Median Arcuate Ligament Syndrome with Orthostatic Intolerance: Intermediate-Term Outcomes following Surgical Intervention.

OBJECTIVES: To report the intermediate-term outcome following surgical intervention for median arcuate ligament syndrome (MALS) in adolescents and young adults with orthostatic intolerance (OI) to assess clinical improvement in the gastrointestinal and 5 other functional domains and if relief of arterial obstruction is associated with resolution of clinical symptoms. STUDY DESIGN: Thirty-one patients were given 2 dysautonomia-designed questionnaires to assess changes in symptoms following operative intervention in 6 functional domains and underwent postoperative repeat abdominal ultrasound examinations. RESULTS: Average follow-up after surgery was 22.4 ± 14.8 months. Self-assessed quality of health on a Likert scale (1-10 with 10 being normal) improved from 4.5 ± 2.1 preoperatively to 5.3 ± 2.4 postoperatively (P = not significant). Gastrointestinal symptoms of abdominal pain, nausea, and vomiting improved in 63% (P = .007), 53% (P = .040), and 62% (P = .014) of patients, respectively. Cardiovascular symptoms of dizziness, syncope, chest pain, and palpitations improved in 45% (P = not significant), 50% (P = not significant), 54% (P = .043), and 54% (P = .037) of patients, respectively. Transabdominal ultrasound peak supine expiratory velocity decreased from 348 ± 105 cm/s preoperatively to 251 ± 109 cm/s at 6 months or more after a ligament release procedure. Decrease of the postoperative celiac artery Doppler velocity was not associated with an improvement in gastrointestinal symptoms (P = .075). CONCLUSIONS: Adolescent and young adult patients with median arcuate ligament syndrome and OI have a good response to surgical intervention. About two-thirds of patients report significant improvement in symptoms of abdominal pain, nausea, and vomiting. Despite these encouraging data, many patients with MALS and OI continue to have an impaired quality of health.

Treadmill exercise testing improves diagnostic accuracy in children with concealed congenital long QT syndrome.

BACKGROUND: Resting electrocardiogram (ECG) identification of long QT syndrome (LQTS) has limitations. Uncertainty exists on how to classify patients with borderline prolonged QT intervals. We tested if exercise testing could help serve to guide which children with borderline prolonged QT intervals may be gene positive for LQTS. METHODS: Pediatric patients (n = 139) were divided into three groups: Controls (n = 76), gene positive LQTS with borderline QTc (n = 21), and gene negative patients with borderline QTc (n = 42). Borderline QTc was defined between 440-470 (male) and 440-480 (female) ms. ECGs were recorded supine, sitting, and standing. Patients then underwent treadmill stress testing with Bruce protocol followed by a 9-minute recovery phase. RESULTS: Supine resting QTc, age, and Schwartz score for the three groups were: (a) gene positive: 446 ± 23 ms, 12.4 ± 3.4 years old, 3.2 ± 1.8; (b) gene negative: 445 ± 20 ms, 12.1 ± 2 years old, 2.0 ± 1.2; and (c) control: 400 ± 24 ms, 15.0 ± 3 years old. The three groups could be differentiated by their QTc response at two time points: standing and recovery phase at 6 minutes. Standing QTc ≥460 ms differentiated borderline prolonged QTc patients (gene positive and gene negative) from controls. Late recovery QTc ≥480 ms distinguished gene positive from gene negative patients. CONCLUSION: Exercise stress testing can be useful to identify children who are gene positive borderline LQTS from a normal population and gene negative borderline QTc children, allowing for selective gene testing in a higher risk group of patients with borderline QTc intervals and intermediate Schwartz scores.

Utility of Diagnostic Studies for Upper Gastrointestinal Symptoms in Children with Orthostatic Intolerance.

OBJECTIVE: To assess the utility of gastrointestinal (GI) diagnostic studies in the evaluation of patients with orthostatic intolerance. STUDY DESIGN: Medical records of 103 consecutive children/young adults with orthostatic intolerance and gastrointestinal symptoms were reviewed. All patients had undergone antroduodenal manometry in conjunction with the tilt table test, autonomic testing, and upper gastrointestinal endoscopy (EGD). A gastric emptying study (GES) was performed in 81 patients. RESULTS: The median age of the cohort was 17 years (IQR, 15-19) with a female predominance (females:males, 3:1). As expected, the tilt table test was abnormal in all patients. Antroduodenal manometry was abnormal in 83 of 103 patients (81%), showing neurogenic intestinal dysmotility in 50%, rumination in 20%, and visceral hyperalgesia in 10%. The GES results were abnormal in 23 of 81 patients (28.4%), mostly (21 of 23) with delayed GES. None of the tilt table test or autonomic results were predictive of abnormal antroduodenal manometry or GES. Analysis of EGD biopsy samples revealed nonspecific esophagitis and/or gastritis in 16 of 103 patients (15%). CONCLUSIONS: Antroduodenal manometry with the tilt table test were the most insightful investigations in adolescents and young adults with orthostatic intolerance and gastrointestinal symptoms. GES and EGD provided limited information. Gastrointestinal symptoms were related more to functional rather than mucosal or organic etiologies, suggesting a limited role of endoscopy alone in evaluating patients with orthostatic intolerance presenting with gastrointestinal symptoms.

Comparison of the Medtronic SelectSecure and conventional pacing leads: Long-term follow-up in a multicenter pediatric and congenital cohort.

INTRODUCTION: The Medtronic SelectSecure™ (Minneapolis, MN, USA) pacing lead (SS) has theoretical advantages compared to conventional (C) transvenous pacing leads (PLs). The study purpose was to determine whether differences in electrical function and lead survival exist between these PLs in a large data set of pediatric and congenital patients. METHODS: A multicenter historical longitudinal cohort study was performed comparing SS and CPL performance over a 72-month follow-up (FU). Ten centers provided data for both SS and CPL, matched for age, implanted pacing chamber, time period of implantation, and presence of heart disease. RESULTS: The cohort consisted of 141 subjects in each group. No statistical differences were observed in age, gender, presence of heart disease, or pacing indication. Atrial and ventricular capture thresholds were stable throughout FU and higher in the SS group (atrial: 0.75 ± 0.02 vs 0.5 ± 0.04 V, ventricular: 1.0 ± 0.04 vs 0.75 ± 0.04 V), P < 0.001. Group PL sensing thresholds did not differ. The SS group required greater energy to pace (atrial: 0.57 ± 0.05 vs 0.32 ± 0.02 mJ, ventricular: 0.83 ± 0.05 vs 0.56 ± 0.06 mJ), P = 0.001. Early lead dislodgement and phrenic nerve stimulation were greater in the SS group (P = 0.03). Long-term lead survival was high and similar between the two groups, P = 0.35. CONCLUSIONS: Long-term survival of both PL was high with a low fracture rate. The SS had excellent electrical function but did show higher capture thresholds and increased energy to pace; these differences are offset by other advantages of the SS PL.

Ablation of the vanishing PVC, facilitated by quantitative morphology-matching software.

BACKGROUND: Ablation of cardiac arrhythmias in children and teenagers often necessitates the use of anesthesia, which can suppress ventricular arrhythmias (VAs), making it difficult to map the site of origin using activation time (AT). Pace mapping, a technique employed to assist with VA origin localization, depends on subjective comparison of paced and targeted QRS morphology. We assessed the utility of a quantitative approach to paced QRS to VA morphology matching using the PaSo software (Carto 3, Biosense Webster), to localize the VA site of origin. METHODS: Twenty-four patients underwent 26 procedures for frequent VAs, 29 for targeted VA. If AT mapping was precluded due to infrequent VA, pace mapping was executed using the PaSo software, after regionalization based on targeted VA QRS morphology. RESULTS: Subjects were aged 1-32 (mean 14 ± 6) years; 10 were male. Heart disease was present in six patients. PVC frequency prior to onset of anesthesia was 15 ± 16/min, decreasing to 0-1 PVC/min in 17 cases prior to ablation. Arrhythmia localization was performed by AT mapping + PaSo (12) or PaSo only (17). Pace mapping exhibited an intraventricular gradient of percent QRS morphology match. Highest achieved QRS match averaged 96 ± 2%. Successful ablation (> 1-month follow-up) was achieved in 24/29 targeted VAs, 11/12 ablated using AT and pace mapping, and 13/17 VA ablated using pace mapping only, P  =  0.29. CONCLUSIONS: (1) Spontaneous VA frequency was markedly reduced following anesthesia, despite catecholamine administration. (2) Notwithstanding the ability to perform AT mapping, successful ablation can still be performed using pace mapping only, facilitated by the PaSo software.

Antroduodenal Manometry Is Abnormal in Children Presenting With Orthostatic Intolerance and Gastrointestinal Symptoms.

OBJECTIVES: Gastrointestinal (GI) symptoms of nausea, vomiting, and abdominal pain are common in patients with orthostatic intolerance (OI), including neurally mediated hypotension (NMH) and postural orthostatic tachycardia syndrome (POTS). Autonomic dysregulation is considered the underlying pathophysiology behind the cardiovascular symptoms of POTS. Because the autonomic nervous system also regulates GI motility, we hypothesized that patients with POTS and GI symptoms will have evidence of autonomic dysmotility of the upper GI tract. METHODS: Thirty-five subjects with OI and GI symptoms were studied. All the subjects had a 24-hour antroduodenal manometry (ADM) study, in conjunction with pharmacologic challenge and autonomic and tilt table testing (TTT). RESULTS: The mean subject age was 16.2 ±â€Š2.8 years (range 10-23.8 years), and male to female ratio was 10:25. TTT was abnormal in all the 35 subjects, whereas Valsalva testing was abnormal (+40 mmHG) in 21 of 34 (62%) subjects, and corrected QT interval was ≥0.44 seconds in 19 of 35 (54%) subjects. During TTT, GI symptoms were reproduced in 31 of 35 (89%) studies. ADM was found to be abnormal at baseline, before the TTT in 5 of 35 (14%) subjects, whereas it became abnormal in 23 of 34 (68%) subjects during TTT. In addition, the expected response to the pharmacologic challenge was limited. Overall, ADM was abnormal in 26 of 35 (74%) patients either at baseline or during TTT in these subjects with OI. CONCLUSIONS: ADM is frequently abnormal in children with OI and GI symptoms. Upper GI motility studies should be a part of the comprehensive evaluation in this population.

Intravenous Hydration for Management of Medication-Resistant Orthostatic Intolerance in the Adolescent and Young Adult.

Orthostatic intolerance (OI) is common in teenagers (T) and young adults (A). Despite treatment with oral fluids, medication, and exercise, a significant number have symptoms from multiple organ systems and suffer low quality of life (QOL). Previous studies showed that acute intravenous (IV) hydration (IH) could help restore orthostatic tolerance; however, no data are available about the intermediate-term effects of IH. We therefore studied the efficacy of IH to improve QOL and manage medication-refractory OI patients. Our study population consisted of 39 patients (mean age = 16.1 ± 3.3) years; thirty-two were female. Average number of medications failed = 3.1. Average QOL score on self-reported OI questionnaire was 4.2 (normal QOL = 10). IV hydration consisted of normal saline (1-2 l/day, 3-7 days/week). 1) Orthostatic testing revealed Postural Orthostatic Tachycardia (24), Neurally Mediated Hypotension (14) or OI (1). 2) Average orthostatic change in heart rate was 48 ± 18 bpm. 3) IH was performed via intermittent IV access (10), PICC line (22), and Port (7). 4) Duration of IH varied from 1 week to 3.8 years (mean = 29 ± 47 weeks). 5) Overall, 79 % (n = 31) demonstrated clinically improved self-reported QOL. 6) Six patients who discontinued IH requested to restart treatment. (7) Complications consisted of upper extremity deep vein thrombosis (n = 3) and infection (n = 4). IH is an effective therapy to improve QOL in T&A with medication-resistant OI. Most patients continued to report improved QOL once IH was discontinued. IH should be considered a therapeutic option in medication-resistant OI patients with low QOL.

Magnesium Lowers the Incidence of Postoperative Junctional Ectopic Tachycardia in Congenital Heart Surgical Patients: Is There a Relationship to Surgical Procedure Complexity?

Magnesium sulfate was given to pediatric cardiac surgical patients during cardiopulmonary bypass period in an attempt to reduce the occurrence of postoperative junctional ectopic tachycardia (PO JET). We reviewed our data to evaluate the effect of magnesium on the occurrence of JET and assess a possible relationship between PO JET and procedure complexity. A total of 1088 congenital heart surgeries (CHS), performed from 2005 to 2010, were reviewed. A total of 750 cases did not receive magnesium, and 338 cases received magnesium (25 mg/kg). All procedures were classified according to Aristotle score from 1 to 4. Overall, there was a statistically significant decrease in PO JET occurrence between the two groups regardless of the Aristotle score, 15.3 % (115/750) in non-magnesium group versus 7.1 % (24/338) in magnesium group, P < 0.001. In the absence of magnesium, the risk of JET increased with increasing Aristotle score, P = 0.01. Following magnesium administration and controlling for body weight, surgical and aortic cross-clamp times in the analyses, reduction in adjusted risk of JET was significantly greater with increasing Aristotle level of complexity (JET in non-magnesium vs. magnesium group, Aristotle level 1: 9.8 vs. 14.3 %, level 4: 11.5 vs. 3.2 %; odds ratio 0.54, 95 % CI 0.31-0.94, P = 0.028). Our data confirmed that intra-operative usage of magnesium reduced the occurrence of PO JET in a larger number and more diverse group of CHS patients than has previously been reported. Further, our data suggest that magnesium's effect on PO JET occurrence seemed more effective in CHS with higher levels of Aristotle complexity.

Postoperative junctional ectopic tachycardia: risk factors for occurrence in the modern surgical era.

BACKGROUND: Postoperative (PO) junctional ectopic tachycardia (JET) can be a life-threatening arrhythmia that follows surgical repair of congenital heart disease (CHD) and results in PO morbidity. METHODS: We reviewed 750 open heart surgeries (OHS) for CHD performed between January 2005 and February 2009. Kaplan-Meier and Cox proportional hazards model analyses were used to estimate the frequency and evaluate risk factors that might predict JET occurrence. RESULTS: The patients ranged in age from 1 day to 36.6 years; half were less than 4.8 months at the time of OHS. JET occurred in 115 of 750 (15.3%) OHS. JET was bimodally distributed by age with a peak incidence between 1-2 weeks and 1-3 years. JET occurred more commonly: (1) in specific types of OHS (single ventricle [19.5%] and cono-truncal defects [19.3%]) (P = 0.03); (2) with increased total surgical time (P = 0.001), aortic cross-clamp time (P < 0.001), cardiopulmonary bypass time (P < 0.001); and (3) followed use of inotropic agents (dopamine or milrinone, P < 0.001). JET lengthened intensive care stay by 3 days (P = 0.0001) and increased mortality (+JET [9.6%] vs -JET [4.6%], P = 0.03). In a multiple variable Cox regression model, total surgical time and PO use of milrinone were the best predictors for JET risk. PO administration of nitroprusside decreased risk of JET. CONCLUSIONS: JET occurred more commonly following OHS associated with prolonged surgical times and PO use of inotropic medications. In contrast to previous reports, our results suggest that mechanical injury to the atrioventricular node area is not strongly associated with JET.

Lower risk of postoperative arrhythmias in congenital heart surgery following intraoperative administration of magnesium.

OBJECTIVES: Postoperative arrhythmias are common in children undergoing congenital heart surgery. We evaluated whether intraoperative administration of magnesium was associated with a reduced occurrence of specific postoperative arrhythmias, as has been described previously, or had a broader effect on multiple arrhythmia types, and whether there existed a dose-effect of intraoperative magnesium. METHODS: We used a historical prospective observational cohort study. Propensity score matching using logistic regression was applied to establish similar populations of treatment groups balanced on 5 important covariates. RESULTS: Based on propensity score-matched groups, magnesium 50 mg/kg administration was associated with a reduced odds of occurrence of postoperative arrhythmias: any arrhythmia (odds ratio [OR] = 0.50, P < .001), junctional ectopic tachycardia (OR = 0.56, P = .004), accelerated junctional rhythm (OR = 0.56, P = .089), atrial tachycardia (OR = 0.48, P = .005), ventricular tachycardia (OR = 0.52, P = .04), and atrioventricular block (OR = 0.47, P = .03). The effect of magnesium on reducing the odds of occurrence of postoperative arrhythmias did not differ between 25 mg/kg versus 50 mg/kg. CONCLUSIONS: As many as one third of our patients undergoing congenital heart surgery had postoperative arrhythmias. Use of intraoperative magnesium was associated with a reduction in the odds of occurrence of all postoperative arrhythmias. Our results do not provide evidence that a greater dose of magnesium is associated with greater arrhythmia risk reduction. Despite the intraoperative use of magnesium, there continued to be a high residual incidence of postoperative arrhythmias, raising the question for studying new or additional agents.

Characterizing the angiogenic activity of patients with single ventricle physiology and aortopulmonary collateral vessels.

OBJECTIVES: Patients with single ventricle congenital heart disease often form aortopulmonary collateral vessels via an unclear mechanism. To gain insights into the pathogenesis of aortopulmonary collateral vessels, we correlated angiogenic factor levels with in vitro activity and angiographic aortopulmonary collateral assessment and examined whether patients with single ventricle physiology have increased angiogenic factors that can stimulate endothelial cell sprouting in vitro. METHODS: In patients with single ventricle physiology (n = 27) and biventricular acyanotic control patients (n = 21), hypoxia-inducible angiogenic factor levels were measured in femoral venous and arterial plasma at cardiac catheterization. To assess plasma angiogenic activity, we used a 3-dimensional in vitro cell sprouting assay that recapitulates angiogenic sprouting. Aortopulmonary collateral angiograms were graded using a 4-point scale. RESULTS: Compared with controls, patients with single ventricle physiology had increased vascular endothelial growth factor (artery: 58.7 ± 1.2 pg/mL vs 35.3 ± 1.1 pg/mL, P < .01; vein: 34.8 ± 1.1 pg/mL vs 21 ± 1.2 pg/mL, P < .03), stromal-derived factor 1-alpha (artery: 1901.6 ± 1.1 pg/mL vs 1542.6 ± 1.1 pg/mL, P < .03; vein: 2092.8 pg/mL ± 1.1 vs 1752.9 ± 1.1 pg/mL, P < .02), and increased arterial soluble fms-like tyrosine kinase-1, a regulatory vascular endothelial growth factor receptor (612.3 ± 1.2 pg/mL vs 243.1 ± 1.2 pg/mL, P < .003). Plasma factors and sprout formation correlated poorly with aortopulmonary collateral severity. CONCLUSIONS: We are the first to correlate plasma angiogenic factor levels with angiography and in vitro angiogenic activity in patients with single ventricle disease with aortopulmonary collaterals. Patients with single ventricle disease have increased stromal-derived factor 1-alpha and soluble fms-like tyrosine kinase-1, and their roles in aortopulmonary collateral formation require further investigation. Plasma factors and angiogenic activity correlate poorly with aortopulmonary collateral severity in patients with single ventricles, suggesting complex mechanisms of angiogenesis.

A preliminary evaluation of the effect of glove use by food handlers in fast food restaurants.

A study was conducted to determine whether the levels of selected microorganisms differed on foods handled by gloved and bare hands at fast food restaurants. Three hundred seventy-one plain flour tortillas were purchased from fast food restaurants and analyzed for Staphylococcus aureus, Escherichia coli, Klebsiella sp., coliform bacteria, and heterotrophic plate count bacteria. Approximately 46% of the samples were handled by workers wearing gloves compared with 52% of samples with bare hand contact. Coliform bacteria were found in 9.6% of samples handled by gloved workers and 4.4% of samples handled by bare hands, although this difference was not statistically significant. The distribution of heterotrophic plate count bacteria, a general measure of hygiene, was also higher in samples handled by gloved workers in one restaurant chain. The presence of E. coli, Klebsiella sp., and S. aureus was detected in one, two, and eight samples, respectively, and there were no significant differences between samples handled by gloved or bare hands. Neither direct contact of the tortilla with the food preparation surface nor gender of the worker affected the level of any organism tested. The observed tendency of food workers to wear the same pair of gloves for extended periods and complacency might account for the apparent failure of gloves to reduce or prevent bacterial contamination. The results further suggest that glove use might be counterproductive because workers might wash their hands less frequently when gloved.

Increased postoperative respiratory complications in heterotaxy congenital heart disease patients with respiratory ciliary dysfunction.

OBJECTIVE(S): Congenital heart disease (CHD) and heterotaxy patients have increased postoperative and respiratory complications. We recently showed CHD-heterotaxy patients can have respiratory ciliary dysfunction (CD) similar to that associated with primary ciliary dyskinesia, including low nasal nitric oxide and abnormal ciliary motion. In this study, we investigated whether CHD-heterotaxy patients with CD may have worse postsurgical outcomes. METHODS: We examined postsurgical outcome in 13 heterotaxy-CHD patients with CD (25 surgeries), compared with 14 heterotaxy-CHD patients without CD (27 surgeries). Outcome data were collected for each surgery, including respiratory complications, tracheostomy, use of inhaled ß-agonists or nitric oxide, length of hospital stay, days on ventilator, and death. RESULTS: The CD versus the no-CD CHD cohorts had similar Risk Adjustment in Congenital Heart Surgery-1 risk categories, repair track, age at surgery, and follow-up evaluation times. Respiratory complications (76% vs 37%; P = .006), need for tracheostomy (16% vs 0%; P = .047), and use of inhaled ß-agonists (64% vs 11%; P = .0001) all were increased significantly in heterotaxy-CHD patients with CD. No significant differences were detected in postoperative hospital stay, days on mechanical ventilation, or surgical mortality. A trend toward increased mortality for the CD group beyond the postoperative period was observed (33% vs 0%; P = .055) in patients younger than age 10 years. CONCLUSIONS: Our findings showed that heterotaxy-CHD patients with CD may have increased risks for respiratory deficiencies. Overall, there was a trend toward increased mortality in CD patients with intermediate follow-up evaluation. Because ß-agonists are known to increase ciliary beat frequency, presurgical screening for CD and perioperative treatment of CD patients with inhaled ß-agonists may improve postoperative outcomes and survival.