Download Imaging Post-Fontan: Why I Do It

Imaging Post-Fontan:
Why I Do It
Cynthia K. Rigsby, MD
Department of Medical Imaging
Post-Fontan Imaging
!
Fontan circuit palliative
!   Good result for patients with ideal hemodynamic profile
!   Most patients are NYHA class I or II
!   Can be early and late morbidity and mortality
!   Complications include exercise intolerance, desaturation, ventricular
dysfunction, rhythm disturbances, hepatomegaly, lymphatic
dysfunction, systemic venous thrombi/pulmonary embolism, ascites,
and peripheral edema
!   Absence of symptoms does not mean ideal hemodynamic profile
!   Potential complications should be pursued and treated prior to
becoming clinically apparent
Gewillig. Heart 2005;91:839–846.
Post-Fontan Imaging
!   Cross-sectional imaging complimentary to echocardiography
!   Echo windows limited as patients grow
! Fontan pathway can be completely delineated
!   Driving force for Fontan cardiac output is pulmonary vascular
resistance
!   Non-obstructive flow to/from lungs essential
!   Functional ventricular, valvular, and flow assessment
!   Lung, liver evaluation
Brown DW. Progress in Pediatric Cardiology 28 (2010) 45–58.
Fontan Anatomy
!   Determine Fontan type
!   RA to PA or variants
!   Generally older patients
!   Stasis secondary to swirling of flow in
dilated RA
!   Poor flow to lungs
!   Arrhythmia
!   Coronary sinus dilation; coronary stasis
!   Lateral tunnel prosthetic baffle and
portion of right atrial wall
!   Can grow with patient
!   Can be performed under 3 years
!   RA tissue exposed to high pressure; RA
sutures; can lead to arrhythmia
!   Extracardiac baffle
!
!
!
!
 
 
 
 
PTFE graft
Does not grow
Older than 3 years
Adequate size for adult IVC blood flow
Fontan Anatomy
!   Determine Fontan type
!   RA to PA or variants
!   Generally older patients
!   Stasis secondary to swirling of flow in
dilated RA
!   Poor flow to lungs
!   Arrhythmia
!   Coronary sinus dilation; coronary stasis
!   Lateral tunnel prosthetic baffle and
portion of right atrial wall
!   Can grow with patient
!   Can be performed under 3 years
!   RA tissue exposed to high pressure; RA
sutures; can lead to arrhythmia
!   Extracardiac baffle; PTFE graft
!   Does not grow
!   Older than 3 years
!   Adequate size for adult IVC blood flow
Fontan Anatomy
!   Determine Fontan type
!   RA to PA or variants
!   Generally older patients
!   Stasis secondary to swirling of flow in
dilated RA
!   Poor flow to lungs
!   Arrhythmia
!   Coronary sinus dilation; coronary stasis
!   Lateral tunnel = prosthetic baffle and
portion of right atrial wall
!   Can grow with patient
!   Can be performed under 3 years
!   RA tissue exposed to high pressure; RA
sutures; can lead to arrhythmia
!   Extracardiac baffle
!
!
!
!
 
 
 
 
PTFE graft
Does not grow
Older than 3 years
Adequate size for adult IVC blood flow
Fontan Anatomy
!   Fenestration
!   Allow for decompression of initial
higher pressures in venae cavae
!   Prevent rapid lung volume overload
!   Increase systemic ventricle preload/
increase cardiac output
!   Can cause desaturation
!   Can be closed percutaneously
!
Venovenous collaterals
!   Decompress from higher pressure
venae cavae to lower pressure
pulmonary or systemic veins that drain
to the common atrium
!   Systemic desaturation
!   Plan for cath treatment
!   Thrombus
!   Massive PE most common cause of
out of hospital death
Gewillig M. Heart 2005; 91(6):839–846.
Fontan Anatomy
!   Fenestration
!   Allow for decompression of initial
higher pressures in venae cavae
!   Prevent rapid lung volume overload
!   Increase systemic ventricle preload/
increase cardiac output
!   Can cause desaturation
!   Can be closed percutaneously
!
Venovenous collaterals
!   Decompress from higher pressure
venae cavae to lower pressure
pulmonary or systemic veins that drain
to the common atrium
!   Systemic desaturation
!   Plan for cath treatment
!   Thrombus
!   Massive PE most common cause of
out of hospital death
Gewillig M. Heart 2005; 91(6):839–846.
Fontan Anatomy
!   Fenestration
!   Allow for decompression of initial
higher pressures in venae cavae
!   Prevent rapid lung volume overload
!   Increase systemic ventricle preload/
increase cardiac output
!   Can cause desaturation
!   Can be closed percutaneously
!
Venovenous collaterals
!   Decompress from higher pressure
venae cavae to lower pressure
pulmonary or systemic veins that drain
to the common atrium
!   Systemic desaturation
!   Plan for cath treatment
!   Thrombus
!   Massive PE most common cause of
out of hospital death
Gewillig M. Heart 2005; 91(6):839–846.
Pulmonary Arteries
!   Stenosis
!   Anastomosis/prior shunt
!   Compression
!   Differential pulmonary blood
flow
!   IVC/SVC blood flow
distribution
!   Pulmonary arteriovenous
malformations
!   Absence of pulsatile flow
!   Absence of “hepatic factor”
!   Desaturation
Pulmonary Arteries
!   Stenosis
!   Anastomosis/prior shunt
!   Differential pulmonary blood
flow
!   IVC/SVC blood flow
distribution
!   Pulmonary arteriovenous
malformations
!   Absence of pulsatile flow
!   Absence of “hepatic factor”
!   Desaturation
Pulmonary Arteries
!   Stenosis
!   Anastomosis/prior shunt
!   Differential pulmonary blood
flow
!   IVC/SVC blood flow
distribution
!   Pulmonary arteriovenous
malformations
!   Absence of pulsatile flow
!   Absence of “hepatic factor”
!   Desaturation
IVC to LPA 64%; IVC to RPA 36%
SVC to LPA 0%; SVC to RPA 100%
Pulmonary Veins
!   Stenosis
Atrioventricular Valves
!   Left, right, common
!   Stenosis
!   Regurgitation
!   Valvular regurgitation can
be estimated using
ventricular volumetrics
and AV valve/great artery
flow analysis
Single Ventricle Function/Scar
!   Single right or left ventricle
!   Morphologic right ventricle
and systemic afterload
!   Multicenter study showed
abnormal ventricular function in
27% patient
!   Associated with older age/
systemic RV morphology
!   Association between myocardial
fibrosis/LGE and higher
ventricular volumes, global and
regional FSV function, and nonsustained VT
Anderson PA. JACC. 2008 July 8;52(2):85–98.
Rathod RH. JACC. 2009;53:A3.
Aortic/Neoaortic Valve
!   Stenosis/Regurgitation
!   Volume load from
regurgitation can be
assessed with phase
contrast
Aorta
!
Aortopulmonary collaterals
!   Volume load systemic
ventricle
!   Detrimental volume/
pressure load on
pulmonary circulation
!   Collateral flow as a
percentage of aortic flow
26±9%
!   Significant contribution of
collateral flow to
pulmonary flow
!   No correlation with
hemodynamic findings at
cath
Grosse-Wortmann L.Circ Cardiovasc Imaging 2009. May;2:219–25.
Liver
!   Venous hypertension
!   Increased sinusoidal
pressure
!   Cirrhosis
!   Coagulation abnormalities
!   Risk for thromboembolism
!   Portal hypertension
!   Risk for hepatocellular
carcinoma
Lymphatic System
!   Early lymphedema/
pulmonary edema and
chylothorax
!   Elevated SVC pressure
!   Protein losing enteropathy
!   Intestinal lymphangiectasia
! Chyle leak into gut/abdomen
!   Ascites, immunodeficiency,
coagulopathy
!   Poor prognosis
!   Plastic bronchitis
!   Secretion of mucoid material
into airways
!   Airway obstruction,
respiratory distress
Fredenberg T. RadioGraphics 2011; 31:453–463.
Lymphatic System
!   Early lymphedema/pulmonary
edema and chylothorax
!   Elevated SVC pressure
!   Protein losing enteropathy
!   Intestinal lymphangiectasia
! Chyle leak into gut/abdomen
!   Ascites, immunodeficiency,
coagulopathy
!   Poor prognosis
!   Plastic bronchitis
!   Secretion of mucoid material
into airways
!   Airway obstruction, respiratory
distress
Fredenberg T. RadioGraphics 2011; 31:453–463.