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1.
BMC Health Serv Res ; 22(1): 153, 2022 Feb 05.
Artigo em Inglês | MEDLINE | ID: mdl-35123454

RESUMO

BACKGROUND: Although transition programs have been evaluated for adolescents with chronic conditions, these have rarely involved process evaluations. Indeed, outcomes of complex interventions are dependent on how the intervention is implemented in practice and evaluations of implementation process are therefore pivotal. The aim of this study was to evaluate the extent to which a transition program for adolescents with congenital heart disease was delivered as intended. Research questions were 1) to what level of fidelity was the program delivered? and 2) what potential moderating factors affected the delivery of the program and overall fidelity? METHODS: A mixed methods design was used, where a process evaluation was embedded in the STEPSTONES randomized controlled trial in Sweden. The implementation fidelity framework by Carrol (2007) and Hasson (2010) was used to design, collect and analyze data. Quantitative data consisted of intervention records on adherence and were analyzed with descriptive statistics. Qualitative data on moderators affecting fidelity were collected through interviews, log-books and focus group interviews with healthcare professionals implementing the intervention and participatory observations of the implementation process. Data were analyzed with deductive content analysis. Triangulation was used to integrate quantitative and qualitative data within the fidelity framework. RESULTS: Six out of eight components of the transition program were delivered to an extent that adhered to the program theory or achieved a high level of fidelity. However, components involving peer support had a low attendance by the participating sample (32.2%), and the joint transfer meeting was challenging to implement, despite achieving high adherence. Moderators affecting the implementation process were the adolescent's and healthcare professional's engagement in the intervention, contextual factors and a lack of standard operating procedures for all components in the program. CONCLUSION: Barriers and facilitators for a future implementation of transition programs have been illuminated in this study. The use of an implementation fidelity framework in the process evaluation proved successful in providing a comprehensive evaluation of factors affecting the implementation process. However, implementation fidelity must be considered in relation to adaptations to the local and personal prerequisites in order to create interventions that can achieve fit.


Assuntos
Atenção à Saúde , Cardiopatias Congênitas , Adolescente , Doença Crônica , Cardiopatias Congênitas/terapia , Humanos , Avaliação de Processos em Cuidados de Saúde , Projetos de Pesquisa
2.
Eur J Cardiovasc Nurs ; 21(6): 620-629, 2022 08 29.
Artigo em Inglês | MEDLINE | ID: mdl-34927192

RESUMO

AIMS: The coronavirus disease-2019 (COVID-19) pandemic poses significant challenges to many groups within societies, and especially for people with chronic health conditions. It is, however, unknown whether and how the pandemic has thus far affected the physical and mental health of patient populations. Therefore, we investigated how the pandemic affected the lives of adults with congenital heart disease (CHD), compared pre- and peri-pandemic patient-reported outcome measures (PROMs) and a patient-reported experience measure (PREM), and investigated whether having had COVID-19 impacted pre-/peri-pandemic differences of the PROMs and PREM. METHODS AND RESULTS: As part of the ongoing APPROACH-IS II project, we longitudinally surveyed 716 adults with CHD from Belgium, Norway, and South Korea. Pre-pandemic measures were administered from August 2019 to February 2020 and the peri-pandemic surveys were completed September 2020-April 2021. The majority of patients indicated that their social lives (80%), mental health (58%), and professional lives/education (51%) were negatively impacted by the pandemic. Patients felt worried (65%), were afraid (55%), reported the pandemic felt 'close' to them (53%), and were stressed (52%). However, differences between pre- and peri-pandemic scores on the PROMs and PREM were negligibly small (Cohen's d < 0.20). Across measures, 5.8-15.8% of patients demonstrated changes (improved or worsened scores) that exceeded the minimal clinically important difference. There were no difference-in-differences for PROMs and PREM between patients who did vs. did not have COVID-19. CONCLUSIONS: Although the COVID-19 pandemic has been disruptive in many ways, pre- to peri-pandemic changes in PROMs and PREM of adults with CHD were negligibly small.


Assuntos
COVID-19 , Cardiopatias Congênitas , Adulto , Bélgica , Humanos , Pandemias , Medidas de Resultados Relatados pelo Paciente , República da Coreia
3.
Tex Heart Inst J ; 48(3)2021 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-34347100

RESUMO

Patients with congenital heart disease frequently have aneurysms or coronary artery fistulae that necessitate treatment. Metal vascular coils have been a mainstay of treatment for these lesions. In 2002, coils coated or filled with expandable hydrogel were introduced to treat cerebral aneurysms; however, the literature on their use in patients with congenital heart disease is limited. We present 5 cases in which large vascular lesions in children or adolescents with congenital heart disease were successfully occluded with hydrogel coils.


Assuntos
Fístula Artério-Arterial/terapia , Cateterismo Cardíaco/métodos , Materiais Revestidos Biocompatíveis , Aneurisma Coronário/terapia , Vasos Coronários/diagnóstico por imagem , Embolização Terapêutica/instrumentação , Cardiopatias Congênitas/complicações , Adolescente , Fístula Artério-Arterial/complicações , Fístula Artério-Arterial/diagnóstico , Criança , Pré-Escolar , Aneurisma Coronário/complicações , Aneurisma Coronário/diagnóstico , Desenho de Equipamento , Feminino , Humanos , Hidrogéis , Masculino
4.
Eur Heart J ; 42(41): 4213-4223, 2021 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-34198319

RESUMO

The vast majority of children with congenital heart disease (CHD) in high-income countries survive into adulthood. Further, paediatric cardiac services have expanded in middle-income countries. Both evolutions have resulted in an increasing number of CHD survivors. Expert care across the life span is necessitated. In adolescence, patients transition from being a dependent child to an independent adult. They are also advised to transfer from paediatrics to adult care. There is no universal consensus regarding how transitional care should be provided and how the transfer should be organized. This is even more challenging in countries with low resources. This consensus document describes issues and practices of transition and transfer of adolescents with CHD, accounting for different possibilities in high-, middle-, and low-income countries. Transitional care ought to be provided to all adolescents with CHD, taking into consideration the available resources. When reaching adulthood, patients ought to be transferred to adult care facilities/providers capable of managing their needs, and systems have to be in place to make sure that continuity of high-quality care is ensured after leaving paediatric cardiology.


Assuntos
Cardiologia , Enfermagem Cardiovascular , Cardiopatias Congênitas , Pediatria , Transição para Assistência do Adulto , Adolescente , Adulto , Ásia , Austrália , Criança , Consenso , Cardiopatias Congênitas/terapia , Humanos , Nova Zelândia , Estados Unidos
5.
Int J Cardiol ; 319: 62-66, 2020 11 15.
Artigo em Inglês | MEDLINE | ID: mdl-32634489

RESUMO

AIMS: The majority of children with complex congenital heart disease (CHD) survive into adulthood due to advances in medical care. Adult patients with CHD have an increased incidence of diagnoses related to ageing such as heart failure, dementia, cancer and sarcopenia, despite a relatively low age. They also have a shorter life expectancy. It is unknown if their bone structures also show signs of premature ageing. We therefore investigated Bone Mineral Content (BMC) and bone mineral density (BMD) in an adult population with complex CHD. METHODS: The total body BMC and BMD was examined using dual energy X-ray absorptiometry (DXA) in 73 adults with complex CHD (mean age 35.8 ± 14.3, women n = 22) and 73 age and sex matched controls. RESULTS: The adults with complex CHD had lower total body BMC (2.6 ± 0.5 kg vs. 2.9 ± 0.5 kg, p < 0.001) and BMD (1.18 ± 0.12 g/cm2vs. 1.26 ± 0.11 g/cm2, p < 0.001) compared to controls. BMD was lower for patients with single ventricle physiology and for the other complex diagnoses, and it persisted after correction for most common risk factors for osteoporosis. CONCLUSION: Adults with complex CHD have reduced total body BMC and BMD compared to healthy controls. These results are a sign of frailty that conforms with other previously reported signs of premature ageing. The risk of osteoporosis is low in our relatively young population, but it is assumed to increase with ageing. We recommend that clinicians pay close attention to risk factors for osteoporosis, and are generous in administering DXA-measurements in order to prevent future fractures among adults with complex CHD.


Assuntos
Cardiopatias Congênitas , Absorciometria de Fóton , Adulto , Densidade Óssea , Feminino , Fraturas Ósseas , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Osteoporose/diagnóstico por imagem , Osteoporose/epidemiologia , Adulto Jovem
6.
Rev. bras. ter. intensiva ; 32(2): 261-267, Apr.-June 2020. tab, graf
Artigo em Inglês, Português | LILACS | ID: biblio-1138498

RESUMO

RESUMO Objetivo: Avaliar a funcionalidade de pacientes pediátricos submetidos à correção cirúrgica de cardiopatia congênita após a alta da unidade de terapia intensiva e as possíveis correlações com variáveis clínicas e risco cirúrgico. Métodos: Estudo transversal, que incluiu crianças entre 1 mês e 18 anos incompletos, que realizaram cirurgia para correção de cardiopatia congênita, no período de outubro de 2017 até maio de 2018. A avaliação da funcionalidade foi realizada por meio da Functional Status Scale, a avaliação do risco cirúrgico se deu pelo Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), e as variáveis clínicas foram obtidas do prontuário eletrônico. Resultados: A amostra foi composta de 57 crianças, com mediana de idade de 7 (2 - 17) meses, sendo 54,4% do sexo masculino. Dentre as crianças, 75,5% apresentaram alteração na funcionalidade, e 45,6% delas tiveram disfunção moderada. Cerca de 47% da amostra apresentou classificação RACHS-1 > 3, indicando maior risco cirúrgico. Maior déficit funcional foi associado a crianças mais novas, com maior duração da ventilação mecânica invasiva e do tempo de internação na unidade de terapia intensiva. Além disso, maior grau de disfunção foi observado entre aqueles classificados com RACHS-1 > 3. Conclusão: A prevalência de disfunção foi elevada em crianças e adolescentes com cardiopatia após cirurgia cardíaca. Maior risco cirúrgico, duração da ventilação mecânica invasiva, permanência na unidade de terapia intensiva e os mais jovens apresentaram associação com pior desempenho funcional.


Abstract Objective: To evaluate the functional status of pediatric patients undergoing congenital heart surgery after discharge from the intensive care unit, and to evaluate the correlations among clinical variables, functional status and surgical risk. Methods: Cross-sectional study including patients aged 1 month to less than 18 years undergoing congenital heart surgery between October 2017 and May 2018. Functional outcome was assessed by the Functional Status Scale, surgical risk classification was determined using the Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), and clinical variables were collected from electronic medical records. Results: The sample comprised 57 patients with a median age of 7 months (2 - 17); 54.4% were male, and 75.5% showed dysfunction, which was moderate in 45.6% of the cases. RACHS-1 category > 3 was observed in 47% of the sample, indicating higher surgical risk. There was a correlation between functional deficit and younger age, longer duration of invasive mechanical ventilation and longer intensive care unit stay. Moreover, greater functional deficit was observed among patients classified as RACHS-1 category > 3. Conclusion: The prevalence of functional deficit was high among children and adolescents with congenital heart disease after cardiac surgery. Higher surgical risk, longer duration of invasive mechanical ventilation, longer intensive care unit stay and younger age were correlated with worse functional status.


Assuntos
Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Adolescente , Respiração Artificial/estatística & dados numéricos , Cardiopatias Congênitas/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Unidades de Terapia Intensiva , Alta do Paciente , Fatores de Tempo , Estudos Transversais , Fatores de Risco , Fatores Etários , Estado Funcional , Tempo de Internação
9.
Cardiovasc Diagn Ther ; 8(6): 754-764, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30740322

RESUMO

Today, more than two thirds of patients with congenital heart disease (CHD) are adults. Cardiac surgery plays an essential role in restoring and maintaining cardiac function, aside from evolving medical treatment and catheter-based interventions. The aim of the present publication was to describe the spectrum of operations performed on adults with CHD (ACHD) by reviewing current literature. Currently, surgery for ACHD is predominantly valve surgery, since valvular pathologies are often either a part of the basic heart defect or develop as sequelae of corrective or palliative surgery. Surgical techniques for valve repair, established in patients with acquired heart disease (non-ACHD), can often be transferred to ACHD. New valve substitutes may help to reduce the number of redo operations. Most of valve operations yield good results in terms of survival and quality of life, with the precondition that the ventricular function is preserved. Heart failure due to end-stage CHD is the most frequent cause of mortality in ACHD. However, surgical treatment by means of mechanical circulatory support (MCS) is still uncommon and the mortality exceeds the one following other operations in ACHD. Currently, different devices are used and new technical developments are in progress. However, there still is no ideal assist device available. Therefore, heart transplantation remains the only valid option for end-stage CHD. Despite higher early mortality following heart transplantation in ACHD compared to non-ACHD, the long-term survival compares favorably to non-ACHD. There is room for improvement by refining the indications, the time of listing, and the perioperative care of ACHD transplant patients. Sudden death is the second most frequent cause of mortality in ACHD. Ventricular tachycardia is the most frequent cause of sudden death followed by coronary artery anomaly. Due to the increasing awareness of physicians and the improved imaging techniques, coronary artery anomalies are coming more into the focus of cardiac surgeons. However, the reported experience is limited and it is currently difficult to provide a standardized and generally applicable recommendation for the indication and the adequate surgical technique. With the increasing age and complexity of ACHD, treatment of rhythm disturbances by surgical ablation, pacemaker or implantable cardioverter defibrillator (ICD) implantation and resynchronisation gains importance. A risk score specifically designed for surgery in ACHD is among the newest developments in predicting the outcome of surgical treatment of ACHD. This evidence-based score, derived from and validated with data from the Society of Thoracic Surgeons Congenital Heart Surgery Database, enables comparison of risk-adjusted performance of the whole spectrum of procedures performed in ACHD and helps in understanding the differences in surgical outcomes. The score is thus a powerful tool for quality control and quality improvement. In conclusion, new developments in surgery for ACHD are currently made with regard to valve surgery, which comprises more than half of all operations in ACHD and in treatment of end-stage CHD, which still yields high mortality and morbidity.

10.
Artigo em Coreano | WPRIM (Pacífico Ocidental) | ID: wpr-81413

RESUMO

Medical and catheter-based ablation therapies have been successfully applied in treating supraventricular tachycardia, supported by abundant evidences from clinical trials. However, Special populations such as pediatric populations, pregnant patients, or patients with congenital heart diseases are easily excluded from clinical trials; thus, caution is needed when adopting results from clinical trials conducted in the usual adult population.


Assuntos
Adulto , Humanos , Gravidez , Cardiopatias Congênitas , Cardiopatias , Pediatria , Taquicardia Supraventricular
11.
Chinese Journal of Ultrasonography ; (12): 1024-1028, 2017.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-707604

RESUMO

Objective To investigate the clinical application valve of prenatal ultrasound in diagnosis of fetal isolated levocardia ( IL ) . Methods Thirty-eight IL fetuses diagnosed in three prenatal diagnosis centers from 2000 to 2016 were reviewed with their prenatal ultrasound features and cardiac and extracardiac malformations . Results Thirty-eight IL cases included 19 cases of left isomerism ( LI) ,14 cases of right isomerism( RI) and 5 non-classified cases . The overall incidence of malformations in IL patients was 97 .4%with 94 .7% in LI patients and 100% in RI patients . The incidence of cardiac malformations was 92 .1%with 84 .2% in LI patients and 100% in RI patients . The most common congenital heart diseases were:right ventricle outlet obstruction , univentricular physiology and atrioventricular septal defect . Anomalous pulmonary venous connection was more commonly seen in LI patients than in RI patients . The most common extracardiac malformation was duodenal obstruction( 86 .9% ) . Inferior vena cava interruption and polysplenia were more commonly seen in LI patients .Juxtaposition of aorta and inferior vena cava and asplenia were more commonly seen in RI patients . Conclusions IL is very rarely seen in population with high incidence of congenital heart diseases and extracardiac malformations .

12.
Chinese Circulation Journal ; (12): 77-80, 2017.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-508134

RESUMO

Objective: To summarize surgical result of single ventricle with total anomalous pulmonary venous connection (TAPVC) in relevant patients. Methods:A total of 25 patients of single ventricle with TAPVC treated in our hospital from 2008-09 to 2016-03 were summarized. The patients were between 5 days to 14 years of age with body weight at (3.3-49.5) kg. There were 22 patients with supracardiac type TAPVC, 1 with infracardiac type TAPVC and 2 with mixed type TAPVC;in addition,17 cases having complete atrio-ventricular canal defect, 2 having tricuspid atresia, 4 having more than moderate atrio-ventricular valve regurgitation, 5 having pulmonary atresia and 9having pulmonary venous obstruction. Results:There were 5/25 (20%) patients died at the early stage for low cardiac output syndrome. Followed-up study was conducted in 20 survivals for (1-65) months and 7 patients had re-surgery including 5 with modified Fontan operation, 1 with atrio-ventricular valve replacement due to severe atrio-ventricular valve regurgitation, 1 with pulmonary venous stenosis repair+atrio-ventricular valve replacement due to pulmonary venousobstruction and severe atrio-ventricular valve regurgitation. 3 patients died during followed-up period because of low cardiac output syndrome, protein lost intestine disease and neurological injury respectively. The rest survivals had good general condition, no residualpulmonary venous obstruction was found. Conclusion: The patients of single ventricle with TAPVC were frequently associated with right atrial isomerism and complex cardiac anomaly;surgical treatment still having the high mortality.

14.
Chinese Circulation Journal ; (12): 993-996, 2016.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-501504

RESUMO

Objective: To study the cause of rapid thrombocytopenia and organ hemorrhage in congenital heart disease (CHD) patients after interventional occlusion. Methods: A total of 665 CHD patients received interventional occlusion in our hospital from 2011-01 to 2015-12 were enrolled. The patients were divided into 3 groups according to the defects: Atrial septal defect (ASD) group,n=100, Ventricular septal defect (VSD) group,n=100 and Patent ductus arteriosus (PDA) group,n=465. Pre- and post-interventional occlusion platelet levels, the relationship between PDA occluder diameter and platelet counts were compared. Pressure difference between both sides of occluder was randomly measured in a part of patients including 50 in ASD group, 50 in VSD group and 102 in PDA group. Based on occluder diameter, the 102 PDA patients were further divided into 2 subgroups: Giant PDA,n=42 and Medium-small PDA,n=60; pressure differences between both sides of occluder were compared between 2 subgroups. Results: No severe thrombocytopenia and organ hemorrhage occurred in ASD group or VSD group. PDA group had 36/465 (7.74%) patients with severe thrombocytopenia, 18 (3.87%) with organ hemorrhage and all of them occurred in giant PDA subgroup; the diameter of PDA occluder was negatively related to post-operative to platelet counts (r=-0.659,P=0.001). For pressure difference on both sides of occluder, compared with prior operation, PDA group showed increased systolic and diastolic pressure differences and increased mean pressure difference at immediately post operation, allP< 0.05; systolic pressure difference in VSD group was (56.57±15.33) mmHg, in Medium-small PDA subgroup was (58.33±26.65) mmHg and in Giant PDA subgroup was (94.66±27.62) mmHg which was much higher than those in VSD group and Medium-small PDA subgroup, allP<0.01. Conclusion: Rapid thrombocytopenia and organ hemorrhage in CHD after interventional occlusion only happened in giant PDA patients. High pressure difference formed high-speed ifltration blood lfow which may cause scouring damage on platelets and it was the main reason for thrombocytopenia occurrence.

15.
Korean Circ J ; 45(3): 216-24, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-26023310

RESUMO

BACKGROUND AND OBJECTIVES: Transcatheter device closure becomes the first option for treating secundum atrial septal defect (ASD), but the conventional method is sometimes unsuccessful even when the defect size indicates the closure to be feasible. To increase the success rate, modified methods have been introduced and used. This study aimed to find predictors for using the modified methods in the device closure of secundum ASDs. SUBJECTS AND METHODS: Between October 2010 and December 2012, 92 patients with ASDs underwent the transcatheter device closure. We analyzed the sizes of the defect, the surrounding rims, and the ratios of the left atrium (LA) dimensions to the device size in the patients who underwent the procedure either using the conventional or modified methods. RESULTS: Among the 88 successful cases (95.7%), 22 patients (25%) required modified methods (12 using pulmonary vein and 10 using balloon). The modified method group had the larger size of ASDs and smaller posterosuperior rim. The mean ratios of the LA anteroposterior diameter, width, and length to the device size were all significantly smaller in the modified methods group than in the conventional group (1.20 vs. 1.56, 1.32 vs. 1.71, and 1.61 vs. 2.07, respectively). We found that the risk factors for the modified methods were smaller retroaortic rim, larger ASD, and smaller LA dimension/device size. CONCLUSION: In addition to larger defects and smaller retroaortic rim, the smaller ratios of the LA dimensions to the device size influenced the need for the application of modified methods in the transcatheter device closure of ASDs.

16.
Korean Circulation Journal ; : 216-224, 2015.
Artigo em Inglês | WPRIM (Pacífico Ocidental) | ID: wpr-19605

RESUMO

BACKGROUND AND OBJECTIVES: Transcatheter device closure becomes the first option for treating secundum atrial septal defect (ASD), but the conventional method is sometimes unsuccessful even when the defect size indicates the closure to be feasible. To increase the success rate, modified methods have been introduced and used. This study aimed to find predictors for using the modified methods in the device closure of secundum ASDs. SUBJECTS AND METHODS: Between October 2010 and December 2012, 92 patients with ASDs underwent the transcatheter device closure. We analyzed the sizes of the defect, the surrounding rims, and the ratios of the left atrium (LA) dimensions to the device size in the patients who underwent the procedure either using the conventional or modified methods. RESULTS: Among the 88 successful cases (95.7%), 22 patients (25%) required modified methods (12 using pulmonary vein and 10 using balloon). The modified method group had the larger size of ASDs and smaller posterosuperior rim. The mean ratios of the LA anteroposterior diameter, width, and length to the device size were all significantly smaller in the modified methods group than in the conventional group (1.20 vs. 1.56, 1.32 vs. 1.71, and 1.61 vs. 2.07, respectively). We found that the risk factors for the modified methods were smaller retroaortic rim, larger ASD, and smaller LA dimension/device size. CONCLUSION: In addition to larger defects and smaller retroaortic rim, the smaller ratios of the LA dimensions to the device size influenced the need for the application of modified methods in the transcatheter device closure of ASDs.


Assuntos
Humanos , Cateterismo Cardíaco , Átrios do Coração , Cardiopatias Congênitas , Comunicação Interatrial , Veias Pulmonares , Fatores de Risco , Dispositivo para Oclusão Septal
18.
Am J Med Genet A ; 164A(12): 3003-9, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-25257999

RESUMO

Since 2004, CHD7 mutations have been a known cause of CHARGE (Coloboma, Heart defects, Atresia of choane, Retardation of growth and development, Genital hypoplasia, Ear anomalies) syndrome, but the full clinical spectrum of CHD7 mutations is only now gradually emerging. CHD7 mutations have been identified in patients who do not fulfill the clinical criteria for CHARGE syndrome and in patients with overlapping syndromes. Variable congenital heart defects occur in the majority of patients with CHD7 mutations, with an overrepresentation of atrioventricular septal defects and conotruncal heart defects. This prompted us to study CHD7 in 46 patients with these heart defects and one other feature of CHARGE syndrome. We identified two CHD7 variants that were inherited from a healthy parent (c.3778 + 17C > T, c.7294G > A), but no pathogenic CHD7 mutations. We conclude that CHD7 mutations are not a major cause of the atrioventricular septal defects and conotruncal heart defects, not even if one extra phenotypic feature of CHARGE syndrome is present. Therefore, CHD7 analysis should not be performed routinely in this group of patients. However, we do recommend adding CHD7 to massive parallel sequencing gene panels for diagnostic work in patients with syndromic heart defects.


Assuntos
DNA Helicases/genética , Proteínas de Ligação a DNA/genética , Cardiopatias Congênitas/genética , Defeitos dos Septos Cardíacos/genética , Fenótipo , Feminino , Humanos , Masculino , Mutação Puntual/genética
19.
J Tehran Heart Cent ; 9(2): 59-63, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-25861320

RESUMO

BACKGROUND: Chylothorax is a rare but serious postoperative condition with a high rate of morbidity and may lead to the mortality of children undergoing congenital heart disease (CHD) surgery. This study evaluated the specific surgical procedures associated with the higher risk of postoperative chylothorax. METHODS: We assessed 435 cases undergoing CHD surgery between April 2003 and May 2006. We detected postoperative chylothorax in 6 patients. The diagnosis of chylothorax was established based on the presence of an odorless fluid with the characteristic milky appearance of the fluid (except when the patients were fasting in the immediate postoperative period), a triglyceride level greater than 110 mg/dL or between 50 and 110 mg/dL with a pleural fluid white cell count greater than 1000, and more than 80% lymphocytes on differential when the pleural fluid was not chylous. RESULTS: Over a 37-month period, 435 (mean age = 51.6 months; 232 males) patients underwent various types of surgical procedures for CHD; 6 patients developed chylothorax after the Fontan operation; one patient died due to severe chylothorax; 3 patients were managed by nutritional modifications, diuretics, and thoracocentesis; and 2 patients required thoracic duct ligation. The Fisher exact test analysis showed a significant association between the Fontan operation and postoperative chylothorax (p value < 0.0001). CONCLUSION: Our study showed a significant association between the Fontan surgery and chylothorax.

20.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-453648

RESUMO

Objective A retrospective review of the use of delayed sterna closure (DSC) after pediatric cardiac operations.The purpose of this study is to access the morbidity of DSC and to analyze the risk factors that may predict the need for DSC.Methods Between January 2008 and December 2011,5 546 infants (2 843 males,2 703 females) underwent cardiac operations through midterm sternotomy in Shanghai Children' s Medical Center.Median age was 5 months(1 day to 11 months) and mean weight was 6.1 kg(2.0-12.5 kg).The pathologies included transposition of great artery(TGA),corctation of aorta (CoA),interruption of aortic arch (IAA),pulmonary atresia (PA),truncus arteriosus (Truncus),complete atrioventricular septal defect(CAVSD),total anormalous pulmonary vein connection (TAPVC),double outlet of right ventricle(DORV),tetralogy of Fallot(TOF),and ventricular septal defect(VSD),et al.All hospital records were reviewed and clinical data were studied and analyzed.Results A total of 313 patients had DSC (5.6%).There were 191 males and 122 females.The reasons for DSC are hemodynamic instability in 296,bleeding in 11,and ECMO(or VAD) in 6 patients.Two hundred and fiftyone (80.2%) patients were less than 5 kg,84 (26.8 %) patients were neonates,and 125 (39.9%) patients aged between one and three months old.The incidences of DSC for these patients were 13.9%,34.4%,and 18.4% respectively.The diagnosis of complex congenital heart defects had a high risk of DSC.The incidences of DSC for TGA,CoA,IAA,PA,Truncus,CAVSD,TAPVC,and DORV were 28.8%,17.8%,43.1%,9.0%,30.4%,6.5%,and 10.8% respectively.The DSC patients had longer duration of CPB time(147 min versus 72 min) and clamping time(81 min versus 40 min).In 282 patients the sternums were closed 1-11 days after the initial operation.In 3 cases the trial of closure failed for the first time and succeeded a second time until several days later.Fouty-six patients died ultimately including 15 patients died after the closure of sternum with a total mortality of 14.7 %.Surgical site infection occurred in 17 patients (5.4%),and surgical intervention were needed in 4 severe cases(1.3%).Conclusion Neonates,an age less than 3 months,weight less than 5 kg,long CPB time and clamping time,and the diagnosis of complex congenital heart defects were risk factors of the need for delayed sterna closure after pediatric cardiac operations.

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