Speech intelligibility is fundamental to social interactions and a critical surgical outcome in patients with cleft palate. Online crowdsourcing is a burgeoning technology, with potential to mitigate the burden of limited accessibility to speech-language-pathologists (SLPs). This pilot study investigates the concordance of online crowdsourced evaluations of hypernasality with SLP ratings of children with cleft palate. Methods Six audio-phrases each from children with cleft palate were assessed by online crowdsourcing using Amazon Mechanical Turk (MTurk), and compared to SLP's gold-standard hypernasality score on the Pittsburgh Weighted Speech Score (PWSS). Phrases were presented to MTurk crowdsourced lay-raters to assess hypernasality on a Likert scale analogous to the PWSS. The survey included clickable reference audio samples for different levels of hypernasality. Results 1,088 unique online crowdsourced speech ratings were collected on 16 sentences of 3 children with cleft palate aged 4-8 years, with audio averaging 6.5 years follow-up after cleft palate surgery. Patient 1 crowd-mean was 2.62 (SLP rated 2-3); Patient 2 crowd-mean 2.66 (SLP rated 3); and Patient 3 crowd-mean 1.76 (SLP rated 2). Rounded for consistency with PWSS scale, all patients matched SLP ratings. Different sentences had different accuracies compared to the SLP gold standard scores.
Background: Bidirectional Glenn shunt (BDG) failure carries high morbidity and mortality but the clinical factors associated with failure and the optimal management strategy are understudied. Methods: A total of 217 patients undergoing BDG at our institution between 1989 and 2020 were retrospectively reviewed and categorized as success or failure. Failure was defined as the need for reoperation (BDG takedown, reoperation for correction of cardiac defect, and/or transplantation) at any time postoperatively; operative mortality (death attributable to BDG malfunction occurring during the index hospitalization for BDG or within 30 days of discharge); or late mortality (death directly attributable to BDG malfunction occurring prior to Fontan or next-stage palliation). Univariate and binary logistic regression analyses were performed. Results: BDG failure occurred in 14 (6.5%) patients. Univariate predictors were: hypoplastic left heart syndrome ( P = .037), right ventricular (RV) dominance ( P = .010), greater pre-BDG pulmonary vascular resistance (PVR) ( P = .012), concomitant atrioventricular valve repair ( P = .020), prolonged pleural drainage ( P = .001), intensive care unit ( P<.001) and hospital ( P = .002) stays, and extracorporeal membrane oxygenation (ECMO) requirement ( P<.001). Multivariate predictors were: RV dominance ( P = .002), greater PVR ( P = .041), ICU ( P<.001) and hospital ( P = .020) stays, and need for ECMO ( P<.001). As many as 10 of 14 (71%) patients with BDG failure died. Reoperation was performed for 10 patients with BDG failure. Five reoperation patients survived until discharge, with four patients alive at last follow-up (mean 7.9 years). Survivors underwent reoperation earlier than nonsurvivors (36 vs. 94 days). Conclusions: BDG failure carries high mortality, but preoperative predictors and postoperative indicators of failure exist. Early BDG takedown and insertion of aorta-pulmonary shunt may allow survival.
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