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OBJECTIVE: To identify delivery room (DR) characteristics of patients with transposition of the great arteries (TGA) who underwent preoperative balloon atrial septostomy (BAS). STUDY DESIGN: Retrospective cohort study of all patients with prenatally diagnosed TGA delivered at our center between 2013 and 2023 who underwent arterial switch operation during the newborn admission. RESULTS: A total of 168 patients were included (median gestational age 39.5 weeks, 64% male, 33% with ventricular septal defect, 8% with aortic arch hypoplasia). BAS was performed in 84 patients (50%). Patients who underwent BAS had higher proportion of intubation in the DR (87% vs 33%, P < .001), lower maximum oxygen saturation in the first 10 minutes (64% vs 74%, P < .001) and 20 minutes (71% vs 81%, P < .001) of life, and lower maximum oxygen saturation at any point in the DR (79% vs 87%, P < .001). Adjusting for confounders (ventricular septal defect, aortic arch anomaly, 5-minute Apgar, birth weight), intubation in the DR (aOR 9.5, 95% CI 3.9, 25.0) and lower maximum oxygen saturation in the DR (aOR 0.9, 95% CI 0.8, 0.9) were independently associated with BAS. By receiver operating characteristic analysis, a maximum oxygen saturation of less than 86% at any time point in the DR discriminated for BAS with a specificity of 0.88, sensitivity of 0.70, and area under the curve of 0.82. CONCLUSIONS: Intubation and lower oxygen saturation in the DR are independently associated with BAS in patients with TGA born at our center. A maximum DR saturation of less than 86% best discriminates patients who undergo BAS in our population.
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BACKGROUND: Observational studies have demonstrated an association between the use of digoxin and reduced interstage mortality after Norwood operation for hypoplastic left heart syndrome (HLHS). Digoxin use has increased significantly but remains variable between different hospitals, independent of case-mix. Instrumental variable analyses have the potential to overcome unmeasured confounding, the major limitation of previous observational studies and to generate an estimate of the attributable benefit of treatment with digoxin. METHODS: A cohort of neonates with HLHS born from January 1, 2007 to December 31, 2021 who underwent Norwood operation at Pediatric Health Information Systems Database hospitals and survived >14 days after operation were studied. Using hospital-specific, 6-month likelihood of administering digoxin as an instrumental variable, analyses adjusting for both unmeasured confounding (using the instrumental variable) and measured confounders with multivariable logistic regression were performed. RESULTS: The study population included 5,148 subjects treated at 47 hospitals of which 63% were male and 46% non-Hispanic white. Of these, 44% (n = 2,184) were prescribed digoxin. Treatment with digoxin was associated with superior 1-year transplant-free survival in unadjusted analyses (85% vs 82%, P = .02). This survival benefit persisted in an instrumental-variable analysis (OR: 0.71, 95% CI: 0.54-0.94, P = .01), which can be converted to an absolute risk reduction of 5% (number needed to treat of 20). CONCLUSIONS: In this observational study of patients with HLHS after Norwood using instrumental variable techniques, a significant benefit in 1-year transplant-free survival attributable to digoxin was demonstrated. In the absence of clinical trial data, this should encourage the use of digoxin in this vulnerable population.
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Sistemas de Información en Salud , Síndrome del Corazón Izquierdo Hipoplásico , Procedimientos de Norwood , Recién Nacido , Humanos , Niño , Masculino , Femenino , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Síndrome del Corazón Izquierdo Hipoplásico/tratamiento farmacológico , Digoxina/uso terapéutico , Resultado del Tratamiento , Factores de Riesgo , Procedimientos de Norwood/métodos , Estudios RetrospectivosRESUMEN
Infants with staged surgical palliation for congenital heart disease are at high-risk for interstage morbidity and mortality; home monitoring programs have mitigated these risks. In 2019, we instituted telemedicine (TM) in our established Infant Single Ventricle Monitoring Program. All consecutive patients discharged following neonatal operation/intervention were monitored until subsequent stage 2 surgical palliation. We offered TM (synchronous video) visits as part of regularly scheduled follow-up, replacing at least one in-person primary care visit with a TM cardiologist visit. We tracked emergency department (ED) visits, hospitalizations, how TM identified clinical concerns, and whether use of TM prevented unnecessary ED visits or expedited in-person assessment. We assessed caregiver and clinician satisfaction. Between 8/2019 and 5/2020, we conducted 60 TM visits for 29 patients. Of 31 eligible patients, 2 families (6.9%) declined. Median monitoring time was 199 days (range 75-264) and median number of TM visits/patient was 2 (range 1-5). In 6 visits (10%), significant clinical findings were identified which avoided an ED visit. Five TM visits led to expedited outpatient assessments, of which 1 patient required hospitalization. There were no missed events or deaths. Median ED visits/patient/month were significantly lower compared to the same calendar period of the prior year (0.0 (0-2.5) vs. 0.4 (0-3.7), p = 0.0004). Caregivers and clinicians expressed high levels of satisfaction with TM. TM for this high-risk population is feasible and effective in identifying clinical concerns and preventing unnecessary ED visits. TM was particularly effective during the COVID-19 pandemic, allowing for easy adaptation of care to ensure patient safety in this fragile cohort.
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COVID-19 , Cardiopatías Congénitas , Telemedicina , Recién Nacido , Lactante , Humanos , Pandemias , Cardiopatías Congénitas/cirugía , Alta del PacienteRESUMEN
Infants with staged surgical palliation for congenital heart disease are at high-risk for interstage morbidity and mortality. Interstage telecardiology visits (TCV) have been effective in identifying clinical concerns and preventing unnecessary emergency department visits in this high-risk population. We aimed to assess the feasibility of implementing auscultation with digital stethoscopes (DSs) during TCV and the potential impact on interstage care in our Infant Single Ventricle Monitoring & Management Program. In addition to standard home-monitoring practice for TCV, caregivers received training on use of a DS (Eko CORE attachment assembled with Classic II Infant Littman stethoscope). Sound quality of the DS and comparability to in-person auscultation were evaluated based on two providers' subjective assessment. We also evaluated provider and caregiver acceptability of the DS. From 7/2021 to 6/2022, the DS was used during 52 TCVs in 16 patients (median TCVs/patient: 3; range: 1-8), including 7 with hypoplastic left heart syndrome. Quality of heart sounds and murmur auscultation were subjectively equivalent to in-person findings with excellent inter-rater agreement (98%). All providers and caregivers reported ease of use and confidence in evaluation with the DS. In 12% (6/52) of TCVs, the DS provided additional significant information compared to a routine TCV; this expedited life-saving care in two patients. There were no missed events or deaths. Use of a DS during TCV was feasible in this fragile cohort and effective in identifying clinical concerns with no missed events. Longer term use of this technology will further establish its role in telecardiology.
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Cardiopatías Congénitas , Síndrome del Corazón Izquierdo Hipoplásico , Estetoscopios , Lactante , Humanos , Estudios de Factibilidad , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/terapia , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Soplos Cardíacos/diagnósticoRESUMEN
We report a case of hypoplastic left heart syndrome and with subsequent aortopathy and then found to have hereditary haemorrhagic telangiectasia/juvenile polyposis syndrome due to a germline SMAD4 pathologic variant. The patient's staged palliation was complicated by the development of neoaortic aneurysms, arteriovenous malformations, and gastrointestinal bleeding thought to be secondary to Fontan circulation, but workup revealed a SMAD4 variant consistent with hereditary haemorrhagic telangiectasia/juvenile polyposis syndrome. This case underscores the importance of genetic modifiers in CHD, especially those with Fontan physiology.
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Cardiopatías , Telangiectasia Hemorrágica Hereditaria , Corazón Univentricular , Humanos , Telangiectasia Hemorrágica Hereditaria/complicaciones , Telangiectasia Hemorrágica Hereditaria/diagnóstico , Telangiectasia Hemorrágica Hereditaria/genética , Corazón Univentricular/complicaciones , Mutación , Cardiopatías/complicaciones , Proteína Smad4/genéticaRESUMEN
OBJECTIVES: To characterize hepatic to systemic lymphatic connections in patients with systemic lymphatic disease using intra-hepatic lymphangiography and to compare outcomes after lymphatic intervention. METHODS: In this retrospective study, patients with intra-hepatic lymphangiography from May 2014 - April 2019 at our institution were included. Imaging review was performed and hepatic lymphatic connections and flow patterns were characterized. Clinical data were reviewed and comparisons between patients undergoing lymphatic intervention with or without abnormal hepatic lymphatics were performed. RESULTS: During the study period, 105 patients underwent intra-hepatic lymphangiography. Primary clinical presentation included ascites (19/105), chylothorax (27/105), plastic bronchitis (PB) (17/105), and protein losing enteropathy (PLE) (42/105). Five categories of hepatic lymphatic connections and flow patterns were identified (%): normal (25%, 26/105), hepatoperitoneal (12%, 13/105), hepatopulmonary (10.5%, 11/105), hepatomesenteric (7.5%, 8/105), and hepatoduodenal (41%, 43/105) with four patients having more than one abnormal pattern. A comparison between clinical presentation and imaging category revealed an increased likelihood of having ascites with hepatoperitoneal (p < .0001), chylothorax/PB with hepatopulmonary (p = .01), and PLE with hepatoduodenal (p < .001) connections. Seventy-six patients had a lymphatic intervention, 24% with normal, and 76% with abnormal liver lymphatics. There was no difference in length of hospital stay or mortality between the two groups, but there was a prolonged time to symptom resolution (p = .006) and persistent symptoms after 6 months (5% vs 44%, p = .002) in the group with abnormal liver lymphatics. CONCLUSION: We identified five liver lymphatic imaging categories with a substantial correlation to presenting lymphatic disease. Abnormal imaging patterns correlated with increased morbidity. Evaluation of liver lymphatics should be considered in patients with a systemic lymphatic disease if central lymphatic imaging is normal. KEY POINTS: ⢠We identified five liver lymphatic imaging patterns: normal, hepatoperitoneal, hepatomesenteric, hepatopulmonary, and hepatoduodenal. ⢠Imaging patterns were correlated with disease presentation (normal - chylothorax/PB, hepatoperitoneal - ascites/chylothorax, hepatopulmonary - chylothorax/PB, hepatoduodenal - PLE). ⢠Abnormal imaging patterns correlated with increased morbidity.
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Quilotórax , Enfermedades Linfáticas , Vasos Linfáticos , Humanos , Hígado/diagnóstico por imagen , Enfermedades Linfáticas/diagnóstico por imagen , Linfografía , Estudios RetrospectivosRESUMEN
Patients with dextro-transposition of the great arteries (d-TGA) require surgical repair as neonates. These patients are at risk for post-operative chylothorax. We sought to describe the presentation, imaging, and outcomes after intervention for patients with d-TGA with post-operative chylothorax. A retrospective chart review was performed in patients with repaired d-TGA who were referred from 1/1/2013 to 4/1/2020 for evaluation of chylothorax. Patient history, lymphatic imaging, and interventional data were collected. Impact of intervention on lymphatic drainage was evaluated with a student's t-test. Eight patients met inclusion criteria for this study. Five patients had a history of central venous thrombus leading to thoracic duct outlet occlusion. Five patients underwent intervention, two were managed conservatively, and one was not a candidate for intervention. Chylothorax resolved in six patients. There was a significant difference in output from 7 days prior to first intervention (114 mL/kg/day) compared to 28 days following final intervention (27 mL/kg/day, p = 0.034). There were no procedural complications. Chylothorax in patients with repaired transposition of the great arteries is often amenable to intervention. Early surveillance and management of central venous thrombosis may reduce the burden of lymphatic disease in these patients.
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Quilotórax , Transposición de los Grandes Vasos , Arterias , Quilotórax/etiología , Quilotórax/cirugía , Humanos , Recién Nacido , Complicaciones Posoperatorias/epidemiología , Estudios Retrospectivos , Transposición de los Grandes Vasos/cirugíaRESUMEN
OBJECTIVE: We compared 5-year outcomes of transcatheter pulmonary valve (TPV) replacement with the Melody TPV in the post-approval study (PAS) and the investigational device exemption (IDE) trial. BACKGROUND: As a condition of approval of the Melody TPV after the IDE trial, the Food and Drug Administration required that a PAS be conducted to evaluate outcomes of TPV replacement in a "real-world" environment. The 5-year outcomes of the PAS have not been published, and the IDE and PAS trials have not been compared. METHODS: The cohorts comprised all patients catheterized and implanted at 5 IDE sites and 10 PAS sites. Differences in trial protocols were detailed. Time-related outcomes and valve-related adverse events were compared between the two trials with Kaplan-Meier curves and log-rank testing. RESULTS: 167 patients (median age, 19 years) were catheterized and 150 underwent TPV replacement in the IDE trial; 121 were catheterized (median age, 17 years) and 100 implanted in the PAS. Freedom from hemodynamic dysfunction (p = .61) or any reintervention (p = .74) over time did not differ between trials. Freedom from stent fracture (p = .003) and transcatheter reintervention (p = .010) were longer in PAS, whereas freedom from explant (p = .020) and TPV endocarditis (p = .007) were shorter. Clinically important adverse events (AEs) were reported in 14% of PAS and 7.2% of IDE patients (p = .056); the incidence of any particular event was low in both. CONCLUSIONS: Hemodynamic and time-related outcomes in the PAS and IDE trials were generally similar, confirming the effectiveness of the Melody TPV with real-world providers. There were few significant complications and limited power to identify important differences in AEs. The lack of major differences in outcomes between the two studies questions the usefulness of mandated costly post-approval studies as part of the regulatory process for Class III medical devices.
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Implantación de Prótesis de Válvulas Cardíacas , Prótesis Valvulares Cardíacas , Insuficiencia de la Válvula Pulmonar , Válvula Pulmonar , Adolescente , Adulto , Cateterismo Cardíaco/efectos adversos , Implantación de Prótesis de Válvulas Cardíacas/efectos adversos , Humanos , Diseño de Prótesis , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/cirugía , Insuficiencia de la Válvula Pulmonar/cirugía , Resultado del Tratamiento , Adulto JovenRESUMEN
Quality improvement efforts have focused on reducing interstage mortality for infants with hypoplastic left heart syndrome (HLHS). In 1/2016, two publications reported that use of digoxin was associated with reduced interstage mortality. The degree to which these findings have affected real world practice has not been evaluated. The discharge medications of neonates with HLHS undergoing Norwood operation between 1/2007 and 12/2018 at Pediatric Health Information Systems Database hospitals were studied. Mixed effects models were calculated to evaluate the hypothesis that the likelihood of digoxin prescription increased after 1/2016, adjusting for measurable confounders with furosemide and aspirin prescription measured as falsification tests. Interhospital practice variation was measured using the median odds ratio. Over the study period, 6091 subjects from 45 hospitals were included. After adjusting for measurable covariates, discharge after 1/2016 was associated with increased odds of receiving digoxin (OR 3.9, p < 0.001). No association was seen between date of discharge and furosemide (p = 0.26) or aspirin (p = 0.12). Prior to 1/2016, the likelihood of receiving digoxin was decreasing (OR 0.9 per year, p < 0.001), while after 1/2016 the rate has increased (OR 1.4 per year, p < 0.001). However, there remains significant interhospital variation in the likelihood of receiving digoxin even after adjusting for known confounders (median odds ratio = 3.5, p < 0.0001). Following publication of studies describing an association between digoxin and improved interstage survival, the likelihood of receiving digoxin at discharge increased without similar changes for furosemide or aspirin. Despite concerted efforts to standardize interstage care, interhospital variation in pharmacotherapy in this vulnerable population persists.
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Antiarrítmicos/uso terapéutico , Digoxina/uso terapéutico , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Procedimientos de Norwood/métodos , Alta del Paciente , Bases de Datos Factuales , Prescripciones de Medicamentos/estadística & datos numéricos , Femenino , Sistemas de Información en Salud , Hospitales Pediátricos , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/tratamiento farmacológico , Recién Nacido , Masculino , Oportunidad Relativa , Farmacoepidemiología/estadística & datos numéricos , Farmacoepidemiología/tendencias , Mejoramiento de la Calidad , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
In patients with total cavopulmonary connections, elevated central venous pressures (CVP) have detrimental effects on the lymphatic system causing an imbalance in fluid production and drainage of the interstitium. This combination may result in life-threatening lymphatic complications including plastic bronchitis (PB), protein losing enteropathy (PLE), chylothorax, and ascites. While embolization of the abnormal lymphatics has greatly improved outcomes from these complications, alternative treatment strategies have been proposed that would result in improved lymphatic drainage while leaving the lymphatic system intact. We report two novel transcatheter approaches for thoracic duct (TD) decompression in two patients who developed PLE after completion of the Fontan procedure as part of staged palliation for congenital heart disease. In addition, one patient had severe concurrent PB. In both patients, a connection was created between a left superior vena cava (LSVC) to the left atrium allowing for a nonsurgical method to decompress the TD. This procedure resulted in significant clinical and laboratory improvement of both patients' PLE and other symptoms of lymphatic dysfunction.
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Bronquitis/terapia , Cateterismo Cardíaco , Procedimiento de Fontan/efectos adversos , Cardiopatías Congénitas/cirugía , Enfermedades Linfáticas/terapia , Enteropatías Perdedoras de Proteínas/terapia , Conducto Torácico/fisiopatología , Bronquitis/diagnóstico , Bronquitis/etiología , Bronquitis/fisiopatología , Cateterismo Cardíaco/instrumentación , Catéteres Cardíacos , Niño , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/fisiopatología , Hemodinámica , Humanos , Enfermedades Linfáticas/diagnóstico por imagen , Enfermedades Linfáticas/etiología , Enfermedades Linfáticas/fisiopatología , Masculino , Cuidados Paliativos , Enteropatías Perdedoras de Proteínas/diagnóstico , Enteropatías Perdedoras de Proteínas/etiología , Enteropatías Perdedoras de Proteínas/fisiopatología , Stents , Conducto Torácico/diagnóstico por imagen , Resultado del TratamientoRESUMEN
OBJECTIVES: To evaluate short- and middle-term outcomes after transcatheter patent ductus arteriosus (TC-PDA) closure in small infants, specifically device-related left pulmonary artery (LPA) stenosis and aortic coarctation, risk factors, and changes over time. BACKGROUND: Recent studies have demonstrated successful transcatheter PDA (TC-PDA) closure in small infants. LPA stenosis and aortic coarctation have been seen after TC-PDA, but it is not clear whether device-related LPA/aortic obstruction persists. METHODS: A single-center retrospective study of infants ≤4 kg who underwent TC-PDA closure from February 1, 2007 to September 1, 2018 was performed, evaluating the incidence and risk factors for LPA stenosis and coarctation. RESULTS: Forty-four patients underwent successful TC-PDA with Amplatzer Vascular Plug II (AVPII; n = 30), Amplatzer Duct Occluder II-Additional Sizes (n = 10), Amplatzer Duct Occluder I (n = 3), and coil-filled AVPI (n = 1) devices, all via an antegrade approach. Median birthweight and procedural weight were 890 g (range: 490-3,250) and 2.8 kg (range: 1.2-4.0), respectively. Median follow-up was 0.7 years (range: 2 days-7 years). Thirty-eight patients had post-procedure echocardiograms assessing LPA/aortic obstruction. Of those, 17 had LPA flow acceleration/stenosis (≥1.5 m/s), which improved or resolved in all patients with available follow-up; 3 developed mild coarctation (>2 m/s), which improved in the two with more than short-term follow-up; 4 developed mild flow acceleration (1.5-2 m/s) in the descending aorta, which resolved in three and increased in one (2.4 m/s). Flow acceleration in the LPA was associated with younger procedural age, larger PDA minimal diameter, and placement of a device other than the AVPII. There was no device-related mortality or need for reintervention. CONCLUSION: TC-PDA in small infants is effective, without significant complications. Device-related LPA/aortic obstruction can improve with time/growth.
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Coartación Aórtica/epidemiología , Cateterismo Cardíaco/efectos adversos , Conducto Arterioso Permeable/terapia , Dispositivo Oclusor Septal/efectos adversos , Estenosis de Arteria Pulmonar/epidemiología , Factores de Edad , Coartación Aórtica/diagnóstico por imagen , Peso al Nacer , Cateterismo Cardíaco/instrumentación , Conducto Arterioso Permeable/diagnóstico por imagen , Femenino , Humanos , Incidencia , Lactante , Recién Nacido de Bajo Peso , Recién Nacido , Masculino , Estudios Retrospectivos , Factores de Riesgo , Estenosis de Arteria Pulmonar/diagnóstico por imagen , Factores de Tiempo , Resultado del TratamientoRESUMEN
The aim of this study was to determine the feasibility of using contrast-enhanced ultrasound (CEUS) evaluation to determine thoracic duct (TD) outlet patency. Nine patients referred for lymphatic imaging and intervention underwent percutaneous intranodal ultrasound contrast injection and conventional lymphangiography (CL). Eight of 9 patients had a patent TD by CEUS and CL. One patient did not have a patent TD. There was 100% agreement between CEUS and CL. These results suggest that CEUS is an imaging modality that might be as accurate as CL in determining TD patency.
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Medios de Contraste/administración & dosificación , Enfermedades Linfáticas/diagnóstico por imagen , Conducto Torácico/diagnóstico por imagen , Ultrasonografía , Adolescente , Niño , Preescolar , Estudios de Factibilidad , Femenino , Humanos , Lactante , Enfermedades Linfáticas/fisiopatología , Masculino , Valor Predictivo de las Pruebas , Estudios Retrospectivos , Conducto Torácico/fisiopatologíaRESUMEN
Pulmonary lymphatic flow disorders involve the abnormal lymphatic flow via lymphatic channels to the lungs and pleural space. Plastic bronchitis and chylothorax are the main complications of this abnormal lymphatic perfusion, which has been termed pulmonary lymphatic perfusion syndrome (PLPS). Following lymphatic access, dynamic contrast MR lymphangiography is the imaging modality of choice to diagnose these disorders. Management includes medical therapy, percutaneous interventions under fluoroscopy, and surgical interventions.
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Bronquitis/diagnóstico por imagen , Quilotórax/diagnóstico por imagen , Vasos Linfáticos/diagnóstico por imagen , Linfografía , Imagen por Resonancia Magnética , Adolescente , Bronquitis/terapia , Niño , Preescolar , Quilotórax/terapia , Medios de Contraste , Dietoterapia , Suplementos Dietéticos , Manejo de la Enfermedad , Embolización Terapéutica , Humanos , Lactante , Enfermedades Pulmonares/diagnóstico por imagen , Enfermedades Pulmonares/terapia , Anomalías Linfáticas/diagnóstico por imagen , Anomalías Linfáticas/terapia , Enfermedades Linfáticas/diagnóstico por imagen , Enfermedades Linfáticas/terapia , Vasos Linfáticos/anomalías , Vasos Linfáticos/cirugía , MicrocirugiaRESUMEN
OBJECTIVES: In-hospital complications after the Norwood operation for single ventricle heart defects account for the majority of morbidity and mortality. Inpatient care variation occurs within and across centers. This multidisciplinary quality improvement project standardized perioperative management in a large referral center. DESIGN: Quality improvement project. SETTING: High volume cardiac center, tertiary care children's hospital. PATIENTS: Neonates undergoing Norwood operation. INTERVENTIONS: The quality improvement team developed and implemented a clinical guideline (preoperative admission to 48 hr after surgery). The composite process metric, Guideline Adherence Score, contained 13 recommendations in the guideline that reflected consistent care for all patients. MEASUREMENTS AND MAIN RESULTS: One-hundred two consecutive neonates who underwent Norwood operation (January 1, 2013, to July 12, 2016) before guideline implementation were compared with 50 consecutive neonates after guideline implementation (July 13, 2016, to May 4, 2018). No preguideline operations met the goal Guideline Adherence Score. In the first 6 months after guideline implementation, 10 of 12 operations achieved goal Guideline Adherence Score and continued through implementation, reaching 100% for the last 10 operations. Statistical process control analysis demonstrated less variability and decreased hours of postoperative mechanical ventilation and cardiac ICU length of stay during implementation. There were no statistically significant differences in major hospital complications or in 30-day mortality. A higher percentage of patients were extubated by postoperative day 2 after guideline implementation (67% [30/47] vs 41% [41/99], respectively; p = 0.01). Of these patients, reintubation within 72 hours of extubation significantly decreased after guideline implementation (0% [0/30] vs 17% [7/41] patients, respectively; p = 0.02). CONCLUSIONS: This initiative successfully implemented a standardized perioperative care guideline for neonates undergoing the Norwood operation at a large center. Positive statistical process control centerline shifts in Guideline Adherence Score, length of postoperative mechanical ventilation, and cardiac ICU length of stay were demonstrated. A higher percentage were successfully extubated by postoperative day 2. Establishment of standard processes can lead to best practices to decrease major adverse events.
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Cardiopatías Congénitas , Síndrome del Corazón Izquierdo Hipoplásico , Procedimientos de Norwood , Niño , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Recién Nacido , Procedimientos de Norwood/efectos adversos , Estándares de Referencia , Factores de Riesgo , Resultado del TratamientoRESUMEN
BACKGROUND: The arterial switch operation (ASO) is the gold standard operative correction of neonates with transposition of the great arteries and intact ventricular septum, with excellent operative survival. The associations between patient and surgeon characteristics and outcomes are well understood, but the associations between variation in preoperative care and outcomes are less well studied. METHODS: A multicenter retrospective cohort study of infants undergoing neonatal ASO between January 2010 and September 2015 at hospitals contributing data to the Pediatric Health Information Systems database was performed. The association between preoperative care (timing of ASO, preoperative use of balloon atrial septostomy, prostaglandin infusion, mechanical ventilation, and vasoactive agents) and operative outcomes (mortality, length of stay, and cost) was studied with multivariable mixed-effects models. RESULTS: Over the study period, 2159 neonates at 40 hospitals were evaluated. Perioperative mortality was 2.8%. Between hospitals, the use of adjuvant therapies and timing of ASO varied broadly. At the subject level, older age at ASO was associated with higher mortality risk (age >6 days: odds ratio, 1.90; 95% CI, 1.11-3.26; P=0.02), cost, and length of stay. Receipt of a balloon atrial septostomy was associated with lower mortality risk (odds ratio, 0.32; 95% CI, 0.17-0.59; P<0.001), cost, and length of stay. Later hospital median age at ASO was associated with higher odds of mortality (odds ratio, 1.15 per day; 95% CI, 1.02-1.29; P=0.03), longer length of stay ( P<0.004), and higher cost ( P<0.001). Other hospital factors were not independently associated with the outcomes of interest. CONCLUSIONS: There was significant variation in preoperative care between hospitals. Some potentially modifiable aspects of perioperative care (timing of ASO and septostomy) were significantly associated with mortality, length of stay, and cost. Further research on the perioperative care of neonates is necessary to determine whether modifying practice on the basis of the observed associations translates into improved outcomes.
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Operación de Switch Arterial , Disparidades en Atención de Salud , Pautas de la Práctica en Medicina , Cuidados Preoperatorios , Transposición de los Grandes Vasos/cirugía , Operación de Switch Arterial/efectos adversos , Operación de Switch Arterial/economía , Operación de Switch Arterial/mortalidad , Cateterismo Cardíaco , Fármacos Cardiovasculares/administración & dosificación , Bases de Datos Factuales , Femenino , Disparidades en Atención de Salud/economía , Costos de Hospital , Mortalidad Hospitalaria , Humanos , Recién Nacido , Tiempo de Internación , Masculino , Pautas de la Práctica en Medicina/economía , Cuidados Preoperatorios/efectos adversos , Cuidados Preoperatorios/economía , Cuidados Preoperatorios/mortalidad , Prostaglandinas/administración & dosificación , Respiración Artificial , Estudios Retrospectivos , Factores de Riesgo , Factores de Tiempo , Tiempo de Tratamiento , Transposición de los Grandes Vasos/economía , Transposición de los Grandes Vasos/mortalidad , Transposición de los Grandes Vasos/fisiopatología , Resultado del TratamientoRESUMEN
Background The Fontan operation is performed for surgical palliation of single ventricle physiology. This operation is usually preceded by a superior cavopulmonary connection (SCPC); lymphatic abnormalities after SCPC may be demonstrated at MRI and prior to the Fontan operation. Purpose To determine if the degree of neck and thoracic lymphatic abnormalities at T2-weighted MRI in patients after superior cavopulmonary connection (SCPC) correlated with surgical outcomes from the Fontan procedure. Materials and Methods Patients for whom SCPC was performed for palliation of single ventricle disease who underwent chest MRI between July 2012 and May 2015 at a single institution were retrospectively reviewed. T2-weighted images were scored as lymphatic type 1 (little or no T2 mediastinal and supraclavicular signal) to type 4 (T2 signal into both the mediastinum and the lung parenchyma). Fontan takedown, duration of post-Fontan hospitalization and pleural effusion, postoperative plastic bronchitis, need for transplant, and mortality were tabulated. The relationship between lymphatic type and clinical outcomes was evaluated by using analysis of variance (ANOVA), the Kruskal-Wallis H test, and the Fisher exact test. Results A total of 83 patients (mean age, 7.9 years ± 2.6) were evaluated. Among these 83 patients, 53 (64%) were classified with type 1 or 2 lymphatic abnormalities, 17 (20%) with type 3, and 12 (16%) with type 4. The rate of failure of Fontan completion was higher in patients with type 4 than in type 1 or 2 (54% vs 2%, respectively; P = .004). Need for cardiac transplant (one of 13 [8%]) and death (three of 13 [23%]) occurred only in type 4. Median postoperative length of stay was longer for patients with type 4 than for those with types 1 or 2 (29 days vs 9 days, respectively; P < .01). Conclusion Greater MRI-based severity of lymphatic abnormalities in patients prior to planned Fontan procedure was associated with failure of Fontan completion and longer postoperative stay. © RSNA, 2019 Online supplemental material is available for this article.
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Procedimiento de Fontan , Anomalías Linfáticas/diagnóstico por imagen , Complicaciones Posoperatorias/diagnóstico por imagen , Niño , Preescolar , Procedimiento de Fontan/efectos adversos , Procedimiento de Fontan/mortalidad , Procedimiento de Fontan/estadística & datos numéricos , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/cirugía , Humanos , Tiempo de Internación , Anomalías Linfáticas/etiología , Sistema Linfático/diagnóstico por imagen , Sistema Linfático/patología , Imagen por Resonancia Magnética , Cuello/diagnóstico por imagen , Cuello/patología , Complicaciones Posoperatorias/etiología , Estudios Retrospectivos , Tórax/diagnóstico por imagen , Tórax/patología , Resultado del TratamientoRESUMEN
BACKGROUND: The risks and benefits of pharmacologic treatment and operative closure of patent ductus arteriosus (O-PDA) in premature infants remain controversial. Recent series have demonstrated the feasibility of transcatheter PDA closure (TC-PDA) in increasingly small infants. The effect of this change on practice has not been evaluated. METHODS: A multicenter observational study of infants treated in neonatal intensive care units in hospitals contributing data to the Pediatric Health Information Systems Database from January 2007 to December 2017 was performed to study trends in the propensities for (1) mechanical closure of PDA and (2) TC-PDA versus O-PDA, as well as interhospital variation in practice. RESULTS: A total of 6,214 subjects at 44 hospitals were studied (5% TC-PDA). Subject median gestational age was 25â¯weeks (interquartile range: 24-27â¯weeks). Median age at closure was 24â¯days (interquartile range: 14-36â¯days). The proportion of all neonatal intensive care unit patients undergoing either O-PDA or TC-PDA decreased (3.1% in 2007 and 0.7% in 2017, Pâ¯<â¯.001), whereas the proportion in which TC-PDA was used increased significantly (0.1% in 2007 to 29.0% in 2017). Case-mix-adjusted multivariable models similarly demonstrated increasing propensity to pursue TC-PDA (odds ratio [OR] 1.66 per year, Pâ¯<â¯.001) with acceleration of the trend after 2014 (OR 2.46 per year, Pâ¯<â¯.001) as well as significant practice variation (Pâ¯<â¯.001, median OR 4.6) across the study period. CONCLUSIONS: In the face of decreasing closure of PDA, the use of TC-PDA increased dramatically with significant practice variability. This demonstrates that there is equipoise for potential clinical trials.
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Análisis de Datos , Conducto Arterioso Permeable/cirugía , Sistemas de Información en Salud/estadística & datos numéricos , Unidades de Cuidado Intensivo Neonatal/tendencias , Grupos Diagnósticos Relacionados , Femenino , Edad Gestacional , Humanos , Lactante , Recién Nacido , Recien Nacido Prematuro , Masculino , Análisis Multivariante , Puntaje de PropensiónRESUMEN
OBJECTIVES: To describe the technique and report on our initial experience with the use of intrahepatic dynamic contrast magnetic resonance lymphangiography (IH-DCMRL) for evaluation of the lymphatics in patients with hepatic lymphatic flow disorders. METHODS: This is a retrospective review of the imaging and clinical findings in six consecutive patients undergoing IH-DCMRL. The technique involves injection of a gadolinium contrast agent into the intrahepatic lymphatic ducts followed by imaging of the abdomen and chest with both heavily T2-weighted imaging and dynamic time-resolved imaging. RESULTS: In six consecutive patients, IH-DCMRL was technically successful. There were four patients with protein-losing enteropathy (PLE) and two with ascites in this study. In the four patients with PLE, IH-DCMRL demonstrated hepatoduodenal connections with leak of contrast into the duodenal lumen not seen by conventional lymphangiography. In one patient with ascites, IH-DCMRL demonstrated lymphatic leakage into the peritoneal cavity not seen by intranodal lymphangiography. In the second patient with ascites, retrograde lymphatic perfusion of mesenteric lymphatic networks and nodes was seen. Venous contamination was seen in two patients. No biliary contamination was identified. There were no short-term complications. CONCLUSIONS: IH-DCMRL is a cross-sectional technique which successfully evaluated hepatic lymphatic flow disorders and warrants further investigation. KEY POINTS: ⢠Intrahepatic dynamic contrast magnetic resonance lymphangiography (IH-DCMRL) is a new imaging technique to evaluate hepatic lymphatic flow disorders such as protein-losing enteropathy. ⢠In comparison to conventional liver lymphangiography, IH-DCMRL offers a 3D imaging technique and better distal lymphatic contrast distribution and does not use ionizing radiation.
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Hígado/irrigación sanguínea , Enfermedades Linfáticas/diagnóstico , Vasos Linfáticos/patología , Linfografía/métodos , Angiografía por Resonancia Magnética/métodos , Compuestos Organometálicos/farmacología , Adolescente , Adulto , Niño , Preescolar , Medios de Contraste/farmacología , Estudios Transversales , Femenino , Humanos , Imagenología Tridimensional , Lactante , Hígado/diagnóstico por imagen , Masculino , Estudios Retrospectivos , Adulto JovenRESUMEN
BACKGROUND: Children with Noonan syndrome are known to have increased risk for lymphatic disorders, the extent and nature of which are poorly understood. OBJECTIVE: Our objective was to describe the imaging findings of the central lymphatic abnormalities in children with Noonan syndrome who underwent central lymphatic imaging. MATERIALS AND METHODS: We conducted a single-center retrospective review of all children with a confirmed history of Noonan syndrome who presented for lymphatic imaging over a 5-year period. Imaging evaluation was performed on unenhanced T2-weighted (T2-W) imaging, dynamic-contrast MR lymphangiography or conventional lymphangiography. Two readers evaluated the imaging in consensus for the distribution of fluid on T2-W imaging and for lymphatic flow of intranodal contrast agent and thoracic duct abnormalities on dynamic-contrast MR lymphangiography and conventional lymphangiography. We performed a chart review for clinical history and outcomes. RESULTS: We identified a total of 10 children, all but one of whom had congenital heart disease. Presenting symptoms included chylothorax (n=9) and ascites (n=1). Nine had T2-W imaging, seven had dynamic-contrast MR lymphangiography, and seven had conventional lymphangiography. All with T2-W imaging had pleural effusions. On both dynamic-contrast MR lymphangiography and conventional lymphangiography, perfusion to the lung was seen (n=6), with intercostal flow also seen on dynamic-contrast MR lymphangiography (n=6). The thoracic duct was not present in three children and the central thoracic duct was not present in three. A double thoracic duct was seen in two children. CONCLUSION: Children with Noonan syndrome and clinical evidence of lymphatic dysfunction have central lymphatic abnormalities characterized by retrograde intercostal flow, pulmonary lymphatic perfusion, and thoracic duct abnormalities.
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Anomalías Linfáticas/diagnóstico por imagen , Anomalías Linfáticas/etiología , Linfografía/métodos , Imagen por Resonancia Magnética/métodos , Síndrome de Noonan/complicaciones , Ascitis/diagnóstico por imagen , Niño , Preescolar , Quilotórax/diagnóstico por imagen , Medios de Contraste , Aceite Etiodizado , Fluoroscopía , Humanos , Imagenología Tridimensional , Lactante , Recién Nacido , Compuestos Organometálicos , Derrame Pleural/diagnóstico por imagen , Estudios Retrospectivos , Conducto Torácico/anomalías , Ultrasonografía IntervencionalRESUMEN
INTRODUCTION: Interstage mortality causes are often unknown in infants with shunt-dependent univentricular defects. For 2 years, screening catheterisation was encouraged before neonatal discharge to determine if routine evaluation improved interstage outcomes. METHODS: Retrospective single-centre review of home monitoring programme from December, 2010 to June, 2012. Composite scores were created for physical examination/echocardiography risk factors; catheterisation risk factors; and interstage adverse events. Composite scores were compared between usual care and screening catheterisation groups. The ability of each risk factor composite to predict interstage adverse events, individually and in combination, was assessed with sensitivity, specificity, and receiver operating characteristic curves. RESULTS: There were 27 usual care and 32 screening catheterisation patients. There were no significant differences between groups except rates of catheterisation before discharge (29.6 versus 100%, p < 0.001). Usual care patients who underwent catheterisation for clinical indications had higher intervention rates (37.5 versus 3.1%, p = 0.004). Physical examination/echocardiography risk factor frequency was similar, but usual care patients with catheterisation had a higher catheterisation risk factor frequency. Interstage adverse event frequency was similar (48.2 versus 53.1%, p = 0.7). For interstage adverse event prediction, sensitivity for the physical examination/echocardiography, catheterisation, and either risk factor composites was 53.3, 72, and 80%, respectively; specificity was 59, 60, and 48%. The area under the receiver operating characteristic curve was 0.56, 0.66, and 0.64. CONCLUSION: Screening catheterisation evaluation offered slightly increased sensitivity and specificity, but no difference in interstage adverse event frequency. Given this small advantage versus known risks, screening catheterisations are no longer encouraged.