Criss cross heart is rare entity: Complete surgical repair in complex CHD with Criss cross heart

1. D R M A N I S H N C H O K H A N D R E
C O N S U L T A N T P E D I A T R I C C A R D I O L O G I S T
Criss cross heart

2. Criss Cross Heart
 Rare congenital anomaly : cardiac rotation resulting in
crossing of ventricular inlets and drainage of the atria
into contra-laterally located ventricles
 <0.1 % of congenital heart disease
 first described by Lev and Rowlatt in 1961
 1974 Anderson et al. used the term “criss-cross heart” for
the first time

5. Introduction
 The atrio-ventricular (AV) and ventriculo-arterial
(VA) connections can be concordant or discordant.
 Atrioventricular concordance and ventriculoarterial
discordance are seen in 80% of cases.
 There can be either side-by-side or superior-inferior
ventricular arrangement (superior-inferior
ventricles, SIV)

6. Etiology
 Abnormal rotation of the ventricular mass occurs
around its long axis and takes place after ventricular
septation.
 Anderson et al. - result of clockwise or counter-
clockwise post-septational rotation of the heart
 connexin 43 (Cx43) gene mutations were associated
with the pathogenesis of the disease

7. AV VA concordance/discordance

9. ECHO diagnosis
 Inability to visualize both the AV valves in a single
imaging plane in Ap4C indicates the presence of crossed
ventricular inflow streams during echocardiography, and
its demonstration is a sine qua non for the diagnosis of
CCH.
 Another of its hemodynamic characteristics is that the
systemic and pulmonary venous systems are crossed at
the AV level, although without mixing.
 Crossing of inlets is seen when posterior to anterior
sweep is made by probe

11.  CCH may be seen in three forms
- Complete Transposition
- Corrected transposition
- Normal hearts

12. Superior-inferior ventricles (SIV)
 SIV arises due to displacement of the ventricular
mass along the horizontal plane
 Although vertical alignment of interventricular
septum has been reported in CCH, horizontal
alignment is much more common.
 SIV, however, is not specific for CCH and may also
be present without CCH

13. Associated anomaly
 DORV: Most common association 20% in SI, Fang
(50%), Yang (50%), Valdes-Crus (30%), Hoffmann
(57%)
 Pulmonary outflow tract obstruction prevalence of
60% in SI, 55% by Valdes-Crus, all cases reported by
Hoffmann

14. Associated Anomalies
 Malpositions (dextrocardia)
 Straddling atrioventricular valve
 Subaortic stenosis
 Aortic arch obstruction
 Mitral stenosis
 Juxtaposition atrial appendages
 Transposition of great arteries

15. Differential diagnosis
 Severe form of straddling AV valve
 Double outlet atrium
 Severe Ebsteins anomaly of tricuspid valve –
tricuspid valve opens into the infundibulum giving
appearance of crossing over

16. Surgical Treatment
 varies from palliative correction to definitive
anatomic correction
 In majority of patients with a criss-cross heart, a
two-ventricle repair may not be possible and
these patients are staged toward a Fontan-type
operation.

17. Literature review

28. Case 1
 12 days old newborn presented with dyspnoea and
cyanosis
 Echo: CCH, D-transposition of great arteries,
muscular ventricular septal defect, rvoto due to
deviation of conal septum
 Treatment- Elective ASO with ventricular septal
defect and atrial septal defect closure

29. Case-2
 12yrs/F/19kg- with a history of progressive
exertional dyspnea and cyanosis of the extremities
since the age of 3
 Echo:Criss-cross heart with discordant
atrioventricular connections, DORV, large VSD and
VPS
 Treatment- Fontan Sx

35. MRI with VA discordance

36. MRI concordance (Fontan) transverse plane

37. MRI concordance (Fontan) Coronal plane

38. Case report
 7 yrs/M/17.5 kg
 Diagnosed with congenital heart disease at infancy
c/o cyanosis, dyspnoea, slow weight gain
 No obvious Dysmorphism, vitals stable, dental
caries+, cyanosis+, pandigital clubbing+,Single S2,
no murmur
 ECHO:Criss cross ventricle, large subaortic
ventricular septal defect, pulmonary atresia,
hypoplastic branch pulmonary arteries, good
biventricular function

39. Pre-op CXR

40. ECHO- AP4C

41. ECHO- Sub-costal 4C view

42. RPA pre-op

43. RPA pre-op

44. CT scan

45. CT scan

46. LPA-CT scan

47. Intra-operative findings
 CCH
 Remote sub-aortic ventricular septal defect
 Adequate size branch pulmonary arteries
 Superio-inferior ventricles

48. Surgery
 Ventricular septal defect closure- enlargement of
ventricular septal defect / LV to aorta baffle
 Relief of right ventricular outflow tract obstruction:
Right ventricular outflow tract resection +
transannular patch (conduit placement could not be
done due to technical reasons)
 Ligation of patent ductus arteriosus and AP
collaterals
 Atrial septal defect closure

49. Hospital stay
 Uneventful postoperative course in PICU and ward

51. ECHO Post OP

53. Conclusion
 CCH – rare / misdiagnosed
 As per literature review case presented here is the
first case of CCH DORV pulmonary atresia who
underwent Biventricular repair

54. Thank You