The Failing Systemic Right Ventricle European Society of Cardiology 2012

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1 The Failing Systemic Right Ventricle European Society of Cardiology 2012

2 I have nothing to disclose.

3 Is the right ventricle an inherently weaker ventricle? Functionally single ventricle TGA (after atrial switch) CCTGA

4 RV Morphology: Functionally Single V 237 Surgery for Congenital Heart Disease The Journal of Thoracic and Cardiovascular Surgery Volume 123 Predictors of outcome after the Fontan operation: Is hypoplastic left heart syndrome still a risk factor? J.William Gaynor, MD Nancy D. Bridges, MD Mitchell Cohen, MD William T. Mahle, MD William M. DeCampli Objective: This study was undertaken to evaluate factors in early mortality and morbidity after the Fontan procedure between January 1, 1992, and December 31, 1999.

5 RV morphology: Functionally Single V Gentles et al. JTCVS 1997;114:376-91

6 Force-frequency relationships LV n=19 RV n=17 Normal n=13 Cheung et al. Heart 2005

7

8

9 ACE inhibition (18pts, R/DB/PC) No change in resting Doppler indices, HR, cardiac index or exercise duration Trend to reduced CI and VO 2 Exercise induced increase in cardiac index significantly reduced with treatment Kouatli et al. Circulation 1997

10

11 Beta blockade? (151 pts R/DB/PC) Shaddy R. et al JAMA Sept 2007

12 RV dilation CCTGA/Mustard-Senning Reduced resting/stressed RVEF Reduced exercise function Neurohormonal activation Systemic RV dilated cardiomyopathy

13 Late RV Failure: Treatment ACE inhibition? - Open label, non randomised - No overall change in RV function (MRI) or exercise function (Hechter et al. Am J Cardiology 2001;87:660-3) Angiotensin receptor blockade? - Multicentre, randomised, placebo-controlled with Losartan - No change in exercise function or NT-BNP -.. implies an alternative mechanism for ventricular dysfunction and impaired ex capacity in these patients.. (Dore et al. Circulation 2005;112: )

14 Aetiology of RV dysfunction Ischemia? 61 patients after atrial switch (7-23 years old) Resting and peak exercise technetium 99 tomography Lubiszewska et al. JACC 2000;36:

15 Aetiology of RV dysfunction: CCTGA 20 patients Resting perfusion defects in all Correlated with RVEF Hornung et al. Am J Cardiol 1999

16 LV after arterial switch 31 patients years after switch Hui et al. Heart 2005

17 Tissue Doppler Normal Mustard Vogel et al. JACC 2004:43; 1006

18 INTRAMYOCARDIAL PRESSURE Ono et al. Circulation 1992: 85;

19 Methods Integrated catheter - micromanometer - conductance electrodes Thermodilution cath IVC occlusion balloon Dobutamine - 0, 5, 10 mcg/kg/min Derrick et al. Circulation 2000;102:III154-9

20 Preload reduction Systolic Contractility ESPVR (Ees) PRSW Coupling Ea/Ees Ees Diastolic relaxation compliance EDPVR, Filling rate dv/dt EDPVR

21 ESPVR Post-Mustard RV myocardial function PRSWI ESPVR p<0. 01 PRSW p<0. 01 Ea/Es p< Ea/Ees P<0.001 P<0.001 P< Dobutamine (mcg/kg/min) Dobutamine (mcg/kg/min) (mcg/kg/min) Dobutamine (mcg/kg/min) (mcg/kg/min) Derrick et al. Circulation 2000;102:III154-9

22 Post-Mustard RV myocardial function 60 Tau TAU p< EDPVR NS dv/dt index(bsa) dv/dt NS (BSA) P< Dobutamine (mcg/kg/min) P=ns Dobutamine (mcg/kg/min) P=ns Dobutamine (mcg/kg/min) Derrick et al. Circulation 2000;102:III154-9

23 Cardiac Heart Index Rate rate Cardiac index p<0. p< Dobutamine ine (mcg/kg/min) (m (m c g c /k g g /k /m g /m i n ) i n ) Stroke Stroke Cardiac volume V Index mls/m index Index 2 p<0. 05 p< Dobutamine Dobutam ine (mcg/kg/min) (m c g /k (m g /m c g i /k n ) g /m i n ) Stroke Stroke volume Volume Index mls/m Sinus p< tachycardia poorly tolerated 40 SD associated 30 with AF? P<0.01 P<0.001 P<0.05 P<0.01 P< Dobutamine Dobutamine 50 (mcg/kg/min) (mcg/kg/min) Dobutamine (mcg/kg/min) (m c g /k g /m i n )

24

25 Late RV Failure: CCTGA?

26 Late RV Failure: CCTGA Prieto et al. Circulation 1998;98:

27 Influence of RV geometry? Progressive RV dilatation Shifted interventricular septum Worsening TR

28 Late RV Failure: CCTGA Prieto et al. Circulation 1998;98:

29 Tricuspid valve replacement Van Son et al. JTCVS 1995;109:

30 Tricuspid valve replacement Hraska et al. JTCVS 2005;129:

31 Retraining Winlaw et al Circulation 2005:111;

32 Retraining Winlaw et al Circulation 2005:111;

33 Retraining CCTGA vs Atrial switch (p=0.91) Pre-existing or induced LV dysfunction (p<0.004) Age >16 (n=12) not an independent risk factor for outcome (p=0.95) Winlaw et al Circulation 2005:111;

34 PV relations during PA Band Pulmonary Artery Band applied

35 PV relations during PA Band Adaptation Failure Pulmonary Artery Band applied

36 Retraining 8 patients over aged 18 (18-56) All had LV pressures <50% systemic 1-3 bandings, 2-32 weeks between procedures 2 of 7 patients had significant TnI release 1 double switch

37 Septal shift Intraoperative PA banding

38 Retraining

39 CCTGA Destination therapy? 56 year old, unoperated CCTGA Asymptomatic until age 50 Severe TR Banded to 45%, 60%, then 85-90% systemic

40 Conclusions Little evidence for intrinsic RV systolic failure Lower tolerance of additional load? The late outcomes of CCTGA are influenced by degree of TR Poor outcomes of LV retraining may be 2 to training protocol PA band as destination therapy?

41 Retraining 8 patients over aged 18 (18-56) All had LV pressures <50% systemic 1-3 bandings, 2-32 weeks between procedures 2 of 7 patients had significant TnI release 1 double switch

42 Systemic RV: Assessment and Treatment Retraining Banded Banded

43 Retraining: 171/2 years old Before Banding

44 Retraining: After 1st Banding

45 Systemic RV: Assessment and Treatment Retraining: 4 days after 2 nd Banding

46 Systemic RV: Assessment and Treatment Retraining: 13 days after 2 nd banding

47 Systemic RV: Assessment and Treatment Retraining: Pre-double switch

48 Systemic RV: Assessment and Treatment Retraining: 11 days after double switch

49 Systemic RV: Assessment and Treatment Retraining: 3 months after double switch

50 Right Ventricular Function in CCTGA

51 Right Ventricular Function in CCTGA

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