Herzinsuffizienz bei EMAH Bedeutung und spezifische Behandlungsschwierigkeiten

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1 Das Bild kann nicht angezeigt werden. Dieser Computer verfügt möglicherweise über zu wenig Arbeitsspeicher, um das Bild zu öffnen, oder das Bild ist beschädigt. Starten Sie den Computer neu, und öffnen Sie dann erneut die Datei. Wenn weiterhin das rote x angezeigt wird, müssen Sie das Bild möglicherweise löschen und dann erneut einfügen. Herzinsuffizienz bei EMAH Bedeutung und spezifische Behandlungsschwierigkeiten Gerhard-Paul Diller EMAH-Zentrum / Kardiologie Zentrum für angeborene und erworbene Herzfehler Medizinische Klinik und Poliklinik C Universitätsklinikum Münster

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3 Chronisch herzkranke Patienten Mehrzahl EMAH haben operative oder interventionelle Behandlungen erhalten. Vollständige Heilung und normale Lebenserwartung ist i.d.r nicht zu erreichen. Großteil dieser Patienten ist chronisch herzkrank Anatomische und funktionelle Rest- und Folgezustände Verbunden mit Einschränkungen der Lebensqualität Leistungs- und Arbeitsfähigkeit Lebenserwartung

4 Eingeschränkte Lebenserwartung TOF Syst RV UVH Nieminen HP, Circulation 2001

5 Nieminen HP et al JACC 2007

6 A state in which the heart fails to maintain an adequate circulation for the needs of the body despite a satisfactory filling pressure Paul Wood, 1950 A clinical syndrome caused by an abnormality of the heart and recognised by a characteristic pattern of haemodynamic, renal, neural and hormonal responses Poole-Wilson, 1985 I. Symptoms of heart failure (at rest or during exercise) and II. objective evidence of cardiac dysfunction (rest) and (in cases where the diagnosis is in doubt) III. response to treatment directed towards heart failure ESC Task Force 2005

7 Zugrundeliegende Herzerkrankung Eingeschrankte körperliche Leistungsfähigkeit Herzinsuffizienzsymptome Neurohormonelle- / Immunaktivierung Bolger et al. Eur Heart J. 2003

8 Diller, G.-P. et al. AHA 2007

9 22% dtga, 32 % cctga, 40% Fontan Herzinsuff.symptome

10 Kempny A. EHJ in press.

11 Neurohormonelle / Immunaktivierung 47 patients with chronic HF due to ischaemic or DCM vs 83 ACHD patients Bolger, A. P. et al. Circulation 2002 Bolger, A. P, EHJ 2003

12 Neurohormonelle / Immunaktivierung Bolger, A. P. et al. Circulation 2002 Sharma et al. Am J Card 2003

13 Neurohormonelle Aktivierung / Autonome Dysfunktion Fontan pt. Younger patients ANP/BNP in the APC vs TCPC group CANA and lower HR variability related to the number of previous surgeries Ohuchi et al Circulation 2002

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15 Hunt et al. JACC 2005

16 Jessup M, NEJM 2003

17 RV LV? Ellipsoid Anderson, Becker 1980

18 1. Medikamentöse Therapie

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20 randomisierte Studie n=18; Fontan pt. 10 Wochen Keine Änderung pvo 2 oder CI Kouatli AA, Circulation 1997

21 n=63, 10 ACE-Hemmer Therapie

22 n=14, 6 Monate AJC 2001

23 n=9, dtga, Enalapril für 12 Monate Ped Cardiol 2002

24 n=7, cross over, EF und Belastungsfähigkeit AJC 2001

25 Multicenter, rand., double-blind, cross-over study 29 pts. (21 dtga, 8 cctga) 15 wk. Losartan 2x50 mg/die

26 Abstract 2031: ACE Inhibitors for Potential PRevention of the Deleterious Effects of Pulmonary Regurgitation In Adults with TEtralogy of Fallot Repair - The APPROPRIATE Study - A Randomised, Double-Blinded, Placebo-Controlled Trial in Adults with Congenital Heart Disease Sonya V Babu-Narayan; Anselm Uebing; Periklis A Davlouros; Michael Kemp; Simon Davidson; Omer Goktekin; Stephanie Bayne; Philip J Kilner; Wei Li; Michael A Gatzoulis Royal Brompton Hosp, London, United Kingdom Background: Angiotensin Converting Enzyme (ACE) inhibitors have been regarded as useful in aortic regurgitation, but their effectiveness with volume loading of the right ventricle (RV) due to pulmonary regurgitation (PR), a cause of late morbidity and mortality after repair of tetralogy of Fallot (ToF), remains unknown. We therefore undertook what we believe to be the first randomised, double-blinded, placebo-controlled drug trial in adults with congenital heart disease, on the effect of ACE inhibition in patients with repaired ToF and PR. Methods and Results: Sixty-Four adults with repaired TOF and at least moderate PR were randomly assigned to six months ramipril 10 mg or six months placebo in a double-blinded fashion. New York Heart Association (NYHA) class, maximal VO 2, pulmonary regurgitant fraction (PRF), RV and left ventricular (LV) end-systolic volume index (ESVi) and ejection fraction (EF) by cardiovascular magnetic resonance, and RV and LV long axis function on M-Mode echocardiography, were similar in both groups at baseline. Treated patients demonstrated significant improvement in RV and LV amplitude of long axis shortening (16.2 ± 4.8 versus 17.8 ± 4.8; P = and 14.6 ± 4.3 versus 16.2 ± 3.6 mm; P = 0.008), RV and LV stroke volume index ( 74.1 ± 18.3 versus 78.9 ± 21.3; P = 0.06, 45.8 ± 7.3 versus 9.8 ± 9.7 mls/m 2 ; P = 0.02) and a trend to improvement in global RVEF (53.5 ± 8.3 versus 55.3 ± 7.0 %; P = 0.08). No significant difference was demonstrated in NYHA class (1.19 ± 0.4 versus 1.19 ± 0.4; P = 1.0), VO 2 max (25.7 ± 5.9 versus 25.3 ± 7.0 ml/min/kg; P = 0.58), PRF (39.2 ± 10.4 versus 37.8 ± 10.9 %; P = 0.17), RVESVi or LVESVi (66.6 ± 25.3 versus 66.9 ± 30.3 ml/m 2 ; P =0.9, 25.1 ± 9.2 versus 24.9 ± 9.0 ml/m 2 ; P = 0.9). Similar increases in atrial and brain natriuretic peptide (ANP;BNP) levels were seen in both groups between baseline and follow up (ANP: +4.2 ± 5.4 placebo versus +2.3 ± 4.6 treatment; P = 0.15 pmol/l, BNP: +3.7 ± 5.8 placebo versus +7.6 ± 8.8 treatment pmol/l; P = 0.25). Conclusions: Six months of ramipril treatment did not reduce pulmonary regurgitant fraction or decrease ventricular size. Increases in RV and LV long axis function, stroke volumes and RV ejection fraction are potentially beneficial but the changes were modest and their clinical significance remains open.

27 NYHA, RVED-area retrospektiv, 60 pt. (31 β-blocker) Doughan A, AJC 2007

28 8 pt. SRV 12 Monate Carvedilol Zieldosis 25 mg/die (erreicht 62% d. Pat.) RVEDV, RVESV & RVEF Giardini A, IJC 2006

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30 Figure 1. Heart rate and peak oxygen consumption (peak VO2) with pre-existing pacemaker (PM) settings and with after active reprogramming. One patient (marked with an asterisk) developed atrial tachycardia at the test with pre-existing pacemaker settings.

31 ECG Phono FT HR 68 bpm t-ivt 17.1 s/min t-ft 24.8 s/min VTI 10.7 cm HR 110 bpm t-ivt 27.0 s/min t-ft 15.6 s/min VTI 5.3 cm HR 140 bpm t-ivt 28.0 s/min t-ft 13.9 s/min VTI 4.2 cm

32 Figure 3. Comparison between systemic (right) ventricular total isovolumic time (t-ivt), total filling time (t-ft) and aortic velocity time integral (VTI) at 3 different heart rates (HR). Non-parametric Wilcoxon tests (paired samples) were used to compare data.

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35 Dubin A, Circulation 2003

36 Uebing A, Circulation 2007

37 A B B C TV RV LA OF LA RV ICV A) Right atrial view of heart specimen showing location and size of coronary sinus (arrow). B) The morphologically right ventricle (RV) viewed from the left shows a good-sized vein (black arrows) coursing obliquely along the lateral wall to connect with the coronary sinus (white arrow). C) A large vein (arrows) with tributaries along the lateral wall of the morpholigically right ventricle. LA= left atrium; ICV= inferior caval vein; OF= oval fossa

38 96.4% Bottega et al, Heart Rhythm 2009;6: Uemura et al, Eur J Cardiothorac Surg 1996;10:

39 Anatomical feasibility of CV lead placement cctga

40 Therapy: - Diuretics (Furosemide, Spironolactone) with only little effect - CRT-D upgrade

41 Outcome Pre CRT-D 3 months after upgrade to CRT-D

42 Outcome Pre CRT-D 3 months after upgrade to CRT-D TAPSE Peak long. 2D strain

43 Case 1 10 cctga pts., mean age 38 (IQR 34-49) 10 NYHA FC IV III II I 0 pre post Work in progress RBH / Data courtes

44 3 pt. SRV (2 cctga, 1dTGA) 3-6 Monate CRT - LVF (subpulm.) trotz initialer haemodyn. Verbesserung PACE 2008

45 Hämodynamik

46 Symptome Schilddrüse, Anämie, Pulmonale Ursache, PHT Lebensstil Extrakardiale Ursachen Hämodynamische Problem Rhythmologisches Problem Chirugie / Intervention Dyssynchronie / Plötzl. Herztod EPU? Schwache Evidenz Medikamentöse Therapie CRT/CRT-D Transplantation Assist devices Palliativmedizin

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49 Zusammenfassung Herzinsuffizienz häufig bei EMAH Patienten Erheblicher Morbidität und Mortalität assoziiert Extrapolation aus Herzinsuffizienzstudien auf EMAH Patienten nur eingeschränkt möglich Möglichkeiten hämodynamische Optimierung (chirurgisch / interventionell) ausschöpfen Multidisziplinäre Versorgung EMAH/Rhythmusexperten/ THG- Chirurgie erforderlich

50 Danke

51 Danke