RESUMO
PURPOSE: Transposition of great arteries with intact ventricular septum and left ventricular outflow tract obstruction (TGA + IVS + LVOTO) is uncommon. We reviewed operations performed in patients with TGA + IVS + LVOTO in the European Congenital Heart Surgeons Association Congenital Database (ECHSA-CD). METHODS: All 109 patients with a diagnosis of TGA + IVS + LVOTO in ECHSA-CD who underwent cardiac surgery during a 21-year period (01/2000-02/2021, inclusive) were included. Preoperative variables, operative data, and postoperative outcomes were collected. RESULTS: These 109 patients underwent 176 operations, including 37 (21.0%) arterial switch operations (ASO), 26 (14.2%) modified Blalock-Taussig-Thomas shunts (MBTTS), 11 (6.2%) Rastelli operations, and 13 (7.3%) other palliative operations (8 superior cavopulmonary anastomosis[es], 4 Fontan, and 1 other palliative procedure). Of 37 patients undergoing ASO, 22 had a concomitant procedure.There were 68 (38.6%) reoperations, including 11 pacemaker procedures and 8 conduit operations. After a systemic-to-pulmonary artery shunt, reoperations included shunt reoperation (n = 4), Rastelli (n = 4), and superior cavopulmonary anastomosis (n = 3).Overall Operative Mortality was 8.2% (9 deaths), including three following ASO, two following "Nikaidoh, Kawashima, or LV-PA conduit" procedures, and two following Rastelli. Postoperative complications occurred after 36 operations (20.4%). The most common complications were delayed sternal closure (n = 11), postoperative respiratory insufficiency requiring mechanical ventilation >7 days (n = 9), and renal failure requiring temporary dialysis (n = 8). CONCLUSION: TGA + IVS + LVOTO is rare (109 patients in ECHSA-CD over 21 years). ASO, MBTTS, and Rastelli are the most common operations performed for TGA + IVS + LVOTO. Larger international studies with long-term follow-up are needed to better define the anatomy of the LVOTO and to determine the optimal surgical strategy.
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Transposição das Grandes Artérias , Procedimentos Cirúrgicos Cardíacos , Transposição dos Grandes Vasos , Obstrução do Fluxo Ventricular Externo , Humanos , Procedimentos Cirúrgicos Cardíacos/métodos , Reoperação , Estudos Retrospectivos , Transposição dos Grandes Vasos/complicações , Resultado do Tratamento , Obstrução do Fluxo Ventricular Externo/complicaçõesRESUMO
The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.
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Cardiologia , Sistema Cardiovascular , Cirurgiões , Humanos , Criança , Qualidade de Vida , Assistência Centrada no PacienteRESUMO
INTRODUCTION: Endobronchial masses such as mucoepidermoid carcinomas or carcinoid tumors are extremely rare in children and they usually originate from large bronchi. These lesions may cause wheezing and dyspnea with poor response to bronchodilators and mimic the airway obstruction caused by asthma. CASE STUDY: We present the case of an 8-year-old girl with tracheal mucoepidermoid carcinoma who was treated as a difficult asthma case with high dose of inhaled corticosteroids. RESULTS: The characteristic stridor, the lack of response to bronchodilators and to inhaled corticosteroid treatment, combined with the characteristic flow loop in spirometry and the hyperinflation seen on the chest radiograph, all raised the clinical suspicion of a tracheal lesion and indicated the need for flexible bronchoscopy. The bronchoscopy revealed a large lesion obstructing totally the trachea lumen. The latter finding was confirmed by chest high resolution CT. The mass was completely excised via sternotomy under cardiopulmonary bypass, and the pathologic examination showed a low-grade mucoepidermoid carcinoma of the trachea. One month after the surgery she was free of symptoms and her spirometry was normal. CONCLUSION: Tracheal lesions mimic the symptoms of airway obstruction caused by asthma and should be always be part of the differential diagnosis in young patients with no response to asthma treatment.
Assuntos
Carcinoma Mucoepidermoide/diagnóstico , Neoplasias da Traqueia/diagnóstico , Administração por Inalação , Corticosteroides/uso terapêutico , Obstrução das Vias Respiratórias , Asma/diagnóstico , Asma/tratamento farmacológico , Carcinoma Mucoepidermoide/diagnóstico por imagem , Carcinoma Mucoepidermoide/patologia , Carcinoma Mucoepidermoide/cirurgia , Criança , Diagnóstico Diferencial , Feminino , Humanos , Neoplasias da Traqueia/diagnóstico por imagem , Neoplasias da Traqueia/patologia , Neoplasias da Traqueia/cirurgiaRESUMO
INTRODUCTION: We sought to determine the surgical outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) enrolled in the European Congenital Heart Surgeons Association (ECHSA) database. MATERIALS AND METHODS: From 1999 to 2019, 907 patients with ALCAPA underwent surgical repair and were included in the current study. The primary outcome was in-hospital mortality. Secondary outcomes included frequency and results of concomitant mitral valve surgery and postoperative mechanical circulatory support (MCS). RESULTS: The overall in-hospital mortality was 6% (54/907) and was significantly higher in neonates (p = .01), patients with lower body surface area (BSA) (p = .01), and those requiring postoperative MCS (p = .001). Associated mitral valve surgery was performed in 144 patients (15.9%) and was associated with longer cardiopulmonary bypass (CPB) and aortic cross-clamp times (AOX) (p ≤ .0001) but was not significantly related to an increase in in-hospital mortality. Postoperative MCS was required in 66 patients (7.3%). These patients were younger (p ≤ .001), had a lower BSA (p ≤ .001), and required a longer CPB (p ≤ .001) and AOX time (p ≤ .001). CONCLUSIONS: ALCAPA repair can be achieved successfully, and with low surgical risk. Concomitant mitral valve procedures can be performed without increasing operative mortality. The use of MCS remains a valuable option, especially in younger patients.
Assuntos
Artéria Coronária Esquerda Anormal , Síndrome de Bland-White-Garland , Anomalias dos Vasos Coronários , Cirurgiões , Humanos , Lactente , Recém-Nascido , Artéria Pulmonar/cirurgia , Estudos Retrospectivos , Resultado do TratamentoRESUMO
OBJECTIVES: We sought to determine the indications, type, and outcomes of reoperations on the aortic root after repair of tetralogy of Fallot (TOF). METHODS: Eleven centers belonging to the European Congenital Heart Surgeons Association contributed to the data collection process. We included 36 patients who underwent surgical procedures on the aortic root, including surgery on the aortic valve and ascending aorta, between January 1975 and December 2017. Original diagnoses included TOF-pulmonary stenosis (n = 18) and TOF-pulmonary atresia (n = 18). The main indications for reoperation were aortic insufficiency (n = 19, 53%), aortic insufficiency and dilatation of the ascending aorta (n = 10, 28%), aortic root dilatation (n = 4, 11%), and ascending aorta dilatation (n = 3, 8%). RESULTS: The median age at reoperation was 30.4 years (interquartile range 20.3-45.3 years), and mechanical aortic valve replacement was the most common procedure performed. Five patients died early after reoperation (14%), and larger ascending aorta diameters were associated with early mortality (P = .04). The median age at the last follow-up was 41.4 years (interquartile range 24.5-51.6 years). Late death occurred in five patients (5/31, 16%). Most survivors (15/26, 58%) were asymptomatic at the last clinical examination (New York Heart Association, NYHA class I). The remaining patients were NYHA class II (n = 7) and III (n = 3). The most common symptoms were fatigue (n = 5), dyspnea (n = 4), and exercise intolerance (n = 3). CONCLUSIONS: Reoperations on the aortic root are infrequent but may become necessary late after TOF repair. The main indications for reoperation are aortic insufficiency, either isolated or associated with a dilatation of the ascending aorta. The surgical risk at reoperation was high and the presence of ascending aorta dilation is related to higher mortality.
Assuntos
Aorta/cirurgia , Insuficiência da Valva Aórtica/cirurgia , Valva Aórtica/cirurgia , Implante de Prótese de Valva Cardíaca , Tetralogia de Fallot/cirurgia , Adulto , Aorta/patologia , Criança , Pré-Escolar , Dilatação Patológica , Feminino , Seguimentos , Humanos , Masculino , Reoperação , Fatores de Tempo , Resultado do TratamentoRESUMO
Surgery for Tetralogy of Fallot progressed rapidly from the palliative arterio-pulmonary Blalock-Taussig shunt, introduced in Baltimore 70 years ago, to the "classic" complete transventricular repair technique, with which excellent early results were achieved soon thereafter. However, as duration of follow-up increased, so did the awareness of development of troubling late complications, including severe pulmonary insufficiency, right ventricular dilatation and dysfunction, and tricuspid valve insufficiency, all contributing to increasing incidence of late reoperations, as well as to arrhythmias and sudden death. This realization fueled the initial introduction of the transatrial-transpulmonary repair technique by Kawashima, as well as the subsequent firm establishment of this technique within the framework of an integrated surgical approach by Roger Mee in Melbourne. In turn, Mee's numerous trainees and associates led the dissemination of this approach and provided the impetus for the current wide adoption of a variety of right ventricular and pulmonary valve preservation techniques. In addition to the outstanding surgical results reported by individual centers adopting this surgical strategy, encouraging multi-institutional data are emerging regarding the benefits of these approaches for more favorable early and, most importantly, late outcome. One student and strong proponent of the Melbourne approach was our late colleague and friend Juan Comas, to whose memory this article can serve as tribute.
Assuntos
Procedimentos Cirúrgicos Cardíacos/história , Tetralogia de Fallot/história , Austrália , Baltimore , História do Século XX , Humanos , Espanha , Tetralogia de Fallot/cirurgiaRESUMO
Across the globe, the implementation of quality improvement science and collaborative learning has positively affected the care and outcomes for children born with CHD. These efforts have advanced the collective expertise and performance of inter-professional healthcare teams. In this review, we highlight selected quality improvement initiatives and strategies impacting the field of cardiovascular care and describe implications for future practice and research. The continued leveraging of technology, commitment to data transparency, focus on team-based practice, and recognition of cultural norms and preferences ensure the success of sustainable models of global collaboration.
Assuntos
Cardiopatias Congênitas/cirurgia , Equipe de Assistência ao Paciente/normas , Atenção Primária à Saúde/métodos , Melhoria de Qualidade/organização & administração , Comportamento Cooperativo , HumanosRESUMO
Breast cancer is often treated with radiotherapy (RT), with two opposing tangential fields. When indicated, supraclavicular lymph nodes have to be irradiated, and a third anterior field is applied. The junction region has the potential to be over or underdosed. To overcome this problem, many techniques have been proposed. A literature review of 3 Dimensional Conformal RT (3D CRT) and older 3-field techniques was carried out. Intensity Modulated RT (IMRT) techniques are also briefly discussed. Techniques are categorized, few characteristic examples are presented and a comparison is attempted. Three-field techniques can be divided in monoisocentric and two-isocentric. Two-isocentric techniques can be further divided in full field and half field techniques. Monoisocentric techniques show certain great advantages over two-isocentric techniques. However, they are not always applicable and they require extra caution as they are characterized by high dose gradient in the junction region. IMRT has been proved to give better dosimetric results. Three-field matching is a complicated procedure, with potential of over or undredosage in the junction region. Many techniques have been proposed, each with advantages and disadvantages. Among them, monoisocentric techniques, when carefully applied, are the ideal choice, provided IMRT facility is not available. Otherwise, a two-isocentric half beam technique is recommended.
Assuntos
Neoplasias da Mama/radioterapia , Radioterapia Conformacional/métodos , Feminino , Humanos , Dosagem Radioterapêutica , Radioterapia de Intensidade ModuladaRESUMO
PURPOSE: To evaluate the impact of splenic irradiation as a palliative treatment for symptomatic splenomegaly due to secondary myelofibrosis. METHODS: Seventeen patients with chronic myelogenous leukemia and 3 with idiopathic polycythaemia presented with splenomegaly, splenic pain and anemia. Due to symptomatic splenomegaly, despite first-line treatment, the patients underwent splenic irradiation. Two patients received two different schedules of external radiotherapy (580 cGy in 5 fractions and 600 cGy in 6 fractions). Eight patients received 980 cGy in 14 fractions. Ten patients received two courses of 360 cGy in 6 fractions, 3 months apart. Median follow-up was 12 months post irradiation. RESULTS: The patients showed excellent response to treatment one month post-radiotherapy, while treatment was well tolerated without severe toxicity. The dimensions of the spleen decreased significantly. Pain-related Visual Analogue Score (VAS) regressed after completion of irradiation. During 12-month follow-up all patients maintained the benefit of radiotherapy. CONCLUSION: This study indicates that splenic irradiation could be a safe and effective palliative treatment for symptomatic splenomegaly due to secondary myelofibrosis.
Assuntos
Cuidados Paliativos , Mielofibrose Primária/complicações , Baço/efeitos da radiação , Esplenomegalia/radioterapia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
PURPOSE: To prospectively assess the efficacy of the selective serotonin inhibitor escitalopram on painful bone metastases, in combination with external beam irradiation. METHODS: Forty-three patients with cancer metastatic to bone and suffering from depression were treated with 3 Dimensional Conformal Radiotherapy (3DCRT) (30 Gy; 3 Gy/fraction, 5 days/week) combined with escitalopram (20 mg/day). Pain relief was evaluated with Wong/Baker Faces Pain Scale. The patients reported outcome using a RTOG-EORTC quality-of-life self-questionnaire (QLQ-C30 v3.0) and the status of depression according to Hamilton Scale (HAM-17). The assessment was performed at baseline and 6-8 weeks after radiotherapy. RESULTS: Patients treated with radiotherapy and escitalopram tended to show a good response to pain and improvement of their quality of life. CONCLUSIONS: Though our data concerned a rather small number of patients, addition of escitalopram to 3DCRT accomplished a high clinical benefit rate on neuropathic pain from bone metastasis.
Assuntos
Neoplasias Ósseas/secundário , Neoplasias Ósseas/terapia , Citalopram/uso terapêutico , Neuralgia/terapia , Qualidade de Vida , Radioterapia Conformacional/métodos , Idoso , Idoso de 80 Anos ou mais , Neoplasias Ósseas/psicologia , Terapia Combinada , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
PURPOSE: The primary endpoint was to assess the late toxicity of a hypofractionated radiotherapy schedule in relation to radiation parameters concerning the rectum and bladder. The second endpoint was to assess a composite of biochemical and clinical failure. METHODS: Sixty-four prospectively selected patients diagnosed with localized low risk prostate cancer, Gleason score (GS) <7, PSA <10, and T1-2N0, were treated with external 3- dimensional conformal radiotherapy (3D-CRT). Patients received 57.75 Gy in 21 daily fractions of 2.75 Gy/fraction. RESULTS: Late gastrointestinal (GI) toxicity was as follows: grade 0: 47 (73.4 %) patients, grade 1: 12 (19.2 %), grade 2: 4 (6.3%), and grade 3: 1 (1.6%). There was a significant correlation between D50, V70 and EORTC/RTOG late rectal toxicity score (p<0.001 and p=0.006, respectively). Grade 1 and 2 late bladder toxicity was seen in 4.7 and 1.6% of the patients, respectively. With a median follow up of 18 months no biochemical relapse was observed. CONCLUSION: The present study supports the use of hypofractionated radiation therapy which showed a high therapeutic ratio with acceptable toxicity and no biochemical relapse during follow-up.
Assuntos
Fracionamento da Dose de Radiação , Neoplasias da Próstata/radioterapia , Idoso , Humanos , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Estudos Prospectivos , Neoplasias da Próstata/mortalidade , Neoplasias da Próstata/patologia , Radioterapia/efeitos adversos , Reto/efeitos da radiação , Bexiga Urinária/efeitos da radiaçãoRESUMO
BACKGROUND: We evaluated outcomes of neonatal cardiac surgery at hospitals affiliated with the European Congenital Heart Surgeons Association (ECHSA). METHODS: All patients ≤30 days of life undergoing a cardiac surgical procedure during a 10 year period between January 2013 and December 2022 were selected from the ECHSA Congenital Database. Reoperations during the same hospitalization, percutaneous procedures, and noncardiac surgical procedures were excluded. A total of 12 benchmark operations were identified. Primary outcomes were 30-day mortality and in-hospital mortality. Multivariable logistic regression analysis was performed to determine independent factors associated with higher mortality. A comparison of mortality between the first 5 years and second 5 years was also performed. RESULTS: The overall number of neonatal operations from 2013-2022 was 30,931, and 22,763 patients met the inclusion criteria of the study. The four most common procedures were: arterial switch operation (3,520/22,763=15.5%), aortic coarctation repair (3,204/22,763=14.1%), shunt procedure (2,351/22,763=10.3%), and Norwood Operation (2,115/22,763=9.23%). 30-day mortality rate was: overall population (1,342/22,763=5.9%), arterial switch (110/3,520=3.13%), Norwood Operation (339/2,115=16.0%), and hybrid operation (94/609=15.4%). In-hospital mortality rate was: overall population (2,074/22,763=9.1%), arterial switch (145/3,520=4.12%), Norwood Operation (523/2,115=24.7%), and hybrid operation (186/609=30.5%). Multivariable analysis revealed that major factors impacting mortality were high risk procedures (adjusted odds ratio=2.74 [95% confidence interval=2.33-3.23]; p<0.001), and the need for extracorporeal membrane oxygenation (11.8 [9.9-14]; p <0.001). CONCLUSIONS: Neonatal cardiac surgery continues to pose a significant challenge, with notable mortality, particularly for neonates with functionally univentricular physiology. These data can serve as important benchmarks across Europe and offer insights regarding opportunities for improvement.
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OBJECTIVES: Symptomatic neonates and infants with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic focusing on risk stratification and management. METHODS: The EA Clinical Congenital Practice Standards Committee is a multinational and multidisciplinary group of surgeons and cardiologists with expertise in EA. A citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to EA. The search was restricted to the English language and the year 2000 or later and yielded 455 results, of which 71 were related to neonates and infants. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of members votes with at least 75% agreement on each statement. RESULTS: When evaluating fetuses with EA, those with severe cardiomegaly, retrograde or bidirectional shunt at the ductal level, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or fetal hydrops should be considered high risk for intrauterine demise and postnatal morbidity and mortality. Neonates with EA and severe cardiomegaly, prematurity (<32 weeks), intrauterine growth restriction, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or cardiogenic shock should be considered high risk for morbidity and mortality. Hemodynamically unstable neonates with a circular shunt should have emergent interruption of the circular shunt. Neonates in refractory cardiogenic shock may be palliated with the Starnes procedure. Children may be assessed for later biventricular repair after the Starnes procedure. Neonates without high-risk features of EA may be monitored for spontaneous closure of the patent ductus arteriosus (PDA). Hemodynamically stable neonates with significant pulmonary regurgitation at risk for circular shunt with normal right ventricular systolic pressure should have an attempt at medical closure of the PDA. A medical trial of PDA closure in neonates with functional pulmonary atresia and normal right ventricular systolic pressure (>20-25 mm Hg) should be performed. Neonates who are hemodynamically stable without pulmonary regurgitation but inadequate antegrade pulmonary blood flow may be considered for a PDA stent or systemic to pulmonary artery shunt. CONCLUSIONS: Risk stratification is essential in neonates and infants with EA. Palliative comfort care may be reasonable in neonates with associated risk factors that may include prematurity, genetic syndromes, other major medical comorbidities, ventricular dysfunction, or sepsis. Neonates who are unstable with a circular shunt should have emergent interruption of the circular shunt. Neonates who are unstable are most commonly palliated with the Starnes procedure. Neonates who are stable should undergo ductal closure. Neonates who are stable with inadequate pulmonary flow may have ductal stenting or a systemic-to-pulmonary artery shunt. Subsequent procedures after Starnes palliation include either single-ventricle palliation or biventricular repair strategies.
Assuntos
Consenso , Anomalia de Ebstein , Humanos , Recém-Nascido , Anomalia de Ebstein/cirurgia , Anomalia de Ebstein/fisiopatologia , Lactente , Medição de Risco , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Fatores de Risco , Resultado do Tratamento , Cirurgia Torácica/normasRESUMO
OBJECTIVES: Anomalous aortic origin of a coronary artery (AAOCA) is a group of rare congenital heart defects with various clinical presentations. The lifetime-risk of an individual living with AAOCA is unknown, and data from multicentre registries are urgently needed to adapt current recommendations and guide optimal patient management. The European AAOCA Registry (EURO-AAOCA) aims to assess differences with regard to AAOCA management between centres. METHODS: EURO-AAOCA is a prospective, multicentre registry including 13 European centres. Herein, we evaluated differences in clinical presentations and management, treatment decisions and surgical outcomes across centres from January 2019 to June 2023. RESULTS: A total of 262 AAOCA patients were included, with a median age of 33 years (12-53) with a bimodal distribution. One hundred thirty-nine (53.1%) were symptomatic, whereas chest pain (n = 74, 53.2%) was the most common complaint, followed by syncope (n = 21, 15.1%). Seven (5%) patients presented with a myocardial infarction, 2 (1.4%) with aborted sudden cardiac death. Right-AAOCA was most frequent (150, 57.5%), followed by left-AAOCA in 51 (19.5%), and circumflex AAOCA in 20 (7.7%). There were significant differences regarding diagnostics between age groups and across centres. Seventy-four (28.2%) patients underwent surgery with no operative deaths; minor postoperative complications occurred in 10 (3.8%) cases. CONCLUSIONS: Currently, no uniform agreement exists among European centres with regard to diagnostic protocols and clinical management for AAOCA variants. Although surgery is a safe procedure in AAOCA, future longitudinal outcome data will hopefully shed light on how to best decide towards optimal selection of patients undergoing revascularization versus conservative treatment.
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BACKGROUND: To evaluate indications and results of surgery for primary cardiac tumors in children. METHODS AND RESULTS: Eighty-nine patients aged ≤18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years). Sixty-three patients (68.5%) presented with symptoms. Surgery consisted of complete resection in 62 (69.7%) patients, partial resection in 21 (23.6%), and cardiac transplant in 4 (4.5%). Most frequent histotypes (93.2%) were benign (rhabdomyoma, myxoma, teratoma, fibroma, and hemangioma). Postoperative complications occurred in 29.9%. Early and late mortality were 4.5% each (mean follow-up, 6.3±4.4 years); major adverse events occurred in 28.2% of the patients; 90.7% of patients are in New York Heart Association class I. There were no statistically significant differences in survival, postoperative complications, or adverse events after complete and partial resection in benign tumors other than myxomas. Cardiac transplant was associated significantly with higher mortality rate (P=0.006). Overall mortality was associated to malignancy (P=0.0008), and adverse events during follow-up (P=0.005). CONCLUSIONS: Surgery for primary cardiac tumors in children has good early and long-term outcomes, with low recurrence rate. Rhabdomyomas are the most frequent surgical histotypes. Malignant tumors negatively affect early and late survival. Heart transplant is indicated when conservative surgery is not feasible. Lack of recurrence after partial resection of benign cardiac tumors indicates that a less risky tumor debulking is effective for a subset of histotypes such as rhabdomyomas and fibromas.
Assuntos
Neoplasias Cardíacas/mortalidade , Neoplasias Cardíacas/cirurgia , Médicos , Complicações Pós-Operatórias/mortalidade , Cuidados Pré-Operatórios/tendências , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Europa (Continente)/epidemiologia , Feminino , Seguimentos , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Humanos , Lactente , Recém-Nascido , Masculino , Complicações Pós-Operatórias/etiologia , Diagnóstico Pré-Natal/tendências , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Resultado do TratamentoRESUMO
BACKGROUND: We previously showed that machine learning-based methodologies of optimal classification trees (OCTs) can accurately predict risk after congenital heart surgery and assess case-mix-adjusted performance after benchmark procedures. We extend this methodology to provide interpretable, easily accessible, and actionable hospital performance analysis across all procedures. METHODS: The European Congenital Heart Surgeons Association Congenital Cardiac Database data subset of 172,888 congenital cardiac surgical procedures performed in European centers between 1989 and 2022 was analyzed. OCT models (decision trees) were built predicting hospital mortality (area under the curve [AUC], 0.866), prolonged postoperative mechanical ventilatory support time (AUC, 0.851), or hospital length of stay (AUC, 0.818), thereby establishing case-adjusted benchmarking standards reflecting the overall performance of all participating hospitals, designated as the "virtual hospital." OCT analysis of virtual hospital aggregate data yielded predicted expected outcomes (both aggregate and for risk-matched patient cohorts) for the individual hospital's own specific case-mix, readily available on-line. RESULTS: Raw average rates were hospital mortality, 4.9%; mechanical ventilatory support time, 14.5%; and length of stay, 15.0%. Of 146 participating centers, compared with each hospital's overall case-adjusted predicted hospital mortality benchmark, 20.5% statistically (<90% CI) overperformed and 20.5% underperformed. An interactive tool based on the OCT analysis automatically reveals 14 hospital-specific patient cohorts, simultaneously assessing overperformance or underperformance, and enabling further analysis of cohort strata in any chosen time frame. CONCLUSIONS: Machine learning-based OCT benchmarking analysis provides automatic assessment of hospital-specific case-adjusted performance after congenital heart surgery, not only overall but importantly, also by similar risk patient cohorts. This is a tool for hospital self-assessment, particularly facilitated by the user-accessible online-platform.
RESUMO
The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.