Management of the Patient

with AV Canal

Lindsey Justice, MSN, RN, CPNP-AC

CICU Nurse Practitioner
The Heart Institute
Cincinnati Children’s Hospital Medical Center
 AV Canal Classifications

• Complete AV canal: primum ASD, inlet VSD, common AV

valve
– Rastelli type A: 75%, the superior common leaflet cleft is over the
crest of the septum, with chordal attachments between the leaflet and
septum
– Rastelli type B: rare, chordal attachments straddle septum to the left
– Rastelli type C: 25%, superior leaflet with no chordal attachments to
the septum, most common type in TOF and other assoc. defects
• Partial AV canal defect: primum ASD, no VSD, cleft mitral
valve
• Transitional AV canal defect: partial defect with inlet VSD
that is partially obstructed by chordal attachments
 Unbalanced AV Canal

• Present when the common AVV opens predominantly

toward one ventricle or the other

• If the common AVV opens predominantly into the

morphologic left ventricle = LV-dominant AV canal
• If the common AVV opens predominantly into the
morphologic right ventricle = RV-dominant AV canal

• Varies from mildly unbalanced with 2 nearly normal-sized

ventricles to severely unbalanced with a single dominant
ventricle and a second hypoplastic ventricle
• Single-ventricle physiology and surgical management
 Physiology
• Hemodynamics depend on:
– Size of the ASD and VSD
– Competency of AV valve
– Pulmonary vascular
resistance (PVR)
• newborn period-PVR  thus
minimal shunting of blood
• PVR falls over time (normal by
6-8 weeks)
– left to right shunting increases and
pulmonary blood flow increases,
results in CHF symptoms
– If no CHF then PVR is 
 Trisomy 21 (Down Syndrome)

• 40% of children with

Down Syndrome have
CHD
– 40% of the defects are AV
canal
– Generally fare better than
non-Down Syndrome AVC
 Considerations with Trisomy 21

• Pulmonary hypertension • Airway abnormalities

– Tracheal stenosis
• Thyroid disorders Loukanov et al. JTCVS 2005
– Laryngo/Tracheomalacia

• GI tract abnormalities
(12%) • CNS abnormalities
– Hypotonia
– TE fistula
– Seizures (<9%)
– Duodenal atresia
– Omphalocele
– Hirschprung disease • Difficulty with sedation
– Use of Precedex
Kalyanaraman et al. Pediatr Cardiol 2007
• Difficulty with vascular
– PCICS Post-op Guideline
access
Sulemanji et al. Anaesthesiol Scand 2009
 Trisomy 21 and Pulmonary
Hypertension

• 42 patients undergoing pulmonary hypertension

study protocol in cath lab
• Baseline, FiO2 1.0, then add 20,40,80 ppm iNO
• 11 Trisomy 21 patients (7 CAVC) vs. 31 without
chromosomal abnormalities
• 42% PVR decrease with O2 + iNO in non Trisomy 21
• 21% PVR decrease with O2 + iNO in Trisomy 21
(p=0.02)
• Conclusion: Patients with Trisomy 21 may be less
responsive to iNO

Cannon et al. Pediatr Cardiol 2005

 Trisomy 21 - Hypothyroidism

• Incidence is 32.5%
Purdy et al. J. Perinatol 2014

• Pre-op thyroid screening is essential

• Subclinical hypothyroidism is common, may
decrease myocardial contractility and cardiac
output
Biondi et al. 1999; Di Bello et al. 2000; Kahaly 2000

• Hypothyroidism associated with:

– longer mechanical ventilation and O2 supplementation
– greater use of epinephrine
– greater use of furosemide
– longer CICU LOS (p<0.0001)
Bettendorf et al. Pediatr Res 1997
 Thyroid Hormone
• Thyroid hormones have important effects on
cardiovascular function
–  Preload
–  Afterload
–  Contractility
–  Heart rate

• T3, T4, and TSH levels are significantly depressed following

cardiac surgery

• Results of T3 repletion in children following cardiac surgery

are mixed and optimal dosing is unknown
King et al, Ir J Med Sci 2014; Bettendorf et al, Lancet 2000; Chowdhury et al, JTCVS 2001
 AV Canal Surgical Repair

• Timing of surgical repair:

– Amount of CHF and
pulmonary overcirculation
– Size and age of infant
– Typically 4-6 months

• Surgery:
– Cardiopulmonary bypass
– Closure of ASD
– Patch closure of VSD
– Valve repair and division
(determined by number of
leaflets on common valve)
– Extent of valve involvement
crucial
 Postoperative Concerns

• Low cardiac output syndrome

• Residual or Recurrent Lesions
– VSD
– Left AV valve stenosis or insufficiency
• Arrhythmias/Heart Block
• Pulmonary Artery Hypertension
 Low Cardiac Output Syndrome

• LCOS: transient decrease in CO due to myocardial

dysfunction, results in inadequate oxygen delivery.
• High risk following cardiac surgery requiring CPB

• Evaluation:
– Tachycardia, diminished urine output, elevated lactate, low SVO2
and hypotension (late sign)

• Treatment:
– Volume administration for inadequate preload
– Inotropic support to improve contractility
– Consider afterload reduction if SVR is elevated or to assist with AV
valve regurgitation
– Support right ventricular function, treat PAH
Koo et al., PCICS Guidelines 2014
 Residual or Recurrent Lesions

• Lesions include:
– LVOTO obstruction (Partial or Transitional AVSD)
– AVV regurgitation and/or stenosis
– Intracardiac shunt (ASD or VSD)
• Intraoperative TEE essential in detecting
residual lesions
– Underestimates AVV regurgitation
• Low threshold for postoperative TTE in patients
not progressing appropriately

Chowdhury et al. JTCS 2009

Honjo et al. Ann Thorac Surg 2006
Kim et al. Ann Thorac Surg 2005
 Medical Treatment of Left AVVR

• Nipride or Milrinone
• ACE inhibition:
– Reduces severity of MR
– Improves cardiac performance
– Promotes ventricular remodeling

Calabro et al. Am Heart J 1999

Momma Pediatr Drugs 2006
Operative Treatment of Left AVVR

Gillespie et al. Cardiol Young 2006

 Post-operative Arrhythmias

• Rhythm disturbances decrease cardiac output

– Cardiac Output = Heart Rate x Stroke Volume
– Bradycardia decreases amount of blood ejected per
minute
– Tachycardia decreases diastolic filling time (stroke
volume) while increasing myocardial oxygen demand

• AV Synchrony assures optimal ventricular filling

(atrial kick = 15% of SV), especially important in
the setting of LCOS

Koo et al., PCICS Guidelines 2014

 Post-operative Arrhythmias

Post-operative arrhythmias are common (15%)

AVSD repair independent risk of arrhythmia

Delaney et al. JTCVS 2006
 Post-operative Arrhythmias - Treatment

• Epicardial pacing wires can be diagnostic and

therapeutic

• Can NOT predict who will need them

– Moltedo et al. 2007

• Transesophageal pacing is a good alternative in

supraventricular arrhythmias
 Post-operative Junctional Tachycardia

• Anatomic substrate is unknown

• “Narrow complex” tachycardia
– AV dissociation (Atrial rate < Ventricular rate)
• Major cause of postoperative morbidity and
mortality
– Increased ventilator time/CICU stay

Dodge-Khatami et. al. 2002

Post-operative Junctional Tachycardia
 Post-operative Junctional Tachycardia

• Address what is modifiable

– Temperature
– Electrolytes (K, Ca+2, Mg+2)
– Catecholamines
– Sedation (Precedex)
Rajput et al. Ann Pediatr Cardiol 2014; Chrysostomou et al. Anesth Analg 2008;
Munoz et al. Anesth Analg 2008

• Restore AV Synchrony
– IV Amiodarone or pacing
• Initiate early treatment
– Faster time to rate and rhythm control
– Less amiodarone
– Shorter ICU LOS Hass et al. JTCVS 2008
 Post-operative Complete Heart Block

• AV synchrony is important
– Transesophageal atrial sensing
• No data on efficacy of steroids

Spontaneous recovery in
66% of patients
• 95% by POD #10

Weindling et. al., Am. J. Cardiol. 1998

 Pulmonary Artery Hypertension
(PAH)

• Most patients with high flow, high pressure and

low PVR lesions (CHF) will not require therapy
for postoperative PAH
• Risk stratification is mainly clinical as cardiac
catheterization and lung biopsy are not
routinely performed
• Risk factors:
– Extracardiac syndromes (Down syndrome)
– LA hypertension
Bando et al. JTCVS 1996
Lindberg et al. JTCVS 2002
 PAH Crisis

• What is it?
– Spasm of pulmonary arteries and vasculature
– Results in decreased pulmonary blood flow
– Decreased pulmonary blood flow leads to
decreased cardiac output

• What can cause it?

– Increased PVR
– Agitation, pain, and suctioning
– Spontaneous
 PAH Crisis

Symptoms
1. No ∆ or HR (early)
2. Acute  in PA pressure **The key is to avoid a crisis!
• No unnecessary
3. CVP/RA
stimulation
4.  End Tidal CO2 • May require pre-
5.  sats medication before care
6.  pulses& perfusion such as ETT suctioning
7. HR BP ( late) • Recognize symptoms and
intervene early
8. Metabolic acidosis
 PAH Crisis Management

• 100% FiO2
• Sedation, +/- paralysis
• Sodium bicarb administration
• Bag patient, slightly hyperventilate
• Minimum amount of PEEP to keep FRC
• Nitric oxide (iNO)
• Volume administration

If these treatments fail: CPR/resuscitation

ECMO
 CAVC and Post-Bypass iNO

• 124 patients, prospective, randomized, placebo-

controlled study of prophylactic iNO 10 ppm
– Infants at high risk for pulmonary hypertensive crises
– 30% had CAVC
• No differences in mortality
– (5 iNO vs. 3 placebo, p=0.49)
• Conclusion: routine
prophylactic use of iNO in
high risk patients reduces
PAH crises and time to
extubation
Miller et al. Lancet 2000
 iNO and Milrinone for Post
CPB PAH

• 90 patients randomized in three groups:

• iNO 30 ppm
• Milrinone 0.5 mcg/kg/min
• iNO 30 ppm + milrinone 0.5 mcg/kg/min
• Mean PAP recorded
• Baseline, 10 and 20 minutes after drug start, 10 minutes
after cessation in drug regimen
• Larger decrease in mPAP with both drugs: 16% greater
(p<0.05)
• Conclusion: both drugs together produce larger
decrease in mPAP in post-CPB
Khazin et al. J Cardiothorac Vasc Anesth 2004
 Conclusions
• AV Canal is associated with Down Syndrome; thus other
considerations of Down Syndrome are relevant for
management (Hypothyroid, Vascular access, Sedation)
• Patients without pre-op CHF indicates increased PVR and
should heighten concern for pulmonary hypertension
symptoms post-operatively
• Common post-operative problems include:
– LCOS
– Arrhythmias (JET or CHB)
– Residual lesions (left AVVR, ASD/VSD, or LVOTO)
– PAH
• Consider the PCICS Postoperative Management Guidelines