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BACKGROUND: Young patients suffering from cryptogenic stroke alongside a patent foramen ovale (PFO) are often considered for percutaneous device closure to reduce the risk of stroke recurrence. Residual right-to-left shunt after device closure may persist in approximately a quarter of the patients at 6 months, and some may close at a later time point. This study aimed to assess the prevalence and persistence of residual right-to-left shunt after percutaneous PFO closure. METHODS: Consecutive patients undergoing transoesophageal echocardiography-guided PFO closure for cryptogenic stroke between 2006 and 2021, with echocardiographic follow-up including contrast bubble study and Valsalva manoeuvre, were enrolled. Follow-up transthoracic echocardiography was performed at 6 months and repeated at 12 months in case of residual right-to-left shunt. Primary outcomes included the prevalence and grade of residual right-to-left shunt at 6 and 12 months after percutaneous PFO closure. RESULTS: 227 patients were included with a mean age of 43±11 years and 50.2% were women. At 6-month follow-up, 72.7% had no residual right-to-left shunt, 12.3% small residual right-to-left shunt, 6.6% moderate residual right-to-left shunt and 8.4% large residual right-to-left shunt. At 12-month follow-up, the presence of residual right-to-left shunt in all patients was 12.3%, of whom 6.6% had small residual right-to-left shunt, 2.6% had moderate residual right-to-left shunt and 3.1% had large residual right-to-left shunt. CONCLUSIONS: Residual right-to-left shunts are common at 6 months after percutaneous closure of PFO. However, the majority are small and two-thirds of residual right-to-left shunts achieve complete closure between 6 and 12 months.
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In patients born with anorectal malformations (ARM), additional congenital heart defects (CHD) can occur. We aimed to provide an overview on disease and treatment details of CHD identified in patients born with ARM, from a unique large cohort of a very rare disease. We performed a retrospective single-center cohort study between January 2000 and July 2023. All consecutive patients with ARM were included. Outcomes were the number of patients with CHD, and screening percentage and percentage of patients diagnosed with CHD over 3 time periods (2000-2006, 2007-2014, 2015-2023). We used uni- and multi-variable logistic regression analyses to search for associations between CHD present and baseline characteristics. In total, 281 patients were included. Some 241 (85.8%) underwent echocardiography, of whom 80 (33.2%) had CHD. Screening percentage with echocardiography increased (74.1% vs. 85.7% vs. 95.9%, p < 0.001) and percentage of patients diagnosed with CHD remained similar over time (30.2% vs. 34.5% vs. 34.0%, p = 0.836). Atrial and ventricular septal defects (n = 36, n = 29), and persistent left superior vena cava (n = 17) were most identified. The presence of VACTERL-association or a genetic syndrome was independently associated with the presence of CHD. CHD were present in 33% of patients with ARM that underwent echocardiography. Over time, the number of CHD identified through screening remained similar. Patients with the presence of VACTERL-association or a genetic syndrome had a higher risk of having CHD. Therefore, acknowledging the potential presence of CHD in patients with ARM remains important.
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BACKGROUND: Balloon atrial septostomy (BAS) is an emergent and essential cardiac intervention to enhance intercirculatory mixing at atrial level in deoxygenated patients diagnosed with transposition of the great arteries (TGA) and restrictive foramen ovale. The recent recall of several BAS catheters and the changes in the European legal framework for medical devices (MDR 2017/745), has led to an overall scarcity of BAS catheters and raised questions about the use, safety, and experience of the remaining NuMED Z-5 BAS catheter. AIMS: To evaluate and describe the practice and safety of the Z-5 BAS catheter, and to compare it to the performance of other BAS catheters. METHODS: A retrospective single-center cohort encompassing all BAS procedures performed with the Z-5 BAS catheter in TGA patients between 1999 and 2022. RESULTS: A total of 182 BAS procedures were performed in 179 TGA-newborns at Day 1 (IQR 0-5) days after birth, with median weight of 3.4 (IQR 1.2-5.7) kg. The need for BAS was urgent in 90% of patients. The percentage of BAS procedures performed at bedside increased over time from 9.8% (before 2010) to 67% (2017-2022). Major complication rate was 2.2%, consisting of cerebral infarction (1.6%) and hypovolemic shock (0.5%). The rate of minor complications was 9.3%, including temporary periprocedural AV-block (3.8%), femoral vein thrombosis (2.7%), transient intracardiac thrombus (0.5%), and atrial flutter (2.2%). BAS procedures performed at bedside and in the cardiac catheterization laboratory had similar complication rates. CONCLUSIONS: BAS using the Z-5 BAS catheter is both feasible and safe at bedside and at the cardiac catheterization laboratory with minimal major complications.
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Transposição dos Grandes Vasos , Humanos , Recém-Nascido , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Transposição dos Grandes Vasos/complicações , Estudos Retrospectivos , Resultado do Tratamento , Catéteres , ArtériasRESUMO
INTRODUCTION: Increasing survival of adult congenital heart disease (ACHD) patients comes at the price of a range of late complications-arrhythmias, heart failure, and valvular dysfunction. Transcatheter valve interventions have become a legitimate alternative to conventional surgical treatment in selected acquired heart disease patients. However, literature on technical aspects, hemodynamic effects, and clinical outcomes of percutaneous atrioventricular (AV) valve interventions in ACHD patients is scarce. METHOD: This is a descriptive cohort from CAHAL (Center of Congenital Heart Disease Amsterdam-Leiden). ACHD patients with severe AV valve regurgitation who underwent a transcatheter intervention in the period 2020-2022 were included. Demographic, clinical, procedural, and follow-up data were collected from patient records. RESULTS: Five ACHD patients with severe or torrential AV valve regurgitation are described. Two patients underwent a transcatheter edge-to-edge repair (TEER), one patient underwent a valve-in-valve procedure, one patient received a Cardioband system, and one patient received both a Cardioband system and TEER. No periprocedural complications occurred. Post-procedural AV valve regurgitation as well as NYHA functional class improved in all patients. The median post-procedural NYHA functional class improved from 3.0 (IQR [2.5-4.0]) to 2.0 (IQR [1.5-2.5]). One patient died 9 months after the procedure due to advanced heart failure with multiorgan dysfunction. CONCLUSION: Transcatheter valve repair is feasible and safe in selected complex ACHD patients. A dedicated heart team is essential for determining an individualized treatment strategy as well as pre- and periprocedural imaging to address the underlying mechanism(s) of AV regurgitation and guide the transcatheter intervention. Long-term follow-up is essential to evaluate the clinical outcomes of transcatheter AV valve repair in ACHD patients.
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BACKGROUND: Congenital abdominal wall defects might be associated with other anomalies, such as atresia in gastroschisis and cardiac anomalies in omphalocele patients. However, in the current literature, an overview of these additional anomalies and potential patient-specific risk factors is missing. Therefore, we aimed to assess the prevalence of associated anomalies and their patient-specific risk factors in patients with gastroschisis and omphalocele. METHODS: A mono-center retrospective cohort study between 1997 and 2023 was performed. Outcomes were the presence of any additional anomalies. Risk factors were analyzed via logistic regression analysis. RESULTS: In total, 122 patients were included, of whom 82 (67.2%) had gastroschisis, and 40 (32.8%) had omphalocele. Additional anomalies were identified in 26 gastroschisis patients (31.7%) and in 27 omphalocele patients (67.5%). In patients with gastroschisis, intestinal anomalies were most identified (n = 13, 15.9%), whereas, in patients with omphalocele, cardiac anomalies were most identified (n = 15, 37.5%). Logistic regression showed that cardiac anomalies were associated with complex gastroschisis (OR: 8.5; CI-95%: 1.4-49.5). CONCLUSIONS: In patients with gastroschisis and omphalocele, intestinal and cardiac anomalies were most identified, respectively. Cardiac anomalies were found to be a risk factor for patients with complex gastroschisis. Therefore, regardless of the type of gastroschisis and/or omphalocele, postnatal cardiac screening remains important.
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BACKGROUND: Cardiac anomalies occur frequently in patients with congenital duodenal obstruction (DO). However, the exact occurrence and the type of associated anomalies remain unknown. Therefore, the aim of this systematic review is to aggregate the available literatures on cardiac anomalies in patients with DO. METHODS: In July 2022, a search was performed in PubMed and Embase.com. Studies describing cardiac anomalies in patients with congenital DO were considered eligible. Primary outcome was the pooled percentage of cardiac anomalies in patients with DO. Secondary outcomes were the pooled percentages of the types of cardiac anomalies, type of DO, and trisomy 21. A meta-analysis was performed to pool the reported data. RESULTS: In total, 99 publications met our eligibility data, representing 6725 patients. The pooled percentage of cardiac anomalies was 29% (95% CI 0.26-0.32). The most common cardiac anomalies were persistent foramen ovale 35% (95% CI 0.20-0.54), ventricular septal defect 33% (95% CI 0.24-0.43), and atrial septal defect 33% (95% CI 0.26-0.41). The most prevalent type of obstruction was type 3 (complete atresias), with a pooled percentage of 54% (95% CI 0.48-0.60). The pooled percentage of Trisomy 21 in patients with DO was 28% (95% CI 0.26-0.31). CONCLUSION: This review shows cardiac anomalies are found in one-third of the patients with DO regardless of the presence of trisomy 21. Therefore, we recommend that patients with DO should receive preoperative cardiac screening. LEVEL OF EVIDENCE: II.
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Síndrome de Down , Obstrução Duodenal , Cardiopatias Congênitas , Humanos , Criança , Síndrome de Down/complicações , Síndrome de Down/epidemiologia , Obstrução Duodenal/congênito , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/epidemiologiaRESUMO
OBJECTIVES: Coronary transfer remains the most crucial part of the arterial switch operation (ASO); yet, certain coronary anatomies prohibit the use of button or trap-door transfer techniques. In the rare setting of 'non-separable' single sinus coronary arteries with intramural course, the modified Yacoub aortocoronary flap technique is a viable option. The aim of this study is to describe this operative technique and review its early- and mid-term outcomes. METHODS: This retrospective analysis included all cases with 'non-separable' single sinus coronary arteries with intramural course where the modified Yacoub aortocoronary flap technique served as a bail-out option. RESULTS: Of 516 patients who underwent ASO at our institution between January 1977 and April 2022, 14 underwent the modified Yacoub aortocoronary flap technique. The median age at ASO was 10 (interquartile range 7-19) days. Hospital mortality occurred in 3 patients (21.4%), all being related to coronary complications. All hospital survivors were still alive at a median of 9.1 (interquartile range 4.2-18.3) years after the ASO. None of them developed complaints of ischaemia, ventricular arrhythmias, ventricular dysfunction or exercise intolerance. Surveillance computed tomography angiography showed stable aortocoronary relationships free from stenosis, compression and kinking. No reoperations for coronary artery problems and/or neoaortic valve or root problems were needed. CONCLUSIONS: Although close monitoring of early coronary events seems crucial to prevent perioperative mortality, the modified Yacoub aortocoronary flap technique may serve as a viable bail-out option in patients with 'non-separable' single sinus coronary anatomy with intramural course, with excellent results among hospital survivors.
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Transposição das Grandes Artérias , Doença da Artéria Coronariana , Anomalias dos Vasos Coronários , Transposição dos Grandes Vasos , Recém-Nascido , Humanos , Transposição das Grandes Artérias/efeitos adversos , Transposição das Grandes Artérias/métodos , Transposição dos Grandes Vasos/cirurgia , Estudos Retrospectivos , Doença da Artéria Coronariana/epidemiologia , Anomalias dos Vasos Coronários/cirurgiaRESUMO
BACKGROUND: In children with anorectal malformations (ARM), additional anomalies can occur within the VACTERL-association. Routine screening is of great importance for early identification and potential treatment. However, uniformity in screening protocols is lacking and only small cohorts have been described in literature. The aim of this study was to assess and describe a unique large cohort of ARM patients who underwent VACTERL screening in the neonatal period. METHODS: A retrospective mono-center cohort study was performed. Included were all neonates born between January 2000 and December 2020 who were diagnosed with ARM and screened for additional anomalies. Full screening consisted of x-ray and ultrasound of the spine, cardiac and renal ultrasound, and physical examination for limb deformities, esophageal atresia, and ARM. Criteria for VACTERL-classification were predefined according to the EUROCAT-definitions. RESULTS: In total, 216 patients were included, of whom 167 (77.3%) underwent full VACTERL-screening (66% in 2000-2006 vs. 82% in 2007-2013 vs. 86% in 2014-2020). Median age at follow-up was 7.0 years (IQR 3.0-12.8). In 103/167 patients (61.7%), additional anomalies were identified. Some 35/216 patients (16.2%) fulfilled the criteria of a form of VACTERL-association. In 37/216 patients (17.1%), a genetic cause or syndrome was found. CONCLUSIONS: The majority of ARM patients underwent full screening to detect additional anomalies (77%), which improved over time to 86%. Yet, approximately a quarter of patients was not screened, with the potential of missing important additional anomalies that might have severe consequences in the future. Forms of VACTERL-association or genetic causes were found in 16% and 17% respectively. This study emphasizes the importance of routine screening. LEVEL OF EVIDENCE: III.
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Malformações Anorretais , Cardiopatias Congênitas , Deformidades Congênitas dos Membros , Recém-Nascido , Criança , Humanos , Pré-Escolar , Malformações Anorretais/diagnóstico , Malformações Anorretais/epidemiologia , Estudos Retrospectivos , Estudos de Coortes , Esôfago/diagnóstico por imagem , Esôfago/anormalidades , Traqueia/anormalidades , Deformidades Congênitas dos Membros/diagnóstico por imagem , Deformidades Congênitas dos Membros/epidemiologia , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/epidemiologia , Rim/anormalidades , Canal Anal/diagnóstico por imagem , Canal Anal/anormalidades , Coluna Vertebral/diagnóstico por imagem , Coluna Vertebral/anormalidadesRESUMO
BACKGROUND: Duodenal obstruction (DO) is a congenital anomaly that is highly associated with other anomalies, such as cardiac anomalies and trisomy 21. However, an overview of additional anomalies and patient-specific risk factors for cardiac anomalies is lacking. Potential association with the vertebral, anorectal, cardiac, trachea-esophageal, renal and limb anomalies (VACTERL) spectrum remains unknown. Therefore, we aim to examine the incidence of associated anomalies, a VACTERL-spectrum association and patient-specific risk factors for cardiac anomalies in patients with DO. METHODS: A retrospective cohort study was performed between 1996 and 2021. Outcomes were the presence of any additional anomalies. Risk factors for cardiac anomalies were analyzed using multivariate logistic regression. RESULTS: Of 112 neonates with DO, 47% (N = 53/112) had one associated anomaly and 38% (N = 20/53) had multiple anomalies. Cardiac anomalies (N = 35/112) and trisomy 21 (N = 35/112) were present in 31%. In four patients, VACTERL-spectrum was discovered, all with cardiac anomalies. Trisomy 21 was found to be a risk factor for cardiac anomalies (OR:6.5; CI-95%2.6-16.1). CONCLUSION: Associated anomalies were present in half of patients with DO, of which cardiac anomalies and trisomy 21 occurred most often, and the VACTERL-spectrum was present in four patients. Trisomy 21 was a significant risk factor for cardiac anomalies. Therefore, we recommend a preoperative echocardiogram in patients with DO. In case a cardiac anomaly is found without trisomy 21, VACTERL-screening should be performed.
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BACKGROUND: During winter, the influenza and Respiratory Syncytial Virus season, children are often seen in the emergency department with fever and respiratory symptoms. CASE DESCRIPTION: We describe the case of a 2-year-old girl with fever, dyspnea and signs of heart failure during physical examination. Echocardiogram showed a large amount of pericardial effusion with signs of cardiac tamponade. Pericardiocentesis showed 200 ml purulent liquid. Surgical drainage was performed. However pericardial effusion recurred. Because constrictive pericarditis was feared, a pericardiectomy was performed after which the patient recovered. Cultures showed Haemophilus influenzae type b and the patient was treated with amoxicillin for 4 weeks in total. CONCLUSION: Fever and dyspnea may also be caused by a problem outside the respiratory system. With physical examination a cardiac cause, such as pericarditis, can be detected. An ECG is easily obtained and can help in the differential diagnosis. An echocardiogram can confirm the diagnosis.
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Pericardite Constritiva , Pericardite , Criança , Pré-Escolar , Dispneia/etiologia , Feminino , Hepatomegalia , Humanos , Pericardiectomia , Pericardiocentese , Pericardite Constritiva/cirurgiaRESUMO
This report describes the case of a symptomatic patient with a right coronary artery fistula draining into the coronary sinus who underwent transcatheter closure, which was deployed in the drainage site to seal off the exit of the fistula. (Level of Difficulty: Advanced.).
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Pathogenic heterozygous NEXN variants are associated with progressive dilated cardiomyopathy (DCM) usually presenting around 50 years of age. We describe an asymptomatic boy who had transient DCM at 3 months of age, that resolved by 4 months. Presently, at 11 years of age, he has normal cardiac function with signs of mild DCM on cardiac MRI. Genetic diagnostics revealed a paternally derived, heterozygous 1949_1951del class 4 variant in NEXN. His father had mild DCM with mildly reduced systolic function. The second patient presented with fetal hydrops at 33 weeks gestation requiring emergency caesarian delivery. Postnatally she required ventilation and continuous inotropic support for left ventricle systolic dysfunction. She died after 2 weeks when therapy was withdrawn. Homozygous c.1174C > T,p.(R392*) class 4 variants in the NEXN gene were found via WES. Microscopic investigation showed endomyocardial fibroelastosis. Her parents, both heterozygous carriers, had normal cardiac function and the family history was normal. These patients show a new clinical spectrum of pediatric cardiac disease seen in heterozygous and homozygous NEXN variants, ranging from mild, transient DCM to a severe, fatal neonatal DCM. These patients support the inclusion of the NEXN gene in the investigation of pediatric patients with DCM, even in cases with transient DCM.
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Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/genética , Heterozigoto , Homozigoto , Proteínas dos Microfilamentos/genética , Mutação , Criança , Eletrocardiografia , Evolução Fatal , Feminino , Estudos de Associação Genética , Predisposição Genética para Doença , Testes Genéticos , Humanos , Lactente , Recém-Nascido , Masculino , Fenótipo , Avaliação de SintomasRESUMO
BACKGROUND: In response to an increased need for patient information in congenital heart disease, we previously developed an online, evidence-based information portal for patients with congenital aortic and pulmonary valve disease. To assess its effectiveness, a stepped-wedge cluster randomised trial was conducted. METHODS: Adult patients and caregivers of paediatric patients with congenital aortic and/or pulmonary valve disease and/or tetralogy of Fallot who visited the outpatient clinic at any of the four participating centres in the Netherlands between 1 March 2016-1 July 2017 were prospectively included. The intervention (information portal) was introduced in the outpatient clinic according to a stepped-wedge randomised design. One month after outpatient clinic visit, each participant completed a questionnaire on disease-specific knowledge, anxiety, depression, mental quality of life, involvement and opinion/attitude concerning patient information and involvement. RESULTS: 343 participants were included (221 control, 122 intervention). Cardiac diagnosis (p=0.873), educational level (p=0.153) and sex (p=0.603) were comparable between the two groups. All outcomes were comparable between groups in the intention-to-treat analyses. However, only 51.6% of subjects in the intervention group (n=63) reported actually visiting the portal. Among these subjects (as-treated), disease-specific knowledge (p=0.041) and mental health (p=0.039) were significantly better than in control subjects, while other baseline and outcome variables were comparable. CONCLUSION: Even after being invited by their cardiologists, only half of the participants actually visited the information portal. Only in those participants that actually visited the portal, knowledge of disease and mental health were significantly better. This underlines the importance of effective implementation of online evidence-based patient information portals in clinical practice.
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Valva Aórtica , Cuidadores/estatística & dados numéricos , Doenças das Valvas Cardíacas/epidemiologia , Portais do Paciente/estatística & dados numéricos , Valva Pulmonar , Qualidade de Vida , Adolescente , Adulto , Feminino , Humanos , Masculino , Morbidade/tendências , Países Baixos/epidemiologia , Inquéritos e Questionários , Adulto JovemRESUMO
Transposition of the great arteries is a congenital heart defect defined by an abnormal connection between the aorta, pulmonary artery, and the ventricles, resulting in parallel systemic and pulmonary circulations. Long-term follow-up data of patients who underwent correction via an arterial switch operation have recently shown that as a result of re-implantation of the coronary arteries in the neo-aorta, coronary stenosis and occlusion are relatively common complications. In this report, we discuss two cases illustrating the added value of myocardial perfusion imaging (MPI) and cardiac CT for the assessment of these patients. Based on the available literature we conclude that MPI and cardiac CT are excellent non-invasive methods to evaluate coronary anatomy and myocardial function also in this specific group of patients.
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Ventrículos do Coração/diagnóstico por imagem , Isquemia Miocárdica/diagnóstico por imagem , Imagem de Perfusão do Miocárdio/métodos , Cintilografia/métodos , Tomografia Computadorizada por Raios X/métodos , Adolescente , Angiografia Coronária/métodos , Estenose Coronária , Vasos Coronários/fisiopatologia , Exercício Físico , Coração/fisiopatologia , Humanos , Masculino , Compostos Organofosforados , Compostos de Organotecnécio , Risco , Tomografia Computadorizada de Emissão de Fóton Único , Transposição dos Grandes Vasos/fisiopatologiaRESUMO
OBJECTIVE: The association between secundum atrial septal defects (ASD) and asthma-like dyspnea with consequent long-term pulmonary inhalant use, is poorly understood in adult ASD patients. Airway hyperresponsiveness is suggested to be the underlying mechanism of cardiac asthma from mitral valve disease and ischemic cardiomyopathy. We hypothesized that airway hyperresponsiveness may also be found in adult ASD patients. Our aim was to study airway responsiveness in adult ASD patients before percutaneous closure and at short-and long-term postprocedural follow-up. METHODS: This prospective study included 31 ASD patients (65% female, mean age 49 ± 15y) who underwent spirometry and bronchoprovocation testing pre-and six-month postprocedurally, with additional bronchoprovocation at 2-year follow-up. Airway hyperresponsiveness was defined as ≥20% fall of forced expiratory volume in 1-second (FEV1 ) following <8.0 mg/mL of inhaled methacholine. RESULTS: Airway hyperresponsiveness was found in 19/30 patients (63%[95%CI 45%-81%]; post hoc statistical power = 89%). Asthma-like symptoms wheezing, chest tightness, and cough were more frequently reported in airway hyperresponsive patients. Airway responsiveness was not influenced by successful percutaneous ASD closure, corresponding to persistence of asthma-like symptoms postclosure. Regardless of airway responsiveness, postprocedural right-sided reverse remodeling significantly improved dyspnea and pulmonary function. CONCLUSIONS: This study is the first to report a high prevalence of airway hyperresponsiveness in a cohort of unrepaired adult ASD patients, and confirms the association between asthma-like symptoms and ASD in adults. Attention to symptoms and pulmonary function should be given during clinical follow-up of adult ASD patients, both before and long after repair.
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Dispneia/etiologia , Comunicação Interatrial/complicações , Pulmão/fisiopatologia , Cateterismo Cardíaco , Dispneia/diagnóstico , Dispneia/fisiopatologia , Ecocardiografia , Feminino , Seguimentos , Fluxo Expiratório Forçado , Comunicação Interatrial/diagnóstico , Comunicação Interatrial/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , EspirometriaRESUMO
BACKGROUND: Infective endocarditis (IE) after transcatheter pulmonary valve implantation (TPVI) in dysfunctioning right ventricular outflow tract conduits has evoked growing concerns. We aimed to investigate the incidence and the natural history of IE after TPVI with the Melody valve through a systematic review of published data. METHODS AND RESULTS: PubMed, EMBASE, and Web of Science databases were systematically searched for articles published until March 2017, reporting on IE after TPVI with the Melody valve. Nine studies (including 851 patients and 2060 patient-years of follow-up) were included in the analysis of the incidence of IE. The cumulative incidence of IE ranged from 3.2% to 25.0%, whereas the annualized incidence rate ranged from 1.3% to 9.1% per patient-year. The median (interquartile range) time from TPVI to the onset of IE was 18.0 (9.0-30.4) months (range, 1.0-72.0 months). The most common findings were positive blood culture (93%), fever (89%), and new, significant, and/or progressive right ventricular outflow tract obstruction (79%); vegetations were detectable on echocardiography in only 34% of cases. Of 69 patients with IE after TPVI, 6 (8.7%) died and 35 (52%) underwent surgical and/or transcatheter reintervention. Death or reintervention was more common in patients with new/significant right ventricular outflow tract obstruction (69% versus 33%; P=0.042) and in patients with non-streptococcal IE (73% versus 30%; P=0.001). CONCLUSIONS: The incidence of IE after implantation of a Melody valve is significant, at least over the first 3 years after TPVI, and varies considerably between the studies. Although surgical/percutaneous reintervention is a common consequence, some patients can be managed medically, especially those with streptococcal infection and no right ventricular outflow tract obstruction.
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Bioprótese/efeitos adversos , Endocardite/epidemiologia , Implante de Prótese de Valva Cardíaca/efeitos adversos , Próteses Valvulares Cardíacas/efeitos adversos , Infecções Relacionadas à Prótese/epidemiologia , Valva Pulmonar/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Endocardite/diagnóstico , Endocardite/mortalidade , Endocardite/terapia , Feminino , Implante de Prótese de Valva Cardíaca/instrumentação , Implante de Prótese de Valva Cardíaca/mortalidade , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Desenho de Prótese , Infecções Relacionadas à Prótese/diagnóstico , Infecções Relacionadas à Prótese/mortalidade , Infecções Relacionadas à Prótese/terapia , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Adulto JovemAssuntos
Obstrução das Vias Respiratórias/diagnóstico por imagem , Obstrução das Vias Respiratórias/fisiopatologia , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/fisiopatologia , Hemodinâmica , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Disfunção Ventricular Direita/diagnóstico por imagem , Adolescente , Obstrução das Vias Respiratórias/etiologia , Feminino , Humanos , Doenças Raras/diagnóstico por imagem , Disfunção Ventricular Direita/diagnósticoRESUMO
BACKGROUND: Dilated cardiomyopathy is a rare but serious disorder in children. No effective diagnostic or treatment tools are readily available. This study aimed to evaluate the efficacy of intravenous immunoglobulins in children with new onset dilated cardiomyopathy. Methods and results In this retrospective cohort study, 94 children with new onset dilated cardiomyopathy were followed during a median period of 33 months. All patients with secondary dilated cardiomyopathy - for example, genetic, auto-immune or structural defects - had been excluded. Viral tests were performed in all patients and 18 (19%) children met the criteria for the diagnosis "probable or definite viral myocarditis". Intravenous immunoglobulins were administered to 21 (22%) patients. Overall transplant-free survival was 75% in 5 years and did not differ between treatment groups. The treatment was associated with a higher recovery rate within 5 years, compared with non-treated children (70 versus 43%, log rank=0.045). After correction for possible confounders the hazard ratio for recovery with intravenous immunoglobulins was not significant (hazard ratio: 2.1; 95% CI: 1.0-4.6; p=0.056). Administration of intravenous immunoglobulins resulted in a greater improvement in the shortening fraction of the left ventricle. CONCLUSION: In our population of children with new onset dilated cardiomyopathy, of either viral or idiopathic origin, intravenous immunoglobulins were administered to a minority of the patients and did not influence transplant-free survival, but were associated with better improvement of systolic left ventricular function and with better recovery. Our results support the concept that children with new onset dilated cardiomyopathy might benefit from intravenous immunoglobulins.
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Cardiomiopatia Dilatada/terapia , Imunoglobulinas Intravenosas/uso terapêutico , Miocardite/complicações , Miocardite/diagnóstico , Pré-Escolar , Ecocardiografia , Feminino , Transplante de Coração , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Miocardite/virologia , Países Baixos/epidemiologia , Estudos Retrospectivos , Volume Sistólico/efeitos dos fármacos , Análise de Sobrevida , Resultado do Tratamento , Função Ventricular Esquerda/efeitos dos fármacos , Carga ViralRESUMO
INTRODUCTION: Since King and Mills' pioneering work in percutaneous closure devices of secundum atrial septal defects (ASD) four decades ago, developments in device shape, material and implantation technique led to adoption of percutaneous ASD closure as current treatment of choice. Not only was the feasibility of such a percutaneous procedure tested altogether, but pursuing the ideal device in terms of safety and efficacy became priority. AREAS COVERED: In this review we present the historical development of ASD devices in design, material and technique with clinical data, and provide the future perspectives in percutaneous ASD closures. EXPERT OPINION: An 'ideal device' requires complete defect closure with negligible risk of complications using a safe, straightforward delivery technique with repositioning and retrieving properties. Some of the devices currently at hand come close to fulfilling these criteria, however none seem to provide those prerequisites completely. By understanding how challenges in device development were overcome in the past, new insights into future improvements are given.