Dysphagia in infants with single ventricle anatomy following stage 1 palliation: Physiologic correlates and response to treatment

Abstract

Background Deficits in swallowing physiology are a leading morbidity for infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliation. Despite the high prevalence of this condition, the underlying deficits that cause this post-operative impairment remain poorly understood. Objective Identify the physiologic correlates of dysphagia in infants with functional single ventricles and systemic outflow tract obstruction following stage 1 palliative surgery. Methods Postoperative fiberoptic laryngoscopies and videofluoroscopic swallow studies (VFSS) were conducted sequentially on infants with functional single ventricles following stage 1 palliative surgery. Infants were dichotomized as having normal or impaired laryngeal function based on laryngoscopy findings. VFSS were evaluated frame-by-frame using a scale that quantifies performance within 11 components of swallowing physiology. Physiologic attributes within each component were categorized as high functioning or low functioning based on their ability to support milk ingestion without bolus airway entry. Results Thirty-six infants (25 male) were included in the investigation. Twenty-four underwent the Norwood procedure and twelve underwent the Hybrid procedure. Low function physiologic patterns were observed within multiple swallowing components during the ingestion of thin barium as characterized by ≥ 4 sucks per swallow (36%), initiation of pharyngeal swallow below the level of the valleculae (83%), and incomplete late laryngeal vestibular closure (56%) at the height of the swallow. Swallowing deficits contributed to aspiration in 50% of infants. Although nectar thick liquids reduced the rate of aspiration (p=0.006), aspiration rates remained high (27%). No differences in rates of penetration or aspiration were observed between infants with normal and impaired laryngeal function. Conclusions Deficits in swallowing physiology contribute to penetration and aspiration following stage 1 palliation among infants with normal and impaired laryngeal function. Although thickened liquids may improve airway protection for select infants, they may inhibit their ability to extract the bolus and meet nutritional needs.

Feeding methods for infants with single ventricle physiology are associated with length of stay during stage 2 surgery hospitalization

“…Systematic enteral feeding algorithms and home nutritional monitoring programs have F I G U R E 1 Flow chart of study population derived from NPC-QIC registry and feeding methods at discharge following S2P. 7,16 To our knowledge, this is the first multicenter study to examine feeding method at S2P discharge for this high-risk population, and define patient characteristics associated with feeding method during S2P hospitalization and discharge. 5,[13][14][15] Evidence-based protocols that encompass all phases of palliative surgery to guide decision making for introduction and advancement of oral feeding are not available.…”

“…23 Of note, many centers try to promptly extubate following 4-5 days or 1 week; therefore, the multicenter data definition of prolonged intubation >14 days was a limitation in our study. 7,24 Additional studies are needed to elucidate long-term implications for RLN injury and recovery, and swallowing outcomes. Therefore, although uncommon, prolonged intubation may be a marker of greater complications during the stage 2 admission (see Table 2).…”

“…4 Improvements in mortality were associated with the use of home monitoring and nutritional assessment programs. [5][6][7] In response, nutrition support has been established by a variety of feeding methods-ranging from total oral feedings, to a combination of oral and tube feedings, to total tube feedings. [5][6][7] In response, nutrition support has been established by a variety of feeding methods-ranging from total oral feedings, to a combination of oral and tube feedings, to total tube feedings.…”

Incidence of aspiration in infants with single‐ventricle physiology following hybrid procedure

“…Oropharyngeal dysphagia is a known complication for infants with congenital heart disease (CHD), though estimates of both incidence and severity of swallowing dysfunction are variable . Many infants born with CHD, including those with hypoplastic left heart syndrome (HLHS), require invasive cardiothoracic surgery within the first days of life.…”

“…Much of the data that exists regarding swallowing in infants with CHD is reported following the Norwood procedure. Only two studies specifically examine feeding and swallowing outcomes following the hybrid procedure . However, both of these studies were limited because rates of swallowing dysfunction were reported only among patients that were already suspected of having dysphagia, increasing the likelihood of subsequently identifying swallowing impairments during instrumental assessment.…”