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Nutrition algorithm for single ventricle infants

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Children with single ventricle congenital heart defects typically undergo a two or three staged palliation surgery to achieve completion of the Fontan or total cavopulmonary connection (TCPC), with a majority of these children requiring a systemic to pulmonary shunt in the neonatal period.

Neonates undergoing surgical repair for single ventricle physiology are at significant risk for growth failure between stage 1 palliation and stage 2 palliation surgery, with most international studies, aside from a few exceptions, reporting an acute decline in weight-for-age z-scores in the early postoperative phase and prior to the second surgery 6,13,16,20-24. Poor nutrition and growth can have significant impacts on post-operative outcomes, and on the long term morbidity and mortality in these children1,4,13,17

Faltering growth in infants with single ventricle physiology is multifactorial13,21,22-24 and includes; a hypermetabolic state, inadequate caloric intake with increased energy expenditure2,4,6,13,14, increased incidence of gastrointestinal morbidity5,7,11-13,oral motor feeding difficulties5,10-12,15,18,20 as well as multiple haemodynamic variances that impact on growth.

International research has demonstrated that growth can be improved with the introduction of standardized feeding protocols that focus on close nutritional surveillance and management particularly in the period between stage 1 and stage 2 palliation surgeries. Feeding guidelines have the potential to mitigate poor growth and have a positive impact on morbidity and potentially on survival of these infants3,7,8,10,19,22,23

The Paediatric and Congenital Cardiac Service has developed the following nutritional algorithms for infants with single ventricle physiology to assist in optimisation of nutrition, growth and establishment of oral feeding between stage one and stage two palliation surgery. These algorithms are based on recommendations from the National Pediatric Cardiology Quality Improvement Collaborative of the Joint Council on Congenital Heart Disease (JCCHD) USA 19, with consideration of the New Zealand health environment.

Nutrition Algorithms for all single ventricle infants

Table 1: Contraindications to enteral feeding

Relative contraindication/caution
Evidence of low cardiac output:
Poor perfusion
Blood pressure instability 
Consideration of NIRs or MVO2
Lactic acidosis 
Inotropic support * (LOW)

Evidence of potential gastrointestinal dysfunction:
Increasing abdominal girth
Gastrointestinal co-morbidities/anatomic abnormality
Excessive vomiting and/or diarrhoea
Excessive NGT aspirate (> 5 mls/kg)
Absolute contraindication
Signs/symptoms N.E.C
Blood in stool 
Bilious emesis 
Inotropic support * (HIGH)
*Inotropic support may differ during various phases of hospitalisation
Inotropic use in pre-operative period can indicate unstable systemic circulation - may not be safe to enterally feed
Inotropic use in infant with stabilised post-operative physiology - may be appropriate / safe to enterally feed when there is evidence of good systemic output while weaning inotropic support

Table 2: Oral Feeding Screen

Feeding screen to be conducted on all single ventricle infants post-operatively
Initial first sucking feed
Bedside nurse to be present for first feed (breast / bottle)
Feed to commence when cueing (e.g. waking, rooting, non-nutritive suck)

Observe feed for signs of physiological instability
Desaturation, cyanosis, tachypnoea, tachycardia, periodic respirations
Low cardiac output
Spillage of milk during feed
Wet breath sounds
Falling asleep during feed / distressed

If no sign of difficulty continue with establishing oral feed

Discontinue feed & referral to SLT for further evaluation if above signs noted

Bedside nurse to document "first feed" in notes
Length of feed
Any signs of physiological instability
Referrals to SLT, Lactation consultant as needed

Observe for abnormality in cry quality and strength (e.g. hoarse weak cry / stridor)
Consider referral to ENT especially if associated with increased work of breathing

Table 3: Promote establishment of oral feeds

Promote positive feeding cues Promote/encourage maternal milk supply

Promote skin to skin time where infant stable

Promote non-nutritive suck with pacifier where infant NBM

Observe for and record positive feeding cues (wakefulness / rooting / non-nutritive suck)

Educate caregivers to recognise positive infant feeding cues

Educate caregiver to recognise infant distress cues
Falling asleep during feed
Distressed or overly agitated
Colour change
Spillage of milk during feed
Wet breath sounds

Provide breast feeding resources for mother
Parents to view DVD Maximising milk production

Promote skin to skin time where infant stable

Assist mother when establishing breast feeding
Lactation referral /support as indicated

Ensure mother eating x3 meals/day plus snacks in between

Expressing aim for 8-12 x day
Minimum of x 7 /24 hrs
1-2 night-time expressions, especially in first 1-2 weeks is recommended
Length of expressions - 20 minutes or until milk stops flowing. Swap breasts frequently

Record maternal milk supply in 24 hours:
Initially small amounts and increases within 72 hrs post-birth and if expressing
Feeding diary for mother

Target volumes
300mls by day 5
500mls by day 7-14
700mls after week 1-3
Consider Domperidone when maternal milk supply limited

Table 4: Energy supplements for all Post-operative single ventricle infants

Referral to dietitian
Start energy supplement early - as soon as infant tolerating 120 mls/kg/day
Medical staff to commence energy supplement over weekend
Breast milk fed infants:
Start: Calogen
Prescribe 5ml/kg/day - total for the day (extra 23 kcal per kg per day):
Given in 8 divided doses (3 hourly feeds)
OR 12 divided doses (2 hourly feeds)

Example: 3kg baby x 5ml Calogen per kg = 15 ml Calogen /day
Baby feeds 8 feeds per day (q 3 hourly) 15÷8 = 1.8ml
Round up to nearest 0.5 ml = 2 mls q3hourly
Formula fed infants:
Ensure infant is on 1kcal/kg/ml infant formula.
Change to another standard formula once feed volume >150mls/kg/day
Contraindications and Cautions
Maximum energy supplementation of extra 30kcal/kg/day from calogen
Do not start energy supplements if infant has altered GI function i.e excessive NGT aspirate, diarrhoea and / or vomiting
Educate family to stop energy supplements if infant develops diarrhoea and / or vomiting

Table 5: Interstage Growth Surveillance


Pre discharge growth parameters are met per Home Monitoring Programme (HMP)
• Must reach 3kg or birth weight whichever the greater
• Gains 150-210grams / week, in week prior to discharge
• Individual infant weight goals are identified per anthropometric percentiles in discharge documentation

Feeding regimen established AND meeting growth percentiles
• minimum oral intake
- breast feed X 6 feeds / 24 hours ( " F" grade feed per breast feeding code)
- OR bottle feed > 120mls/kg/day
• Maximum volume if bottle feeding - 180mls/kg/day* (*volume depends on signs of CHF, tolerance, growth )
• Maximum energy supplement of extra 30kcal/kg/day from calogen
• Where receiving NGT feed top up - discharges to regional hospital (???)

Caregiver education
• trained to obtain and record daily weight and volume of enteral intake (bottle fed) OR number and quality of breast feeds / 24 hrs (breast fed)
• trained to notify community nurse / paediatrician for breaches in following nutritional criteria
- loss of 50 grams/ 24 hours
- failure to gain 20 grams weight in 3 day period
- feeding less than 6 feeds / 24 hrs (breast fed) OR < 120mls/kg/day (bottle fed)
• Review of services / supports / equipment needed
• Plan & goals communicated to local health team includes
- breeches in nutritional criteria
- AND expected weight gain per individual infant
Outpatient Surveillance

Daily: weight, quality of feeding (and volume if bottle fed) by Caregiver/ Parent
X2 weekly: review of weight, quality of feeding (and volume if bottle fed), energy supplement by Community nursing team
Monthly Interstage Nutritional Evaluation: review of weight, height, HC and plot anthropometic percentiles by NS PCCS and Dietitian, +/- Lead Consultant
 Faltering Growth

Infant breaches Nutritional Criteria
- loss of 50 grams/ 24 hours
- not achieving 20 grams weight gain over 3 day period
- feeding less than 6 feeds / 24 hrs (breast fed) OR < 120mls/kg/day (bottle fed)
- OR not achieving individual infant weight goals

Discuss with Community team

Discuss with Lead Cardiologist

When breach of HMP nutritional criteria limited to faltering growth without concurrent symptoms of progressive heart failure , anatomic problems or intercurrent illness then adjustments to nutritional plan can be attempted
- Review by community dietitian - check intake.
- Increase energy supplement to maximum of extra 30kcal/kg/day with calogen
- If breast fed consider bottle top ups
- If bottle fed consider increase volume (max 180mls/kg/day *increase volume only after consultation with cardiology team )
- Consider hospitalisation for NGT and/or nutritional intervention

Consideration of other causes
- Cardiac concerns (CHF / residual lesion etc)
- Vocal cord injury
- G.I. concerns (GORD /delayed gastric emptying)
- Syndromes
- Neurodevelopmental concerns
 Cardiac evaluation if

CHF or poor systemic output
Dropping oxygen saturations
Ongoing growth failure
Consider 12 lead ECG / ECHO / Catheter / CT /MRI as indicated
Consider Stage II surgery if faltering growth / weight plateau despite intervention
Nutrition Red Flags

Weight loss >50grams in 1 day
Not achieving 20 grams weight gain over 3 day period
Reduced feeding: frequency, duration, quality, volume if bottle fed
Feed intolerance, excessive vomiting and /or diarrhoea 


  1. Anderson, et al. (2009). Lower weight-for-age z score adversely affects hospital length of stay after the bidirectional Glenn procedure in 100 infants with a single ventricle. Journal of Thoracic & Cardiovascular Surgery; 138(2), 397-404.e391. 
  2. Anderson et al, (2012). Variation in growth of infants with a single ventricle. Journal of Pediatrics; 161:16-21.
  3. Braudis, N et al. (2009). Enteral feeding algorithm for infants with hypoplastic left heart syndrome poststage I palliation. Pediatric Critical Care; 10(4) 460-466.
  4. Bodil M.K. et al. (2013). Low energy intakes are associated with adverse outcomes in infants after open heart surgery. J Parenter Enteral Nutrition; 11, 37(2):254-60. 
  5. Davis, et al (2008). Feeding Difficulties and Growth Delay in Children with Hypoplastic Left Heart Syndrome versus d-Transposition of the Great Arteries. Pediatric Cardiology; 29(2), 328-333.

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Document Control

  • Date last published: 18 July 2016
  • Document type: Clinical Guideline
  • Services responsible: Paediatric Cardiology
  • Owner: Marion Hamer
  • Editor: Marion Hamer
  • Review frequency: 2 years