Concern for dysphagia/aspiration

Infants with CHD are at risk for feeding and swallowing difficulties both pre- and postoperatively, with 18–56% of infants with CHD receiving a dysphagia diagnosis, depending on the cardiac lesion. ^{Reference Jones, Desai and Fogel34} Safe, efficient oral intake involves the integration of a complex system of sensory and motor neural pathways. Infants with CHD, however, may have poor state regulation, ^{Reference Gakenheimer-Smith, Glotzbach and Ou35} abnormal muscle tone, and difficulty coordinating breathing and swallowing due to neurodevelopmental delay and increased work of breathing. ^{Reference Butler, Sadhwani and Stopp36} These challenges may contribute to limited and overlooked breastfeeding opportunities.

Infants with CHD may also require noninvasive ventilation (e.g., nasal continuous positive airway pressure; high-flow nasal cannula) pre- and/or postoperatively. The risk of aspiration attributable to oral feeding during noninvasive ventilation is unclear. Evidence in preterm populations is inconsistent, ^{Reference Desai, Jones and Fogel37,Reference Canning, Clarke, Thorning, Chauhan and Weir38} while studies of term infants with bronchiolitis have described few adverse events and no aspiration pneumonia related to oral feeding on noninvasive ventilation. ^{Reference Gray, Lee and Levy39,Reference Conway, Halaby, Akerman and Asuncion40} Currently, there are no evidence-based oral feeding guidelines for infants on noninvasive ventilation; thus, clinical practices are driven by institutional guidelines, practice traditions, and clinician experience. Further research is needed to clarify the relationship between oral feeding (including breastfeeding) and aspiration during noninvasive ventilation of infants with CHD.

Interventions to treat CHD (e.g., surgery in proximity to the recurrent laryngeal nerve, ^{Reference Barr, Bowman and Deshpande41} prolonged intubation, extracorporeal membrane oxygenation) may increase the risk of vocal fold motion impairment, with subsequent increased risk of dysphagia and aspiration. ^{Reference Raulston, Smood and Moellinger42} The prevalence of aspiration-related complications in CHD is unknown, ^{Reference Raulston, Smood and Moellinger42} however, and there is little evidence about breastfeeding safety in the context of dysphagia or aspiration. One study including infants <6 months old reported significantly greater oral control, less nasopharyngeal backflow, and less laryngeal penetration during breastfeeding compared to bottle feeding in the same infants, using videofluoroscopic swallowing study (VFSS). ^{Reference Hernandez and Bianchini43} Similarly, Hersh et al. ^{Reference Hersh, Sorbo, Moreno, Hartnick, Fracchia and Hartnick44} found that 90% of infants aged 1–6 months with VFSS-diagnosed dysphagia (n = 80) had no pulmonary complications with continued breastfeeding and/or bottle-feeding human milk. In contrast, Duncan et al. ^{Reference Duncan, Golden, Larson, Williams, Simoneau and Rosen45} reported higher bronchoalveolar lavage markers of pulmonary inflammation in infants diagnosed with aspiration before age 12 months who were counselled to continue breastfeeding (n = 12), compared to those who were told to stop (n = 7). However, this study has a high risk for bias due to 75% loss to follow-up, no measurement of actual breastfeeding occurrence up to 1 year before follow-up lavage, and low statistical power. Given the inconsistent evidence, there are no accepted practice guidelines for breastfeeding infants with CHD who have dysphagia or aspiration, but the limited evidence suggests that dysphagia/aspiration are not automatic contraindications to breastfeeding.

For infants with CHD and confirmed dysphagia, we recommend early involvement of a feeding/swallowing specialist and an international board-certified lactation consultant (IBCLC) to evaluate several factors: (1) maternal milk supply and flow rate, (2) the infant’s capacity to latch effectively and coordinate with the milk flow, (3) the presence of specific etiological factors such as vocal fold motion impairment, (4) dysphagia severity, (5) the infant’s medical complexity, and (6) parent feeding goals. As demonstrated by Kenny et al.’s ^{Reference Kenny, McIntosh and Jardine46} implementation of a clinical pathway, collaborative and multidisciplinary assessment of these factors can inform appropriate use of targeted strategies to facilitate safe and efficient breastfeeding. Strategies to allow an infant to breastfeed while decreasing dysphagia/aspiration risk include adjustments to positioning, flow rate, and breastfeeding duration/frequency. In the absence of consensus-based pathways, we recommend close monitoring for signs and symptoms of aspiration, instrumental diagnostic assessment as indicated, and multidisciplinary creation of family-centred feeding plans individualised for an infant’s physiological stability and dysphagia risk.

Concerns related to weight gain

Infants with CHD may struggle to achieve age-appropriate growth, experience periods of energy deficit, and have higher rates of malnutrition than the general population. A 2022 meta-analysis found that 16.2% of North American children with CHD were underweight and 14.8% had wasting (i.e., weight-for-length z-score < −2), with higher rates preoperatively and a decrease to near-population-level malnutrition rates by 12 months postoperatively. ^{Reference Diao, Chen and Wei47}

The perioperative time may be a period of particularly poor growth for infants with CHD due to feeding interruption, ^{Reference Jones, Desai and Fogel34} systemic inflammation, ^{Reference Floh, Nakada and La Rotta48} and perioperative catabolism. ^{Reference Schricker and Lattermann49} Moza et al. ^{Reference Moza, Truong and Lambert50} found that 98.6% of US infants with single ventricle physiology experienced a decrease in weight for age z-score (WAZ) between stage 1 palliation and discharge. The authors concluded that most factors associated with this WAZ decrease were unavoidable as part of the infant’s clinical course or as a measure of disease severity. NEC was one potentially modifiable factor associated with poor growth, suggesting that feeding plans targeting NEC prevention (e.g., human milk) could improve growth for infants with CHD. ^{Reference Moza, Truong and Lambert50}

Common misconceptions among clinicians include that breastfeeding is more “work,” requires more energy and does not support adequate weight gain. The available evidence does not support these concerns, although data are somewhat limited. Studies of critically ill infants have demonstrated significantly lower cardiorespiratory stress with breastfeeding compared to bottle feeding; ^{Reference Marino, O’Brien and LoRe27,Reference Chen, Wang, Chang and Chi51} no difference in energy expenditure between breastfeeding and bottle feeding for preterm infants—despite longer breastfeeding session duration; ^{Reference Berger, Weintraub, Dollberg, Kopolovitz and Mandel52} and lower energy expenditure in gavage-fed preterm infants fed human milk compared to formula feeding in the same infants. ^{Reference Lubetzky, Vaisman, Mimouni and Dollberg53} Emerging evidence demonstrates similar or improved growth for infants with CHD fed human milk compared to formula ^{Reference Davis, Baumgartel and Baust24,Reference Elgersma, McKechnie and Schorr54,Reference Trabulsi, Lessen and Siemienski55} and no difference in 1–month and 12–month weight trajectories related to breastfeeding, ^{Reference Davis, Baumgartel and Baust24,Reference Elgersma, McKechnie and Schorr54,Reference Trabulsi, Lessen and Siemienski55} suggesting that concern about weight gain should not automatically preclude breastfeeding.

While high-energy fortification is common for infants with CHD, the literature on the topic is inconsistent. Moza et al. ^{Reference Moza, Truong and Lambert50} reported that higher caloric density was not associated with better growth by stage 1 palliation discharge, a finding echoed by Cui et al. ^{Reference Cui, Li and Hu56} and McCrary et al. ^{Reference McCrary, Clabby and Mahle57} Conversely, other studies have reported increases in weight status related to energy-enriched formulas or fortifiers, ^{Reference Blanco, Hair and Justice19,Reference Chen, Zhang and Song58–Reference Goday, Lewis and Sang62} although many of these studies exhibit risk for bias due to small and/or heterogeneous samples, ^{Reference Sahu, Singal and Menon59,Reference Scheeffer, Ricachinevsky and Freitas60,Reference Goday, Lewis and Sang62,Reference Zhang, Gu, Mi, Jin, Fu and Latour63} comparison of weight rather than WAZ, or substantial attrition (e.g., 68% in one study). ^{Reference Scheeffer, Ricachinevsky and Freitas60} Most studies reported more feeding intolerance in the high-energy intervention groups, with varying severity and significance. ^{Reference Cui, Li and Hu56,Reference Scheeffer, Ricachinevsky and Freitas60–Reference Zhang, Gu, Mi, Jin, Fu and Latour63} Many studies did not include infants who were breastfeeding or fed human milk, and only two studies were conducted in North America, ^{Reference Blanco, Hair and Justice19,Reference Goday, Lewis and Sang62} limiting generalisability. Consequently, optimal fortification strategies in the context of breastfeeding are unknown.

Furthermore, while studies have reported associations between low weight status and postoperative morbidity/mortality for infants with CHD, nearly all studies are retrospective, limiting causal inference. Studies display high variability in patient age range (e.g., neonates; ^{Reference Mitting, Marino, Macrae, Shastri, Meyer and Pathan64} 30 days–18 years ^{Reference Silva-Gburek, Marroquín and Flores65} ), weight conceptualisation (e.g., continuous WAZ, ^{Reference Ross, Latham and Joffe66} WAZ > or ≤ −2, ^{Reference Silva-Gburek, Marroquín and Flores65} WAZ decrease >0.67 ^{Reference Eskedal, Hagemo and Seem67} ), and measurement timing (e.g., birth, ^{Reference Steurer, Peyvandi and Costello68} admission, ^{Reference Silva-Gburek, Marroquín and Flores65} preop, ^{Reference Ross, Latham and Joffe66} postop ^{Reference Eskedal, Hagemo and Seem67} ), limiting comparability among studies. Ross et al.’s ^{Reference Ross, Latham and Joffe66} large, single-site study of children ages 0–5 found that the relationship between WAZ and postoperative mortality was not linear, with increased mortality risk only in cases of WAZ ≤ −2. In contrast, a large national registry analysis demonstrated an association between even mildly low WAZ at birth (−0.5 to −1) and increased risk of postoperative mortality. ^{Reference Steurer, Peyvandi and Costello68} Interestingly, excess weight was associated with mortality and adverse outcomes in another analysis of the same registry, ^{Reference Ross, Radman and Jacobs69} suggesting that not all weight gain is beneficial for children with CHD.

To date, a causal link between population-level higher weight status and improved clinical outcomes has not been established. A 2022 meta-analysis ^{Reference Singal, Sahu, Trilok Kumar and Kumar70} of the effect of energy- and/or protein-dense enteral feeding interventions on postoperative outcomes demonstrated no significant reduction in ventilator duration or length of stay for infants with CHD. Similarly, reports of large-scale feeding protocol implementation have described improved post-protocol WAZ but no differences in length of stay or mortality. ^{Reference O.’Neal Maynord, Johnson, Xu, Slaughter and Killen71,Reference Lisanti, Savoca and Gaynor72} While optimising nutrition, including age-appropriate weight gain, for critically ill infants remains a crucial developmental goal, it is plausible that poor weight status is largely a symptom of critical illness, and the benefit of targeting weight gain as a primary outcome for all infants with CHD is unclear.

Given the limits of the evidence on nutrition, growth, and breastfeeding for infants with CHD, several questions remain. First, it is unclear whether specific groups may benefit more or less from high-energy nutrition intervention; thus, tools for risk stratification are needed. Second, little is known about the optimal timing of high-energy intervention or the relationship between hospital weight gain trajectory and safe discharge/readmission outcomes. Third, there is a need for relevant measures of nutritional adequacy beyond weight gain (e.g., body composition, metabolic biomarkers, developmental indicators), which are currently underexplored in this population. Finally, research and quality improvement efforts are needed to identify methods of incorporating breastfeeding into nutrition plans for infants with CHD, particularly in the context of nutrition fortification. Individualised nutrition plans that consider both clinical needs and caregiver goals should be prioritised. The current evidence does not support automatic withholding of breastfeeding for all infants with CHD due to concern about weight gain.

Clinical instability/sickness

The CHD clinical course varies, and infants may experience periods of physiological instability and sickness. Historical concerns about preoperative feeding safety for newborns with critical CHD have been challenged by studies reporting no increased risk for NEC or other adverse outcomes associated with preoperative feeding. ^{Reference Mills, Kim and Fogg73–Reference Penk, Cagle and Holloway75} Writing for the Neonatal Cardiac Care Collaborative, Mills et al.’s ^{Reference Mills, Kim and Fogg73} comprehensive review concluded that evidence does not support universal preoperative feeding restriction, including for infants with ductal-dependent circulation or umbilical arterial catheters. Two subsequent publications provide further evidence to support this conclusion. First, a retrospective, multicentre study of preoperatively fed newborns with prostaglandin-dependent lesions reported NEC or adverse events in only 2 of 127 (1.6%) patients, with no lasting clinical implications. ^{Reference Penk, Cagle and Holloway75} Although 56% of these infants had an umbilical arterial catheter, most (56%) were primarily orally fed, with a median peak daily volume of 29 mL/kg/day. Similarly, Pappas et al. ^{Reference Pappas, Erickson, Ricketts, Moehlmann, Hahn and Daniel74} reported no increase in NEC following a preoperative enteral feeding protocol for infants with single ventricle physiology (65% with umbilical arterial catheter; 56% exclusive oral feeding), despite an increase in preoperative feeding from 39.5 to 75% and a mean volume of 28 mL/kg/day. These data suggest that most infants with critical CHD can safely feed preoperatively, with close clinical monitoring and stable or decreasing haemodynamic support. ^{Reference Mills, Kim and Fogg73} Moreover, these cohorts demonstrate that early oral feeding is often possible for infants with critical forms of CHD.

To date, most preoperative oral feeding evidence does not delineate between breast and bottle feeding, limiting clinical recommendations for preoperative breastfeeding support in the context of critical illness. Two additional clinical considerations impacting breastfeeding initiation and continuation for infants with CHD are volume restriction related to pulmonary overcirculation, low systemic circulation, or general haemodynamic instability; and safe out-of-bed mobilisation.

Developmental considerations

Primitive infant feeding reflexes facilitate the development of neuromotor pathways for successful feeding (suck-swallow-breathe) coordination. ^{Reference Lisanti, Demianczyk and Costarino91,Reference Glodowski, Thompson and Martel92} There is a sensitive 8–12 week “window of opportunity” to establish neuromotor pathways for feeding in neonates prior to infant feeding reflex integration. ^{Reference Lau93} For infants with CHD, life-saving medical and surgical interventions may interfere with this crucial timeline (Figure 2). ^{Reference Jones, Desai and Fogel34} Newborns with CHD may also exhibit neurobehavioural differences, including lower attention and state dysregulation, which are associated with poorer oral feeding outcomes. ^{Reference Gakenheimer-Smith, Glotzbach and Ou35}

Figure 2. Medical and surgical interventions for infants with CHD can interfere with the finite developmental window for learning oral feeding skills.

In light of these challenges, responsive, infant-driven, early oral feeding support is a critical component of individualised developmental care for infants with CHD. ^{Reference Delaney and Arvedson94} The developmentally supportive care model promotes individualised interventions that modify both caregiving practices and the environment, based on an infant’s behaviour and responses to stimuli, to optimise neurodevelopmental outcomes. ^{Reference Delaney and Arvedson94,Reference Lisanti, Uzark and Harrison95} Delayed oral feeding initiation has been associated with poor subsequent development of oral feeding skills for infants with CHD. ^{Reference Peterson and Evangelista96,Reference Dabbagh, Miller, McCulloch, Rosenthal, Conaway and White97} Breastfeeding is the biologically-normative, developmentally-appropriate first method of newborn oral feeding, and evidence supports a theory of “feeding imprinting,” in which an infant’s earliest oral feeding experience is foundational. ^{Reference Elgersma, Trebilcock and Whipple98,Reference Mobbs, Mobbs and Mobbs99} Studies in preterm and CHD populations align with this feeding imprinting theory, with breastfeeding as the first oral feeding method predicting not only breastfeeding at hospital discharge and beyond, but also human milk dose and duration throughout infancy. ^{Reference Mobbs, Mobbs and Mobbs99–Reference Pinchevski-Kadir, Shust-Barequet and Zajicek101} Considering that many infants with CHD experience nasogastric tube feeding both pre- and postoperatively, strategies to facilitate breastfeeding while transitioning from tube feeding are key. However, recommendations for initiation of breastfeeding after tube feeding are sparse and limited to preterm populations. ^{Reference Elgersma, Wolfson and Fulkerson102,Reference Medeiros, Oliveira and Fernandes103} Moreover, parents report that this transition is stressful, ^{Reference Davanzo, Strajn, Kennedy, Crocetta and De Cunto104} suggesting a critical need for cohesive, multidisciplinary clinical guidelines to support the transition from tube feeding to breastfeeding for infants with CHD.

Infants with CHD are never “too sick” or “too unstable” for individualised developmental care, including pre-feeding interventions to support oral feeding and breastfeeding that are appropriate for the infant’s physiologic state. The Newborn Individualized Developmental Care and Assessment Program (NIDCAP) and other developmental care frameworks emphasise that even the most critically ill infants benefit from individualised, neuroprotective, and family-integrated care strategies, which support physiologic stability and neurodevelopment. ^{Reference Madiba and Sengane105,Reference Als106} Interventions and strategies should evolve dynamically as the infant’s status changes throughout hospitalisation. Strategies to facilitate breastfeeding, including pre-feeding interventions, can be found in Table 2. Given the relatively fixed care requirements in the immediate postoperative time and considering the short window of opportunity before feeding reflex integration, we recommend initiating developmentally supportive breastfeeding interventions as early and as frequently as possible during the preoperative time. Two recent quality improvement projects suggest that allowing postnatal time for family bonding and skin-to-skin care is feasible and safe as early as the first hour after birth, with approximately 70% ^{Reference Westrup107} and 91% ^{Reference Ball, Seabrook and Corbitt108} of infants born with critical CHD successfully receiving this postnatal care.

Table 2. Breastfeeding facilitators for hospitalised infants, with considerations for use in CHD

Abbreviations: BF = breastfeeding; CHD; CLC = Certified Lactation Counselor; ECMO = extracorporeal membrane oxygenation; IBCLC = International Board Certified Lactation Consultant; RN = registered nurse; RT = respiratory therapist.

General breastfeeding challenges

Supportive breastfeeding care requires both clinicians and lactation specialists who have a comprehensive understanding of common breastfeeding challenges and can implement strategies to mitigate breastfeeding issues. ^{Reference Gelehrter, Blonsky, Kataria-Hale, Thomas, Strohacker and Laventhal109} Evidence-based lactation education for cardiac clinicians should focus on the importance of establishment and protection of milk supply via manual expression or pumping, assessment of adequate latch and milk transfer, and supportive communication about breastfeeding initiation and continuation. ^{Reference Gelehrter, Blonsky, Kataria-Hale, Thomas, Strohacker and Laventhal109,Reference Mercado, Vittner and McGrath110} For higher-level support of complicated breastfeeding challenges, IBCLCs and/or breastfeeding medicine specialists are imperative. ^{Reference Gelehrter, Blonsky, Kataria-Hale, Thomas, Strohacker and Laventhal109,Reference Mercado, Vittner and McGrath110} An IBCLC can offer expertise regarding the use of nipple shields, positioning for infants with ankyloglossia and tethered oral tissue, or pump flange sizing, and can explicitly support the breastfeeding dyad without other clinical requirements. ^{Reference Gelehrter, Blonsky, Kataria-Hale, Thomas, Strohacker and Laventhal109} Breastfeeding medicine specialists are board-certified physicians with additional training in lactation and breastfeeding. These specialists provide advanced lactation support, including diagnosis and medical management of lactation-related disorders such as mastitis. ^{Reference Mercado, Vittner and McGrath110}

The literature describes several successful models of unit-based human milk and breastfeeding support for preterm infants, including the Spatz 10-Step and Breastfeeding Resource Nurse model, ^{Reference Rosen-Carole, Coyle and Eglash111} the Rush Mother’s Milk Club, ^{Reference Spatz D.L.Beyond112} the Encourage, Assess, Transition (EAT) protocol, ^{Reference Meier, Patel, Bigger, Rossman and Engstrom113} and Maastrup et al.’s ^{Reference Swanson, Elgersma and McKechnie114} nurse training programme focused on breastfeeding-supportive practices. These models incorporate staff lactation education and training, which aligns with parent reports of clinician advocates as key to breastfeeding success for infants with critical CHD. ^{Reference Elgersma, McKechnie, Sommerness, Tanner and Swanson10} A recent quality improvement project to improve human milk feeding in a paediatric cardiac ICU similarly incorporated broad-scale staff lactation training, along with more intensive “Lactation Superuser” education. ^{Reference Maastrup, Rom, Walloee, Sandfeld and Kronborg115} Taken together, the success of these ICU-based initiatives reinforces the need for specialised breastfeeding care to mitigate breastfeeding challenges in the context of maternal/infant separation, a noxious feeding environment, and competing care priorities in an acute setting.

Workflow and implementation

Implementing clinician education and multidisciplinary breastfeeding support may be challenging in the context of heavy clinical acuity, staff turnover, and specialised medicine. Postnatal physical separation of the infant/lactating parent dyad is common and can be exacerbated by separate birth and children’s hospitals. Parents of medically complex infants are often the strongest advocates for their child’s feeding plan, yet parents have described some clinicians as ambivalent, dismissive, uncomfortable, and even hostile towards their breastfeeding goals. ^{Reference Elgersma, McKechnie, Sommerness, Tanner and Swanson10,Reference Gauntt, Tucker, Dolan, Gajarski and Krawczeski116} The currently limited CHD-specific clinician lactation education may contribute to systemic barriers such as unhelpful or inaccurate advice, delayed lactation equipment provision, inadequate developmental care, inconsistent feeding documentation, and ignorance of institutional breastfeeding policy. ^{Reference Hookway, Brown and Grant117,Reference Hookway and Brown118} Lactation education opportunities for parents of infants with CHD are also limited. Given that prenatal lactation education is increasingly recognised as the gold standard, based on evidence of factors that facilitate breastfeeding, ^{Reference Miller, Elhoff and Alexander119} initiatives to incorporate CHD-focused feeding and lactation consultation into prenatal care (e.g., into fetal cardiology consults or prenatal CHD classes) may be particularly effective. We strongly recommend assessment and documentation of parent feeding intention and preparation for breastfeeding during the prenatal period. We also recommend that institutions explore video-based education, mobile lactation apps, and peer mentorship programmes to reach more families. Furthermore, there has been little investigation into best practices to support breastfeeding in the context of CHD beyond hospital discharge. Considering that parents of infants with CHD have reported conflicting messaging and inconsistent breastfeeding support throughout the prenatal, hospitalisation, and post-discharge periods, ^{Reference Elgersma, McKechnie, Sommerness, Tanner and Swanson10} we strongly recommend further research and practice initiatives to develop congruent, collaborative approaches to breastfeeding support across the care continuum.