Garick D. Hill

Restrictive Lung Disease is an Independent Predictor of Exercise Intolerance in the Adult with Congenital Heart Disease

BACKGROUND/OBJECTIVES Following repair of congenital heart disease (CHD), adult patients are at risk for reduced exercise capacity. Restrictive lung disease (RLD) may contribute to reduced exercise capacity in this population. The aim of this study was to determine the prevalence of RLD and its impact on exercise tolerance in the adult with CHD. METHODS One hundred consecutive adult patients with CHD, who underwent routine cardiopulmonary exercise testing with spirometry, were evaluated. Clinical data were obtained by retrospective chart review. RESULTS Patients from 10 major diagnostic groups were identified. The median age for the cohort was 31 years (range 18-63) and included 43 males and 57 females. Most patients, 79%, had at least one previous surgical procedure. Based on spirometry and flow/volume loops, 50 patients were classified as normal pulmonary function, 44 patients had patterns suggestive of RLD, 4 suggestive of mixed (obstructive and restrictive), and 2 indeterminate. Risk factors associated with RLD include history of multiple thoracotomies (odds ratio = 9.01, P =.05) and history of atrial arrhythmias (odd ratio = 4.25, P =.05). Overall, 56% of the patients had abnormal exercise capacity. Spirometry suggestive of RLD was a significant risk factor for decreased exercise capacity (odds ratio = 3.65, P =.03). Patients with spirometry suggesting RLD also had lower exercise duration (P =.004) and a higher New York Heart Association Functional Class (P =.02). History of previous surgery and decreased heart rate reserve were also significant risk factors for decreased exercise capacity. CONCLUSION Abnormal spirometry suggestive of RLD is common in the adult with CHD and is a significant risk factor for decreased exercise tolerance in this population. Further studies are needed to evaluate the relationship between RLD and exercise intolerance and its relationship to mortality in the adult with CHD.

Effect of feeding modality on interstage growth after stage I palliation: A report from the National Pediatric Cardiology Quality Improvement Collaborative

OBJECTIVES Achieving adequate growth after stage 1 palliation for children with single-ventricle heart defects often requires supplemental nutrition through enteral tubes. Significant practice variability exists between centers in the choice of feeding tube. The impact of feeding modality on the growth of patients with a single ventricle after stage 1 palliation was examined using the multiinstitutional National Pediatric Cardiology Quality Improvement Collaborative data registry. METHODS Characteristics of patients were compared by feeding modality, defined as oral only, nasogastric tube only, oral and nasogastric tube, gastrostomy tube only, and oral and gastrostomy tube. The impact of feeding modality on change in weight for age z-score during the interstage period, from stage 1 palliation discharge to stage 2 palliation, was evaluated by multivariable linear regression, adjusting for important patient characteristics and postoperative morbidities. RESULTS In this cohort of 465 patients, all groups demonstrated improved weight for age z-score during the interstage period with a mean increase of 0.3±0.8. In multivariable analysis, feeding modality was not associated with differences in the change in weight for age z-score during the interstage period (P=.72). Risk factors for poor growth were a diagnosis of hypoplastic left heart syndrome (P=.003), vocal cord injury (P=.007), and lower target caloric goal at discharge (P=.001). CONCLUSIONS In this large multicenter cohort, interstage growth improved for all groups and did not differ by feeding modality. With appropriate caloric goals and interstage monitoring, adequate growth may be achieved regardless of feeding modality and therefore local comfort and complication risk should dictate feeding modality.

Disparities in the prenatal detection of critical congenital heart disease

Prenatal diagnosis of critical congenital heart disease, which requires surgical or catheter intervention in the first 30 days of life, allows for delivery at a specialized center and can reduce preoperative morbidity and mortality. We sought to identify the risk factors for a missed prenatal diagnosis of critical congenital heart disease.

Feeding Dysfunction in Children with Single Ventricle Following Staged Palliation

OBJECTIVE To determine the prevalence of feeding dysfunction in children with single ventricle defects and identify associated risk factors. STUDY DESIGN Patients aged 2-6 years with single ventricle physiology presenting for routine cardiology follow-up at the Childrens Hospital of Wisconsin were prospectively identified. Parents of the patients completed 2 validated instruments for assessment of feeding dysfunction. Chart review was performed to retrospectively obtain demographic and diagnostic data. RESULTS Instruments were completed for 56 patients; median age was 39 months. Overall, 28 (50%) patients had some form of feeding dysfunction. Compared with a normal reference population, patients with single ventricle had statistically significant differences in dysfunctional food manipulation (P < .001), mealtime aggression (P = .002), choking/gagging/vomiting (P < .001), resistance to eating (P < .001), and parental aversion to mealtime (P < .001). Weight and height for age z-scores were significantly lower in subjects with feeding dysfunction (-0.84 vs -0.33; P < .05 and -1.46 vs -0.56; P = .001, respectively). Multivariable analysis identified current gastrostomy tube use (P = .02) and a single parent household (P = .01) as risk factors for feeding dysfunction. CONCLUSION Feeding dysfunction is common in children with single ventricle defects, occurring in 50% of our cohort. Feeding dysfunction is associated with worse growth measures. Current gastrostomy tube use and a single parent household were identified as independent risk factors for feeding dysfunction.

Surgical Valvotomy Versus Balloon Valvuloplasty for Congenital Aortic Valve Stenosis: A Systematic Review and Meta‐Analysis

Background Optimal initial treatment for congenital aortic valve stenosis in children remains unclear between balloon aortic valvuloplasty (BAV) and surgical aortic valvotomy (SAV). Methods and Results We performed a contemporary systematic review and meta‐analysis to compare survival in children with congenital aortic valve stenosis. Secondary outcomes included frequency of at least moderate regurgitation at hospital discharge as well as rates of aortic valve replacement and reintervention. Single‐ and dual‐arm studies were identified by a search of PubMed (Medline), Embase, and the Cochrane database. Overall 2368 patients from 20 studies were included in the analysis, including 1835 (77%) in the BAV group and 533 (23%) in the SAV group. There was no difference between SAV and BAV in hospital mortality (OR=0.98, 95% CI 0.5–2.0, P=0.27, I2=22%) or frequency of at least moderate aortic regurgitation at discharge (OR=0.58, 95% CI 0.3–1.3, P=0.09, I2=54%). Kaplan–Meier analysis showed no difference in long‐term survival or freedom from aortic valve replacement but significantly more reintervention in the BAV group (10‐year freedom from reintervention of 46% [95% CI 40–52] for BAV versus 73% [95% CI 68–77] for SAV, P<0.001). Results were unchanged in a sensitivity analysis restricted to infants (<1 year of age). Conclusions Although higher rates of reintervention suggest improved outcomes with SAV, indications for reintervention may vary depending on initial intervention. When considering the benefits of a less‐invasive approach, and clinical equipoise with respect to more clinically relevant outcomes, these findings support the need for a randomized controlled trial.

Feeding Dysfunction in Single Ventricle Patients with Feeding Disorder

PURPOSE To determine whether caregivers of children with single ventricle heart defects identified as having feeding disorder will report more frequent feeding dysfunction, or maladaptive mealtime behavior and/or interactions, when compared with reference populations. METHODS As part of routine evaluation, parents of children evaluated at the Feeding, Swallowing, and Nutrition Center at the Childrens Hospital of Wisconsin completed previously validated questionnaires to assess feeding dysfunction and parental stress. Parental responses for single ventricle patients were compared with all other children evaluated with a feeding disorder. RESULTS Questionnaires were completed in eight patients with single ventricle heart defects. The mean age was 36 ± 23 months, with five females (63%). Mean weight-for-age z-score was -1.4 ± 0.9. Compared with noncardiac feeding clinic children, there was more reported child resistance to eating (83 ± 15% vs. 44 ± 2%; P = .05). Single ventricle parents were more likely to report distress (50 ± 18% vs. 21 ± 2%; P = .04) and a difficult child (63 ± 17% vs. 31 ± 2%; P = .05). There was also more defensive responding among parents of single ventricle children (63 ± 17% vs. 29 ± 2%; P = .04). CONCLUSION Single ventricle patients evaluated for disordered feeding more frequently showed resistance to eating and parental distress than noncardiac feeding clinic patients. Parents of these children underestimated the degree of feeding difficulty by defensive responding and had more parental distress. These findings suggest that feeding dysfunction can contribute to longer-term feeding and growth problems in single ventricle patients with feeding disorder.

The Impact of Obesity on Postoperative Outcomes in Adults with Congenital Heart Disease Undergoing Pulmonary Valve Replacement

OBJECTIVE The impact of obesity on surgical morbidity in adults with congenital heart disease is currently unknown. The aim of our study was to investigate the impact of obesity on postoperative outcomes in adults with congenital heart disease undergoing reoperation for pulmonary valve replacement. METHODS A retrospective analysis was performed assessing the influence of obesity on surgical outcomes. Obesity was defined as a body mass index ≥30 kg/m2. RESULTS The mean body mass index of the cohort was 25.9 ± 6.9 kg/m2 . The cohort included 71 patients with 17 patients (24%) being obese. There was no postoperative mortality. Obese patients had a longer hospital length of stay (6.6 vs. 4.7 days; P < .001) and increased incidence of postoperative arrhythmias (29% vs. 5.6%; P = .003) compared with nonobese patients. Multivariable analysis performed using logistic regression with backwards elimination demonstrated obesity was independently associated with hospital length of stay >5 days (odds ratio [OR] = 5.2; 95% confidence interval [CI]: 1.5-18.2, P = .01) and with increased postoperative arrhythmias (OR = 4.2; 95% CI: 1.7-40, P < .01). CONCLUSIONS Obesity is associated with increased morbidity in adults with congenital heart disease undergoing pulmonary valve replacement, including longer hospitalization and higher risk for postoperative arrhythmias.

Assessment of Growth 6 Years after the Norwood Procedure.

&NA; At 6 years of age, patients with hypoplastic left heart syndrome had mean age‐adjusted z‐scores for weight and height below the normative population, and body mass index was similar to the normative population. Males had the greatest increase in z‐scores for body mass index. Trial registration ClinicalTrials.gov: NCT00115934.

Single-Stage Repair of Coarctation of the Aorta and Ventricular Septal Defect: A Comparison of Surgical Strategies and Resource Utilization:

Background: We sought to compare clinical outcomes and resource utilization for two surgical approaches for single-stage repair of coarctation of the aorta and ventricular septal defect (VSD). Methods: This was a retrospective chart review of 21 consecutive neonates and infants undergoing single-stage repair of coarctation of the aorta and VSD. Group 1 included 13 patients with both arch repair and VSD repair completed via sternotomy. Group 2 included eight patients with off-pump arch repair via left thoracotomy followed by repositioning and VSD repair via sternotomy. Primary clinical outcome was arch reintervention. Secondary outcomes included various measures of resource utilization. Results: Group 1 patients demonstrated younger age at repair (median of 10 days vs 57 days for group 2; P = .05) and lower proximal arch z scores (−4.2 vs −2.3 for group 2; P = .003). Arch reintervention occurred in 0 of 8 patients in group 2 and 1 (7.7%) of 13 patients in group 1 (P = nonsignificant). Group 2 was associated with lower total charges (US

Transplacental Treatment of Fetal Tachycardia: A Systematic Review and Meta-analysis

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