Echocardiography in Fontan Physiology
Echocardiography in Fontan Physiology
Echocardiography in Fontan Physiology
Echocardiography in
Fontan Circulation
Jan Marek
Great Ormond Street Hospital
&
Institute of Child Health
London, United Kingdom
No disclosures
Fontan palliation
Atrio-ventricular & atrio- pulmonary
connection using conduits (valved, non-valved)
J Thorac Cardiovasc Surg. 1983 (100 pts with TA, 1968-1983)
myocardium
Absence of respiratory sucking
Pro-coagulation enviroment
Pro-arhythmogenic environment
Thrombosis, AFlu+AFib, lung and systemic embolisation,
protein loosing enteropathy, plastic bronchitis,
right heart failure, low cardiac output
Extracardiac
conduit
Fontan Palliation
CLASSICAL
< 1990
LATERAL TUNNEL
1990-2000
EXTRACARDIAC COND.
> 2000
Atrio-Pulmonary
Connection
Total
Cavo-Pulmonary
Connection
Fontan
Physiology
Fontan Physiology
Push & Pull mechanism
Elevated CVP drives blood into lungs
Systemic ventricle & Inspiration is sucking blood from lungs
Fontan Physiology
SVCp 12 mmHg
SVCp 17 mmHg
Fenestrated TCPC
Increased pulmonary vascular resistance
85
%
95
%
17
14
Hijazi, Circulation 1992; Mavroudis, Circulation 1992; Harke, JACC 1994; Kuhn, JACC 1995
Fontan Physiology
CVp 13 mmHg
LAp 5
TPG 8 mmHg
CVp 18 mmHg
LAp 12
TPG 6 mmHg
Non-LV Chamber
Fontan Circulation:
Ventricular function
Cause of ventricular
dysfunction:
Architecture of myocardium
Incoordination
Abnormal loading condition
Intrinsic myocardium abnormality
Ischaemic dysfunction
Rhythm disturbance
>60%
0.25
<40%
40-60%
0.00
0.50
0.75
1.00
Fontan circulation
RV function in HLHS
an alysis tim e
RV EF _Cat ego ry = 1
RV EF _Cat ego ry = 3
GOSH
Non-invasive Assessment of
Myocardial Function in
Non-LV Setting
Fontan Circulation
Ventricular function: good or bad?
Non-LV function
Echocardiographic challenges
Eye balling
Planimetry
(T)APSE
M-mode (AMM)
+dP/dt (systemic AVVR)
Tei index
TDI (Strain, -SR)
RT-3DE
2D/3D Strain
Non-LV function
Echocardiographic challenges
Eye balling
Planimetry
(T)APSE
M-mode (AMM)
+dP/dt (systemic AVVR)
Tei index
TDI (Strain, -SR)
RT-3DE
2D/3D Strain
[mmHg]
Time [s]
1,117 mmHg.s-1
Off ECMO
+
Adr.0.01, Mil.0.5
611 mmHg.s-1
1,085 mmHg.s-1
Spectral TDI
Lengthening
SYS
DIAST
Strain Rate
(sec-1)
Strain
(%)
IVA
(cm / sec-1)
-20
Courtesy P.Claus, Leuven
Direct TDI
Mechanical dyssynchrony in
single ventricle morphology
Atrial
channel
LA
DDD
pacemaker
Ventricular
channel
RA
OT
SV
RFW
Apex
(T)APSE (>15cm)
CW Doppler
Fontan connections
Ventricular function
AVVR quantification
Ruling out pulmonary vein
obstruction/compression
Ruling out thrombus
3D roadmap for
intervention
Late gadolinium
- ventricular and atrial scarring
Echocardiography
MRI
CT