Echocardiographic assessment of the right ventricle in paediatric pulmonary hypertension.

Transcription

1 Echocardiographic assessment of the right ventricle in paediatric pulmonary hypertension. Mark K. Friedberg, MD No disclosures

2 Outline RV response to increased afterload Echo assessment of RV function in PAH 2-D Annular motion: M-mode, TDI Myocardial function Ventricular-vascular coupling Both systole and diastole are important Both the RV and LV are important

3 The RV response to increased afterload determines outcomes Pulmonary Circulation 2017;

4 The RV does poorly in PAH Courtesy Mieke Driessen, In Progress

5 2 layers Superficial layer Circumferential Deep layer: longitudinal RV function Longitudinal shortening Inward (bellows) free wall motion. inlet to outlet propulsion RV Free Wall IVS

6 Physiology of the hypertensive RV RV Free Wall IVS Anderson, Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann :76 Chin, Coronary Artery Disease 2005, 16:13 18

7 RV response to increased afterload Dilatation Decreased output/ ejection Prolonged systole

8 RV dimensions Lopez, J Am Soc Echocardiogr 2010;23:465-95

9 RV dimensions

10 RV/LV ratio Jone PN,. J Am Soc Echocardiogr (2014) 27:172 8.

11 3-D assessment of RV volumes and EF

12 RV response to increased afterload Dilatation Decreased output/ ejection Prolonged systole

13 Subjective (Eyeball) assessment still most common method

14 Parameters of longitudinal function

15 Tricuspid annular plane systolic excursion (TAPSE) Koestenberger, JASE 2009

16 TAPSE correlates with survival Forfia, Am J Respir Crit Care Med, :1034

17 Early Decline in TAPSE Predicts Outcome in Children With Pulmonary Hypertension IPAH (18) PH-CHD (25) Lung disease (5) Hauk, Euroecho, 2014

18 13y, ipah, PAH worsening, pneumonia

19 Tricuspid tissue Doppler velocities Lammers, J Am Soc Echocardiogr 2012;25:504

20 Tissue Doppler Imaging: Severe PAH

21 TAPSE pitfalls: Pre-tricuspid Eisenmenger Normal TAPSE Low FAC% due to apical dysfunction

22 Fractional area of change Mertens & Friedberg. Nat. Rev. Cardiol, 2010

23 RV apical remodeling Control Pulmonary stenosis Pulmonary Hypertension Driessen, Echocardiography 2017

24 PS patients do better than PAH 16 PS pts (10.3±4.7 yrs), 18 ipah pts (10.8±5.6 yr) and 18 controls (11.2 ± 5.0 yrs). RV: LV pressure ratio: 0.71 [ ] mmhg in PS and 1.09 [ ] in ipah (p=0.004). Driessen, Physiological Reports, 2017

25 RV contraction under stress PAH HLHS cctga Increased circumferential/ radial thickening

26 Septal bowing

27 Inefficient RV transverse motion PS PAH RV Free Wall IVS Driessen, Physiological Reports, 2017

28 Conventional echo parameters in children with PAH ipah All Kassem E, Am Heart J. 2013;165:

29 Strain and strain rate

30 RV strain in pediatric ipah patients Okumura, JASE 2014; 27:1344

31 Systolic prolongation and MPI Tricuspid Pulmonary

32 The S:D Duration ratio Friedberg, JASE, 2006;19:1326

33 S:D ratio in PAH Alkon, Am J Cardiol 2010;106:430

34 13y, ipah, pneumonia, PAH worsening

35 Another 13 year old with PAH

36 Prolonged RV systole=reduced RV and LV filling

37 Further deterioration

38 12 year old severe ipah

39 Diastolic function Right atrial size Bustamante-Labarta, JASE 2002;15:1160 Kassem E, Am Heart J. 2013;165:

40 Right Ventricular Diastolic Performance in Children With Pulmonary Arterial Hypertension Associated With Congenital Heart Disease: Correlation of Echocardiographic Parameters With Invasive Reference Standards by High-Fidelity Micromanometer Catheter Kenichi Okumura, Cameron Slorach, Dariusz Mroczek, Andreea Dragulescu, Luc Mertens, Andrew N. Redington and Mark K. Friedberg Circ Cardiovasc Imaging. 2014;7: ; originally published online February 27, 2014; doi: /CIRCIMAGING Circulation: Cardiovascular Imaging is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX Copyright 2014 American Heart Association, Inc. All rights reserved. Print ISSN: Online ISSN: The online version of this article, along with updated information and services, is located on the World Wide Web at: Permissions: Requests for permissions to reproduce figures, tables, or portions of articles originally published in Circulation: Cardiovascular Imaging can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office. Once the online version of the published article for which permission is being requested is located, click Request Permissions in the middle column of the Web page under Services. Further information about this process is available in the Permissions and Rights Question and Answer document. Reprints: Information about reprints can be found online at: Subscriptions: Information about subscribing to Circulation: Cardiovascular Imaging is online at: Okumura, CircCV Imag 2014

41 Future directions

42 RV stroke work DiMaria, Heart 2014

43 Function Low High Ventricular-vascular matching Low Group 1 Normal Group 2 Exercise Resistance High Group 4 Uncompensated PH Group 3 Compensated PH DiMaria, ASE, 2016

44 4-D flow in a PAH patient Courtesy Siri-Anne Nayrnes

45 RV response to exercise Baseline 60 watts Pieles, In Progress

46 Summary Assessment of RV function is important to assess for RV decompensation. It is important to qualitatively evaluate RV function as well as use quantitative measures Newer methods can be combined with conventional ones for more comprehensive evaluation. Further validation of newer methods is needed (especially in terms of increased sensitivity).

47 Thank you