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BACKGROUND: Children with advanced pulmonary disease due to cystic fibrosis (CF) are at risk of acute respiratory failure due to pulmonary exacerbations leading to their admission to pediatric intensive care units (PICU). The objectives of this study were to determine short and medium-term outcomes of children with CF admitted to PICU for acute respiratory failure due to pulmonary exacerbation and to identify prognosis factors. METHODS: This retrospective monocentric study included patients less than 18 years old admitted to the PICU of a French university hospital between 2000 and 2020. Cox proportional hazard regression methods were used to determine prognosis factors of mortality or lung transplant. RESULTS: Prior to PICU admission, the 29 patients included (median age 13.5 years) had a severe lung disease (median Forced Expiratory Volume in 1 s percentage predicted at 29%). Mortality rates were respectively 17%, 31%, 34%, 41% at discharge and at 3, 12 and 36 months post-discharge. Survival rates free of lung transplant were 34%, 32%, 24% and 17% respectively. Risk factors associated with mortality or lung transplant using the univariate analysis were female sex and higher pCO2 and chloride levels at PICU admission, and following pre admission characteristics: home respiratory and nutritional support, registration on lung transplant list and Stenotrophomonas Maltophilia bronchial colonization. CONCLUSION: Children with CF admitted to PICU for acute respiratory failure secondary to pulmonary exacerbations are at high risk of death, both in the short and medium terms. Lung transplant is their main chance of survival and should be considered early.
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Fibrosis Quística , Unidades de Cuidado Intensivo Pediátrico , Insuficiencia Respiratoria , Humanos , Fibrosis Quística/mortalidad , Fibrosis Quística/complicaciones , Fibrosis Quística/diagnóstico , Femenino , Masculino , Estudios Retrospectivos , Niño , Adolescente , Insuficiencia Respiratoria/mortalidad , Insuficiencia Respiratoria/diagnóstico , Insuficiencia Respiratoria/etiología , Unidades de Cuidado Intensivo Pediátrico/estadística & datos numéricos , Factores de Riesgo , Progresión de la Enfermedad , Francia/epidemiología , Preescolar , Resultado del TratamientoRESUMEN
AIMS: The implantable cardioverter defibrillator (ICD) has been increasingly used in children. Both epicardial and transvenous approaches are used, with controversy regarding the best option with no specific recommendations. We aimed to compare outcomes associated with epicardial vs. transvenous ICDs in children. METHODS AND RESULTS: Data were analysed from a retrospective study including all patients <18-year-old implanted with an ICD in a tertiary centre from 2003 to 2021. Outcomes were compared between epicardial and transvenous ICDs. A total of 122 children with an ICD (mean age 11.5 ± 3.8 years, 57.4% males) were enrolled, with 84 (64.1%) epicardial ICDs and 38 (29.0%) transvenous ICDs. Early (<30 days) ICD-related complications were reported in 17 (20.2%) patients with an epicardial ICD vs. 0 (0.0%) with a transvenous ICD (P = 0.002). Over a mean follow-up of 4.8 ± 4.0 years, 25 (29.8%) patients with an epicardial ICD and 9 (23.7%) patients with a transvenous ICD experienced at least one late ICD-related complication [hazard ratio (HR) 1.8, 95% confidence interval (CI) 0.8-4.0]. Implantable cardioverter defibrillator lead dysfunction occurred in 19 (22.6%) patients with an epicardial ICD vs. 3 (7.9%) with a transvenous ICD (HR 5.7, 95% CI 1.3-24.5) and was associated with a higher incidence of ICD-related reintervention (HR 3.0, 95% CI 1.3-7.0). After considering potential confounders, especially age and weight at implantation, this association was no longer significant (P = 0.112). The freedom from ICD lead dysfunction was greater in patients with pleural coils than in those with epicardial coils (HR 0.38, 95% CI 0.15-0.96). CONCLUSION: In children, after a consideration of patient characteristics at implantation, the burden of complications and ICD lead dysfunction appears to be similar in patients with epicardial and transvenous devices. Pleural coils seem to be associated with better outcomes than epicardial coils in this population. CLINICAL TRIAL REGISTRATION: NCT05349162.
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Desfibriladores Implantables , Adolescente , Niño , Femenino , Humanos , Masculino , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/terapia , Arritmias Cardíacas/etiología , Muerte Súbita Cardíaca/epidemiología , Desfibriladores Implantables/efectos adversos , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Patients with end-stage pulmonary arterial hypertension due to congenital heart disease have limited access to heart-lung transplantation or double-lung transplantation. We aimed to assess the effects of a high-priority allocation program established in France in 2007. We conducted a retrospective study to compare waitlist and posttransplantation outcomes before versus after implementation of the high-priority allocation program. We included 67 consecutive patients (mean age at listing, 33.2 ± 10.5 years) with pulmonary arterial hypertension due to congenital heart disease listed for heart-lung transplantation or double-lung transplantation from 1997 to 2016. At one month, the incidences of transplantation and death before transplantation were 3.5% and 24.6% in 1997-2006, 4.8% and 4.9% for patients on the regular list in 2007-2016, and 41.2% and 7.4% for patients listed under the high-priority allocation program (p < .001 and p = .0001, respectively). Overall survival was higher in patients listed in 2007-2016 (84.2% and 61.2% at 1 and 10 years vs. 36.8% and 22.1%, p = .0001). Increased incidence of transplantation, decreased waiting list mortality, and improved early and long-term outcomes were observed in patients with pulmonary arterial hypertension due to congenital heart disease listed for transplantation in the recent era, characterized by implementation of a high-priority allocation program.
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Cardiopatías Congénitas , Trasplante de Corazón , Hipertensión Arterial Pulmonar , Obtención de Tejidos y Órganos , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/cirugía , Humanos , Estudios Retrospectivos , Tasa de Supervivencia , Listas de EsperaRESUMEN
INTRODUCTION: With the increase and refinement of video assisted mitral valve surgery, cristalloïd cardioplegia started regaining popularity. The aim of our study was to evaluate the effectiveness of Celsior®, a crystalloid cardioplegic solution, on myocardial protection in elective surgical mitral valve repair in comparison to blood based hyperkalemic cardioplegia. METHODS: In this observational retrospective study, all consecutive elective isolated surgical mitral valve repair where Celsior® or normothermic hyperkalemic blood cardioplegia were used were included. Primary endpoint was any sign of myocardial protection failure (troponin levels, need for inotropic or mechanical support, rhythm disturbances, mortality). Secondary endpoint was Celsior® safety (allergic reactions, bleeding, organ toxicities). RESULTS: From January 2009 to August 2016, 382 patients underwent elective isolated mitral valve repair in whom normothermic hyperkalemic blood cardioplegia (n = 181) or Celsior® (n = 201) were used. There were no statistically significant differences in baseline characteristics including Euroscore 2. Peak troponin (pg/ml) release and 30-days mortality were not statistically different. Need for cardioversion was significantly more frequent in the Celsior® group (47% vs 13%, p < 0.001). There was no statistical difference in post-operative atrial fibrillation, permanent pacemaker implantation, reoperation for bleeding, transfusion, acute kidney injury, haemoglobin at discharge or length of stay. No allergic reaction to Celsior® occurred. CONCLUSION: Effective myocardial protection was achieved with the Celsior® cardioplegic solution with no unexpected toxicity. Celsior® may be an efficacious and safe cardioprotective strategy in mitral valve repair.
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Paro Cardíaco Inducido , Válvula Mitral , Soluciones Cardiopléjicas/uso terapéutico , Soluciones Cristaloides , Disacáridos , Electrólitos , Glutamatos , Glutatión , Histidina , Humanos , Manitol , Estudios RetrospectivosRESUMEN
OBJECTIVE: More than 30% of European pediatric cardiac surgery centers use single-dose cold histidine-tryptophan-ketoglutarate cardioplegia (Custodiol; Dr Franz Köhler Chemie GmbH, Bensheim, Germany). In neonates with transposition of the great arteries, arterial switch surgery (ASO) implies aortic division, and it is unknown whether repeated ostial cannulation causes intimal insult and affects long-term results, and therefore, single-dose Custodiol is appealing. The present study investigated the association among myocardial no-flow duration, postoperative troponins, and postoperative outcomes in neonates undergoing ASO with Custodiol cardioplegia. DESIGN: Retrospective analysis of the association among myocardial no-flow duration, postoperative troponin release (concentration magnitudeâ¯×â¯measurement duration within 48 h), and outcomes using stratification according to coronary anatomy and attending surgeon. SETTING: Single-institutional, tertiary pediatric cardiac surgery unit of a university hospital. PARTICIPANTS: The study comprised 101 neonates undergoing ASO. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The mean age of patients was 6.1 ± 5.4 days, the cardiopulmonary bypass duration was 108.7 ± 54.1 minutes, the temperature during cross-clamping was 31.1°C ± 1.7°C, the duration of mechanical ventilation was 4 (3-6) days, the length of intensive care unit stay was 7 (5-8) days, delayed sternal closure occurred in 32 (31.7%) patients, and no patients died. The myocardial no-flow duration averaged 62.3 ± 14.6 minutes and was linked with both troponin release (pâ¯=â¯0.04) and low cardiac output syndrome, as assessed by the requirement for delayed sternal closure (pâ¯=â¯0.03), regardless of cardiopulmonary bypass duration and temperature. Eighty-two percent of the patients with myocardial no-flow duration >74 minutes necessitated delayed sternal closure. CONCLUSIONS: Single-dose Custodiol may be inadequate for prolonged cross-clamping durations without myocardial perfusion in neonates.
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Soluciones Cardiopléjicas/uso terapéutico , Histidina/uso terapéutico , Ácidos Cetoglutáricos/uso terapéutico , Transposición de los Grandes Vasos/cirugía , Triptófano/uso terapéutico , Constricción , Alemania , Paro Cardíaco Inducido , Humanos , Recién Nacido , Estudios RetrospectivosRESUMEN
Dilated cardiomyopathy (DCM) is the most common childhood cardiomyopathy and is associated with considerable early mortality. Heart transplantation is often the only viable life-saving option. Pulmonary artery banding (PAB) has been recently proposed as a bridge or alternative to transplantation for DCM. In our cohort, PAB was selectively addressed to heritable DCM or DCM with congenital left ventricle aneurysm (CLVA). This study aimed to describe the clinical evolution and left ventricle reverse remodeling (LVRR) over time (6 months and 1 year after surgery). Ten patients with severe DCM received PAB between 2016 and 2021 and underwent clinical and postoperative echocardiography follow-ups. The median age at PAB was <1 year. The in-hospital mortality was zero. Two patients died two months after PAB of end-stage heart failure. The modified Ross class was improved in the eight survivors with DCM and remained stable in the two patients with CLVA. We observed a positive LVRR (LV end-diastolic diameter Z-score: 8.4 ± 3.7 vs. 2.8 ± 3; p < 0.05; LV ejection fraction: 23.8 ± 5.8 to 44.5 ± 13.1 (p < 0.05)). PAB might be useful as part of the armamentarium available in infants and toddlers with severe DCM not sufficiently responding to medical treatment with limited probability of spontaneous recovery.
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OBJECTIVE: Aortic valvuloplasty frequency has significantly increased over the past 15 years. Surgical repair varies in complexity depending on valvular lesions. Our aim is to report results on the whole spectrum of aortic valvuloplasty techniques. METHODS: All children who consecutively underwent aortic valvuloplasty for aortic stenosis and/or aortic insufficiency between January 2006 and December 2020 at Necker Sick Children's Hospital (Paris, France) were included in a retrospective cohort study. Aortic valvuloplasty techniques were classified into 3 difficulty levels: (1) simple repair, corresponding to commissurotomy and/or shaving in aortic stenosis (AS) in neonates (group 1) and children >1 month (group 2); (2) intermediate-complexity repair, corresponding to commissuroplasty, leaflet resuspension, and fenestration closure in aortic insufficiency (leaflet prolapse in connective tissue disease, isolated leaflet prolapse and Laubry-Pezzi groups); and (3) complex repair requiring a pericardial patch to restore a functional aortic valve in mixed aortic valve disease (bicuspidization with neocommissure and cusp extension groups). RESULTS: During the study period, 324 children underwent aortic valvuloplasty. Survival and freedom from aortic valve reintervention at 10 years were, respectively, 86.1% and 50.9% in neonates with AS, 95.2% and 71.7% in children >1 month with AS, 93.8% and 79.5% in leaflet prolapse in connective tissue disease, 97.7% and 91.9% in isolated leaflet prolapse, 100% and 88% in those with Laubry-Pezzi syndrome, 97.4% and 84.8% in bicuspidization with neocommissure, and 100% and 54.2% in the cusp extension. CONCLUSIONS: Durability of aortic valvuloplasty techniques is satisfactory and offers the possibility to delay the Ross procedure, regardless of the lesion's complexity.
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Insuficiencia de la Válvula Aórtica , Estenosis de la Válvula Aórtica , Enfermedades del Tejido Conjuntivo , Niño , Recién Nacido , Humanos , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/cirugía , Válvula Aórtica/patología , Estudios Retrospectivos , Insuficiencia de la Válvula Aórtica/diagnóstico por imagen , Insuficiencia de la Válvula Aórtica/cirugía , Insuficiencia de la Válvula Aórtica/patología , Estenosis de la Válvula Aórtica/cirugía , Prolapso , Resultado del TratamientoRESUMEN
OBJECTIVES: The purpose of this study is to describe the long-term results of the 'réparation à l'étage ventriculaire' (REV) technique for double-outlet right ventricle and transposition of the great arteries (TGA) with pulmonary stenosis (PS). METHODS: Between 1980 and 2021, 157 patients underwent a REV procedure (median age and weight: 20.8 months and 7.7 kg). The most frequent anatomical presentation was the association between TGA, ventricular septal defect and PS (n = 116, 73.9%). RESULTS: Sixty-seven patients (42.7%) underwent a Rashkind procedure, and 67 patients (42.7%) a prior surgical palliation (including 62 systemic-to-pulmonary artery shunts). Resection of the conal septum and/or ventricular septal defect enlargement was performed in 109 patients (69.4%). Thirteen patients (8.3%) died, including 4 during the first postoperative month and 2 after heart transplant. Overall survival at 40 years was 89.3%. Thirty-seven patients (23.6%) required 68 reinterventions on the right ventricular outflow tract (RVOT), including 49 reoperations, with a median delay of 9 years after the REV (8 months to 27 years). Twenty patients (12.7%) underwent RVOT valvulation (16 surgical and 4 interventional). Freedom from RVOT reintervention and reoperation at 40 years were 60.3% and 62.6%, respectively. Four patients (2.5%) required reoperation for left ventricular outflow tract obstruction, with a median delay of 4.8 years. CONCLUSIONS: The REV procedure is a good alternative for TGA and double-outlet right ventricle with PS patients. Only a quarter of the patients required redo surgery on the RVOT. Reoperations for left ventricular outflow tract obstruction are scarce.
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Procedimientos Quirúrgicos Cardíacos , Ventrículo Derecho con Doble Salida , Defectos del Tabique Interventricular , Estenosis de la Válvula Pulmonar , Transposición de los Grandes Vasos , Obstrucción del Flujo de Salida Ventricular Izquierda , Humanos , Lactante , Transposición de los Grandes Vasos/cirugía , Ventrículo Derecho con Doble Salida/cirugía , Procedimientos Quirúrgicos Cardíacos/métodos , Resultado del Tratamiento , Estenosis de la Válvula Pulmonar/cirugía , Defectos del Tabique Interventricular/cirugía , ArteriasRESUMEN
OBJECTIVE: Difficult to repair aortic valve lesions, requiring the use of a valve substitute, remain controversial in the face of the Ross procedure, despite undeniable technical advances. This study was undertaken to compare midterm outcomes of children treated using the Ross procedure or aortic valvuloplasty for complex aortic valve lesions. METHODS: Between January 2006 and December 2017, 126 patients aged younger than 18 years were treated for complex aortic stenosis and/or aortic insufficiency and were included in this retrospective study. Only aortic valve lesions requiring repair with an autologous or heterologous pericardial patch were considered complex lesions. Propensity score framework analyses were used to compare outcomes of the Ross and aortic valvuloplasty groups while controlling for confounders. RESULTS: Among the 126 patients with complex aortic valve lesions, propensity score matching selected 34 unique pairs of patients with similar characteristics. Survival (aortic valvuloplasty, 94.1%; Ross, 91%; P = .89), freedom from overall reintervention (aortic valvuloplasty, 50.1%; Ross, 69%; P = .32), and freedom from infective endocarditis at 8 years (aortic valvuloplasty, 100%; Ross, 85.9%; P = .21) were similar. However, freedom from reintervention in the left ventricular outflow tract at 8 years was lower after aortic valvuloplasty than after the Ross procedure (50.1% vs 100%, respectively; P = .001). CONCLUSIONS: Aortic valvuloplasty and the Ross procedure yielded similar 8-year outcomes regarding death, reoperation, and infective endocarditis although aortic valvuloplasty tended to be associated with fewer cases of infective endocarditis. Aortic valvuloplasty using a pericardial patch can be chosen as a first-line strategy for treating complex aortic valve lesions and might offer the possibility of a later Ross procedure.
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Insuficiencia de la Válvula Aórtica/cirugía , Estenosis de la Válvula Aórtica/cirugía , Procedimientos Quirúrgicos Cardíacos , Implantación de Prótesis de Válvulas Cardíacas , Pericardio/trasplante , Adolescente , Factores de Edad , Insuficiencia de la Válvula Aórtica/diagnóstico por imagen , Insuficiencia de la Válvula Aórtica/fisiopatología , Estenosis de la Válvula Aórtica/diagnóstico por imagen , Estenosis de la Válvula Aórtica/fisiopatología , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Niño , Preescolar , Toma de Decisiones Clínicas , Femenino , Implantación de Prótesis de Válvulas Cardíacas/efectos adversos , Humanos , Lactante , Recién Nacido , Masculino , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Factores de Tiempo , Resultado del TratamientoRESUMEN
Effusions can show some surprises. We document the case of a fourteen-month-old male patient with short-bowel syndrome, hospitalized in a cardiology unit, receiving a chronic parenteral nutrition by a Broviac® catheter. The patient presented several thrombosis following iterative catheter replacements. In parallel with superior vena cava plasty, a right intra-atrial Broviac® catheter was placed in the absence of other peripheral venous accesses. This device has a cutaneous exit site to allow for infusion of a hyperosmolar lipid emulsion. Seven days later, a milky liquid was secreted from pericardial/mediastinal redon. A gel lipoprotein electrophoresis of the fluid suggested a preliminary diagnosis of chylopericardium. However, biochemical testing of certain analytes evoked a parenteral nutrition-related pericardial effusion and a possible pseudochyloperitoneum caused by the shearing of a migrated Broviac® in pericardium. The patient, on a fat-free diet, was admitted to the ICU to drain the effusion and reposition the catheter, with success. In the light of new datas on the interference of parenteral lipid emulsions with the lipoproteins gel electrophoresis, we will try to determine whether the apparent presence of chylomicrons in the gel would be the sign of a lesion of the lymphatic system, or rather the result of a contamination by artificial chylomicron in the lipid emulsion, if not the sign of contaminated blood. In our article, we highlight several considerations in identifying and confirming cases of pericardial effusion, such as chylopericardium and parenteral nutrition-related one, as well as points concerning the use of lipid emulsions for pediatric patients with short-bowel syndrome.
Les liquides d'épanchements peuvent renfermer quelques surprises. Nous documentons le cas d'un patient de quatorze mois, hospitalisé en cardiologie, présentant un syndrome de grêle court et recevant une nutrition parentérale au long cours par cathéter Broviac®. Le patient présentait de multiples occlusions veineuses consécutives aux changements itératifs du dispositif. En parallèle d'une plastie de la veine cave supérieure, un Broviac® a été posé en intra-atrial droit devant l'absence d'autres abords veineux périphériques. Ce dispositif comporte un orifice de sortie sous-cutané pour apporter une solution de nutrition hyperosmolaire de type émulsion lipidique. Le liquide recueilli dans les drains péricardiques en post-opératoire est lactescent, particulièrement à partir du septième jour. Le lipidogramme du liquide d'épanchement péricardique semble conclure à la présence de chylomicrons - un chylopéricarde. Cependant, le dosage de certains analytes penche en faveur d'un perfusopéricarde, probablement pseudochyleux, lié au cisaillement du Broviac® dont l'extrémité a migré de l'oreillette droite au péricarde. Le patient, sous régime sans graisses, sans nutrition parentérale, sera réopéré pour drainer l'épanchement et repositionner le cathéter, avec succès. À la lumière de données originales quant à l'interférence des émulsions lipidiques sur le lipidogramme, nous tâcherons de déterminer si l'apparente présence de chylomicrons sur le gel serait le témoin d'une réelle lésion du lymphatique, ou plutôt le fruit d'une contamination par l'émulsion, si ce n'est par le sang. Des considérations au sujet des épanchements péricardiques, dont les chylopéricarde et nutripéricarde, ainsi que sur les émulsions lipidiques pédiatriques dans le contexte du grêle court émailleront ce travail.
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Derrame Pericárdico , Síndrome del Intestino Corto , Humanos , Masculino , Niño , Lactante , Derrame Pericárdico/diagnóstico , Derrame Pericárdico/etiología , Emulsiones , Vena Cava Superior , Nutrición Parenteral , LípidosRESUMEN
Blunt traumatic aortic tear is an extremely rare and life-threatening paediatric emergency. Percutaneous repair is highly debateable in children due to anatomic limitations. Herein, we report an important case of a 5-year-old boy diagnosed with grade 3 aortic isthmus disruption following fifth-floor defenestration. The 96-h control computerized tomography (CT) scan showed an increase in the pseudoaneurysm size with small active bleeding motivating endovascular covered stenting that was safely and efficiently performed with good outcomes.
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Rotura de la Aorta , Procedimientos Endovasculares , Heridas no Penetrantes , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/lesiones , Aorta Torácica/cirugía , Rotura de la Aorta/diagnóstico por imagen , Rotura de la Aorta/cirugía , Preescolar , Humanos , Masculino , Estudios Retrospectivos , Stents , Resultado del Tratamiento , Heridas no Penetrantes/diagnóstico por imagen , Heridas no Penetrantes/cirugíaRESUMEN
Anomalous aortic origin of coronary arteries is a rare congenital heart disease that can be associated with sudden death. We present the case of a young patient who sustained a cardiac arrest revealing an anomalous origin of the right coronary artery. Unroofing and pericardial enlargement of the coronary artery ostia was performed and the patient is actually asymptomatic.
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Anomalías de los Vasos Coronarios/cirugía , Vasos Coronarios/cirugía , Paro Cardíaco/etiología , Revascularización Miocárdica/métodos , Angiografía por Tomografía Computarizada , Angiografía Coronaria , Anomalías de los Vasos Coronarios/complicaciones , Anomalías de los Vasos Coronarios/diagnóstico , Vasos Coronarios/diagnóstico por imagen , Humanos , MasculinoRESUMEN
OBJECTIVES: Aortic root and ascending aorta replacements (AARs) are rarely required in the paediatric population. We report here a series of AAR performed in young children using different surgical techniques. METHODS: Between 1995 and 2017, 32 children under the age of 10 years (median age 5.4 years) underwent AAR procedures at our institution. Twenty-two (69%) had a connective tissue disease (infantile Marfan syndrome or Loeys-Dietz syndrome). We performed 11 AAR using a composite graft with a mechanical prosthesis and 21 valve-sparing procedures (10 Yacoub operations and 11 David operations). Median follow-up for operative survivors was 7.7 years (interquartile range 4.2-12.8 years). RESULTS: The cardiac-related early mortality rate was 6%. Patient survival was 91% at both 1 and 10 years. Eleven survivors (38%), all with a status of post-valve-sparing procedure, required an aortic root reintervention with an aortic valve replacement after a median interval of 4.2 years. Interestingly, only patients with infantile Marfan syndrome tended to be associated with risk of reoperation. CONCLUSIONS: Aortic root and AARs are safe in young children whatever the surgical procedure. Aortic valve-sparing procedures show good long-term results except in children with infantile Marfan syndrome whose ineluctable aortic annulus dilatation or aortic valve regurgitation requires reintervention after a short period.
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Insuficiencia de la Válvula Aórtica , Implantación de Prótesis Vascular , Síndrome de Marfan , Aorta/cirugía , Válvula Aórtica/cirugía , Insuficiencia de la Válvula Aórtica/cirugía , Implantación de Prótesis Vascular/efectos adversos , Niño , Preescolar , Humanos , Síndrome de Marfan/complicaciones , Síndrome de Marfan/cirugía , Reimplantación , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVES: Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden death in children and young adults. The most threatening anatomy is an interarterial and an intramural course, both probably involved in ischaemic phenomena and sudden death. The treatment of interarterial AAOCA remains controversial. Most of the published studies describe the results of the unroofing technique. Our study aims to evaluate the results of a different surgical approach. METHODS: From 2005 to 2019, 61 patients were operated on for an interarterial AAOCA (median age 14.7 years). Forty patients had a right AAOCA, and 21 patients had a left AAOCA including 5 patients with intraseptal course. Seventy percent of patients were symptomatic. Five patients had an aborted sudden cardiac death. Two surgical techniques were used: an 'anatomical' repair for 35 patients (15 left and 22 right AAOCA) or a coronary translocation with creation of a neo-ostia in 19 patients (1 left and 18 right AAOCA). The 5 left AAOCA patients with an intra-septal course required a complete release of the coronary artery from the septum. RESULTS: There was no early or late postoperative death. Three patients had an acute postoperative ischaemic event. Two patients required immediate angioplasty and stenting: 1 patient (7 years) with a hypoplastic right AAOCA and 1 patient (66 years) for inadequate tailoring after septal release. The third patient required an immediate surgical revision (H-2) for left AAOCA thrombosis at the level of the pericardial patch with full myocardial recovery at discharge. During follow-up, 1 patient with right AAOCA translocation and chronic chest pain required subsequent stenting and finally a coronary artery bypass grafting 2 years after initial surgery. One patient who had an asymptomatic mild right coronary stenosis 1 year after anatomical repair was successfully treated by angioplasty alone. All patients but 1 who underwent coronary translocation are totally asymptomatic. All patients with anatomical repair or septal release are free from ischaemic symptoms. CONCLUSIONS: Anatomical repair might provide a better protective option for these patients. Unlike unroofing, it treats the entire intramural segment, relocates the ostium at the appropriate sinus level and corrects any acute take-off angle.
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Anomalías de los Vasos Coronarios , Adolescente , Aorta , Dolor en el Pecho , Niño , Anomalías de los Vasos Coronarios/cirugía , Humanos , Adulto JovenRESUMEN
OBJECTIVES: A double orifice of the left atrioventricular valve (LAVV) associated with atrioventricular septal defects (AVSD) can significantly complicate surgical repair. This study reports our experience of AVSD repair over 3 decades, with special attention to the zone of apposition (ZoA) of the main orifice, and presents a technique of hemivalve pericardial extension in specific situations. METHODS: We performed a retrospective study from 1987 to 2016 on 1067 patients with AVSD of whom 43 (4%) had a double orifice, plus 2 additional patients who required LAVV pericardial enlargement. Median age at repair was 1.3 years. Mean follow-up was 8.2 years (1 month-32 years). RESULTS: Associated abnormalities of the LAVV subvalvular apparatus were found in 7 patients (5 parachute LAVV and 2 absence of LAVV subvalvular apparatus). ZoA was noted in 4 patients (9%): partially closed in 15 (35%) and completely closed in 24 (56%). Four patients required, either at first repair or secondarily, a hemivalve enlargement using a pericardial patch without closure of the ZoA. The early mortality rate was 7% (n = 3), all before 2000. Two patients had unbalanced ventricles and the third had a single papillary muscle. There were no late deaths. Six patients (14%) required 7 reoperations (3 early and 4 late reoperations) for LAVV regurgitation and/or dysfunction, of whom 4 (9%) required mechanical LAVV replacement (all before 2000). Freedom from late LAVV reoperation was 97% at 1 year, 94% at 5 years and 87% at 10, 20 and 30 years. Unbalanced ventricles (P = 0.045), subvalvular abnormalities (P = 0.0037) and grade >2 LAVV postoperative regurgitation (P = 0.017) were identified as risk factors for LAVV reoperations. Freedom from LAVV mechanical valve replacement was 95% at 1 year, 90% at 5 years and 85% at 10, 20 and 30 years. An anomalous LAVV subvalvular apparatus was identified as a risk factor for mechanical valve replacement (P = 0.010). None of the patients who underwent LAVV pericardial extension had significant LAVV regurgitation at the last follow-up examination. CONCLUSIONS: Repair of AVSD and double orifice can be tricky. Preoperative LAVV regurgitation was not identified as an independent predictor of surgical outcome. LAVV hemivalve extension appears to be a useful and effective alternate surgical strategy when the ZoA cannot be closed.
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Defectos de los Tabiques Cardíacos/patología , Procedimientos Quirúrgicos Cardíacos/métodos , Procedimientos Quirúrgicos Cardíacos/mortalidad , Femenino , Defectos de los Tabiques Cardíacos/mortalidad , Defectos de los Tabiques Cardíacos/cirugía , Humanos , Lactante , Masculino , Reoperación , Estudios Retrospectivos , Válvula Tricúspide/anomalías , Válvula Tricúspide/cirugíaRESUMEN
OBJECTIVES: Repair of tetralogy of Fallot (ToF) can be challenging in the presence of an abnormal coronary artery (CA) in 5-12% of cases. The aim of this study was to report our experience with ToF repair without the systematic use of a right ventricle-to-pulmonary artery (RV-PA) conduit. METHODS: We conducted a monocentric retrospective study from 2000 to 2016, including 943 patients with ToF who underwent biventricular repair, of whom 8% (n = 76) presented with an abnormal CA. Mean follow-up time was 50 months (1 month-18 years). RESULTS: The most frequent CA anomaly was the left descending artery arising from the right CA (n = 47, 61.8%). The median age at repair was 7.7 months (1.8 months-16 years). Thirteen patients (17%) required prior palliation, mostly systemic pulmonary shunts for anoxic spells in the neonatal period. Surgical repair allowed us to preserve the annulus in 40 patients (53%) by combining PA trunk plasty, commissurotomy and infundibulotomy under the abnormal CA. If the annulus had to be opened (n = 35, 46%), a transannular patch was inserted after a vertical incision of the PA trunk and extended obliquely on the RV over the anomalous crossing CA (with an infundibulotomy under the abnormal CA). Three patients (4%) required the insertion of an RV-PA conduit (1 valved tube and 2 RV-PA GORE-TEX tubes with annulus conservation). The early mortality rate was 4% (n = 3); none of the deaths was coronary related. Four patients (5%) required reoperation (2 early and 2 late reoperations) for residual pulmonary stenosis, 3 of whom had annulus preservation during the initial repair. The mean RV/left ventricle (LV) pressure ratio and an RV/LV pressure ratio >2/3 were identified as risk factors for right ventricular outflow tract (RVOT) reinterventions (P = 0.0026, P = 0.0085, respectively), RVOT reoperations (P = 0.0002 for both) and reoperation for RVOT residual stenosis (P = 0.0002, P = 0.0014, respectively). Two patients underwent pulmonary valve replacement. Freedom from late reoperation was 100% at 1 year, 97% at 5 years and 84% at 10 and 15 years. CONCLUSIONS: Repair of ToF and abnormal CA can be performed without an RV-PA conduit, with an acceptable low reintervention rate. The high early mortality rate in this series remains a concern. If any doubt remains about the surgical relief of the RVOT obstruction, the RV/LV pressure ratio should always be measured in the operating room.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos , Anomalías de los Vasos Coronarios/complicaciones , Tetralogía de Fallot , Adolescente , Prótesis Vascular , Procedimientos Quirúrgicos Cardíacos/métodos , Procedimientos Quirúrgicos Cardíacos/mortalidad , Niño , Preescolar , Femenino , Ventrículos Cardíacos/cirugía , Humanos , Lactante , Masculino , Arteria Pulmonar/cirugía , Válvula Pulmonar/cirugía , Reoperación/estadística & datos numéricos , Estudios Retrospectivos , Factores de Riesgo , Tetralogía de Fallot/complicaciones , Tetralogía de Fallot/epidemiología , Tetralogía de Fallot/mortalidad , Tetralogía de Fallot/cirugía , Resultado del TratamientoRESUMEN
We present the case of a 60-year old woman with a ruptured thoraco-abdominal aortic aneurysm (TAAA). It was a Type IV TAAA in the Crawford Classification. A mycotic origin was suspected as she had a known history of lymphocytic lymphoma. She underwent thoraco-abdominal aortic replacement with a good surgical result. Histopathological examination revealed destruction of the aortic layers due to inflammatory lymphomatous aortic infiltration. The patient fully recovered.
Asunto(s)
Aneurisma de la Aorta Abdominal/etiología , Aneurisma de la Aorta Torácica/etiología , Rotura de la Aorta/etiología , Implantación de Prótesis Vascular/métodos , Leucemia Linfocítica Crónica de Células B/complicaciones , Aneurisma de la Aorta Abdominal/diagnóstico , Aneurisma de la Aorta Abdominal/cirugía , Aneurisma de la Aorta Torácica/diagnóstico , Aneurisma de la Aorta Torácica/cirugía , Rotura de la Aorta/diagnóstico , Rotura de la Aorta/cirugía , Biopsia , Femenino , Humanos , Leucemia Linfocítica Crónica de Células B/diagnóstico , Persona de Mediana Edad , Tomografía Computarizada por Rayos XRESUMEN
The two main objectives of this single-center, retrospective study were to analyze the outcomes and to identify the independent predictors of 30 day and long-term mortality in case of cardiopulmonary resuscitation requiring extracorporeal life support (ECLS) in the elderly patients. From October 2004 to May 2014, 163 patients with a mean age of 75.5 years (range 70-91) required veno-arterial ECLS. The main indication was postcardiotomy cardiogenic shock (79.6%). Extracorporeal life support duration averaged 5.6 ± 4.3 days. Thirty-day mortality was 72% (n = 117) and follow-up survival rate was 14.1% with a median follow-up of 23.7 months (range 1.5-102.3). Lactatemia was identified as an independent risk factor of 30 day mortality. Previous stroke, lactatemia, bilirubinemia, and ECLS implantation under cardiac massage were identified as independent risk factors for long-term mortality. Extracorporeal life support after TAVI procedure and intra-aortic balloon pump support were identified as protective factors for both 30 day and long-term survival. The 23 patients, still alive at last follow up, have a good quality of life, doing well, and self-catering. Although mortality rate at 30 days is more than 70% in elderly patients requiring circulatory support with ECLS, survivors can pretend to an acceptable long-term survival with a good quality of life. Extracorporeal life support implantation in the elderly patients should remain scarce, but should still be considered as a therapeutic option in well-selected patients.
Asunto(s)
Oxigenación por Membrana Extracorpórea , Choque Cardiogénico/terapia , Anciano , Anciano de 80 o más Años , Oxigenación por Membrana Extracorpórea/efectos adversos , Femenino , Humanos , Contrapulsador Intraaórtico/efectos adversos , Masculino , Calidad de Vida , Estudios Retrospectivos , Choque Cardiogénico/mortalidad , Choque Cardiogénico/psicologíaRESUMEN
OBJECTIVES: We describe in a prospective study, a novel surgical technique for the management of hypoplastic left heart syndrome inspired by the hybrid Norwood approach. METHODS: This new neonatal palliation comprises replacement of the patent ductus arteriosus (PDA) and aortic arch plasty with a pulmonary homograft associated with the banding of both pulmonary arteries and atrial septectomy, under cardiopulmonary bypass without aortic clamping and cardioplegia. Initial results led to tightening of the pulmonary artery band from 3.5 mm to 2.5 mm. RESULTS: From July 2014 to May 2016, 15 patients were initially palliated (13 patients with aortic atresia/mitral atresia, 1 with double inlet right ventricle, 1 with tricuspid atresia) at a median age of 5 days (1-8 days) and a median weight of 3.0 kg (2.3-3.9 kg). All but 2 patients were discharged from the hospital: There was 1 early in-hospital death and 1 patient required hospitalization in the intensive care unit until stage 2. There were also 4 interstage deaths. Nine patients underwent Stage 2 and 1 patient is still waiting. Compared to the classic Norwood procedure, early mortality decreased significantly (43.3-6.7%; P = 0.0074) and overall mortality before Stage 2 was halved (61.6-33.3%). Postoperative morbidity was also reduced. CONCLUSIONS: This new surgical palliative approach to hypoplastic left heart syndrome, particularly useable technically, seems to combine the advantages of the hybrid procedure by avoiding cardiac ischaemia and ventriculotomy without the complications of PDA stenting and restrictive atrial septectomy. Although the mortality rate decreased significantly, it remains substantial in small-volume centres, especially in the interstage period.
Asunto(s)
Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Procedimientos de Norwood , Aloinjertos/cirugía , Aloinjertos/trasplante , Aorta Torácica/cirugía , Femenino , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/mortalidad , Recién Nacido , Masculino , Procedimientos de Norwood/efectos adversos , Procedimientos de Norwood/métodos , Procedimientos de Norwood/mortalidad , Procedimientos de Norwood/estadística & datos numéricos , Complicaciones Posoperatorias , Estudios Prospectivos , Arteria Pulmonar/cirugía , Arteria Pulmonar/trasplanteRESUMEN
OBJECTIVES: The presence of single coronary artery (CA) in the arterial switch operation (ASO) for neonatal treatment with transposition of the great arteries (TGA) has been reported to be an independent risk factor for early death after surgical repair and late reintervention. The study objective was to evaluate the mortality and the CA stenosis risk at early and long term in neonatal ASO for TGA and single CA. METHODS: Between January 1987 and January 2010, 979 neonates underwent an ASO, of which 73 had a single CA (7.5% of all cohort): right ostium with posterior left CA loop was the most frequent pattern (63%), followed by left ostium with an anterior right CA loop (26%). Mean age at operation was 9.3 ± 5.7 days. Mean follow-up was 9.8 years (range: 1-20 years). RESULTS: Eight patients (11%) died, 6 of coronary-related death. Overall, survival was 90.1 ± 1.9% at 1 year and 88.6% ± 3.8% at 2, 5, 10 and 15 years, respectively. Independent risk factor for mortality was associated surgery before 2001. Freedom from coronary events was 91.6 ± 3.3% at 1 year and 88.7 ± 3.8% at 5, 10 and 15 years respectively. No patients required late reintervention for CA surgery or angioplasty. CONCLUSIONS: All coronary-related death occurred within the first 6 months after ASO, and all patients but 1 were operated before 2001. In our experience, it appears that a single CA is not any more a risk factor for early and late mortality after ASO for TGA. Mortality has drastically reduced since 2001 and is now close to that found in TGA with standard coronary patterns. The acquired experience shared between the surgeons and the institution offsets the undeniable surgical difficulty.