Echocardiographic assessment of the right ventricle in paediatric pulmonary hypertension. Mark K. Friedberg, MD No disclosures
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
The RV response to increased afterload determines outcomes Pulmonary Circulation 2017; 7 361 371
The RV does poorly in PAH Courtesy Mieke Driessen, In Progress
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
Physiology of the hypertensive RV RV Free Wall IVS Anderson, Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2007 10:76 Chin, Coronary Artery Disease 2005, 16:13 18
RV response to increased afterload Dilatation Decreased output/ ejection Prolonged systole
RV dimensions Lopez, J Am Soc Echocardiogr 2010;23:465-95
RV dimensions
RV/LV ratio Jone PN,. J Am Soc Echocardiogr (2014) 27:172 8.
3-D assessment of RV volumes and EF
RV response to increased afterload Dilatation Decreased output/ ejection Prolonged systole
Subjective (Eyeball) assessment still most common method
Parameters of longitudinal function
Tricuspid annular plane systolic excursion (TAPSE) Koestenberger, JASE 2009
TAPSE correlates with survival Forfia, Am J Respir Crit Care Med, 2006 174:1034
Early Decline in TAPSE Predicts Outcome in Children With Pulmonary Hypertension IPAH (18) PH-CHD (25) Lung disease (5) Hauk, Euroecho, 2014
13y, ipah, PAH worsening, pneumonia
Tricuspid tissue Doppler velocities Lammers, J Am Soc Echocardiogr 2012;25:504
Tissue Doppler Imaging: Severe PAH
TAPSE pitfalls: Pre-tricuspid Eisenmenger Normal TAPSE Low FAC% due to apical dysfunction
Fractional area of change Mertens & Friedberg. Nat. Rev. Cardiol, 2010
RV apical remodeling Control Pulmonary stenosis Pulmonary Hypertension Driessen, Echocardiography 2017
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 [0.41-1.57] mmhg in PS and 1.09 [0.46-1.50] in ipah (p=0.004). Driessen, Physiological Reports, 2017
RV contraction under stress PAH HLHS cctga Increased circumferential/ radial thickening
Septal bowing
Inefficient RV transverse motion PS PAH RV Free Wall IVS Driessen, Physiological Reports, 2017
Conventional echo parameters in children with PAH ipah All Kassem E, Am Heart J. 2013;165:1024-31
Strain and strain rate
RV strain in pediatric ipah patients Okumura, JASE 2014; 27:1344
Systolic prolongation and MPI Tricuspid Pulmonary
The S:D Duration ratio Friedberg, JASE, 2006;19:1326
S:D ratio in PAH Alkon, Am J Cardiol 2010;106:430
13y, ipah, pneumonia, PAH worsening
Another 13 year old with PAH
Prolonged RV systole=reduced RV and LV filling
Further deterioration
12 year old severe ipah
Diastolic function Right atrial size Bustamante-Labarta, JASE 2002;15:1160 Kassem E, Am Heart J. 2013;165:1024-31
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;7:491-501; originally published online February 27, 2014; doi: 10.1161/CIRCIMAGING.113.001071 Circulation: Cardiovascular Imaging is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright 2014 American Heart Association, Inc. All rights reserved. Print ISSN: 1941-9651. Online ISSN: 1942-0080 The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circimaging.ahajournals.org/content/7/3/491 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: http://www.lww.com/reprints Subscriptions: Information about subscribing to Circulation: Cardiovascular Imaging is online at: http://circimaging.ahajournals.org//subscriptions/ Okumura, CircCV Imag 2014
Future directions
RV stroke work DiMaria, Heart 2014
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
4-D flow in a PAH patient Courtesy Siri-Anne Nayrnes
RV response to exercise Baseline 60 watts Pieles, In Progress
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).
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