Complications in CHD with a little help from our friends

Transcription

1 Complications in CHD with a little help from our friends Systemic ventricular failure how can the surgeon help? Assist device or transplantation? Michael Huebler (Zurich, CH)

2 Survivors of CHD Curative No further treatment Palliative Further treatment Medical treatment Surgical treatment Permanent improvement No permanent improvement 10-20% of patients with CHD Heart failure Heart Transplantation Heart/Lung Transplantation Mechanical Circulatory Support: Bridge to transplantation Bridge to transplantibility Permanent support

3 Potential Mechanisms of HF in CHD Multifactorial- CHD related ineffective cardiovascular system, even after repair Volume overload: Pressure overload: Ventricular failure Pulmonary hypertension Systemic arterial hypertension Coronary artery disease shunt lesions ± valvular regurgitation valvular disease ± other obstructive lesions intrinsic myocardial dysfunction Ventricular incision, large patch CHD lesions ventricular dysfunction comorbidities ie.sleep apnea CHD malperfusion issues Neurohormonal Activation Myocardial Fibrosis Remodeling Geometric and Anatomic Disadvantage Cyanosis Intractable atrial arrhythmias

4 Treatment Option:VAD Therapy Current FDA Approved VAD s for Pediatric Use Berlin EXCOR (Berlin Heart) HVAD (HeartWare) SynCardia TAH 50 cc (CardioWest) SynCardia TAH 70 cc (CardioWest) HeartMate II (Thoratec) Para-corporeal Pulsatile No limit Pericardial Continuous >1.5 m 2 Corporeal Pulsatile >1.2 m 2 Corporeal Pulsatile >1.7m 2 Preperitoneal pocket Continuous >1.5 m 2 4

5 Berlin Heart Versatility

6 Berlin Heart Versatility LVAD/RVAD/BVAD Single Ventricle VAD

7 Number of Implantations >1,700 pediatric Berlin Heart EXCOR implantations worldwide pediatric heart centers in 37 countries worldwide /03 04/05 06/07 08/09 10/11 12/13 14/15 Year Cumulative time on device: 412 years Mean time on device: 92 days (up to 3.5 years) EXCOR Pediatric Update, Berlin Heart, Berlin, Germany, Jan 2016

8 EXCOR Pediatric Update, Berlin Heart, Berlin, Germany, Jan

9 EXCOR Pediatric Update, Berlin Heart, Berlin, Germany, Jan

10 Adverse Events after BH Excor Pediatric VAD Zafar et al,

11 Freedom from First Neurological Event after BH Excor Implantation <50 days Jordan et al,

12 Quality of Life?! BERLIN HEART Patient confined to driver and hospital

13 Initial series from Berlin

14 HeartWare Ventricular Assist System (HVAD ) HVAD miniaturized implantable blood pump Pericardial placement no pump pocket Provides up to 10 L/min of flow Centrifugal design, continuous flow Hybrid magnetic / hydrodynamic impeller suspension free from mechanical contact points Thin (4.2 mm), flexible driveline with fatigue resistant cables Zero cable fractures

15 LV-Apical Implantation Anastomose Outflow Graft to Aorta Aorta sideclamped Oblique anastomose end-toside outflow graft to aorta Outflow graft

16 Patient 1 Patient 2 Patient 3 Patient 4 Patient 5 Patient 6 Patient Age (years) Weight (kg) BSA (m2) Gender m m m f m m f f Diagnosis DCM HLHS/ TCPC 7 Patient DCM DCM DCM DCM DCM DCM LVEDD (mm) EF (%) inotrops 2 E/M D/M E/M D/M D/M E/M E/M E/M Sec. organ dysfunction HVAD Experience in Pediatric Patients renal renal/ hepatic since 2009 hepatic gastrointestinal renal Renal/ heaptic hepatic 8 renal DCM = dilatative cardiomyopathy HLHS = hypoplastic left heart syndrom TCPC = totalo cavopulmonary connection 2 E = Epinephrine M = Milrinone D = Dobutamine

17 HVAD Experience in Pediatric Patients Since 2009 Pat. 1 Pat. 2 Pat. 3 Pat. 4 Pat. 5 Pat. 6 Pat. 7 Pat. 8 Age (years) Weight (kg) BSA (m2) Extubation (post-op day) Pump speed (rpm, median) Pump flow (L/min, median) VAD support (days) Complications Pericardial tamponade Outcome htx htx htx htx htx htx htx htx HIT

18 Minimal Patient Size? Smallest child intital series Personal communication 6years,17kg, BSA 0.7 2years, 10kg Outcome:Successfully transplanted

19 Minimal Patient Size? CT Scan 6years, 17kg

20 Minimal Patient Size? 2.5cm Explanted heart after HTX

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22 HVAD Implantation in Failing FONTAN: Hypoplastic left heart syndrom S/P Norwood I and II Total cavopulmonary connection (extracardiac conduit) Short term support: Htx after 24h

23 HVAD Implantation systemic right ventricle (Transposition complex) Right ventricular diaphragmatic site Transposition of the great arteries Congenitally corrected transposition of the great arteries Joyce at al, J Heart Lung Transplant 2010;

24 VAD Therapy in GUCH ACHD Patients CASE #1 A 24-year-old male with D-TGA s/p months Past medical history: supraventricular arrhythmias ablation and implantation of a pacemaker (atrial lead) in 2003 tricuspid valve incompetence, stenosis of the superior caval vein Biventricular dysfunction NYHA III

25 HVAD Therapy in ACHD Patients CASE #1 - Surgery CPB connection RA - ascending aorta Heartware VAD (HVAD) inflow cannula insertion at free wall of anatomically right ventricle Outflow graft anastomosis with ascending aorta

26 HVAD Implantation in TGA Patients Technical Aspects Implantation Site

27 HVAD Implantation in TGA Patients Technical Aspects Implantation Site

28 HVAD Implantation in TGA Patients Technical Aspects Implantation Site

29 OUTCOME Case #1: at home, support ongoing after over 12 months, NYHA 2 Catheterization 12 months later

30 HVAD as BiVAD, 10 years, 26 kg, BSA 0.97 m2 LVAD 2300 RPMs, 3.2 L/min 2.7 Watt RVAD 2080 RPMs, 3.3 L/min 2.1 Watt Anticoagulation Heparin (i.v.) target anti-xa IU/ml

31 Atrial Implantation of BiHVAD LV-Aorta, RA-PA Outflow graft to PA Inflow cannula in RA

32 Discharge home rate on device as high as 60% Favourable outcome in this cohort (0% mortality) Mean readmission 2.5/patient Main cause for readmission Driveline infection 25% Coagulation issues 20% VAD alarm 20% Others 35%

33 Quality of Life! Smallest HVAD Patient in initial series

34 HeartWare Worldwide Pediatric Experience As of December, 2015 Over 300 implants worldwide under 18 years of age Over 80 implants in patients under 12 years of age Smallest patients published: 6 years - BSA of 0.7 and 17 kg (Miera et al, 2011) 3 years - BSA of 0.5 and 13.5kg (Peng et al, 2016) Implants in smaller patients have been performed - 10 BVAD Implantations Courtesy of HeartWare Inc

35 implants HVAD in Pediatric Patients World Experience (incomplete - courtesy HeartWare) Age range: BSA: 2 years 17 years 0.5 sqm 2.5 sqm years

36 Experiences Heartware Conway et al. ISHLT patients, 28 sites (16 North America, 8 Europe, 4 others) from 11 countries Variable N (%) Age Gender (male) 104 (61) 13 ( ) years Weight at implant (kg) 40 (26-58) Diagnosis CHD 28 (16%) CM 139 (81%) Dil CM 110 (79%) Previous sternotomy 40 (23%) 36

37 Experiences Heartware Conway et al. ISHLT 2016 Support Times Discharged home 96 (56%) Duration of Hospital Stay 42d (28-73) Duration of VAD support 111d (45-243) 1,2 1 North American vs. Europe/others: 131 (48-273) vs. 89 (42-203), p=0.19 Time Post Implant Survival% 6 months 86% Year 1 84% Year 2 84% Year 3 84% Year 4 84% 2 Longest: 1642 d (4.5yrs) 37

38 Pediatric Patients supported by Pulsatile vs Continuous-flow ventricular assist devices Pulsatile VAD Continuous-flow VAD E.Blume et al,

39 Pediatric and Adult Patients supported by Continuous-flow ventricular assist devices J. Rossano et al,

40 Cerebrovascular Complications EUROMACS Neurologic events Kaplan Maier analyses for neurologic events (embolic and bleeding) in children provided with intra-corporeal ventricular assist device 10% cerebro-vascular complications Schweiger M, Hübler M, Mohacsi P, et al. ISHLT, 2016

41 SynCardia Total Artificial Heart 7

42 Indications TAH Patients s/p htx with acute or chronic rejection Patients s/p htx with vasculopathy Difficult/impossible placement of apical cannula in severe restrictive or hypertrophic CM If multiple/complex cardiac repair are needed for conventional VAD implantation Intractable arrhythmias Intractable/severe/recurrent endocarditis Thrombembolism of cardiac origin Pre- and postpulmonary failing fontan patients 42

43 Implantation aspects Smaller device 50cc for minimal BSA 0.9 sqm Virtual fit mandatory for secure implantation Cave: compression of pulmonary veins (esp.leftsideded) Cave: bleeding complications Still high mortality and morbidity 43

44 The Worldwide Experience of SynCardia Total Artificial Heart in Patients with Congenital Heart Disease (Morales et al. ISHLT 2016) 44

45 Conclusions (1) Berlin Heart Excor Pediatric VAD even 26 years after its initial use: - first choice in children <0.5sqm BSA - best alternative for BVAD use - concern about adverse neurological outcome (2) Adult contiuous flow VAD (Heartware HVAD, Thoratec Heartmate II): - first choice in children >0.5sqm BSA - results in children equal results in adults - improved quality of life and discharge home on device feasible (3) Syncardia TAH 50cc: - clinical trial underway for children >0.9sqm BSA - advantages in specific indications - virtual fit mandatory - concern about adverse neurological outcome

46 BUT

47 MCS is no long-term solution for heart failure so far

48 Actuarial freedom from any major event

49 Survival (%) Pediatric Heart Transplants Kaplan-Meier Survival (Transplants: January 1982 June 2013) 100 <1 Year (N = 2,777) 1-5 Years (N = 2,556) 6-10 Years (N = 1,637) Years (N = 4,402) Overall (N = 11,384) vs : p = vs : p = No other pair-wise comparisons were significant at p < Median survival (years): <1=20.6; 1-5=17.2; 6-10=13.9; 11-17= Years 2015 JHLT Oct; 34(10): 33(10):

50 Neonate,, 3300g, 40GW Fetal echocardiography 32GW no pathologies After Birth: Huge left ventricular tumor LVEF ductal depending systemic perfusion: Retrograde coronary perfusion severe mitral regurgitation ASD bicuspide aortic valve Ca. 90% stenosis left main bronchus (external compression) age 120min On dobutamin

51 Chest X-Ray

52 day 2 LV- Tumor LVOT- Obstruction

53 day 2 Compression left main bronchus Compression left main bronchus

54 @ day 6:Surgery Resection/Debulking of LV-tumor Mitral valve resection, ASD closure LVAD Implantation: Berlin Heart Excor Pediatric 10ml Pump chamber 6mm Apex cannula 6mm Graft cannula

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56 Post-op X-Ray

57 @ day24:heart transplantation Size missmatch >300%

58 @ 4 weeks after heart transplantation

59 Primary HTX High risk CHD lesions pulmonary atresia with intact ventricular septum/rv dependent coronary circulation Heterotaxy syndrome with single-ventricle physiology High risk HLHS/C - Norwood candidates (1) significant ventricular dysfunction (2) tricuspid regurgitation (3) pulmonary valve dysfunktion (4) left ventricle coronary fistulae (5) restrictive interatrial septum 59

60 Conclusions No lower age limits for heart transplantation for proper indication Even patient size miss matches > 300% can be successfully transplanted Primary heart transplantation for intractable or high risk congenital heart disease procedures has to be evaluated in individual cases HTX is still the treatment of choice for treatment of heart failure in the context of CHD

61 Thank you for your attention 61