SHA24/027003

Balanced vs.. Unbalanced Atrioventricular Septal Defects SHA 24, February 13-16, 2013 Riyadh Saudi Arabia

Ziad R. Bulbul, MD King Faisal Heart Center

SHA 24, February 13-16,2013 Riyadh KSA

Atrioventricular Septal Defects hmmm.. If you thought It is confusing, wait till I am done!!!

SHA 24, February 13-16,2013 Riyadh KSA

Outline Introduction ♥ History ♥ Nomenclature ♥ Embryology ♥ Anatomy and distinctive features ♥ Balanced vs. Unbalanced ♥ Surgical Considerations ♥ Conclusion ♥

SHA 24, February 13-16,2013 Riyadh KSA

Introduction ♥

Incidence: ♥

7-10% of patients with congenital heart disease ♥

♥

Prevalence: ♥

♥

♥ ♥

10% are unbalanced

The New England Regional Infant Cardiac Program: 0.11/1000 live birth Baltimore-Washington Infant Study: 0.342/1000 live birth

80% have trisomy 21 syndrome Frequent seen in hearts with isomerism of the atrial appendages

SHA 24, February 13-16,2013 Riyadh KSA

Outline Introduction ♥ History ♥ Nomenclature ♥ Embryology ♥ Anatomy and distinctive features ♥ Balanced vs.. Unbalanced ♥ Surgical Considerations ♥ Conclusion ♥

SHA 24, February 13-16,2013 Riyadh KSA

History ♥

Karl Von Rokitansky (1804-1878) ♥

Professor of pathological anatomy at the University of Vienna

“The Defects of Cardiac Septation: A pathological Anatomical Treatise” Karl Von Rotitansky, W. Braumüller, 1875

SHA 24, February 13-16,2013 Riyadh KSA

History ♥

Abbott: “Ostium Primum septal defect” and “Common AtrioVentricular Canal Defect”

♥

1948 Rogers and Edwards: those defects are morphologically similar.

♥

1956-1958 Wakai and Edwards: Partial and Complete AVCD,

♥

1958 Lev: Ostium Primum Defect and Common Atrioventricular Orifice and position of the AV node and the His bundle

♥

Wakai, Edwards, and later Lev and Bharati Intermediate and Transitional

♥

1966-Rastelli: morphology of the AV valve leaflets

♥

1967-Ugarte :emphasized the idea of bridging leaflets

♥

Later in the 60’s : Anderson, Piccoli and colleagues :absence of the AVS introduced the term: Atrio Ventricular Septal Defects SHA 24, February 13-16,2013 Riyadh KSA

Outline Introduction ♥ History ♥ Nomenclature ♥ Embryology ♥ Anatomy and distinctive features ♥ Balanced vs. Unbalanced ♥ Surgical Considerations ♥ Conclusion ♥

SHA 24, February 13-16,2013 Riyadh KSA

Nomenclature ♼

Which of the following has been used to describes the lesion in this echo still frame?

1.

2.

3.

4.

5.

6.

7.

Atrioventricular Communis Endocardiac Cushion defect Atrioventricular Canal Atrioventricular defect Common Atrioventricular Orifice defect Common Atrioventricular Septal defect ALL of the Above SHA 24, February 13-16,2013 Riyadh KSA

Nomenclature ♥

Classification ♥ ♥ ♥ ♥

Complete and Incomplete Common and Partial Common and Partitioned Ostium Primum and Common ♥

Intermediate ♥

♥

♥

Type I , II and III ♥ A, B and C

Transitional

Categorization ♥

Balanced and Unbalanced ♥

♥

Right and Left Dominant

Simple and Complicated SHA 24, February 13-16,2013 Riyadh KSA

Outline Introduction ♥ History ♥ Nomenclature ♥ Embryology ♥ Anatomy and distinctive features ♥ Balanced vs. Unbalanced ♥ Surgical Considerations ♥ Conclusion ♥

SHA 24, February 13-16,2013 Riyadh KSA

Embryology Anderson: ♥

The fusing cushions provide focal point for appropriate alignment of the atrial and ventricular septums

♥

Mesenchymal cap on the Primary septum and its role in the closure of the atrial foramen

♥

Forward expansion of the vestibular spine between the atrial septum and the cushions Circ Res. 1998;82:625656 SHA 24, February 13-16,2013 Riyadh KSA

Outline Introduction ♥ History ♥ Nomenclature ♥ Embryology ♥ Anatomy and distinctive features ♥ Balanced vs. Unbalanced ♥ Surgical Considerations ♥ Conclusion ♥

SHA 24, February 13-16,2013 Riyadh KSA

Distinctive Features ♼

Atrioventricular septum: ♼

Almost always, there is a defect in the structures interposed between the right atrium and the left ventricle

SHA 24, February 13-16,2013 Riyadh KSA

Distinctive Features ♥

Atrioventricular junction: ♥

Ovoid shape of the common atrioventriclar junction, with un-wedging of the aortic valve

SHA 24, February 13-16,2013 Riyadh KSA

Distinctive Features ♼

Valve Leaflets: ♼

Abnormal arrangement and number of the leaflets that guard the common atrioventricular junction

SHA 24, February 13-16,2013 Riyadh KSA

Distinctive Features ♥

Papillary muscles: ♥ ♥

Unusual arrangement of the left ventricular papillary muscles Counter clockwise rotation

SHA 24, February 13-16,2013 Riyadh KSA

Outline Introduction ♥ History ♥ Nomenclature ♥ Embryology ♥ Anatomy and distinctive features ♥ Balanced vs. Unbalanced ♥ Surgical Considerations ♥ Conclusion ♥

SHA 24, February 13-16,2013 Riyadh KSA

Balanced vs. Unbalanced ♥

Balanced

♥

Unbalanced ♥ ♥

Right dominant Left dominant

SHA 24, February 13-16,2013 Riyadh KSA

Balanced vs. Unbalanced ♥

Historically, the ventricular size and volume have been the dominant measure of unbalance: ♥

Thanopoulos BD, Fisher EA, DuBrow IW, Hastreiter AR. Right and left ventricular volume characteristics in common atrioventricular canal. Circulation. 1978:57:991-995

♥

Thies WR, Bargeron LM Jr., Bini RM, Colvin EV, Soto B. Spectrum of heart with one underdeveloped and one dominant ventricle. Pediatr Cardiol.1986;7:129-139

SHA 24, February 13-16,2013 Riyadh KSA

Balanced vs. Unbalanced ♥

Lev and Bharati: “ ….. Balanced type of Atrioventricular canal, we imply that both ventricles are hypertrophied and enlarged” “ ……Dominant Right type, we mean that the right ventricle is hypertrophied and enlarged and the left ventricle is smaller than normal”

♥

Unbalanced variant.., be it Right or left dominant, are found in both, the complete and the intermediate types

SHA 24, February 13-16,2013 Riyadh KSA

Balanced vs. Unbalanced Anderson: “….In AVSD be there a common or separate valvular orifices, the left and right components of the common AV junction are of comparable circumferences and the ventricles are then of similar size, this arrangement produces the so called balanced form of defect” SHA 24, February 13-16,2013 Riyadh KSA

Balanced vs.. Unbalanced ♼

Balanced CAVSD

SHA 24, February 13-16,2013 Riyadh KSA

Balanced vs.. Unbalanced ♼

Unbalanced CAVSD (extreme)

SHA 24, February 13-16,2013 Riyadh KSA

Balanced vs.. Unbalanced ♥

Unbalanced CAVSD(“gray” zone)

SHA 24, February 13-16,2013 Riyadh KSA

Balanced vs.. Unbalanced

Predictive criteria for determining the viability of the function of the left sided structures after biventricular repair of this lesion are lacking, however,‌..

Are there objective tools that help discriminate between balanced and unbalanced ?

SHA 24, February 13-16,2013 Riyadh KSA

Balanced vs.. Unbalanced Walter et al ♥

Eur J Cardiothorac Surg 33(2008) 40-47

Basis of angiographic measurements, ♥

expressed as left ventricle / right ventricle long axis ratio

(LAR) Tool 1: Angiography, Left to right ventricular LAR<1

SHA 24, February 13-16,2013 Riyadh KSA

Balanced vs.. Unbalanced Owens et al,

Pediatr Cardiol(2009) 30:431-435

â&#x20AC;&#x153;An unbalanced defect was defined as one with more than 60% of the atrioventricular valve are committed to one ventricle, as determined by echocardiographyâ&#x20AC;?

Tool 2: Echocardiography 60% or more of the AVV committed to one ventricle or the other

SHA 24, February 13-16,2013 Riyadh KSA

Balanced vs.. Unbalanced Cohen et al

J Am Coll Cardiol 1996;28:1017-23

♥

“ AVVI”

♥

Sub-costal 30 degrees LAO by 2D echo Area of the smaller /larger AV component

♥

Tool 3: Echocardiography, AVVI <0.76

LAVVA

RAVVA

SHA 24, February 13-16,2013 Riyadh KSA

Surgical Considerations Van Son, et al ♥ Potential LV volume ♥

Ann Thorac Surg 63:1657-1663

Post-op LV volume=0.57 x pre-op true volume +6.83

Tool 4: LV V indexed of ≥15 ml/m2

SHA 24, February 13-16,2013 Riyadh KSA

Balanced vs.. Unbalanced Szwast et al ♥ ♥ ♥

Am J Cardiol 2011;107:103-109

“ LVII” left ventricular inflow index Four Chamber view by 2D echo Diameter of the smallest color flow /LAVV annulus diameter

Tool 5: Echocardiography LVII >0.5

SHA 24, February 13-16,2013 Riyadh KSA

Outline Introduction ♥ History ♥ Nomenclature ♥ Embryology ♥ Anatomy and distinctive features ♥ Balanced vs.. Unbalanced ♥ Surgical Considerations ♥ Conclusion ♥

SHA 24, February 13-16,2013 Riyadh KSA

Surgical Considerations

Biventricular Repair “To Be or not to Be”

SHA 24, February 13-16,2013 Riyadh KSA

Surgical Considerations

SHA 24, February 13-16,2013 Riyadh KSA

Surgical Considerations ♥

“ AVVI”

♥

Echocardiograms of 103 patients of CAVSD over 4 years: ♥ 26 have uAVSD by angiography, echo or surgery ♥

♥ ♥ ♥

All patients with bAVSD had AVVI > 0.67 Non with AVVI <0.3 had biventricular repair In the uAVSD group ♥ ♥

♥

♥ ♥

15 had Biventricular Repair ♥ 6 early death

Wide overlap in the AVVI values No difference between survivals and non-survivals

LAVVA

RAVVA

3 of 6 non survivals had low AVVI and “normal” ventricular cavity ♥ Small Ventricular size alone was not associated with poor outcome All non-survivals have large VSD’s Concluded: ♥ If AVVI<0.67 in the presence of large VSD; single ventricular palliation should be considered SHA 24, February 13-16,2013 Riyadh KSA

Surgical Considerations

SHA 24, February 13-16,2013 Riyadh KSA

Surgical Considerations

SHA 24, February 13-16,2013 Riyadh KSA

Surgical Considerations

SHA 24, February 13-16,2013 Riyadh KSA

Surgical Considerations “LAR” ♥ ♥

♥

♥

18 years period- retrospective report Diagnosis of uAVSD based on the angiographic LV/RV Long axis ratio < 1 19 pts R dominant uAVSD (out of 259 pts AVSD) underwent Biventricular repair 3 early failure and 3 late reoperation

All have LAR< 0.65 Conclusion: caution should be exercised when LAR<0.65 ♥

♥

SHA 24, February 13-16,2013 Riyadh KSA

Surgical Considerations

Van Son, et al

Ann Tho ra c S u r g the Potential LV volume 63:1657 -1663 Five neonates with R. Dominant uCAVC Mean Measured LVED volume of 14.8 ml/m2 ♥ Potential LV volume of 30 ml/m2 LAVV to total AVV diameter ratio of 0.3 LAR (LV to RV long dimension ratio) of 0.65 ♥ All Survived Biventricular repair, with one late death ♥

♥ ♥

♥ ♥

Concluded:

Pre-op indexed LV volume of 15 ml/m2 allowed biventricular repair SHA 24, February 13-16,2013 Riyadh KSA

Surgical Considerations

SHA 24, February 13-16,2013 Riyadh KSA

Surgical Considerations

♥ ♥

AVVI Modified!!

AVVI revisited!!

SHA 24, February 13-16,2013 Riyadh KSA

Surgical Considerations ♥ ♥ ♥ ♥ ♥

♥ ♥

♥

356 echocardiograms with AVSD over 6 yrs period Four cardiac centers AVVI was measured in 315 patients Classified unbalanced if AVVI <0.4 or AVVI>0.6 Majority had bAVSD 0.4<AVVI<0.6 and underwent biventricular repair AVVI< 0.19 all underwent single ventricular repair Heterogeneous repair strategy was present 0.19< AVVI<0.39

Concluded: ♥ ♥

AVVI cannot as a sole discriminator identify all forms of u AVSD, AVVI effectively characterized the transition between balanced and unbalanced AVSD

SHA 24, February 13-16,2013 Riyadh KSA

Surgical Considerations

Szwast, et al LVII • •

Am J Cardiol 2011;107 :103-109

1994-2007 45 subjects with uAVSD diagnosed on the basis of AVVI 23 had SV based on severe LV hypoplasia or AVVI<0.27 • 22 had BV repair, non had an AVVI <0.41 4 deaths • No subject in the Biv group had a LVII <0.5 SLRA: A greater LVII(>0.55) most accurately predicted survival following Biventricular repair-R=0.46 •

SHA 24, February 13-16,2013 Riyadh KSA

Surgical Considerations

Am J Cardiol 2011;107 :103-109

•

Deviating from the conclusions in previous publication by Cohen • Mortality in BV group was significantly associated with • • • •

•

Younger age at repair Abnormal left AVV morphology Reoperation within 30 days Retrograde flow in the transverse arch in patients with PDA

No difference between survivals and non-survivals in the: • • •

Degree of AVV regurgitation LV volume Size of the VSD SHA 24, February 13-16,2013 Riyadh KSA

Outline History ♥ Nomenclature ♥ Embryology ♥ Anatomy and distinctive features ♥ Balanced vs.. Unbalanced ♥ Surgical Considerations ♥ Conclusion ♥

SHA 24, February 13-16,2013 Riyadh KSA

Conclusion ♥

Objective criteria ♥ ♥ ♥ ♥

•

AVVI> 0.41 LVII> 0.55 LAR> 0.65 Potential LVV of 15 ml/m2

Additional variable: • • • • •

Young age at repair Large non restrictive VSD, in the face of small LAVV Abnormal left AVV morphology Severe left ventricular outflow tract obstruction Retrograde flow in the transverse arch in patients with PDA SHA 24, February 13-16,2013 Riyadh KSA

Conclusion

Am J Cardiol 2011;107:103-109

SHA 24, February 13-16,2013 Riyadh KSA

Conclusion

Is a “bad” biventricular repair better than a “good” single ventricular repair???

SHA 24, February 13-16,2013 Riyadh KSA

Thank You

SHA 24, February 13-16,2013 Riyadh KSA