surgical perspective

1. HETEROTAXY
&
SINGLE
VENTRICLE
Dr.Khaled Alhawri

2. Definitions
■ Rare
■ Hetero = different. Taxy = arrangement
■ Heterotaxy : the internal thoraco-abdominal
organs demonstrate abnormal arrangement
across the left-right axis of the body.
■ Synonyms asplenia, polysplenia,Situs
ambigus
■ Isomerism
– . Greek: isomeaning equal and meros-
meaning part.
– structures that are themselves mirror-
imaged
■ Situs Ambigus : situs solitus and situs
inversus in the same person.
■ heart defects of varying types and severity
■ involves the heart and other organs : renal,
biliary

5. Some Facts
 right isomerism : recognized in infancy RVOT and PS
 Always check spleen Abd US.
 Septicemia can develop in asplenic and polysplenic
patient ,often fulminant
 Klebsiella and Escherichia coli < 6 months
 pneumococcus and Haemophilus infl. > 6 months
 AB prophylaxis, oral penicillin V is recommended.
 H. influenzae vaccine ,hepatitis B vaccine
 AV block is exceedingly rare in right isomerism
 Complete AV block : in 1/10 of left isomerism.

6. Some Facts,cont’d
■ Most cardiac operations are palliative in
nature.
■ mortality and morbidity still high.
■ Factors with increased operative risk
– abnormalities of the systemic venous
connection,
– PAPVD, TAPVD Sp Obst.
– AVSD
– a morphologic right ventricle
supporting the systemic circulation
■ Left isomerism have less severe cardiac
malformations
– 1/3 go for biventricular repair.
– progressive valvar regurgitation is
common post repair
– A few biventricular repair for right
isomerism do exist.

9. SURGICAL PERSPECTIVE
■ Cardiopulmonary Bypass
– systemic venous anomalies e g
■ LSVC. 3venous cannula
■ Interrupted IVC larger SVC cannula
■ Direct caval cannulation rather than atrial cannulation for
ibtraatrial repair
■ Multiple cardiac procedure needed eg band, shunt, Glenn,
Fontan
■ Need for AVV repair
■ Need for Branch PA reconstruction due to discontinous PA
Branch or stenoses
■ Need for Kawashima in case of interrupted IVC
■ Increased risk of infection

10. RESULTS
■ Wide morphologic variability so difficult to comment on results
– e.g. AVSD, DORV with PA, malposed great arteries, heart block
■ Death almost certain if important cardiac defects
were present.
■ even in patients with normal hearts 18% mortality
early in life : non-cardiac abnormalities although their surgical
repair has improved
■ Overall survival is better with LEFT at 1yr
■ Left Isomerism 94%
■ Right isomerism : 79% and 53% in other series.
■ Survival is better when a biventricular repair rather than SV.
■ In the large series reported by Serraf and colleagues, however,
mortality in these two categories was similar
– But they still concluded that Bivent. is still preferred.

12. – Left Atrial Isomerism Outcomes
■ 1/3 received a biventricular repair.
■ both short- and long-term outcomes were
substantially better in those undergoing
biventricular repair
■ Risk factors for death
– Low Birth wt
– Single vent.
– GI abnormality
– AV block
– Coar
– AVSD
– one study of 91 biventricular repairs
suggests that attempting biventricular
repair in the setting of unbalanced AV
septal defect is associated with
increased mortality
RESULTS

13.  Right Atrial Isomerism Outcomes
 short- and long-term outcomes are worse
than for left
 Death is almost certain if no intervention.
 overall results have improved substantially over
time, with current-era outcomes suggesting 7% to
15% early mortality, and long-term survival of 75%
 Non cardiac abnormality contribute to mortality and
morbidity
RESULTS

14. RESULTS
 Fontan outcome:
 Heterotaxic pt
 Undergo operation at an
older age
 Receive more previous
operations
 Higher incidence of sinus
node dysfunction and atrial
arrhythmias
 More AVV reg.

15. Initial reports of the outcomes of the modified
Fontan operation for heterotaxy syndrome
revealed early mortality of 13% to 80%
extracardiac conduit technique for
construction of a Fontan circuit has a
particular benefit for this group
technical advances and modifications of
the Fontan operation have led to markedly
improved Fontan procedure results in
heterotaxy syndrome

16. Outcomes Of Multistage Palliation Of Infants
With Functional Single Ventricle And
Heterotaxy Syndrome
Bahaaldin Alsoufi, MD, Courtney McCracken, PhD, Brian Schlosser, BS, RDCS, Ritu
Sachdeva, MBBS, Andrew Well, PhD, Brian Kogon, MD, William Border, MBChB,
MPH, Kirk Kanter, MD
■ 2002-2012
■ 67 infants with heterotaxy syndrome
■ Primary outcome: Death( or transplant)VS reaching Glenn.
■ (58Pt) 87% : neonatal palliation
– modified Blalock-Taussig shunt 51%
– Norwood 18%
– pulmonary artery band 18%
– PrimaryGlenn (13%)
■ 50% (33pt) hadTAPVD repair
■ By one year :
– 29% died or transplanted
– 63% reached Glenn
■ By 5 yr : 64% undergone Fontan

17. ■ 8 pt needed ECMO survival ( 1/8)
– 5 post TAPVD rep and BT
– 2 post Norwood
– 1BT shunt Unifocalization
■ Overall hospital Mortality (15pt) 22%
– 9 post BT shunt
– 3 post PAB
– 3 post Norwood
– 60% of them had TAPVD reapir
– 30% of them had AVV repair
■ Risk factors for mortality
– Unplanned re-operation e.g. BT revision
– TAPVD rep.
■ 1st palliation in Heterotaxy vs non heterotaxy
– Higher mortality 27% VS 10% (p=0.02)
– Longer ventilation & ICU stay
■ Inter-stage results are comparable bet. heterotaxy and non heterotaxy

18. Conclusions
• The management of heterotaxy infants with functional single ventricle
remains challenging .
• First-stage palliation is associated with high operative mortality and
increased resource utilization due to surgical morbidity.
• Nonetheless, outcomes beyond hospital discharge are comparable to other
single ventricle patients.
• Efforts to improve survival in those patients should focus on perioperative
care

19. THANKYOU