Martha L. Clabby

Gastric fundoplication is effective in promoting weight gain in children with severe congenital heart defects.

AIM The aim of this study was to determine outcomes, including weight gain, morbidity, and mortality, of children with severe congenital heart disease who underwent fundoplication (FP) for gastroesophageal reflux disease. METHODS An institutional review board-approved retrospective review was conducted on all children with congenital heart disease who underwent FP from 1999 to 2005. Preoperative age, weight, cardiac procedures, postoperative weight, and mortality were extracted from medical records. The Wilcoxon signed rank, Wilcoxon rank sum, and log-rank tests were used; P value less than .05 was significant. All procedures were performed with dedicated cardiac anesthesia personnel with recovery in a cardiac intensive care unit. RESULTS Of 112 subjects identified, 37 (33%) had single ventricle (SV) physiology. The most frequent cardiac procedures performed were Norwood (33), pulmonary artery band (11), and systemic pulmonary artery shunt (11). A total of 104 laparoscopic FPs (with 2 conversions to open) and 8 open FPs were performed. The median preoperative age was 3 months, and weight percentile was 1.5%. From baseline, postoperative median weight percentiles increased to 4% at 3 months (P < .001) and to 20% at 5 years postoperatively (P = .004). Single ventricle physiology had no significant effect on outcomes. Postoperative mortality (< or =30 days) was 4.5% (5/112); 5-year survival was 74% (83/112). Five-year survival of SV subjects (59%) was significantly lower (P = .03) than that of the other subjects (81%). No significant difference in survival was seen between SV subjects with FP and all SV patients seen at our center during the study period. Only one death was directly related to antireflux surgery (SV subject). There were 8 patients who had recurrent gastroesophageal reflux disease: 4 were treated with reoperation, and 4 were treated medically. CONCLUSION Weight gain in this high-risk population can be expected after antireflux surgery. Mortality is high because of intrinsic disease, especially in the SV population. Fundoplications performed with the assistance of dedicated pediatric cardiac anesthesia personnel followed by recovery in a cardiac intensive care unit is possible with acceptable postoperative morbidity and mortality.

Association of Interstage Home Monitoring With Mortality, Readmissions, and Weight Gain A Multicenter Study from the National Pediatric Cardiology Quality Improvement Collaborative

Background— Daily home monitoring of oxygen saturation and weight has been reported to improve outcomes for patients with single-ventricle heart disease during the period between stage I palliation and stage II palliation. However, these studies have been limited to single institutions and used historical control subjects. Our objective was to determine the association of various interstage home monitoring strategies with outcomes using a multicenter cohort with contemporary control subjects. Methods and Results— We performed a retrospective cohort study using prospectively collected data from the National Pediatric Cardiology Quality Improvement Collaborative from 2008 to 2012. We compared interstage mortality, unscheduled readmissions, and change in weight-for-age Z score for various home monitoring strategies of oxygen saturation (n=494) or weight (n=472), adjusting for sex, syndrome, tricuspid regurgitation, arch obstruction, and shunt type. Overall interstage mortality was 8.1%, and 47% had ≥1 unscheduled readmission. We did not find any associations of home oxygen saturation or weight monitoring with mortality or readmission. Although there was no difference in weight-for-age Z score for daily (0.33±0.12) versus weekly (0.34±0.18, P=0.98) weight monitoring, daily home weight monitoring was superior to no home weight monitoring (−0.15±0.18; P<0.01). Conclusions— Home weight monitoring is associated with improved weight gain during the interstage period, but we did not find any benefits in other clinical outcomes for either home oxygen saturation monitoring or home weight monitoring.

Variation in Feeding Practices following the Norwood Procedure

OBJECTIVES To assess variation in feeding practice at hospital discharge after the Norwood procedure, factors associated with tube feeding, and associations among site, feeding mode, and growth before stage II. STUDY DESIGN From May 2005 to July 2008, 555 subjects from 15 centers were enrolled in the Pediatric Heart Network Single Ventricle Reconstruction Trial; 432 survivors with feeding data at hospital discharge after the Norwood procedure were analyzed. RESULTS Demographic and clinical variables were compared among 4 feeding modes: oral only (n = 140), oral/tube (n = 195), nasogastric tube (N-tube) only (n = 40), and gastrostomy tube (G-tube) only (n = 57). There was significant variation in feeding mode among sites (oral only 0%-81% and G-tube only 0%-56%, P < .01). After adjusting for site, multivariable modeling showed G-tube feeding at discharge was associated with longer hospitalization, and N-tube feeding was associated with greater number of discharge medications (R(2) = 0.65, P < .01). After adjusting for site, mean pre-stage II weight-for-age z-score was significantly higher in the oral-only group (-1.4) vs the N-tube-only (-2.2) and G-tube-only (-2.1) groups (P = .04 and .02, respectively). CONCLUSIONS Feeding mode at hospital discharge after the Norwood procedure varied among sites. Prolonged hospitalization and greater number of medications at the time of discharge were associated with tube feeding. Infants exclusively fed orally had a higher weight-for-age z score pre-stage II than those fed exclusively by tube. Exploring strategies to prevent morbidities and promote oral feeding in this highest risk population is warranted.

Results of Palliation With an Initial Modified Blalock-Taussig Shunt in Neonates With Single Ventricle Anomalies Associated With Restrictive Pulmonary Blood Flow

BACKGROUND We report our current-era results after placement of a modified Blalock-Taussig shunt (mBTS) as the first surgery in the multistage palliation strategy of neonates born with single ventricle (SV) anomalies associated with restrictive pulmonary blood flow. METHODS Between 2002 and 2012, 174 neonates with SV underwent mBTS. Competing risks analysis modeled events after mBTS (death or transplantation, transition to Glenn) and subsequently after Glenn (death or transplantation, transition to Fontan) and examined risk factors affecting outcomes. RESULTS Competing risks analysis showed that 2 years after mBTS 27% of patients had died or received transplantation and 73% had undergone Glenn. Five years after Glenn, 6% had died, 73% had undergone Fontan, and 21% were alive awaiting Fontan. Overall survival 8 years after mBTS was 68%. On multivariable analysis, risk factors for mortality were increased shunt size to weight ratio (hazard ratio [HR]:1.2 per 0.1 ratio increase [1.0 to 1.4], p = 0.015), postoperative extracorporeal membrane oxygenation (HR: 4.0 [2.2 to 7.4], p < 0.001), chromosomal and extracardiac malformations (HR: 2.5 [1.3 to 5.0], p = 0.008), cardiopulmonary bypass use (HR: 2.5 [1.3 to 4.5], p = 0.004), and underlying pulmonary atresia with intact ventricular septum (PAIVS) or atrial isomerism (HR: 2.1 [1.1 to 4.2], p = 0.035). CONCLUSIONS Palliation outcomes with mBTS in SV patients trail behind the generally improved results of congenital heart surgery. Several anatomic and patient-related factors continue to affect survival. Mortality is high in low weight neonates in whom a balance between overcirculation due to large shunt and low pulmonary blood flow and risk of occlusion due to small shunt is difficult to attain. Patients with chromosomal and extracardiac malformations, atrial isomerism, and PAIVS continue to be the most challenging and are associated with higher hospital and interstage mortality.

Heterotaxy syndrome and malrotation: Does isomerism influence risk and decision to treat

PURPOSE Controversy remains regarding the management of the asymptomatic heterotaxy syndrome (HS) patient with suspected intestinal rotational abnormalities. We evaluated the outcomes for our HS population to identify frequency of malrotation and identify characteristics of children who might benefit from expectant management. METHODS After IRB approval, a retrospective review of all patients treated for HS at a large tertiary care childrens hospital between January 2008 and June 2012 was performed. For the purpose of this paper, malrotation was defined as an operative note that described the presence of Ladds bands and a narrow mesentery. RESULTS Thirty-eight patients with HS were identified, including 18 who underwent abdominal exploration. Left atrial isomerisation (LAI) was identified in 13 individuals, and right atrial isomerisation (RAI) was noted in 25. The rate of surgical intervention did not vary between the 2 groups (54%). Malrotation was found in 8 patients: one with LAI and 7 with RAI. This difference in incidence was statistically significant (p=0.04). CONCLUSION These data suggest that the direction of atrial isomerisation influences the likelihood of true malrotation, where RAI patients are more likely to be malrotated. Given the inherent risk of surgery on this medically fragile patient population, surgeons should consider expectant management for asymptomatic LAI patients.

Caloric intake during the perioperative period and growth failure in infants with congenital heart disease.

Infants with congenital heart disease have impaired weight gain during the first several months of life. Efforts have focused on improving weight gain and nutritional status during the first months of life. Close examination of the data suggests that the immediate postoperative period is problematic. Etiology of this early growth failure should be identified to develop effective interventions. This is a retrospective study of neonates who underwent modified systemic–to–pulmonary artery shunt, including Norwood palliation, at Children’s Healthcare of Atlanta between January 2009 and July 2011. We analyzed growth from time of surgical intervention to hospital discharge. Measures of calculated weight-for-age Z-score (WAZ score) were performed using the World Health Organization’s Anthro Software (version 3.2.2, January 2011; WHO, Geneva, Switzerland). Seventy-three patients were identified. Eight patients did not meet inclusion criteria. Complete data were collected on the remaining 65 patients. Median caloric intake patients received was 50.4 [interquartile range (IQR) 41.6 to 63.6] calories/kg/day while exclusively on parental nutrition. At hospital discharge, the median WAZ score was −2.0 (IQR −2.7 to −1.2) representing an overall median WAZ score decrease of −1.3 (IQR −1.7 to −0.7) from time of shunt palliation to hospital discharge. Despite studies showing poor weight gain in infants with congenital heart disease after neonatal palliation, this study reports the impact of hospital-based nutritional practices on weight gain in infants during the immediate postoperative period. Our data demonstrate that actual caloric intake during the cardiac intensive care unit stay is substantially below what is recommended.

Severe aortopathy due to fibulin-4 deficiency: molecular insights, surgical strategy, and a review of the literature

Mutations in the EFEMP2 (alias FBLN4) gene, which encodes the extracellular matrix protein fibulin-4, lead to severe aortopathy with aneurysm formation and vascular tortuosity. The disease phenotype, termed autosomal recessive cutis laxa type 1B (ARCL 1B), is rare among heritable connective tissue diseases but becomes more likely when noting family consanguinity and loose, inelastic skin in the patient. Our patient presented with an intercurrent illness exacerbating upper airway obstruction due to compression from a large aortic aneurysm. Genetic testing eventually revealed the causative mutation. She was initially treated with an angiotensin II receptor blocker and beta-blocker and eventually underwent total thoracic aortic replacement via a two-stage elephant trunk-type procedure. She recovered well and is currently asymptomatic but will require lifetime follow-up due to residual vascular tortuosity and aneurysm risk. Conclusion: Better understanding of the importance of transforming growth factor beta signaling in the pathophysiology of aortopathies such as ARCL 1B has led to targeted medical therapies. Specific surgical techniques can lead to optimal outcomes in these patients.

Factors affecting death and progression towards next stage following modified Blalock-Taussig shunt in neonates.

OBJECTIVES The modified Blalock-Taussig shunt (BTS) is utilized to palliate neonates born with restrictive pulmonary blood flow including those with single ventricle (SV) or biventricular (BV) cardiac anomalies. We aim in the current study to report palliation outcomes of neonates with BTS and to examine factors affecting death and progression to the subsequent stage of palliation or repair. METHODS Between 2002 and 2012, 341 patients underwent BTS including 175 with SV and 166 with BV anomalies. Competing risk analysis modelled events after BTS (death or transplantation, transition to Glenn shunt or biventricular repair) and examined risk factors affecting outcomes. RESULTS SV patients had a higher incidence of extracorporeal membrane oxygenation (ECMO) support requirement (12 vs 4%, P = 0.004) and unplanned cardiac reoperation (14 vs 7%, P = 0.051) than their BV counterparts. Additionally, hospital mortality was higher in SV than in BV patients (15 vs 3%, P < 0.001). In SV patients, competing risk analysis showed that, 2 years following BTS, 27% of patients had died or received transplantation and 73% had undergone the Glenn shunt. On multivariable analysis, factors associated with time until death or transplantation prior to Glenn were cardiopulmonary bypass [hazard ratio (HR) 3.6 (2.0-6.4), P < 0.001], unplanned cardiac reoperation [HR 2.4 (1.3-4.6), P = 0.007], pulmonary atresia [HR 2.0 (1.1-3.7), P = 0.026] and the shunt size/weight ratio [HR 1.3 (1.1-1.4) per 0.1 increase, P = 0.001]. In BV patients, competing risk analysis showed that, 2 years following BTS, 13% of patients had died or received transplantation, 85% had undergone biventricular repair and 2% were alive without biventricular repair. On multivariable analysis, factors associated with time until death or transplantation prior to biventricular repair were genetic syndromes and extracardiac malformations [HR 6.1 (2.0-18.2), P = 0.001], weight ≤2.5 kg [HR 5.6 (2.0-16.0), P = 0.001] and male gender [HR 3.4 (1.1-11.0), P = 0.041]. CONCLUSIONS Palliation with BTS continues to be associated with significant operative morbidity and mortality. In addition to hospital death, there is an important interstage attrition risk prior to subsequent palliation or biventricular repair. Inherent patient characteristics (i.e. genetic syndromes and low weight) and anatomical details (i.e. SV, pulmonary atresia and concomitant cardiac anomalies) are associated with worse survival.

Patient and practice factors affecting growth of infants with systemic-to-pulmonary shunt.

BACKGROUND On recognising poor growth following neonatal palliation with a systemic-to-pulmonary shunt, we sought to determine how patient- and procedure-related factors impact growth, paying attention to the role of the primary cardiologist in this process. METHODS In a retrospective review, neonates (133 patients) receiving modified systemic-to-pulmonary artery shunts from 2002 to 2009 were studied and outpatient visits were reviewed. Patients with single- and two-ventricle circulations after shunt takedown were compared using weight-for-age z-score. RESULTS Single-ventricle patients had a higher weight-for-age z-score at neonatal surgery than two-ventricle patients (-0.4 ± 1.0 compared with -1.2 ± 0.9, with p < 0.001), but they had a greater drop in the weight-for-age z-score to the first outpatient visit (-1.1 ± 0.7 compared with -0.8 ± 0.7, with p = 0.02). After the first outpatient visit, the weight-for-age z-score was not significantly different between single-ventricle and two-ventricle patients. From multivariate analysis, a lower number of nutritional interventions by cardiologists was significantly associated with poor growth (p = 0.03). Poor growth was not associated with race, use of feeding tube, exclusive formula use, or proximity to surgical centre. CONCLUSION The significant drop in the weight-for-age z-score from neonatal surgery to first outpatient visit suggests that these patients may receive inadequate nutrition. The poorest performers received the least number of outpatient changes to their diet. This finding underscores the critical role of the primary cardiologist in optimising weight gain through adjustments in nutrition.

Is there a benefit to postoperative fluid restriction following infant surgery

OBJECTIVE Fluid restriction is often employed immediately following cardiac surgery in children. The goal of this approach is to achieve an early negative fluid balance, which theoretically should lead to less interstitial edema and earlier extubation. The purpose of this study was to determine whether time to negative fluid balance in infants after undergoing systemic-to-pulmonary artery shunt palliation impacts duration of mechanical ventilation and hospital length of stay. DESIGN This is a retrospective study of neonates who underwent a modified systemic-to-pulmonary artery shunt at a single institution. SETTING University hospital pediatric cardiac intensive care unit (CICU). PATIENTS Neonates who underwent a modified systemic-to-pulmonary artery shunt between January 1, 2009 and June 1, 2011. OUTCOME MEASURES Information collected included time to negative fluid balance (in hours), CICU and hospital length of stay (in days), and the number of patients who had delayed sternal closure and/or underwent cardiopulmonary bypass. RESULTS Data were available for 65 subjects. Median fluid administration in the 24 hours postoperatively was 43.9 cc/kg/day (interquartile range: 32.9-61.0). Mean time to negative fluid balance was 25.0 ± 12.8 hours. Time to negative fluid balance was not associated with time to extubation, CICU and hospital length of stay, or change in weight-for-age z-score at intensive care unit discharge. CONCLUSION Time to negative fluid balance is not associated with duration of mechanical ventilation, CICU, and hospital length of stay in patients after undergoing systemic-to-pulmonary artery shunt palliation. The utility of a restricted fluid strategy immediately following infant heart surgery is questionable.