Abnormalities in serum biomarkers correlate with lower cardiac index in the Fontan population.

BACKGROUND: Fontan survivors have depressed cardiac index that worsens over time. Serum biomarker measurement is minimally invasive, rapid, widely available, and may be useful for serial monitoring. The purpose of this study was to identify biomarkers that correlate with lower cardiac index in Fontan patients. Methods and results This study was a multi-centre case series assessing the correlations between biomarkers and cardiac magnetic resonance-derived cardiac index in Fontan patients ⩾6 years of age with biochemical and haematopoietic biomarkers obtained ±12 months from cardiac magnetic resonance. Medical history and biomarker values were obtained by chart review. Spearman's Rank correlation assessed associations between biomarker z-scores and cardiac index. Biomarkers with significant correlations had receiver operating characteristic curves and area under the curve estimated. In total, 97 cardiac magnetic resonances in 87 patients met inclusion criteria: median age at cardiac magnetic resonance was 15 (6-33) years. Significant correlations were found between cardiac index and total alkaline phosphatase (-0.26, p=0.04), estimated creatinine clearance (0.26, p=0.02), and mean corpuscular volume (-0.32, p<0.01). Area under the curve for the three individual biomarkers was 0.63-0.69. Area under the curve for the three-biomarker panel was 0.75. Comparison of cardiac index above and below the receiver operating characteristic curve-identified cut-off points revealed significant differences for each biomarker (p<0.01) and for the composite panel [median cardiac index for higher-risk group=2.17 L/minute/m2 versus lower-risk group=2.96 L/minute/m2, (p<0.01)]. CONCLUSIONS: Higher total alkaline phosphatase and mean corpuscular volume as well as lower estimated creatinine clearance identify Fontan patients with lower cardiac index. Using biomarkers to monitor haemodynamics and organ-specific effects warrants prospective investigation.

Prevalence and patterns of retention in cardiac care in young adults with congenital heart disease.

The population with adult congenital heart disease is expanding. Cardiac care retention and follow-up patterns were assessed in 153 adults with congenital heart disease (median age, 24.5 years), previously compliant as teenagers. The majority (125; 81.7%) were retained in care, most often by a pediatric cardiologist (69%). The rate of retention was surprisingly high.

Late dysphagia after radiotherapy-based treatment of head and neck cancer.

BACKGROUND: Changing trends in head and neck cancer (HNC) merit an understanding of the late effects of therapy, but few studies examine dysphagia beyond 2 years of treatment. METHODS: A case series was examined to describe the pathophysiology and outcomes in dysphagic HNC survivors referred for modified barium swallow (MBS) studies ≥ 5 years after definitive radiotherapy or chemoradiotherapy (January 2001 through May 2011). Functional measures included the penetration-aspiration scale (PAS), performance status scale-head and neck (PSS-HN), National Institutes of Health Swallowing Safety Scale (NIH-SSS), and MBS impairment profile (MBSImp). RESULTS: Twenty-nine patients previously treated with radiotherapy (38%) or chemoradiotherapy (62%) were included (median years posttreatment, 9; range, 5-19). The majority (86%) had oropharyngeal cancer; 52% were never-smokers. Seventy-five percent had T2 or T3 tumors; 52% were N+. The median age at diagnosis was 55 (range, 38-72). Abnormal late examination findings included: dysarthria/dysphonia (76%), cranial neuropathy (48%), trismus (38%), and radionecrosis (10%). MBS studies confirmed pharyngeal residue and aspiration in all dysphagic cases owing to physiologic impairment (median PAS, 8; median NIH-SSS, 10; median MBSImp, 18), whereas stricture was confirmed endoscopically in 7 (24%). Twenty-five (86%) developed pneumonia, half requiring hospitalization. Swallow postures/strategies helped 69% of cases, but no patient achieved durable improvement across functional measures at last follow-up. Ultimately, 19 (66%) were gastrostomy-dependent. CONCLUSIONS: Although functional organ preservation is commonly achieved, severe dysphagia represents a challenging late effect that may develop or progress years after radiation-based therapy for HNC. These data suggest that novel approaches are needed to minimize and better address this complication that is commonly refractory to many standard dysphagia therapies.

What is a clinically relevant difference in MDADI scores between groups of head and neck cancer patients?

OBJECTIVE/HYPOTHESIS: To describe clinically relevant between-group differences in MD Anderson Dysphagia Inventory (MDADI) scores among head and neck cancer (HNC) patients. STUDY DESIGN: Retrospective cross-sectional study was conducted in 1,136 HNC patients seen for modified barium swallow (MBS) studies. METHODS: The MDADI was administered by written questionnaire at the MBS appointment. MD Anderson Dysphagia Inventory global, composite, and subscale scores were calculated. Anchor-based methods were employed to determine clinically meaningful between-group differences by feeding tube status, aspiration status (per MBS study), and diet level. RESULTS: Mean MDADI scores for the 1,136 patients were: emotional 65.8 ± 17.3, functional 68.1 ± 19.6, physical 60.1 ± 18.6, global 59.3 ± 28.3, and composite 64.0 ± 17.1. Three hundred seventy-eight patients (33%) were feeding tube-dependent; 395 (34.8%) were aspirators; 122 (11%) were nothing per oral (Performance Status Scale-Head and Neck [PSS-HN] diet = 0); and 249 (22%) ate unrestricted, regular diets (PSS-HN diet = 100). Statistically significant (P < 0.0001) between-group differences (feeding tube vs. no feeding tube, aspirator vs. nonaspirator, oral vs. nonoral diet, PSS-HN diet levels) were observed for all mean MDADI scores (global, composite, and subscales). A mean difference of 10 points in composite MDADI scores differentiated feeding tube-dependent from nontube-dependent patients, aspirators from nonaspirators, and distinct PSS-HN diet levels. CONCLUSIONS: We identify that a 10-point between-group difference in composite MDADI scores was associated with clinically meaningful between-group differences in swallowing function. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:1108-1113, 2016.

Long-term functional and survival outcomes after induction chemotherapy and risk-based definitive therapy for locally advanced squamous cell carcinoma of the head and neck.

BACKGROUND: The purpose of this study was to evaluate long-term outcomes after induction chemotherapy followed by "risk-based" local therapy for locally-advanced squamous cell carcinoma of the head and neck (SCCHN). METHODS: Forty-seven patients (stage IV; ≥N2b) were enrolled in a phase II trial. Baseline and 24-month functional measures included modified barium swallow (MBS) studies, oropharyngeal swallow efficiency (OPSE), and the MD Anderson Dysphagia Inventory (MDADI). Functional status was assessed at 5 years. RESULTS: Five-year overall survival (OS) was 89% (95% confidence interval [CI], 81% to 99%). A nonsignificant 13% average reduction in swallowing efficiency (OPSE) was observed at 24 months relative to baseline (p = .191). MDADI scores approximated baseline at 24 months. Among 42 long-term survivors (median, 5.9 years), 3 patients (7.1%) had chronic dysphagia. The rate of final gastrostomy dependence was 4.8% (2 of 42). CONCLUSION: Sequential chemoradiotherapy achieved favorable outcomes among patients with locally advanced SCCHN, mainly of oropharyngeal origin. MBS and MDADI scores found modest swallowing deterioration at 2 years, and chronic aspiration was uncommon in long-term survivors.

Gastrostomy tube placement in patients with oropharyngeal carcinoma treated with radiotherapy or chemoradiotherapy: factors affecting placement and dependence.

BACKGROUND: Although many patients require nutritional support during radiotherapy or chemoradiotherapy for oropharyngeal cancer, little is known regarding the risk factors that predispose to gastrostomy tube (g-tube) placement and prolonged dependence, or the therapeutic interventions that may abrogate these effects. METHODS: We performed a retrospective medical chart review of patients who were treated for primary oropharyngeal cancer at a tertiary care center from 2003 to 2008. Patients who had a complete response at the primary site at 1-year posttreatment were included. G-tube placement and dependence ≥6 months were evaluated in relationship to site and stage of primary tumor, baseline characteristics, treatment type, smoking status, and swallowing intervention. RESULTS: We evaluated 474 patients (79%) with oropharyngeal cancer; 215 patients (40%) had concurrent chemotherapy, 73 patients (15%) had induction chemotherapy, and 69 patients (15%) had induction chemotherapy followed by concurrent chemotherapy. Two hundred ninety-three patients (62%) received g-tubes, of which 238 (81%) received the g-tube during radiation. At 1-year follow-up, 41 patients (9%) remained dependent on enteral feedings. Placement of g-tubes and prolonged g-tube dependence were significantly more likely in patients with T3 to 4 tumors (p < .001), baseline self-reported dysphagia (p < .001), odynophagia (p < .001), >10% baseline weight loss (p < .001), and in those treated with concurrent chemoradiotherapy. Patients who reported adherence to exercises had significantly lower rates of g-tube placement (p < .001), and duration of dependence was significantly shorter in those who reported adherence to swallowing exercises (p < .001). CONCLUSION: Almost 40% of patients with oropharyngeal cancer treated with nonsurgical organ preservation modalities may avoid feeding tube placement. Factors that predispose to g-tube placement and prolonged dependence include T3 to T4 tumors, concurrent chemotherapy, current smoking status, and baseline swallowing dysfunction or weight loss. Adherence to an aggressive swallowing regimen may reduce long-term dependence on enteral nutrition and limit the rate of g-tube placement overall.

Customization of the voice prosthesis to prevent leakage from the enlarged tracheoesophageal puncture: results of a prospective trial.

OBJECTIVES/HYPOTHESIS: Customization of the tracheoesophageal (TE) voice prosthesis (VP) is often preferred over surgical closure to prevent aspiration around the VP in laryngectomized patients with an enlarged tracheoesophageal puncture (TEP), but it has not been thoroughly evaluated. STUDY DESIGN: Single-institution prospective trial. METHODS: A prospective trial was conducted to evaluate the effectiveness of a customized VP with the addition of an enlarged tracheal and/or esophageal collar in patients with leakage around an enlarged TEP. Absence of leakage around the VP after placement defined immediate effectiveness. Long-term success was defined by the prevention of adverse events related to leakage during the study period. Events that defined failure included: permanent gastrostomy dependence, aspiration pneumonia, and/or surgical TEP closure. RESULTS: Twenty-one patients with enlarged TEP were enrolled (2003-2006). Insertion of a customized VP was unsuccessful in one patient; 145 customizations were performed in the remaining 20 patients (median, 3.5 customizations) during the trial period. Of the customizations, 77% (112/145) prevented leakage immediately after VP insertion. The most common adverse event was dislodgement of the prosthesis (11%) or the collar alone (7%) in 18% (26/145) of customized VP placements. Six patients who died of disease were not evaluable for long-term outcomes. Long-term success was achieved in 80% (12/15) of evaluable patients who avoided permanent gastrostomy, aspiration pneumonia, and surgical TEP closure. CONCLUSIONS: Prosthetic customization offers an effective method to prevent leakage around the VP in many patients with an enlarged TEP, thereby preserving TE voice while avoiding surgical closure in this high-risk population.

One slide fits all: the versatility of slide tracheoplasty with cardiopulmonary bypass support for airway reconstruction in children.

OBJECTIVES: This study describes results of tracheal reconstruction in children with slide tracheoplasty with cardiopulmonary bypass and identifies predictors for adverse outcomes. METHODS: Preoperative characteristics, operative variables, and outcome measures were collected for children undergoing slide tracheoplasty with cardiopulmonary bypass between April 2001 and October 2009. Predictors of worse outcomes were identified by bivariate analysis. Multiple regression analysis was performed for predictors of prolonged hospital stay. RESULTS: Cohort included 80 patients (median age, 8.7 months; 7 days-21 years). Forty-eight patients had associated cardiac or great vessel anomalies; 24 had simultaneous repair of cardiovascular anomaly at tracheal reconstruction. Fifty (63%) were extubated within 48 hours after operation. Median stay was 18.5 days (range, 7-119 days). Twenty-three patients (29%) required significant airway reintervention during median follow-up of 12 months (range, 4 months-7.8 years). There were 4 deaths, 2 early and 2 late. In bivariate analysis, age (P = .017), cardiopulmonary bypass duration (P = .025), and duration of mechanical ventilation (P < .05) were associated with mortality; duration of postoperative mechanical ventilation was associated with need for significant airway reintervention (P = .009). Multiple regression analysis indicated preoperative ventilatory support (P < .001), longer cardiopulmonary bypass (P = .002), previous airway operation (P = .01), and need for significant airway reintervention (P < .001) as predictors of longer hospital stay. CONCLUSIONS: Slide tracheoplasty with cardiopulmonary bypass can be performed with low mortality in a diverse pediatric population. This technique minimizes need for early significant airway reintervention in most cases.