AVSD in review for PICU resident

1. AVSD in review
By : dr. Sh.A.O.Bawazir
General pediatric resident
R3

2. Objectives:-
Introduction and Types
The pathophysiology
perioperative management issues
potential complications
expected early postoperative outcomes

3. Introduction
• AVSDs
• atrioventricular valves ( common valve ) (pathognomonic)
• atrial septum deficit
• the ventricular septum deficit
• 4-5% of all congenital heart deficit
• Prevalence 0.3- 0.4/1000 living birth

4. Introduction
• Common associated with Down syndrome 50-60% (44% CHD & 39%
AVSD)
• Around 50% isolated with no genetic abnormality
• (M : F) 1.57
• Recurrent risk :
• in first degree 3-4% if have on affected child
• 10% if mother affected
• No risk if father affected

5. Types

7. pathophysiology

8. Perioperative
management issues

9. perioperative management issues
• Perioperative morbidity and mortality for repair of AVSD improved
over time secondary to:
• improved screening methods
• early repair
• better surgical techniques
• enhanced understanding of postoperative complications.
• Infants usually presented with systemic pulmonary artery pressures
and congestive heart failure required anti congestive medication to
prevent Eisenmenger heart
• Severe CHF in small infants (treated palliatively AP band) care higher
Mortality .

10. preoperative management issues
• Optimal management requires a multidisciplinary team approach
• Optimal time for repair CAVSD between (3 - 6 m) age.
• Optimal Weight is 4kg and more
• Deciding how to manage this complex group of patients requires assessment of
clinical imaging and hemodynamic data .
• Ventricular volumes determined by three dimensional ECHO
• MRI scans may guide therapy with the ability to recruit ventricles with volume as low as 15–
30 ml/m2.

11. 1. Knowledge of the underlying lesion?
2. Evidence of long-term complications and other features that put children into a high-risk category?
3. General medical condition & Evidence of recent upper or lower respiratory tract infections?
4. Routine drug therapy and previous surgery ?
Key features in preoperative assessment

12. Key features in preoperative
assessment
6.Venous access
7.Basic lab. Study and imaging ?
8. Sedative premedication: commonly used to avoid distress, minimize oxygen
consumption, and may also reduce the amount of induction agent so minimizing
reductions in SVR
9. Anesthesiologist assessment

13. Special consideration for down syndrome
• high incidence of extracardiac anomalies
• cohort of patients is known to develop early severe pulmonary
hypertension (PH) and pulmonary vascular obstructive disease
(PVOD)
• Particularly difficult sedition & anesthesia because of abnormal
concentrations of opioid receptors in the cerebral cortex, nociception,
anxiety levels, and neurotransmission,
• Preoperative screening for thyroid function is essential
• Vascular access continues to be challenging secondary to
abnormalities of the vasculature, skin, and subcutaneous fat.

14. Intra operative

15. Intra operative
Detail of significant :
• ASA
• CPB time
• Cross clamp time
• Blood transfusion
• End of OP rhythm (NSR)
• Medication : Inotrope used and doses ,Heparin
• TEE – ECHO for any residual

16. Intra operative
the modified single-patch technique
pledgeted sutures

17. Pulmonary artery (PA) banding
• no longer used in the elective routine repair
of complete AVSD in favor of a 1 stage repair.
• 5%–10% of patients coming for AVSD surgery
will require PA banding
• Indication of PA band:
• cases with pulmonary over circulation (AVSD)
Low weight and extremely septic infant
• patient with a contraindication for CPB, eg.,CNS
and recent intracranial bleed, necessitate
palliative procedure

18. Single ventricle physiologies (uAVSD)
GOALS :
• Unobstructed systemic blood flow
• Unobstructed pulmonary venous return
• Limited pulmonary blood flow
• Undistorted pulmonary arteries (PVR)
• Minimal AVV regurgitation
“Children with single-ventricle anatomy will ultimately undergo some
variation of the Fontan operation as their final surgical palliation”

19. BT - shunt
• the Blalock–Taussig shunt:
• is a surgical procedure used to increase
pulmonary blood flow for palliation in
duct dependent cyanotic heart defects
as patient with hypoplastic left ventricle

20. Glenn system
• Aim too separate the systemic and pulmonary circuits,
resulting in normal or near normal oxygen saturation.
• After these procedures, the single ventricle ejects blood
only to the systemic circuit, with pulmonary blood flow
derived by “passive flow” into the pulmonary vascular bed
at the expense of higher central venous pressure.
• connections improve cyanosis and minimize ventricular
work, the elevated PVR in the neonate precludes their use
until approximately 3 months of age.

21. • At 2 to 5 years of age,
• The inferior vena cava is connected to the pulmonary artery.
directed the venous return passively to the pulmonary arteries.
• A small communication is often left in the baffle to allow some residual right-to-left
mixing, which improves perioperative hemodynamics.
This communication (fenestration) often closes spontaneously or can be closed
percutaneously some time later
• increasing the systemic oxygen saturation to >95%

22. postoperative period

23. Postoperative Evaluation by ICU physician:
• Review of patient’s underlying cardiac defect(s),
• history of prior interventions,& preOp pathophysiology & clinical status
• Review of anesthesia record & operative note
• Verifications :
• current medication dosages
• ventilator settings
• IV fluid rates
• Physical examinations focusing (CVS.RESP)
• Interpretation of data from bedside monitoring & initial lab testing
• Documentation in the medical record of physical exam & lab findings,
• evaluation of the adequacy of the procedure,
• plans for the first postop night

24. postoperative issue (high light) :
-The issue (patients with a complete AVSD )may have a reactive
pulmonary arterial bed .
• These patients may benefit from a 1- to 3-day period of sedation
careful ventilator management
to minimize the chances of a pulmonary hypertensive crisis,
HOW?
In the current era with most patients undergoing repair at
approximately 4 months old, pulmonary hypertension is usually not a
major problem in the postoperative period

25. low cardiac output syndrome (LCOS)
• typically occurs 6 to 18 hours after cardiopulmonary bypass (CPB)
surgery
• Prevent by using inotropic support
• monitor cardiac output postoperatively
• Lactate
• physical examination
• intermittent or continuous venous saturation
• ECHO
• Temp. (peripheral and core )

26. • Eighty-five respondents (85/98 = 87%) answered this question and indicated
increasing either milrinone (42%) or adrenaline/epinephrine (36%) when cardiac
output is worsening
• If this treatment fails, then in most of the cases adrenaline/epinephrine (40%) will
be added as the second drug of choice.

28. sedative and analgesic
• Selection and appropriate use of sedative and analgesic medications
remain challenging in all patients in ICU, but postoperative cardiac
patients present some unique challenges.
• Two alternatives to opiates and benzodiazepines have evolved that
show promising results and improved profiles of side effects:
• dexmedetomidine
• caudal anesthesia (promising new technique)

29. caudal anesthesia
• blunt the stress response to CPB
• resulting in:
- decreased intraoperative narcotic
requirements,
- no impact on hemodynamics.
- Caudal anesthesia may facilitate early
extubation.

30. suture disruption
-they should be carefully assessed for suture disruption
• Why?
• leading to left or right atrioventricular valve insufficiency and/or
recurrent ventricular or atrial level shunting.

31. potential complications

32. potential complications
• Children with AVSD (complete AVSD) have a significant morbidity and
mortality 20%
• postoperative left atrioventricular valve regurgitation (LAVV)
• residual intracardiac shunts
• postoperative pulmonary hypertension
• various life-threatening cardiac arrhythmias
• left ventricular outflow tract obstruction.

33. expected early
postoperative outcomes

34. Shunt Lesions Part I: Patent Ductus Arteriosus,
Atrial Septal Defect, Ventricular Septal Defect, and Atrioventricular Septal Defect
Carl L. Backer, MD1,2; Osama Eltayeb, MD1,2; Michael C. Mongé, MD1,2; Mjaye L. Mazwi MD3,4;
John M. Costello, MD, MPH3,4
• Complete AVSD:
• Median age at repair was 4 months.
• one early mortality (4 mo postoperatively).
• There were no patients with heart block requiring a pacemaker.
• The median hospital stay was 9 days.
• Two patients ultimately underwent reoperation for left ventricular outflow tract
obstruction.
• Three patients underwent reoperation for left atrioventricular valve insufficiency.
Congenital Heart Disease

35. Shunt Lesions Part I: Patent Ductus Arteriosus,
Atrial Septal Defect, Ventricular Septal Defect, and Atrioventricular Septal Defect
Carl L. Backer, MD1,2; Osama Eltayeb, MD1,2; Michael C. Mongé, MD1,2; Mjaye L. Mazwi MD3,4;
John M. Costello, MD, MPH3,4
• partial AVSD:
• Between 1990 and 2015, 89 children underwent repair
• median age of 1.6 years.
• There was no operative mortality.
• 2 patients required pacemakers
• 3 patients underwent reoperation for left atrioventricular valve
insufficiency,
• 6 patient for subaortic stenosis.
Congenital Heart Disease

36. • Major complications occurred 9.8%
• permanent pacemaker implantation in 2.7%.
• Overall mortality was 3%.
• associated with higher mortality, longer postoperative length of stay in (ICU)
and higher incidence of major complications.
• Weight <3.5 kg
• age <2.5 months
• Surprisingly, children with Down's syndrome had lower morbidity and
mortality than other children, and their duration of ICU stay was similar.

37. Outcomes of repair of complete atrioventricular septal
defect in the current era.
Xie O, Brizard CP, d'Udekem Y, Galati JC, Kelly A, Yong MS, Weintraub RG, Konstantinov IE
Eur J Cardiothorac Surg. 2014 Apr; 45(4):610-7.
Royal children's Hospital Melbourne
• Age >6 months at repair was associated with higher rates of
reoperation
• operating at age <6 months was associated with longer ICU stay.
• Moderate AVV regurgitation at discharge was a risk factor for
reoperation.
• Down's syndrome present in 75% of patients was not at higher risk

38. At the end
• The natural history of complete AVSD includes:
• premature death due to complications of congestive heart failure and
or PA hypertension.
• Repair during infancy is recommended for all children.
• The age at elective repair 3–6 months at most centers today.
• Early repair minimizes the risk of premature death or pulmonary
vascular obstructive disease.
• CAVSD is frequently associated with Down's syndrome.

39. Thank you