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

Courtesy Mieke Driessen, In Progress

The RV does poorly in PAH

RV function • 2 layers

– Superficial layer

• Circumferential

– Deep layer:

• longitudinal

• Longitudinal shortening

• Inward (bellows) free wall

motion.

• inlet to outlet propulsion

RV Free

Wall

IVS

Physiology of the hypertensive RV

Chin, Coronary Artery Disease

2005, 16:13–18

Anderson, Semin Thorac Cardiovasc Surg

Pediatr Card Surg Ann 2007 10:76

RV Free

Wall

IVS

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

Forfia, Am J Respir Crit Care Med, 2006 174:1034

TAPSE correlates with survival

Early Decline in TAPSE Predicts Outcome in Children With Pulmonary Hypertension

0 5 1 0 1 5

0

5 0

1 0 0

S u rv iv a l B y T A P S E a t D ia g n o s is

Y e a rs

Pe

rc

en

t s

urv

iva

l

Z -s c o re > -4

Z -s c o re 4

0 5 1 0 1 5

0

5 0

1 0 0

S u rv iv a l B y T A P S E a t D ia g n o s is

Y e a rs

Pe

rc

en

t s

urv

iva

l

Z -s c o re > -4

Z -s c o re 4

Hauk, Euroecho, 2014

IPAH (18)

PH-CHD (25)

Lung disease (5)

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

Increased circumferential/ radial thickening

ccTGA HLHS PAH

Septal bowing

Inefficient RV transverse motion PS PAH

RV Free

Wall

IVS

Driessen, Physiological Reports, 2017

Conventional echo parameters in children with PAH

Kassem E, Am Heart J. 2013;165:1024-31

iPAH All

Strain and strain rate

RV strain in pediatric iPAH patients

Okumura, JASE 2014; 27:1344

Tricuspid Pulmonary

Systolic prolongation and MPI

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

Right atrial size

Bustamante-Labarta, JASE 2002;15:1160

Diastolic function

Kassem E, Am Heart J. 2013;165:1024-31

Okumura, CircCV Imag 2014

Andrew N. Redington and Mark K. FriedbergKenichi Okumura, Cameron Slorach, Dariusz Mroczek, Andreea Dragulescu, Luc Mertens,

Micromanometer CatheterEchocardiographic Parameters With Invasive Reference Standards by High-Fidelity

Hypertension Associated With Congenital Heart Disease: Correlation of Right Ventricular Diastolic Performance in Children With Pulmonary Arterial

Print ISSN: 1941-9651. Online ISSN: 1942-0080 Copyright © 2014 American Heart Association, Inc. All rights reserved.

Dallas, TX 75231is published by the American Heart Association, 7272 Greenville Avenue,Circulation: Cardiovascular Imaging

doi: 10.1161/CIRCIMAGING.113.0010712014;7:491-501; originally published online February 27, 2014;Circ Cardiovasc Imaging.

http://circimaging.ahajournals.org/content/7/3/491

World Wide Web at: The online version of this article, along with updated information and services, is located on the

http://circimaging.ahajournals.org//subscriptions/

is online at: Circulation: Cardiovascular Imaging Information about subscribing to Subscriptions:

http://www.lww.com/reprints Information about reprints can be found online at: Reprints:

document. Permissions and Rights Question and Answer information about this process is available in the

requested is located, click Request Permissions in the middle column of the Web page under Services. FurtherCenter, not the Editorial Office. Once the online version of the published article for which permission is being

can be obtained via RightsLink, a service of the Copyright ClearanceCirculation: Cardiovascular Imagingin Requests for permissions to reproduce figures, tables, or portions of articles originally publishedPermissions:

by guest on May 20, 2014http://circimaging.ahajournals.org/Downloaded from by guest on May 20, 2014http://circimaging.ahajournals.org/Downloaded from

Future directions

RV stroke work

DiMaria, Heart 2014

Resistance

Low High

Fun

ctio

n

Low Group 1 Normal

Group 4 Uncompensated PH

High Group 2 Exercise

Group 3 Compensated PH

Ventricular-vascular matching

DiMaria, ASE, 2016

Courtesy Siri-Anne Nayrnes

4-D flow in a PAH patient

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).

Thank you