Double outlet right ventricle: navigation of surgeon to chose best treatment strategy

Transcription

1 Double outlet right ventricle: navigation of surgeon to chose best treatment strategy Jan Marek Great Ormond Street Hospital & Institute of Cardiovascular Sciences, University College London

2 Double outlet right ventricle = > Right ventricle with two outlets Infundibular septum is RV structure!

3 Double outlet right ventricle Double conus does not determine DORV!

4 Complex DORV: Major dilemma Conventional repair (DORV/CCTGA VSD cl. ± LVOTO relief ± TV pl.) Survival at 32yrs 62.4% Freedom from re-operation 64.2% Conventional Rastelli Survival at 30yrs 78.5% Freedom from re-operation 25-50% Arterial switch Survival at 20yrs 90.0% Freedom from re-operation 65% Double switch Survival at 15yrs 74% Freedom from re-operation 84.4% Atrial switch + Rastelli Survival at 16yrs 80% Freedom from re-operation 89.6% R.E.V. Survival at 20yrs 87.5% Freedom from re-operation 92.5% Fontan Survival at 22yrs 79.3% Freedom from re-operation 91.3% Hazekamp MG, EJCTS 2010, Hayes DA, Circulation 2013, Shin`oka T, J Thorac Cardiovasc Surg 2007 Schwartz F, EJCTS 2013, Sarkar D, Circulation, 999, Gellat M, JACC 1997

5 Limitations of current imaging techniques Individualised anatomy up to surgeons spatial imagination and experiences Precise measuring not possible Intracardiac exploration for right decision making on empty heart in diastasis Some surgeon choices are not correct univentricular repair vs. biventricular repair

6 Major concerns for optimal biventricular outcome Restrictive VSD even after enlarged (limited room for enlargement, risk of AVB) Major tricuspid attachment on the pathway of tunnel Marked LVOTO concern after repair Major AV valve straddling

7 Surgical danger areas RH Anderson & A Cook 2005

8 Double outlet right ventricle + VSD TOF Ao Double outlet right ventricle RV LV Ao Pa Pa Ao Pa Ao TGA Courtesy RH Anderson & A. Cook

9 Complex DORV: Ventricular septal defect Located anywhere in septum Any type can occur but PM inlet & outlet VSD are common Inlet defects may be associated with coexisting AV straddling Malaligned outlet defects can cause R or L outflow obstruction

10 DORV/TGA- associated anomalies + (AV/VA [double] discordance [=CCTGA]) + Juxtaposed atrial appendage + (Sub) Pulmonary / Aortic obstruction + Right / Left atrioventricular valve anomaly + Supero-inferior ventricles (Criss-Cross heart)

11 DORV + remote VSD Considering VSD baffle to left side great artery Arterial switch or modified Nikaidoh procedure (no TGA) Possible VSD baffle to right side great artery and Rastelli or R.E.V. Univentricular

12 DORV: Subcostal view DORV + right side-by-side aorta, VSD?: what to do?

13 DORV: Subcostal view DORV + subaortic VSD: for VSD closure

14 DORV: Long sweep DORV + subaortic VSD: for VSD closure

15 DORV: Subcostal view DORV+subpulmonary VSD: for ASO+VSD closure

16 DORV: Parasternal SAX

17 Double outlet right ventricle + non-committed VSD

18 DORV with bilateral conuses and PS

19 DORV + (sub)aortic obstruction Always with COA, Hypoplasia rarely IAA in 5% of complex DORV/TGA, most often with anterior malaligned VSD

20 DORV + (sub)pulmonary obstruction Procedure: R.E.V. + IS resection, VSD patch, RVOT-PA patch

21 Procedure: ASO + subao VSD closure, subpa defect enlargement to provide unobstructed neosubaortic pathway DORV/2xVSD

22 DORV + Right anterior AO + (Sub) Pulmonary obstruction Procedure: ASO + Lecompte, LVOT relief + IS resection, VSD patch

23 DORV + Multiple VSD: Biventricular or Univentricular repair? BiV Uni

24 DORV + VSD with abnormal attachment of tricuspid valve: Biventricular or Univentricular repair? Univentricular

25 Straddling AV valves Straddling of TV

26 Straddling AV valves Straddling of MV

27 DORV/TGA + VSD + (Sub)Pulmonary obstruction PA RV LV Occurs in 25% In 20% of patients with IVS, but only 5% have significant obstruction In 30% of patients with VSD LV AO PA LV

28 DORV/TGA + VSD + (Sub)Pulmonary obstruction S/P Rastelli operation

29 En face view: AV valves (straddling) Septal surface VSD size & position LV-AO pathway

30 DORV: 3D Echocardiography Pushparajah K, et al. Echocardiography. 2013

31 Multimodality imaging

32 3D Printing Understanding 3D topology Choosing best strategy Access to VSD(s) Identification outflow tracts Intraventricular baffling Sizing VSD patch Suturing 3D Printing: practising operation Potential issues Time cost Labor intensive Processing software not fully developed More cases needed No fine structures valves! Courtesy Glen Van Arsdell

33 DORV/TGA in Criss Cross

34 DORV/TGA in Criss Cross AO PA

35 Double outlet RV Tips & Tricks for 2D assessment First scan in newborn or infant decisive Subcostal views crucial for assessing pathways for intraventricular baffling Parasternal short axis most important precordial projection Long sweeps helpful to understand spatial relationship of VSD and great arteries

36 Thank you