Transcript Surgical Considerations for Patients with Heterotaxy Syndrome

PCICS
December 10, 2014
Surgical Considerations for Patients with
Heterotaxy Syndrome
Jeffrey P. Jacobs, M.D., FACS, FACC, FCCP
Professor of Surgery, Johns Hopkins University
Director, Andrews/Daicoff Cardiovascular Program,
Surgical Director of Heart Transplantation and Extracorporeal Life Support Programs,
Johns Hopkins All Children’s Heart Institute
Disclosure
1. Chair of the STS Database Access and
Publications Taskforce
2. Chair of the STS Database Task Force on
Longitudinal Follow-up and Linked Registries
3. Chair of the STS Database Public Reporting
Task Force
Objectives
• Define Heterotaxy Syndrome
• Understand the “outcomes” of surgical procedures
commonly performed patients with Heterotaxy
Syndrome
Two Manuscripts
Jacobs JP, Anderson RH, Weinberg P, Walters III HL, Tchervenkov CI, Del Duca D,
Franklin RCG, Aiello VD, Béland MJ, Colan SD, Gaynor JW, Krogmann ON,
The nomenclature,
definition and classification of cardiac structures in the setting
of heterotaxy. In 2007 Supplement to Cardiology in the Young: Controversies
Kurosawa H, Maruszewski B, Stellin G, Elliott MJ.
and Challenges Facing Paediatric Cardiovascular Practitioners and their Patients,
Anderson RH, Jacobs JP, and Wernovsky G, editors. Cardiology in the Young. 2007
Sept; 17(Suppl 2):1–28, doi: 10.1017/S1047951107001138..
Jacobs JP, Pasquali SK, Morales DLS, Jacobs ML, Mavroudis C, Chai PJ, Tchervenkov CI,
Heterotaxy: Lessons
Learned About Patterns of Practice and Outcomes From the
Congenital Heart Surgery Database of the Society of Thoracic
Surgeons. The World Journal for Pediatric and Congenital Heart Surgery
Lacour-Gayet FG, Walters III H, Quintessenza JA.
(WJPCHS). 2011 April; 2(2):278-286. DOI: 10.1177/2150135110397670.
Cardiac Connections – Sequential
Segmental Approach (Anderson)
1. Atrial arrangement,
2. Atrioventricular connections,
and
3. Ventriculoarterial connections
Atrial Arrangement
Robert
Anderson
Atrioventricular Connections
Robert
Anderson
Atrioventricular Connections
• Normal atrioventricular connections (Concordant atrioventricular
connections in biventricular heart)
• Discordant AV connections
• Absent left sided AV connection
• Absent right sided AV connection
• Double inlet AV connection
• Double inlet AV connection, Double inlet LV
• Double inlet AV connection, Double inlet RV
• Mixed ("Ambiguous") AV connections (biventricular)
Ventriculoarterial Connections
•
•
•
•
•
•
•
•
Normal ventriculoarterial connections (Concordant ventriculoarterial connections in
biventricular heart)
Discordant VA connections (TGA)
Single outlet VA connection, Common arterial trunk
Single outlet VA connection, Single outlet VA connection via aorta (pulmonary atresia)
Single outlet VA connection, Single outlet VA connection via pulmonary trunk (aortic atresia)
Double outlet VA connections, Double outlet LV
Double outlet VA connections, Double outlet RV
Concordant VA connections with parallel great arteries (anatomically corrected malposition)
Concordant Atrioventricular Connections with
Discordant Ventriculoarterial Connections
(TGA)
Robert H. Anderson, Paul M. Weinberg. The clinical anatomy of transposition. In:
Jacobs JP, Wernovsky G, Gaynor JW, and Anderson RH (editors). 2005 Supplement to
Cardiology in the Young: Controversies of the Ventriculo-Arterial Junctions and Other
Topics, Cardiology in the Young, Volume 15, Supplement 1: 1 - 198, February 2005.
Jacobs JP, Anderson RH, Weinberg P, Walters III HL, Tchervenkov CI, Del Duca D, Franklin RCG, Aiello VD, Béland
MJ, Colan SD, Gaynor JW, Krogmann ON, Kurosawa H, Maruszewski B, Stellin G, Elliott MJ. The
nomenclature, definition and classification of cardiac structures in the setting of
heterotaxy. In 2007 Supplement to Cardiology in the Young: Controversies and Challenges Facing
Paediatric Cardiovascular Practitioners and their Patients, Anderson RH, Jacobs JP, and Wernovsky G,
editors. Cardiology in the Young. 2007 Sept; 17(Suppl 2):1–28, doi: 10.1017/S1047951107001138..
•
Specification of the atrial arrangement, the
atrioventricular connections, and the
ventriculoarterial connections, does not specify nor
imply the ventricular topology, or the relationships of
the cardiac chambers and great arteries in space.
These variables are separately specified.
Cardiac Relations - Segmental
Approach (Van Praagh & Vlad)
1. the sidedness of the atrial chambers, or, in other
words, the atrial “situs”,
2. the ventricular topology, in other words, the
ventricular “loop”, and
3. the relationships of the arterial trunks in space
Cardiac Relations - Segmental
Approach (Van Praagh & Vlad)
• The viscera and atria: The types of visceroatrial situs
– S
solitus
– I
inversus
– A
ambiguus
• The ventricular topology
– D
D-loop ventricles,
– L
L-loop ventricles
• Great vessels: The great arterial position
– S
solitus
– I
inversus
– D
dextro
– L
levo
Visceral Situs
William T. Mahle, MD
Associate Professor of
Pediatrics
Emory University School of
Medicine
Norman H Silverman
Stanford University
Lucile Packard Children’s Hospital
Robert Anderson
Morphologically Right Ventricle
Robert
Anderson
Morphologically Left Ventricle
VENTRICULAR TOPOLOGY – Robert Anderson
Right hand
Left hand
Jaggers JJ, Cameron DE, Herlong JR, et al: The Society of Thoracic Surgeons Congenital
Heart Surgery Nomenclature and Database Project: transposition of the great arteries,
Ann Thorac Surg 69(suppl):S205, 2000. In: Mavroudis C, Jacobs JP. Congenital Heart
Surgery Nomenclature and Database Project: Ann Thorac Surg. 2000 Apr;69(4 Suppl):S1372.
Anatomic types of transposition of the great arteries (TGA) as
described by Van Praagh and colleagues
The anatomic subtypes that are included in this nomenclature include:
» TGA {S,D,D};
» TGA {S,D,A};
» TGA {S,D,L};
» TGA {I,L,L};
» TGA {I,L,D};
» {A,L,L}
» {A,D,D}
(Those with situs ambiguous technically cannot be TGA as described by Van
Praagh, but may be physiologically uncorrected and appropriately treated by
existing standard therapies for TGA)
Jacobs JP, Anderson RH, Weinberg P, Walters III HL, Tchervenkov CI, Del Duca D, Franklin RCG, Aiello VD, Béland
MJ, Colan SD, Gaynor JW, Krogmann ON, Kurosawa H, Maruszewski B, Stellin G, Elliott MJ. The
nomenclature, definition and classification of cardiac structures in the setting of
heterotaxy. In 2007 Supplement to Cardiology in the Young: Controversies and Challenges Facing
Paediatric Cardiovascular Practitioners and their Patients, Anderson RH, Jacobs JP, and Wernovsky G,
editors. Cardiology in the Young. 2007 Sept; 17(Suppl 2):1–28, doi: 10.1017/S1047951107001138..
•
Specification of the atrial “situs”, the
ventricular “loop”, and the relationships of the
arterial trunks in space does not specify the
atrioventricular and ventriculoarterial
connections.
These variables are separately specified.
Cardiac Position
Cardiac Position
Can be surgically important – for example –
Pathway for extracardiac Fontan
•
•
•
Dextrocardia (right sided ventricular mass)
Levocardia (left sided ventricular mass)
Mesocardia (central/midline ventricular mass)
•
•
Dextroversion (ventricular apex points to the right)
Levoversion (ventricular apex points to the left)
Jacobs JP, Anderson RH, Weinberg P, Walters III HL, Tchervenkov CI, Del Duca D, Franklin RCG, Aiello VD, Béland
MJ, Colan SD, Gaynor JW, Krogmann ON, Kurosawa H, Maruszewski B, Stellin G, Elliott MJ. The
nomenclature, definition and classification of cardiac structures in the setting of
heterotaxy. In 2007 Supplement to Cardiology in the Young: Controversies and Challenges Facing
Paediatric Cardiovascular Practitioners and their Patients, Anderson RH, Jacobs JP, and Wernovsky G,
editors. Cardiology in the Young. 2007 Sept; 17(Suppl 2):1–28, doi: 10.1017/S1047951107001138..
“Heterotaxy is synonymous with “visceral heterotaxy” and
“heterotaxy syndrome”. Heterotaxy is defined as an
abnormality where the internal thoraco-abdominal
organs demonstrate abnormal arrangement across
the left-right axis of the body. By convention,
heterotaxy does not include patients with either the
expected usual or normal arrangement of the internal
organs along the left-right axis, also known as “situs
solitus”, nor patients with complete mirror-imaged
arrangement of the internal organs along the left-right
axis also known as “situs inversus”.”
LATERALIZED BODILY ARRANGEMENT
1
2
3
RAA
1
1
2
2
2
3
LAA
LAA
Usual
1
Robert Anderson
RAA
Mirror-imagery
Supported by:
Isomerism
In chemistry, isomers are defined as different compounds that have the same molecular
formula.
Isomers are classified as enantiomers when their molecules are mirror reflections of each
other .
Jacobs JP, Anderson RH, Weinberg P, Walters III HL, Tchervenkov CI, Del Duca D, Franklin RCG, Aiello VD, Béland
MJ, Colan SD, Gaynor JW, Krogmann ON, Kurosawa H, Maruszewski B, Stellin G, Elliott MJ. The
nomenclature, definition and classification of cardiac structures in the setting of
heterotaxy. In 2007 Supplement to Cardiology in the Young: Controversies and Challenges Facing
Paediatric Cardiovascular Practitioners and their Patients, Anderson RH, Jacobs JP, and Wernovsky G,
editors. Cardiology in the Young. 2007 Sept; 17(Suppl 2):1–28, doi: 10.1017/S1047951107001138..
“Isomerism in the context of the
congenitally malformed heart is defined
as a situation where some paired
structures on opposite sides of the
left-right axis of the body are, in
morphologic terms, symmetrical
mirror images of each other”
VISCERAL HETEROTAXY
1
1
2
2
3
3
1
1
2
2
??
??
Mid-line Liver
Mid-line Liver
Malrotated gut
Malrotated gut
“Polysplenia”
“Asplenia”
Robert Anderson
Supported by:
Isomeric Lungs
Isomeric Bronchial Arrangements
Jacobs JP, Anderson RH, Weinberg P, Walters III HL, Tchervenkov CI, Del Duca D, Franklin RCG, Aiello VD, Béland
MJ, Colan SD, Gaynor JW, Krogmann ON, Kurosawa H, Maruszewski B, Stellin G, Elliott MJ. The
nomenclature, definition and classification of cardiac structures in the setting of
heterotaxy. In 2007 Supplement to Cardiology in the Young: Controversies and Challenges Facing
Paediatric Cardiovascular Practitioners and their Patients, Anderson RH, Jacobs JP, and Wernovsky G,
editors. Cardiology in the Young. 2007 Sept; 17(Suppl 2):1–28, doi: 10.1017/S1047951107001138..
“Isomerism of the right atrial appendages is a
subset of heterotaxy where the atrial
appendages on both sides of the body have the
appearance of the morphologically right atrial
appendage.”
Jacobs JP, Anderson RH, Weinberg P, Walters III HL, Tchervenkov CI, Del Duca D, Franklin RCG, Aiello VD, Béland
MJ, Colan SD, Gaynor JW, Krogmann ON, Kurosawa H, Maruszewski B, Stellin G, Elliott MJ. The
nomenclature, definition and classification of cardiac structures in the setting of
heterotaxy. In 2007 Supplement to Cardiology in the Young: Controversies and Challenges Facing
Paediatric Cardiovascular Practitioners and their Patients, Anderson RH, Jacobs JP, and Wernovsky G,
editors. Cardiology in the Young. 2007 Sept; 17(Suppl 2):1–28, doi: 10.1017/S1047951107001138..
“Isomerism of the left atrial appendages is a
subset of heterotaxy where the atrial
appendages on both sides of the body have the
appearance of the morphologically left atrial
appendage.”
Jacobs JP, Anderson RH, Weinberg P, Walters III HL, Tchervenkov CI, Del Duca D, Franklin RCG, Aiello VD, Béland
MJ, Colan SD, Gaynor JW, Krogmann ON, Kurosawa H, Maruszewski B, Stellin G, Elliott MJ. The
nomenclature, definition and classification of cardiac structures in the setting of
heterotaxy. In 2007 Supplement to Cardiology in the Young: Controversies and Challenges Facing
Paediatric Cardiovascular Practitioners and their Patients, Anderson RH, Jacobs JP, and Wernovsky G,
editors. Cardiology in the Young. 2007 Sept; 17(Suppl 2):1–28, doi: 10.1017/S1047951107001138..
“Situs ambiguus is defined as an abnormality in which there
are components of situs solitus and situs inversus in the
same person. Situs ambiguus, therefore, can be
considered to be present when the thoracic and abdominal
organs are positioned in such a way with respect to each
other as to be not clearly lateralised and thus have neither
the usual, or normal, nor the mirror-imaged
arrangements.”
Jacobs JP, Anderson RH, Weinberg P, Walters III HL, Tchervenkov CI, Del Duca D, Franklin RCG, Aiello VD, Béland
MJ, Colan SD, Gaynor JW, Krogmann ON, Kurosawa H, Maruszewski B, Stellin G, Elliott MJ. The
nomenclature, definition and classification of cardiac structures in the setting of
heterotaxy. In 2007 Supplement to Cardiology in the Young: Controversies and Challenges Facing
Paediatric Cardiovascular Practitioners and their Patients, Anderson RH, Jacobs JP, and Wernovsky G,
editors. Cardiology in the Young. 2007 Sept; 17(Suppl 2):1–28, doi: 10.1017/S1047951107001138..
“Asplenia syndrome” can be defined as a
subset of heterotaxy with components of
bilateral right-sidedness, usually
associated with absence of the spleen.
Jacobs JP, Anderson RH, Weinberg P, Walters III HL, Tchervenkov CI, Del Duca D, Franklin RCG, Aiello VD, Béland
MJ, Colan SD, Gaynor JW, Krogmann ON, Kurosawa H, Maruszewski B, Stellin G, Elliott MJ. The
nomenclature, definition and classification of cardiac structures in the setting of
heterotaxy. In 2007 Supplement to Cardiology in the Young: Controversies and Challenges Facing
Paediatric Cardiovascular Practitioners and their Patients, Anderson RH, Jacobs JP, and Wernovsky G,
editors. Cardiology in the Young. 2007 Sept; 17(Suppl 2):1–28, doi: 10.1017/S1047951107001138..
“Polysplenia syndrome” can be defined
as a subset of heterotaxy with
components of bilateral left-sidedness,
usually associated with multiple spleens.
Complete description of both the cardiac
relations and connections:
1.
2.
3.
4.
5.
6.
7.
8.
9.
the arrangement of the atrial appendages
the ventricular topology
the atrioventricular connections
the ventriculoarterial connections
the infundibular morphologies
the relationships of the arterial trunks in space
the position of the heart in the chest
the orientation of the cardiac apex
In heterotaxy, particular attention is required for the venoatrial
connections, since these are so often abnormal.
Heterotaxy
•
•
Nomenclature is important
Less than perfect association between the state of the
spleen and the form of heart disease .
• Splenic morphology should be investigated in all forms of
heterotaxy, regardless of the type of cardiac disease.
• The splenic morphology should not be used to stratify the form
of disease within the heart
• The form of cardiac disease should not be used to stratify the
state of the spleen.
The Report of the 2010 STS Congenital Heart Surgery Practice and
Manpower Survey
♥ undertaken by the Society of Thoracic
Surgeons Workforce on Congenital Heart
Surgery
♥ 125 centers in the United States of America
perform pediatric and congenital heart
surgery
♥ 8 centers in Canada perform pediatric and
congenital heart surgery
Jacobs ML, Daniel M, Mavroudis C, Morales DLS, Jacobs JP, Fraser CD,
Turek JW, Mayer JE, Tchervenkov C, Conte JV. Report of the 2010
Society of Thoracic Surgeons Congenital Heart Surgery Practice
and Manpower Survey. The Annals of Thoracic Surgery, 2011;92:762–
9, August 2011.
Growth in the STS Congenital Heart Surgery Database
Participating Centers Per Harvest
120
100
80
60
40
20
0
Participating Centers
2002
16
2003
18
2004
21
2005
34
2006
47
2007
58
2008
68
2009
79
2010
93
2011
101
2012
105
2013
111
2014
114
Jacobs JP, Jacobs ML, Mavroudis C, Lacour-Gayet FG, Tchervenkov CI, Pasquali SK. Executive
Summary: The Society of Thoracic Surgeons Congenital Heart Surgery Database Twentieth Harvest – (January 1, 2009 – December 31, 2013). The Society of Thoracic
Surgeons (STS) and Duke Clinical Research Institute (DCRI), Duke University Medical Center,
Durham, North Carolina, United States, Spring 2014 Harvest.
Growth in the STS Congenital Heart Surgery Database
Operations per averaged 4 year data collection cycle
160000
140000
120000
100000
80000
60000
40000
20000
0
Operations
2002
12787
2003
16461
2004
28351
2005
37093
2006
45635
2007
61014
2008
72002
2009
91639
2010
103664
2011
114041
2012
130823
2013
136617
2014
142933
Jacobs JP, Jacobs ML, Mavroudis C, Lacour-Gayet FG, Tchervenkov CI, Pasquali SK. Executive
Summary: The Society of Thoracic Surgeons Congenital Heart Surgery Database Twentieth Harvest – (January 1, 2009 – December 31, 2013). The Society of Thoracic
Surgeons (STS) and Duke Clinical Research Institute (DCRI), Duke University Medical Center,
Durham, North Carolina, United States, Spring 2014 Harvest.
Growth in the STS Congenital Heart Surgery Database
Cumulative operations over time
350000
300000
250000
200000
150000
100000
50000
0
Cumulative Operations
2000
2001
2002
2003
2004
2005
2006
2007
2008
2009
2010
2011
2012
2013
2014
4237
9747
16537
26404
39988
58181
79399
98406
119266
148110
179697
213416
257932
292828
314674
Jacobs JP, Jacobs ML, Mavroudis C, Lacour-Gayet FG, Tchervenkov CI, Pasquali SK. Executive
Summary: The Society of Thoracic Surgeons Congenital Heart Surgery Database Twentieth Harvest – (January 1, 2009 – December 31, 2013). The Society of Thoracic
Surgeons (STS) and Duke Clinical Research Institute (DCRI), Duke University Medical Center,
Durham, North Carolina, United States, Spring 2014 Harvest.
STS Database Penetrance in USA
• The STS Congenital Heart Surgery Database (STSCHSD) is the largest clinical database in the world
for congenital and pediatric cardiac surgery.
• The Report of the 2010 STS Congenital Heart Surgery
Practice and Manpower Survey, undertaken by the STS
Workforce on Congenital Heart Surgery, documented
that 125 hospitals in the United States of America
and 8 hospitals in Canada perform pediatric and
congenital heart surgery.
• The STS-CHSD contains data from 119 of the 125
hospitals (95.2% penetrance by hospital) in the
United States of America and 3 of the 8 centers in
Canada.
Heterotaxy in the EACTS-STS Congenital
Heart Surgery Databases:
10 TERMS
• 3 Preoperative Factors
• 7 Diagnostic terms
Heterotaxy in the EACTS-STS Congenital
Heart Surgery Databases:
10 TERMS
• 3 Preoperative Factors:
1. Heterotaxy syndrome
2. Heterotaxy syndrome, Asplenia syndrome
3. Heterotaxy syndrome, Polysplenia syndrome
Heterotaxy in the EACTS-STS Congenital
Heart Surgery Databases:
10 TERMS
• 7 Diagnostic terms
1.
2.
3.
4.
5.
6.
7.
Single ventricle, Heterotaxia syndrome
Atrial Isomerism, Left {CAN NOT BE PRIMARY DIAGNOSIS}
Atrial Isomerism, Right {CAN NOT BE PRIMARY DIAGNOSIS}
Dextrocardia {CAN NOT BE PRIMARY DIAGNOSIS}
Levocardia {CAN NOT BE PRIMARY DIAGNOSIS}
Mesocardia {CAN NOT BE PRIMARY DIAGNOSIS}
Situs inversus {CAN NOT BE PRIMARY DIAGNOSIS}
Heterotaxy syndrome
• Heterotaxy is synonymous with ‘visceral heterotaxy’
and ‘heterotaxy syndrome’. Heterotaxy is defined as
an abnormality where the internal thoracoabdominal organs demonstrate abnormal
arrangement across the left-right axis of the body. By
convention, heterotaxy does not include patients with
either the expected usual or normal arrangement of
the internal organs along the left-right axis, also known
as ‘situs solitus’, nor patients with complete mirrorimaged arrangement of the internal organs along the
left-right axis also known as ‘situs inversus’.
Heterotaxy syndrome, Asplenia syndrome
• “Asplenia syndrome” can be defined
as a subset of heterotaxy with
components of bilateral rightsidedness, usually associated with
absence of the spleen.
Heterotaxy syndrome, Polysplenia syndrome
• “Polysplenia syndrome” can be
defined as a subset of heterotaxy
with components of bilateral leftsidedness, usually associated with
multiple spleens.
Single Ventricle
1.
2.
3.
4.
5.
6.
7.
8.
Single ventricle, DILV
Single ventricle, DIRV
Single ventricle, Mitral atresia
Single ventricle, Tricuspid atresia
Single ventricle, Unbalanced AV canal
Single ventricle, Heterotaxia syndrome
Single ventricle, Other
Single Ventricle + Total anomalous pulmonary
venous connection (TAPVC)
STS Congenital Heart Surgery Database
• All Index Operations in Database over 12 years
– January 1, 1998 - December 31, 2009
• Excludes participants with > 15% missing for
discharge mortality or non-cardiac abnormalities,
and individual records missing discharge mortality
or non-cardiac abnormalities
• Excludes patients weighing less than or equal to
2,500 g undergoing PDA ligation as their primary
procedure
STS Congenital Heart Surgery Database
• 77,153 total operations
• 1,144 with asplenia
• 361 with polysplenia
Percentage
Discharge
Mortality
All Index
Operations in
Database
Asplenia
Polysplenia
No Asplenia or
Polysplenia
STS-EACTS
Category 1
0.5%
5.5%
10.6%
0.5%
STS-EACTS
Category 2
1.7%
4.6%
7.6%
1.6%
STS-EACTS
Category 3
2.7%
4.3%
1.7%
2.6%
STS-EACTS
Category 4
8.2%
15.1%
14.1%
7.8%
STS-EACTS
Category 5
20.8%
30.8%
16.1%
20.4%
STS Congenital Heart Surgery Database
• All Index Operations in Database over 12 years
– January 1, 1998 - December 31, 2009
• Include all patients with Single Ventricle who
underwent Shunt, Glenn, or Fontan
• Excludes participants with > 15% missing for
discharge mortality
STS Congenital Heart Surgery Database
• 804 Shunt (148 with heterotaxy)
• 1669 Glenn (201 with heterotaxy)
• 1936 Fontan (240 with heterotaxy)
Percentage
Discharge
Shunt
Mortality
All Single
Ventricle
All Single
Ventricle
except
Heterotaxy
Single
ventricle,
Heterotaxia
syndrome
Glenn
Fontan
7.3%
1.4%
2.1%
6.6%
1.4%
1.8%
10.8% 1.5%
4.2%
Heterotaxy:
Lessons learned from the STS Database
1. STS Database is largest Congenital Heart
Surgery Database in North America
2. Review of Data allows for unique picture of
Patterns of Practice and Outcome
3. Heterotaxy is a challenging problem with
increased discharge mortality in most
subgroups.
DATA
DATA
DATA
DATA is Power