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OBJECTIVE: To test the hypothesis that neonates with symptomatic tetralogy of Fallot (TOF) and absent ductus arteriosus (ADA) have worse clinical outcomes compared with those with a ductus arteriosus (DA), and that this difference is driven by those born with ADA and with critically deficient pulmonary blood flow (CDPBF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of neonates who underwent intervention for symptomatic TOF comparing death and reintervention between subjects with and without a DA identified on fetal echocardiogram or on echocardiogram performed in the first postnatal day. Exclusion criteria were as follows: inability to define DA status, collaterals supplying pulmonary blood flow, atrioventricular septal defect, and absent pulmonary valve. We defined CDPBF as undergoing a procedure to augment pulmonary blood flow on the date of birth or extracorporeal membrane oxygenation prior to such a procedure. RESULTS: The study cohort included 519 patients, among whom 11% had ADA. Patients with ADA were more likely to have a genetic syndrome and had smaller branch pulmonary artery size. In analyses adjusting for center, interventional treatment strategy, genetic syndrome, and minimum branch pulmonary artery size, ADA was associated with higher mortality risk (adjusted hazard ratio of 2.37 (95% CI: 1.07,5.27; P = .034). Seven patients had CDPBF (1.3% of the entire cohort and 12% of patients with ADA). CONCLUSIONS: A minority of symptomatic TOF neonates have ADA, which is associated with higher adjusted mortality risk compared with those with a DA. CDPBF appears to be a rare but important entity in this population.
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Tetralogía de Fallot , Humanos , Tetralogía de Fallot/mortalidad , Estudios Retrospectivos , Recién Nacido , Femenino , Masculino , Ecocardiografía , Estudios de Cohortes , Conducto Arterial/diagnóstico por imagen , Resultado del TratamientoRESUMEN
Objectives: Aortic valve repair can be limited by inadequate leaflet tissue for proper coaptation. Various kinds of pericardium have been used for cusp augmentation, but most have failed because of tissue degeneration. A more durable leaflet substitute is needed. Methods: In this report, 8 consecutive cases are presented in which autologous ascending aortic tissue was used to augment inadequate native cusps during aortic valve repair. Biologically, aortic wall is a living autologous tissue that could have exceptional durability as a leaflet substitute. Techniques for insertion are described in detail, along with procedural videos. Results: Early surgical outcomes were excellent, with no operative mortalities or complications, and all valves were competent with low valve gradients. Patient follow-up and echocardiograms to a maximum of 8 months' postrepair remain excellent. Conclusions: Because of superior biologic characteristics, aortic wall has the potential to provide a better leaflet substitute during aortic valve repair and to expand patient categories amenable to autologous reconstruction. More experience and follow-up should be generated.
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OBJECTIVE: To evaluate early growth following primary or staged repair of neonatal symptomatic tetralogy of Fallot (sTOF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of consecutive infants with sTOF who underwent initial intervention at age ≤30 days, from 2005 to 2017. Management strategies were either primary repair or staged repair (ie, initial palliation followed by complete repair). The primary outcome was change in weight-for-age z-score (ΔWAZ) from the initial intervention to age 6 ± 2 months. Secondary outcomes included method and mode of feeding, feeding-related medications, and feeding-related readmissions. Propensity score adjustment was used to account for baseline differences between groups. A secondary analysis was performed comparing patients stratified by the presence of adequate growth (6-month ΔWAZ > -0.5) or inadequate growth (6-month ΔWAZ ≤ -0.5), independent of treatment strategy. RESULTS: The study cohort included 143 primary repair subjects and 240 staged repair subjects. Prematurity was more common in the staged repair group. After adjustment, median ΔWAZ did not differ between treatment groups over the first 6 months of life (primary: -0.43 [IQR, -1.17 to 0.50]; staged: -0.31 [IQR, -1.31 to 0.71]; P = .55). For the entire cohort, ΔWAZ was negative (-0.36; IQR, -1.21 to 0.63). There were no between-group differences in the secondary outcomes. Secondary analysis revealed that the subjects with adequate growth were more likely to be orally fed at initial hospital discharge (P = .04). CONCLUSIONS: In neonates with sTOF, growth trajectory over the first 6 months of life was substandard, irrespective of treatment strategy. Those patients with adequate growth were more likely to be discharged from the index procedure on oral feeds.
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Procedimientos Quirúrgicos Cardíacos , Tetralogía de Fallot , Humanos , Lactante , Recién Nacido , Tetralogía de Fallot/cirugía , Estudios Retrospectivos , Estudios de Cohortes , Resultado del Tratamiento , Procedimientos Quirúrgicos Cardíacos/métodosRESUMEN
OBJECTIVE: Neonatal interventional strategies for pulmonary atresia with intact ventricular septum are based on tricuspid valve hypoplasia and right ventricle-dependent coronary circulation. We sought to evaluate long-term outcomes comparing biventricular (BiV) versus single-ventricle (SV) strategies. METHODS: Retrospective review was performed of 119 patients diagnosed with pulmonary atresia with intact ventricular septum from 1995 to 2018. Descriptive statistics summarized patient characteristics and a multivariable Cox survival model was used to compare treatment strategies. RESULTS: Of 119 patients, 62 (52.1%) were male and 13 (10.9%) had a chromosomal abnormality. BiV was pursued in 53.8% (64 out of 119) and SV in 46.2% (55 out of 119) with median tricuspid valve z scores of -1.59 (interquartile range, -3.03 to 0.21) and -5.12 (interquartile range, -5.60 to -4.06), respectively. The median follow-up was 6 years (interquartile range, 2-15 years). Overall survival at 1, 3, and 10 years was 82.4% (98 out of 119), 80.6% (96 out of 119) and 79.8% (95 out of 119), respectively. End states include 36 (30.3%) BiV, 33 (27.7%) SV, 22 (18.5%) alive without definitive end state, 21 (17.6%) death before end state, 4 (3.4%) 1-and-a-half ventricle, and 3 (2.5%) transplants. No SV were converted to BiV, whereas 4 out of 64 (6.3%) BiV were converted to SV. After adjusting for gender, chromosomal abnormalities, gestational age, and birth weight, SV patients had a significantly higher hazard of mortality (hazard ratio, 9.0; 95% CI, 2.65-30.69; P < .001). Mortality was higher in those with right ventricle-dependent coronary circulation (41.9% [13 out of 31]) compared with those without right ventricle-dependent coronary circulation (7.3% [6 out of 82]) (P < .001). CONCLUSIONS: Pulmonary atresia with intact ventricular septum remains a challenging lesion for those patients on the SV pathway, particularly with right ventricle-dependent coronary circulation.
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Cardiopatías Congénitas , Atresia Pulmonar , Tabique Interventricular , Femenino , Cardiopatías Congénitas/cirugía , Ventrículos Cardíacos/anomalías , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/cirugía , Humanos , Recién Nacido , Masculino , Atresia Pulmonar/cirugía , Resultado del TratamientoRESUMEN
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: We sought to identify patient and surgical factors associated with time to hospital discharge in patients undergoing complete repair for tetralogy of Fallot. METHODS AND RESULTS: We performed a prospective cohort study of patients with tetralogy of Fallot admitted for complete repair between May 1, 2012 and June 2, 2017 at Children's Hospital of Philadelphia with detailed demographic, clinical, and operative characteristics. The primary outcome was time to hospital discharge. Cox proportional hazards models were used to identify patient and operative predictors of time to hospital discharge. We enrolled 151 subjects, 62.8% male, 65.6% non-Hispanic white, and 9.9% non-Hispanic black. The median time to hospital discharge was 7 days (interquartile range 4, 12). Five patients died in the hospital, all of whom underwent tetralogy of Fallot repair beyond the neonatal period. Greater birth weight was associated with higher rate of hospital discharge (hazard ratio [HR]=1.35, 95% confidence interval (CI) =1.11, 1.64), while absent pulmonary valve versus pulmonary stenosis (HR=0.27, 95% CI=0.08, 0.91), pulmonary valve atresia versus pulmonary stenosis (HR=0.57, 95% CI=0.33, 0.97), presence of aortopulmonary collaterals (HR=0.44, 95% CI=0.24, 0.84), complete repair performed in the neonatal period (<30 days of life) (HR=0.45, 95% CI=0.27, 0.75), more than 1 cardiopulmonary bypass run (HR=0.33, 95% CI=0.18, 0.61), and longer aortic cross-clamp time (HR [per 10 minutes]=0.88, 95% CI=0.79, 0.97) were associated with lower rate of hospital discharge. CONCLUSIONS: Postoperative hospital stay after complete repair of tetralogy of Fallot is in part determined by patient and operative factors. Some (eg, surgical strategy for the symptomatic neonate) may be modifiable. These results may impact patient counseling, choice of surgical approach, and postoperative care.
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Procedimientos Quirúrgicos Cardíacos , Tiempo de Internación , Tetralogía de Fallot/cirugía , Factores de Edad , Peso al Nacer , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Procedimientos Quirúrgicos Cardíacos/mortalidad , Femenino , Mortalidad Hospitalaria , Humanos , Lactante , Recién Nacido , Masculino , Complicaciones Posoperatorias/mortalidad , Complicaciones Posoperatorias/terapia , Estudios Prospectivos , Factores de Riesgo , Tetralogía de Fallot/mortalidad , Factores de Tiempo , Resultado del TratamientoRESUMEN
OBJECTIVES: To describe the frequency, characteristics, and outcomes of heart failure-related emergency department (ED) visits in pediatric patients. We aimed to test the hypothesis that these visits are associated with higher admission rates, mortality, and resource utilization. STUDY DESIGN: A retrospective analysis of the Nationwide Emergency Department Sample for 2010 of patients ≤18 years of age was performed to describe ED visits with and without heart failure. Cases were identified using International Classification of Disease, Ninth Revision, Clinical Modification codes and assessed for factors associated with admission, mortality, and resource utilization. RESULTS: Among 28.6 million pediatric visits to the ED, there were 5971 (0.02%) heart failure-related cases. Heart failure-related ED patients were significantly more likely to be admitted (59.8% vs 4.01%; OR 35.3, 95% CI 31.5-39.7). Among heart failure-related visits, admission was more common in patients with congenital heart disease (OR 5.0, 95% CI 3.3-7.4) and in those with comorbidities including respiratory failure (OR 78.3, 95% CI 10.4-591) and renal failure (OR 7.9, 95% CI 1.7-36.3). Heart failure-related cases admitted to the hospital had a higher likelihood of death than nonheart failure-related cases (5.9% vs 0.32%, P < .001). Factors associated with mortality included respiratory failure (OR 4.5, 95% CI 2.2-9.2) and renal failure (OR 7.8, 95% CI 2.9-20.7). Heart failure-related ED visits were more expensive than nonheart failure-related ED visits ($1460 [IQR $861-2038] vs $778 [IQR $442-1375] [P < .01].) CONCLUSIONS: Heart failure-related visits represent a minority of pediatric ED visits but are associated with increased hospital admission and resource utilization.
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Servicio de Urgencia en Hospital/estadística & datos numéricos , Insuficiencia Cardíaca/epidemiología , Aceptación de la Atención de Salud/estadística & datos numéricos , Adolescente , Niño , Preescolar , Bases de Datos Factuales , Servicio de Urgencia en Hospital/economía , Femenino , Insuficiencia Cardíaca/economía , Insuficiencia Cardíaca/mortalidad , Hospitalización/estadística & datos numéricos , Humanos , Lactante , Masculino , Estudios Retrospectivos , Factores de Riesgo , Estados Unidos/epidemiologíaRESUMEN
BACKGROUND: Intramural ventricular septal defects (VSDs), residual interventricular communications occurring after repair of conotruncal defects, are associated with poor postoperative outcomes. The ability of intraoperative transesophageal echocardiography (TEE) to identify intramural VSDs has not yet been evaluated. METHODS: Intraoperative TEE and postoperative transthoracic echocardiography (TTE) data in all patients undergoing all biventricular repair of conotruncal anomalies in our hospital between January 1, 2006, and June 30, 2013, were reviewed. The ability of TEE to accurately identify residual defects was assessed using postoperative TTE as the reference imaging modality. RESULTS: Intramural VSDs occurred in 34 of 337 patients evaluated; 19 were identified by both TTE and TEE, and 15 were identified by TTE only. Sensitivity was 56% and specificity was 100% for TEE to identify intramural VSDs. Peripatch VSDs were identified in 90 patients by both TTE and TEE, in 53 by TTE only, and in 15 by TEE only, yielding a sensitivity of 63% and specificity of 92%. Of the VSDs requiring catheterization or surgical reintervention, 6 of 7 intramural VSDs and all 5 peripatch VSDs were identified by intraoperative TEE. TEE guided the intraoperative decision to return to cardiopulmonary bypass (CPB) in an attempt to close residual defects in 12 patients with intramural VSDs and in 4 patients with peripatch VSDs seen after initial CPB; of these, 10 intramural VSDs and all 4 peripatch VSDs resolved or became smaller on final intraoperative TEE. CONCLUSIONS: TEE has modest sensitivity but high specificity for identifying intramural VSDs and can detect most defects requiring reintervention. Repeat attempts at closure in the index operation may successfully correct intramural VSDs identified by TEE.
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Ecocardiografía Transesofágica , Cardiopatías Congénitas/cirugía , Defectos del Tabique Interventricular/diagnóstico por imagen , Complicaciones Posoperatorias/diagnóstico por imagen , Puente Cardiopulmonar , Ecocardiografía , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Reoperación , Estudios Retrospectivos , Sensibilidad y EspecificidadRESUMEN
BACKGROUND: Intramural ventricular septal defects (VSDs) are interventricular communications through right ventricular free wall trabeculations that can occur after repair of conotruncal anomalies. We assessed the prevalence of residual intramural VSDs and their effect on postoperative course. METHODS AND RESULTS: Children who underwent biventricular repair of a conotruncal anomaly from January 1, 2006, to June 30, 2013, and had a postoperative transthoracic echocardiogram were included. Images were reviewed for residual intramural or nonintramural VSDs. The primary outcome was a composite of mortality, extracorporeal membrane oxygenation use, and need for subsequent catheter or surgical VSD closure. The secondary outcome was postoperative hospital length of stay. A residual VSD was present in 256 of the 442 subjects (58%), of which 231 (90%) were <2 mm in size. Forty-nine patients (11%) had intramural VSDs, and 207 (47%) had nonintramural VSDs. Patients with intramural VSDs were more likely to reach the primary composite outcome compared with those with nonintramural VSDs or no residual VSD (14 of 49 [29%] versus 15 of 207 [7%] versus 6 of 186 [3%]; P<0.0001). In addition, those with intramural VSDs had longer postoperative hospital length of stay compared with those with nonintramural VSDs or no residual VSD (20 days [interquartile range, 11-42 days] versus 7 days [interquartile range, 5-14 days] versus 6 days [interquartile range, 4-11 days]; P=0.0001). These associations remained significant after adjustment for known risk factors for poor outcomes, including residual VSD size and operative complexity. CONCLUSIONS: Among residual VSDs after repair of conotruncal anomalies, intramural VSDs are uniquely associated with postoperative morbidity, mortality, and longer postoperative hospital length of stay. It is important to recognize intramural VSDs in the postoperative period.