1. 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

2. Disclosure Chair of the STS Database Access and Publications Taskforce
Chair of the STS Database Task Force on Longitudinal Follow-up and Linked Registries
Chair of the STS Database Public Reporting Task Force

3. Objectives Define Heterotaxy Syndrome
Understand the “outcomes” of surgical procedures commonly performed patients with Heterotaxy Syndrome

4. 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, 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 Sept; 17(Suppl 2):1–28, doi: /S
Jacobs JP, Pasquali SK, Morales DLS, Jacobs ML, Mavroudis C, Chai PJ, Tchervenkov CI, Lacour-Gayet FG, Walters III H, Quintessenza JA. 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 (WJPCHS) April; 2(2): DOI: /

5. Cardiac Connections – Sequential Segmental Approach (Anderson)
Atrial arrangement,
Atrioventricular connections, and
Ventriculoarterial connections

6. Atrial Arrangement
Robert Anderson

7. Atrioventricular Connections
Robert Anderson

8. 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)

9. 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)

10. 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) Supplement to Cardiology in the Young: Controversies of the Ventriculo-Arterial Junctions and Other Topics, Cardiology in the Young, Volume 15, Supplement 1: , February 2005.

11. These variables are separately specified.
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 Sept; 17(Suppl 2):1–28, doi: /S
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.

12. Cardiac Relations - Segmental Approach (Van Praagh & Vlad)
the sidedness of the atrial chambers, or, in other words, the atrial “situs”,
the ventricular topology, in other words, the ventricular “loop”, and
the relationships of the arterial trunks in space

13. 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

14. Visceral Situs Associate Professor of Pediatrics
William T. Mahle, MD
Associate Professor of Pediatrics
Emory University School of Medicine

15. Norman H Silverman
Stanford University
Lucile Packard Children’s Hospital

16. Morphologically Right Ventricle
Robert Anderson
Morphologically Right Ventricle

17. Morphologically Left Ventricle
Robert Anderson
Morphologically Left Ventricle

18. VENTRICULAR TOPOLOGY – Robert Anderson
Right hand
Left hand

19. The anatomic subtypes that are included in this nomenclature include:
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, In: Mavroudis C, Jacobs JP. Congenital Heart Surgery Nomenclature and Database Project: Ann Thorac Surg Apr;69(4 Suppl):S1-372.
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)

21. These variables are separately specified.
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 Sept; 17(Suppl 2):1–28, doi: /S
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.

22. Cardiac Position

23. 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)

24. 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 Sept; 17(Suppl 2):1–28, doi: /S
“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”.”

25. LATERALIZED BODILY ARRANGEMENT1 2 3 1 2 3
RAA
LAA
RAA
LAA
Liver
Stomach
Spleen
Liver
Stomach
Spleen
Mirror-imagery
Usual
Robert Anderson
Supported by:

26. 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 .

27. 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 Sept; 17(Suppl 2):1–28, doi: /S
“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”

28. ? ? ? ? VISCERAL HETEROTAXY 1 1 2 3 2 “Polysplenia” “Asplenia”
Mid-line Liver
Mid-line Liver
Malrotated gut
Malrotated gut
“Polysplenia”
“Asplenia”
Robert Anderson
Supported by:

29. Isomeric Lungs

30. Isomeric Bronchial Arrangements

31. 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 Sept; 17(Suppl 2):1–28, doi: /S
“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.”

32. 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 Sept; 17(Suppl 2):1–28, doi: /S
“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.”

33. 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 Sept; 17(Suppl 2):1–28, doi: /S
“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.”

34. 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 Sept; 17(Suppl 2):1–28, doi: /S
“Asplenia syndrome” can be defined as a subset of heterotaxy with components of bilateral right-sidedness, usually associated with absence of the spleen.

35. 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 Sept; 17(Suppl 2):1–28, doi: /S
“Polysplenia syndrome” can be defined as a subset of heterotaxy with components of bilateral left-sidedness, usually associated with multiple spleens.

36. Complete description of both the cardiac relations and connections:
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.

37. 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.

38. 8 centers in Canada perform pediatric and congenital heart surgery
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.

39. 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.

40. 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.

41. 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.

42. STS Database Penetrance in USA
The STS Congenital Heart Surgery Database (STS-CHSD) 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.

43. Heterotaxy in the EACTS-STS Congenital Heart Surgery Databases: 10 TERMS
3 Preoperative Factors
7 Diagnostic terms

44. Heterotaxy in the EACTS-STS Congenital Heart Surgery Databases: 10 TERMS
3 Preoperative Factors:
Heterotaxy syndrome
Heterotaxy syndrome, Asplenia syndrome
Heterotaxy syndrome, Polysplenia syndrome

45. Heterotaxy in the EACTS-STS Congenital Heart Surgery Databases: 10 TERMS
7 Diagnostic terms
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}

46. Heterotaxy syndrome
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’.

47. Heterotaxy syndrome, Asplenia syndrome
“Asplenia syndrome” can be defined as a subset of heterotaxy with components of bilateral right-sidedness, usually associated with absence of the spleen.

48. Heterotaxy syndrome, Polysplenia syndrome
“Polysplenia syndrome” can be defined as a subset of heterotaxy with components of bilateral left-sidedness, usually associated with multiple spleens.

49. Single Ventricle 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)

50. STS Congenital Heart Surgery Database
All Index Operations in Database over 12 years
January 1, 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

51. STS Congenital Heart Surgery Database
77,153 total operations
1,144 with asplenia
361 with polysplenia

52. Percentage Discharge Mortality
All Index Operations in Database
Asplenia
Polysplenia
No Asplenia or Polysplenia
STS-EACTS Category 1
0.5%
5.5%
10.6%
STS-EACTS Category 2
1.7%
4.6%
7.6%
1.6%
STS-EACTS Category 3
2.7%
4.3%
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%

53. STS Congenital Heart Surgery Database
All Index Operations in Database over 12 years
January 1, December 31, 2009
Include all patients with Single Ventricle who underwent Shunt, Glenn, or Fontan
Excludes participants with > 15% missing for discharge mortality

54. STS Congenital Heart Surgery Database
804 Shunt (148 with heterotaxy)
1669 Glenn (201 with heterotaxy)
1936 Fontan (240 with heterotaxy)

55. Percentage Discharge Mortality
Shunt
Glenn
Fontan
All Single Ventricle
7.3%
1.4%
2.1%
All Single Ventricle except Heterotaxy
6.6%
1.8%
Single ventricle, Heterotaxia syndrome
10.8%
1.5%
4.2%

56. Heterotaxy: Lessons learned from the STS Database
STS Database is largest Congenital Heart Surgery Database in North America
Review of Data allows for unique picture of Patterns of Practice and Outcome
Heterotaxy is a challenging problem with increased discharge mortality in most subgroups.

57. DATA DATA DATA

58. DATA is Power