith adequate TV-PV distance: intraventricular baffle technique PA-VSD in Tassig Bing without PS: ASO with VSD closure Doubly committed VSD: Baffle Noncommitted VSD: baffle with myocardial flap reconstruction of straddling TV
- 28. INTRAVENTRICULAR BAFFLE FOR UNCOMPLICATEDDORV Standard midline sternotomy/cardioplegic arrest RA approach: technically difficult, No significant studies to show that safer than ventricular approach Opened cephalocaudal near AV groove
- 29. INTRAVENTRICULAR BAFFLE RVapproach: Transverse ventriculotomy in low RVOT Vertical infundibular ventriculotomy: if the distance between LAD and RCA small Assessment of anatomy: VSD Distance between TV and PV
- 30.
- 31. INTRAVENTRICULAR REPAIR Enlargementof VSD: anteriorly, Risk of damaging LAD Risk of damaging MV VSD patch: Polyester tube graft diameter >20% of Ascending aorta, Length: Anterior edge of VSD to sub Ao conus Neonates and infants: standard pledgetted interrupted suture technique Older: continuous
- 32. VSD PATCH ININTRAVENTRICULAR BAFFLE Flat patch : Knitted Daron Velour Tube: PTFE
- 33.
- 34. REPAIR OF TAUSSIGBING ANOMALY Closure of VSD followed by ASO Approach Perimembranous VSD: RA Rvoutlet VSD and PA not overriding : Proximal aorta (after harvesting coronary buttons) PA overridin: PA
- 35. TAUSSIG BING REPAIRBY INTRAVENTRICULAR APPROACH Intraventricular tunnel repair: Kawashima Method If insufficient space between TV and PV: Patrick-McGoon method Lecompte Inraventricular repair
- 36. KAWASHIMA METHOD RV openedthrough vertical or transverse incision
- 37. KAWASHIMA METHOD VSD enlargedby incision anteriorly and excision of some portion of infundibular septum
- 38. KAWASHIMA METHOD Intraventricular tunnelposterior to PA
- 39. PATRICK-MCGOON METHOD Transverse ventriculotomyand VSD enlargement of inlet septum
- 40. PATRICK-MCGOON METHOD PTFE tubegraft with diameter >20% of ascending aorta;
- 41. PATRICK-MCGOON METHOD Continuous sutuiringof the tube graft
- 42. PATRICK-MCGOON METHOD Completion ofspiralled tube graft
- 43. RESULTS Early survival:current hospital mortality <2% for simple baffle procedure. Taussig Bing heart ~ 3.7% Time related survival: >95% @ 15 yrs Age at repair: Older age is a risk factor: ecplained by absence or presencec of pulmonary vascular disease
- 44. RESULTS Type ofrepair: Favourable subtypes: Simple DORV, DORV with doubly committed VSD Unfavourable: DORV with subpulmonic VSD Major cardiac anomalies Pulmonary stenosis: incremental risk factor CAVCD, Hypoplasia of cardiac chamber, mitral valve anomalies
- 45. COMPLICATIONS OF REPAIR Major reoperation are mainly for Leakage and obstruction of baffle: <1% catheter based evidence of incidence LVOTO: manly due to potentially obstructing muscle in the LVOTO
- 46.
Editor's Notes
- #4 Diagnosis was correctly made at operation and the term DORV was coined in operating room.
- #5 Artery is assigned to the ventricle it overrides more than 50%. aorta overrides >50% to RV in TOF. Taussig bing heart: PA arises wholely or nearly so from RV, Eaqually from right and left ventricles and >50% but not entirely from the Left ventricle When it arises entirely from LV then : TGS with VSD.
- #7 Lev’s theory: Conotruncus which ultimately forms PA and Ao forms as spiral septation. If septum doesnot spiral in usual fashion at all: All great vessels in parallel: TGA If septum spiralling slightly abnormal: dextroposition of aorta relative to its usual location: TOF, Spiralling is more than TOF and less than TGA: DORV
- #8 TOF: underdevelopment of pulmonary conus: AO is more anterior and to the right and superior, Conoventricular VSD with anterior malalignment of conal septum: RVOTO. Extension of Van Praagh:
- #9 AV, TV, MV are in fibrous continuity PV is separate by sub pulmonary conus (infundibulum) DORV: Bilateral Coni: both AV and PV are lifted away from the AV valves TGA: No sup pulm conus and PV is in fibrous continuity with TV and MV, AoV is lifted away by the sub ao conus Greater digree of conal underdevelopment result in great arteries to lie side by side. Particularly if there is compensatory development of subaortic conus. No subpulmonary counus but aortic conus: TGA
- #10 Conoventriccular: between the two limbs of TSM. Sub aortic: TOF end: no sub aortic conus: superior margin of VSD is the Ao valve itself. Sub pulmonary: TGA end: Progressive Subaortic conus; Ao Valve moves cephalad; and PV more intimately associated: Subpulmonary. Non committed: VSD not in conal septum or Jn of conal and interventricular septum(subaortic) : remote from aorta: difficult to direct LV flow to aorta:: inlet type Sub aortic VSD: VIF: posterior margin of VSD. Bundle away Subaortic Perimembranous VSD near TV : Posterio division of TSM is deficient: Bundle lies in poesterior inferior margin of VSD.
- #12 LAD anterior excludes an intraventricular repair and suggests that the defect is closer to the transposition end of the spectrum and requires ASO.
- #13 Richard John Bing (October 12, 1909 Nuremberg, Germany – November 8, 2010 La Cañada Flintridge, California) was a cardiologist who made significant contributions to his field of stud Helen Brooke Taussig (May 24, 1898 – May 20, 1986) was an American cardiologist, working in Baltimore and Boston who founded the field of pediatric cardiology. Notably, she is credited with developing the concept for a procedure that would extend the lives of children born with Tetralogy of Fallot (the most common cause of blue baby syndrome). This concept was applied in practice as a procedure known as the Blalock-Taussig shunt. The procedure was developed by Alfred Blalock and Vivien Thomas, who were Taussig's colleagues at the Johns Hopkins Hospital.
- #14 Physiological: similar to transposition. Saturations higher in PA than in Aorta. Because of preferential blood flow from LV to PA.
- #15 Both Subaortic and sub pulmonary conus present, separate both aortic and pulmonary valves from Atrioventricular valves. Semilunar valves lie side by side. And are at the same height Large subpulmonaryVSD present True DORV: Aorta arises entirely from RV and PA overrides the ventricular septum, but doesnot override the left ventricular cavity.
- #18 Simple DORV: flow of highly oxygenated LV blood through VSD is directed prefrencially benieth infundibular septum to adjuscant aorta. Systemic venous blood to PA Taussig bing heart: LV blood into Adjascent PA by vertically positioned infundibular septum. Venous blood from RV going into Ao.
- #19 Simple DORV: simple large VSD Also for DORV with doubly committed and non committed VSD Taussig bing: TGA DORV with pulm stenosis/ atresia: TOF with pulmonary stenosis/atresis
- #23 1st repair was TOF type.
- #25 Baffle from VSD to Aorta. RVOT passesaround the LV baffle but still within the RV. As AV moves superiorly and away from TV, PV moves closer to TV. Baffle pathway must pass between the PV and TV Adequate separation Separation less than the dia of Ao valve: likely to result in stenosis PV very close to TV: Precludes intraventricular repair.
- #30 Assessment: whether VSD abutts or has muscular fold
- #32 Continuous suture in infants cause unacceptable incidences of residual VSD
- #33 Marking stitch: most anterior part of the repair 1st matress stitch: Atria->ventricle-> base of Tricuspid commissure between ATL and STL Flat patch whe VSD is closely related to Aorta Tube when baffle has to travel long.
- #41 Initial sutures in usual mannar Preventing damage to the bundle of His
- #42 Leftward arm of the continuous tuture continoud posterior then left margin of aortopulmonary trunk. Then finally along the anterior margin