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1.
Acta Obstet Gynecol Scand ; 95(11): 1273-1280, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27496177

RESUMO

INTRODUCTION: As the prenatal detection rates of congenital heart defects (CHDs) increase, obstetricians are more frequently faced with pregnancies complicated by a fetal CHD. Congenital anomalies in general are associated with preterm birth and fetal demise. The aim of this study was to gain insight into the prevalence of preterm birth and fetal demise in singleton pregnancies with fetuses with isolated CHDs. MATERIAL AND METHODS: A geographical cohort study was performed in a large region in the Netherlands. Fetuses and infants from singleton pregnancies diagnosed with severe isolated CHD, born between 1 January 2002 and 1 January 2012, were included. All cases in the CHD cohort were assessed for preterm birth or fetal demise. The proportions of preterm birth and fetal demise were compared with those in a control group and odds ratios were calculated. RESULTS: The proportion of preterm births in the CHD cohort (n = 1013) was 9.1% (95% CI 7.3-10.9) compared with 5.6% (95% CI 5.4-5.8) in the control group, with an odds ratio of 1.7 (95% CI 1.4-2.1). The preterm birth started spontaneously in 49.5% and 38.4% were induced. In 15 cases fetal demise occurred (1.5%; 95% CI 0.8-2.2), compared with 0.7% (95% CI 0.6-0.8) in the control group, odds ratio 2.0 (95% CI 1.2-3.4). CONCLUSIONS: Higher rates of preterm birth and fetal demise occur in fetuses with isolated CHD compared with the general population. Prenatal specialists should be vigilant for signs of heart failure, premature closure of the foramen ovale or fetal distress in fetuses with isolated CHDs.


Assuntos
Morte Fetal/etiologia , Cardiopatias Congênitas/complicações , Nascimento Prematuro/etiologia , Estudos de Casos e Controles , Feminino , Seguimentos , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/mortalidade , Humanos , Países Baixos , Gravidez , Nascimento Prematuro/epidemiologia , Diagnóstico Pré-Natal , Prevalência , Fatores de Risco
2.
Prenat Diagn ; 36(2): 177-85, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26716421

RESUMO

OBJECTIVES: To demonstrate the spectrum of copy number variants (CNVs) in fetuses with isolated left-sided congenital heart defects (CHDs), and analyse genetic content. METHODS: Between 2003 and 2012, 200 fetuses were identified with left-sided CHD. Exclusion criteria were chromosomal rearrangements, 22q11.2 microdeletion and/or extra-cardiac malformations (n = 64). We included cases with additional minor anomalies (n = 39), such as single umbilical artery. In 54 of 136 eligible cases, stored material was available for array analysis. CNVs were categorized as either (likely) benign, (likely) pathogenic or of unknown significance. RESULTS: In 18 of the 54 isolated left-sided CHDs we found 28 rare CNVs (prevalence 33%, average 1.6 CNV per person, size 10.6 kb-2.2 Mb). Our interpretation yielded clinically significant CNVs in two of 54 cases (4%) and variants of unknown significance in three other cases (6%). CONCLUSIONS: In left-sided CHDs that appear isolated, with normal chromosome analysis and 22q11.2 FISH analysis, array analysis detects clinically significant CNVs. When counselling parents of a fetus with a left-sided CHD it must be taken into consideration that aside from the cardiac characteristics, the presence of extra-cardiac malformations and chromosomal abnormalities influence the treatment plan and prognosis.


Assuntos
Aberrações Cromossômicas , Variações do Número de Cópias de DNA/genética , Cardiopatias Congênitas/genética , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/genética , Estenose da Valva Aórtica/congênito , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/genética , Estudos de Coortes , Hibridização Genômica Comparativa , Bases de Dados Factuais , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/genética , Hibridização in Situ Fluorescente , Gravidez , Ultrassonografia Pré-Natal
3.
J Cardiovasc Magn Reson ; 17: 18, 2015 Feb 19.
Artigo em Inglês | MEDLINE | ID: mdl-25827288

RESUMO

BACKGROUND: To characterize and directly quantify regurgitant jets of left atrioventricular valve (LAVV) in patients with corrected atrioventricular septal defect (AVSD) by four-dimensional (4D)Flow Cardiovascular Magnetic Resonance (CMR), streamline visualization and retrospective valve tracking. METHODS: Medical ethical committee approval and informed consent from all patients or their parents were obtained. In 32 corrected AVSD patients (age 26 ± 12 years), echocardiography and whole-heart 4DFlow CMR were performed. Using streamline visualization on 2- and 4-chamber views, the angle between regurgitation and annulus was followed throughout systole. On through-plane velocity-encoded images reformatted perpendicular to the regurgitation jet the cross-sectional jet circularity index was assessed and regurgitant volume and fraction were calculated. Correlation and agreement between different techniques was performed with Pearson's r and Spearman's rho correlation and Bland-Altman analysis. RESULTS: In 8 patients, multiple regurgitant jets over the LAVV were identified. Median variation in regurgitant jet angle within patients was 36°(IQR 18-64°) on the 2-chamber and 30°(IQR 20-40°) on the 4-chamber. Regurgitant jets had a circularity index of 0.61 ± 0.16. Quantification of the regurgitation volume was feasible with 4DFlow CMR with excellent correlation between LAVV effective forward flow and aortic flow (r = 0.97, p < 0.001) for internal validation and moderate correlation with planimetry derived regurgitant volume (r = 0.65, p < 0.001) and echocardiographic grading (rho = 0.51, p = 0.003). CONCLUSIONS: 4DFlow CMR with streamline visualization revealed multiple, dynamic and eccentric regurgitant jets with non-circular cross-sectional shape in patients after AVSD correction. 4DFlow with retrospective valve tracking allows direct and accurate quantification of the regurgitation of these complex jets.


Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Defeitos dos Septos Cardíacos/cirurgia , Hemodinâmica , Interpretação de Imagem Assistida por Computador/métodos , Imagem Cinética por Ressonância Magnética/métodos , Insuficiência da Valva Mitral/diagnóstico , Valva Mitral/fisiopatologia , Adolescente , Adulto , Estudos de Viabilidade , Feminino , Defeitos dos Septos Cardíacos/diagnóstico , Defeitos dos Septos Cardíacos/fisiopatologia , Humanos , Masculino , Insuficiência da Valva Mitral/etiologia , Insuficiência da Valva Mitral/fisiopatologia , Valor Preditivo dos Testes , Estudos Prospectivos , Resultado do Tratamento , Adulto Jovem
4.
Case Rep Pediatr ; 2013: 426923, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23424699

RESUMO

We present 3 patients with 4 causes of mediastinal fluid collection after congenital cardiac surgery in this extended case report. Volumetric computed tomography played an essential role in diagnosing causes and extent, relevant to subsequent management. Recent advances in volumetric computed tomography allow fast and accurate imaging of cardiovascular and extravascular structures in children with acceptable radiation dose, providing a powerful imaging tool for the evaluation of complications after congenital cardiac surgery.

5.
Ann Thorac Surg ; 94(6): 2077-82, 2012 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22981253

RESUMO

BACKGROUND: Current surgical strategies that aim at preventing pulmonary regurgitation in patients with corrected tetralogy of Fallot (cToF) may result in a certain grade of residual pulmonary stenosis (PS). The clinical implications of a postoperative residual PS in cToF patients remain unclear. Pulmonary valve replacement (PVR) is frequently needed during follow-up of cToF patients. The aim of the current study was to determine the role of residual PS in the need for PVR during follow-up in cToF patients. METHODS: cToF patients were included if clinical follow-up after primary surgical correction had taken place for a minimum of 5 years. Patient characteristics, surgical factors, and postoperative factors were reviewed, with a special focus on the transpulmonic systolic gradient. Cox proportional hazards regression analysis was performed to identify predictors of PVR. RESULTS: Of 171 cToF patients, 71 (41.5%) underwent PVR after 24.2 years (interquartile range, 16.8-31.6 years). Year of birth, older age at corrective operation, and patch use significantly predicted PVR during follow-up. By contrast, a mild residual PS in cToF patients (peak systolic gradient, 15-30 mm Hg) independently reduced the risk of PVR, as compared with patients without PS (hazard ratio, 0.47; p=0.02) and with moderate-to-severe PS (hazard ratio, 0.35; p=0.01). CONCLUSIONS: In addition to the known risks factors for PVR, a postoperative mild residual PS reduces the risk of PVR during follow-up of cToF patients. This finding provides clinical evidence for a conservative PS relief during correction of ToF.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Implante de Prótese de Valva Cardíaca , Estenose da Valva Pulmonar/etiologia , Tetralogia de Fallot/cirurgia , Função Ventricular Direita/fisiologia , Adolescente , Adulto , Contraindicações , Ecocardiografia , Feminino , Seguimentos , Humanos , Masculino , Prognóstico , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/prevenção & controle , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/fisiopatologia , Adulto Jovem
6.
J Thorac Cardiovasc Surg ; 144(6): 1315-22, 2012 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22405962

RESUMO

OBJECTIVES: Borderline left ventricle is the left ventricular morphology at the favorable end of the hypoplastic left heart syndrome. In contrast to the severe end, it is suitable for biventricular repair. Wondering whether it is possible to identify cases suitable for biventricular repair from a developmental viewpoint, we investigated the myocardial histology of borderline and severely hypoplastic left ventricles. METHODS: Postmortem specimens of neonatal, unoperated human hearts with severe hypoplastic left heart syndrome and borderline left ventricle were compared with normal specimens and hearts from patients with transposition of the great arteries. After tissue sampling of the lateral walls of both ventricles, immunohistochemical and immunofluorescence stainings against cardiac troponin I, N-cadherin, and connexin 43, important for proper cardiac differentiation, were done. RESULTS: All severely hypoplastic left hearts (7/7) and most borderline left ventricle hearts (4/6) showed reduced sarcomeric expressions of troponin I in left and right ventricles. N-cadherin and connexin 43 expressions were reduced in intercalated disks. The remaining borderline left ventricle hearts (2/6) were histologically closer to control hearts. CONCLUSIONS: Four of 6 borderline left ventricle hearts showed myocardial histopathology similar to the severely hypoplastic left hearts. The remainder were similar to normal hearts. Our results and knowledge regarding the role of epicardial-derived cells in myocardial differentiation lead us to postulate that an abnormal epicardial-myocardial interaction could explain the observed histopathology. Defining the histopathologic severity with preoperative myocardial biopsy samples of hearts with borderline left ventricle might provide a diagnostic tool for preoperative decision making.


Assuntos
Antígenos CD/análise , Caderinas/análise , Conexina 43/análise , Ventrículos do Coração/química , Síndrome do Coração Esquerdo Hipoplásico/metabolismo , Miocárdio/química , Autopsia , Biomarcadores/análise , Procedimentos Cirúrgicos Cardíacos , Regulação para Baixo , Imunofluorescência , Ventrículos do Coração/anormalidades , Ventrículos do Coração/patologia , Ventrículos do Coração/cirurgia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/patologia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Imuno-Histoquímica , Recém-Nascido , Miocárdio/patologia , Seleção de Pacientes , Fenótipo , Estudos Retrospectivos , Sarcômeros/química , Sarcômeros/patologia , Índice de Gravidade de Doença , Transposição dos Grandes Vasos/metabolismo , Transposição dos Grandes Vasos/patologia , Troponina I/análise
7.
Eur J Cardiothorac Surg ; 42(4): 680-4, 2012 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-22402454

RESUMO

OBJECTIVES: The Senning or Mustard procedure combined with the arterial switch operation (ASO) (± VSD and no left ventricular (LV) outflow tract obstruction) or the Rastelli operation (VSD and LV outflow tract obstruction) has become the preferred strategy over conventional repair as it is thought to prevent long-term dysfunction of the right ventricle (RV). More recently, hemi-Mustard rerouting of blood from the inferior vena cava to the RV in combination with bidirectional cavopulmonary anastomosis (BCPA) has been adopted by some centres for potential benefits over the classic atrial switch procedure. The aim of this study was to analyse our experience with hemi-Mustard and BCPA as part of an anatomical repair of congenitally corrected transposition of the great arteries (CCTGA) in selected patients. METHODS: Between 2004 and 2011, eight patients underwent hemi-Mustard/BCPA with the Rastelli operation (n = 6) or ASO (n = 2). The median age was 2.9 (range: 1.2-9.1) years. Positional anomalies were present in 75% of the patients. Both patients with ASO had dysplastic and insufficient tricuspid valves. In the Rastelli group, four patients had previously received shunts followed by BCPA in one patient. In the ASO group, both patients underwent pulmonary artery banding initially. RESULTS: There was one in-hospital death and no late mortality. Two patients received a pacemaker. One patient from the Rastelli group required conduit change 6 years later. At the mean follow-up of 4.5 years, six and one patients are in NYHA classes I and II, respectively; six patients showed good biventricular function, while one had LV dysfunction. Systemic venous obstruction and sinus node dysfunction were not observed, and BCPA was functioning well in all patients. CONCLUSIONS: Hemi-Mustard/BCPA is useful in anatomical repair of CCTGA in selected patients. When compared with the classic atrial switch operation, it is technically easier which makes it especially helpful in atrio-apical discordance; it unloads an RV with limited size or function, and avoids complications related to the upper limb of the classic atrial switch procedure. Mid-term results of this approach are favourable. Further follow-up is needed to prove long-term benefits.


Assuntos
Aorta Torácica/cirurgia , Derivação Cardíaca Direita/métodos , Artéria Pulmonar/cirurgia , Transposição dos Grandes Vasos/cirurgia , Criança , Pré-Escolar , Transposição das Grandes Artérias Corrigida Congenitamente , Feminino , Seguimentos , Átrios do Coração/cirurgia , Derivação Cardíaca Direita/mortalidade , Mortalidade Hospitalar , Humanos , Lactente , Masculino , Estudos Retrospectivos , Transposição dos Grandes Vasos/mortalidade , Resultado do Tratamento , Valva Tricúspide/cirurgia , Veia Cava Inferior/cirurgia
8.
Ann Thorac Surg ; 93(3): 849-55, 2012 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-22265201

RESUMO

BACKGROUND: Long-term results of reoperation for left atrioventricular valve regurgitation (LAVVR) after previous correction of atrioventricular septal defect (AVSD) are scarce. We evaluated long-term outcome of reoperation for LAVVR and identified risk factors for reoperation. METHODS: Between December 1976 and July 2006, 45 of 312 patients with correction of different AVSDs underwent reoperation for LAVVR. The cohort of 267 patients who did not need reoperation for LAVVR allowed for the identification of risk factors for reoperation and evaluation of overall survival after primary AVSD repair in a competing risk scenario. Clinical data were obtained by retrospective review. RESULTS: The left atrioventricular valve (LAVV) was repaired in 31 patients (68.9%) and replaced in 14 (31.1%). There were 3 in-hospital deaths (6.7%) and 2 late deaths (4.4%). Estimated overall survival was 88.1% at 15 years after the reoperation, and estimated incidence of death after reoperation in the total patient cohort was 2% at 15 years after the primary AVSD repair. Overall survival was significantly higher after LAVV repair than after replacement (p=0.010). Ten patients with LAVV repair required a second reoperation for LAVVR. At follow-up, survivors were in New York Heart Association functional class I (n=36) or II (n=4). Independent risk factors for first reoperation for LAVVR were associated cardiovascular anomalies (p<0.001), LAVV dysplasia (p<0.001), and nonclosure of the cleft (p=0.027). CONCLUSIONS: After previous correction of AVSD, LAVVR can usually be corrected by valve repair. A very dysplastic valve may necessitate replacement. Overall survival is higher after repair than after replacement. In general, overall survival of patients reoperated on for LAVVR is favorable. The overall mortality rate after primary repair of AVSD is explained only for a small part by mortality after reoperation for LAVVR.


Assuntos
Comunicação Interatrial/cirurgia , Comunicação Interventricular/cirurgia , Insuficiência da Valva Mitral/cirurgia , Complicações Pós-Operatórias/cirurgia , Pré-Escolar , Feminino , Comunicação Interatrial/mortalidade , Comunicação Interventricular/mortalidade , Humanos , Masculino , Recidiva , Reoperação , Estudos Retrospectivos , Fatores de Tempo
9.
Eur J Hum Genet ; 19(3): 247-52, 2011 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-21063442

RESUMO

The most common mutations found in FBN1 are missense mutations (56%), mainly substituting or creating a cysteine in a cbEGF domain. Other mutations are frameshift, splice and nonsense mutations. There are only a few reports of patients with marfanoid features and a molecularly proven complete deletion of a FBN1 allele. We describe the clinical features of 10 patients with a complete FBN1 gene deletion. Seven patients fulfilled the Ghent criteria for Marfan syndrome (MFS). The other three patients were examined at a young age and did not (yet) present the full clinical picture of MFS yet. Ectopia lentis was present in at least two patients. Aortic root dilatation was present in 6 of the 10 patients. In three patients, the aortic root diameter was on the 95th percentile and in one patient, the diameter of the aortic root was normal, the cross-section, however, had a cloverleaf appearance. Two patients underwent aortic root surgery at a relatively young age (27 and 34 years). Mitral valve prolapse was present in 4 of the 10 patients, and billowing of the mitral valve in 1. All patients had facial and skeletal features of MFS. Two patients with a large deletion extending beyond the FBN1 gene had an extended phenotype. We conclude that complete loss of one FBN1 allele does not predict a mild phenotype, and these findings support the hypothesis that true haploinsufficiency can lead to the classical phenotype of Marfan syndrome.


Assuntos
Alelos , Síndrome de Marfan/genética , Proteínas dos Microfilamentos/genética , Mutação , Adolescente , Adulto , Criança , Pré-Escolar , Cisteína/metabolismo , Ectopia do Cristalino/genética , Ectopia do Cristalino/patologia , Feminino , Fibrilina-1 , Fibrilinas , Haploinsuficiência , Humanos , Masculino , Prolapso da Valva Mitral/genética , Fenótipo , Deleção de Sequência , Adulto Jovem
10.
Ann Thorac Surg ; 90(5): 1554-61, 2010 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-20971263

RESUMO

BACKGROUND: Outcome of surgical correction of atrioventricular septal defects (AVSD) still varies despite enhanced results. We reviewed our 30-year experience with AVSD repair and identified risk factors for mortality and reoperation. METHODS: Between 1975 and 2006, 312 patients underwent surgery for complete AVSD (n = 209; 67.0%), partial AVSD (n = 76; 24.4%), or intermediate AVSD (n = 27; 8.6%). Mean age was 2.4 ± 3.9 years; 142 patients (45.5%) were younger than 6 months. Follow-up was 99.0% complete. RESULTS: There were 26 in-hospital deaths (8.3%) and 6 late deaths (2.1% of 283). Estimated overall survival for the total study population was 91.3%, 90.6%, and 88.6% at 1, 5, and 15 years, respectively. In the multivariable logistic regression analysis, surgical era 1975 to 1995 (p < 0.001) and younger age (p = 0.004) were found to be independent risk factors for early mortality, whereas preoperative AV valve insufficiency showed a tendency toward statistical significance (p = 0.052). Of the hospital survivors, 43 patients required a late reoperation. Estimated freedom from late reoperation was 96.4%, 89.3%, and 81.8% at 1, 5, and 15 years, respectively. Multivariable Cox regression analysis showed associated cardiovascular anomalies (p < 0.001), left AV valve dysplasia (p < 0.001), and absence of cleft closure (p = 0.003) to be independent risk factors for late reoperation. CONCLUSIONS: AVSD repair can be accomplished with good long-term results. Early surgical era, associated cardiovascular anomalies, left AV valve dysplasia, and absence of cleft closure negatively influence survival and risk of reoperation.


Assuntos
Comunicação Interatrial/cirurgia , Comunicação Interventricular/cirurgia , Pré-Escolar , Feminino , Comunicação Interatrial/mortalidade , Comunicação Interventricular/mortalidade , Mortalidade Hospitalar , Humanos , Lactente , Recém-Nascido , Masculino , Reoperação , Estudos Retrospectivos
11.
Hum Mutat ; 31(12): E1915-27, 2010 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-20886638

RESUMO

Marfan syndrome (MFS) is a dominant disorder with a recognizable phenotype. In most patients with the classical phenotype mutations are found in the fibrillin-1 gene (FBN1) on chromosome 15q21. It is thought that most mutations act in a dominant negative way or through haploinsufficiency. In 9 index cases referred for MFS we detected heterozygous missense mutations in FBN1 predicted to substitute the first aspartic acid of different calcium-binding Epidermal Growth Factor-like (cbEGF) fibrillin-1 domains. A similar mutation was found in homozygous state in 3 cases in a large consanguineous family. Heterozygous carriers of this mutation had no major skeletal, cardiovascular or ophthalmological features of MFS. In the literature 14 other heterozygous missense mutations are described leading to the substitution of the first aspartic acid of a cbEGF domain and resulting in a Marfan phenotype. Our data show that the phenotypic effect of aspartic acid substitutions in the first position of a cbEGF domain can range from asymptomatic to a severe neonatal phenotype. The recessive nature with reduced expression of FBN1 in one of the families suggests a threshold model combined with a mild functional defect of this specific mutation.


Assuntos
Ácido Aspártico/genética , Fator de Crescimento Epidérmico/química , Genes Recessivos/genética , Proteínas dos Microfilamentos/química , Proteínas dos Microfilamentos/genética , Mutação de Sentido Incorreto/genética , Adolescente , Adulto , Substituição de Aminoácidos/genética , Criança , Família , Feminino , Fibrilina-1 , Fibrilinas , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Modelos Moleculares , Linhagem , Estrutura Terciária de Proteína , Relação Estrutura-Atividade , Adulto Jovem
12.
Pediatr Cardiol ; 31(2): 229-33, 2010 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-19937010

RESUMO

To assess the prevalence, history, and treatment of arrhythmias, in particular preexcitation and Wolff-Parkinson-White (WPW) syndrome, in patients with Ebstein anomaly (EA) during childhood and adolescence, we performed a multicenter retrospective study of all consecutive live-born patients with EA, diagnosed, and followed by pediatric cardiologists between 1980 and 2005 in The Netherlands. During a follow-up after EA diagnosis of 13 years 3 months (range: 6 days to 28 years 2 months), 16 (17%) of the 93 pediatric EA patients exhibited rhythm disturbances. Nine patients showed arrhythmic events starting as of the neonatal period. Supraventricular tachycardia was noted in 11 patients. One patient died in the neonatal period due to intractable supraventricular tachycardia resulting in heart failure and one patient died at 5 weeks of age most probably due to an arrhythmic event. The 14 surviving patients all show preexcitation, albeit 4 of them intermittently, and all have a right-sided accessory pathway location. Nine patients underwent catheter ablation of an accessory pathway. Only four patients are currently on antiarrhythmic drugs. The 17% prevalence of rhythm disturbances in pediatric EA patients, most commonly supraventricular arrhythmias, is significantly lower than in adult EA patients. Life-threatening rhythm disturbances are not frequent early in life. Symptomatic patients are well treated with radiofrequency catheter ablation.


Assuntos
Arritmias Cardíacas/epidemiologia , Anomalia de Ebstein/epidemiologia , Adolescente , Adulto , Criança , Pré-Escolar , Eletrocardiografia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Países Baixos/epidemiologia , Síndromes de Pré-Excitação/epidemiologia , Estudos Retrospectivos , Taquicardia Supraventricular/epidemiologia , Síndrome de Wolff-Parkinson-White/epidemiologia
13.
Eur J Cardiothorac Surg ; 35(4): 582-7; discussion 587-8, 2009 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-19223194

RESUMO

OBJECTIVE: To analyze size and function of aortic root and left ventricle as well as quality of life in patients 20 years after arterial switch procedure. METHODS: Thirty-nine patients who underwent arterial switch operation between 1977 and 1989 were examined. Perioperative and follow-up data were analyzed. Evaluation included clinical assessment, ECG, echocardiography and quality of life questionnaire. RESULTS: Patients had simple transposition (24), transposition with ventricle septal defect (7), Taussig-Bing anomaly (4) or transposition with ventricle septal defect and aortic arch obstruction (4). Mean age at evaluation was 19.9+/-2.6 years. Seven patients required reintervention for pulmonary stenosis (4), coarctation (2) and subaortic stenosis, followed by valve replacement 10 years later (1). Arrhythmia occurred in four patients. Patients were in New York Heart Association functional class I (38) or II (1). Quality of life scores were comparable to normal controls except for the lower score in the domains of vitality, aggressive and depressive mood. Diameters of aortic annulus, sinus of Valsalva and sinotubular junction were 26.5+/-4.1, 36.5+/-5.6 and 29.2+/-6.6mm respectively. Sixty-five percent of sinus of Valsalva and 38% of sinotubular junction indexed to body surface area fall outside the 95% confidence interval. Aortic regurgitation was absent in 72%, mild in 13% and moderate in 15%. No patient had severe regurgitation. Patients without regurgitation had smaller diameters of annulus (p=0.005) and sinus of Valsalva (p=0.01). Left ventricular end-diastolic and end-systolic diameters were 51+/-7mm and 34+/-6mm respectively. Fractional shortening was 34+/-5%; no regional wall motion abnormalities were observed. CONCLUSIONS: Clinical outcome is good 20 years after arterial switch operation and aortic valve function remains preserved in most patients. However, aortic root dilatation is present in two thirds of patients emphasizing the need for careful follow-up.


Assuntos
Aorta/patologia , Transposição dos Grandes Vasos/cirurgia , Função Ventricular Esquerda/fisiologia , Adolescente , Adulto , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/etiologia , Insuficiência da Valva Aórtica/patologia , Arritmias Cardíacas/etiologia , Eletrocardiografia , Seguimentos , Cardiopatias Congênitas/cirurgia , Humanos , Complicações Pós-Operatórias , Qualidade de Vida , Reoperação , Seio Aórtico/patologia , Transposição dos Grandes Vasos/fisiopatologia , Transposição dos Grandes Vasos/reabilitação , Resultado do Tratamento , Ultrassonografia , Adulto Jovem
14.
Interact Cardiovasc Thorac Surg ; 7(3): 517-8, 2008 May.
Artigo em Inglês | MEDLINE | ID: mdl-18375450

RESUMO

We report on an uncommon case of a 10-month-old patient who required early surgical closure of an ostium secumdum atrial septal defect due to the concomitant presence of a big subcarinal bronchogenic cyst compressing the left atrium and, therefore, increasing the left-to-right shunt. It led to refractory congestive heart failure symptoms, establishing thereby an earlier indication of surgical treatment.


Assuntos
Cisto Broncogênico/diagnóstico , Procedimentos Cirúrgicos Cardíacos , Insuficiência Cardíaca/etiologia , Comunicação Interatrial/cirurgia , Cisto Mediastínico/diagnóstico , Cisto Broncogênico/complicações , Cisto Broncogênico/cirurgia , Ponte Cardiopulmonar , Átrios do Coração/cirurgia , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/cirurgia , Comunicação Interatrial/complicações , Comunicação Interatrial/diagnóstico por imagem , Humanos , Lactente , Cisto Mediastínico/complicações , Cisto Mediastínico/cirurgia , Esterno/cirurgia , Fatores de Tempo , Tomografia Computadorizada por Raios X
15.
Eur Heart J ; 28(21): 2661-6, 2007 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-17895254

RESUMO

AIMS: The objective of this study is to establish factors associated with death after diagnosis of Ebstein's anomaly (EA) during childhood and adolescence. METHODS AND RESULTS: This study is a retrospective chart review. All paediatric patients were diagnosed with EA and followed in tertiary-care university hospitals between 1980 and 2005. Factors associated with death were obtained using the Cox regression and log-rank tests. Of the 93 patients with EA, 18 (19%) died and 75 (81%) survived. The median age at EA diagnosis and follow-up was 0 (range 0-162) and 86 months (range 0-216), respectively. After 35 months of diagnosis, the Kaplan-Meier survival probability remains stable at 80%. Young age at presentation (< or =12 months), hepatomegaly, the need for medication (diuretics and Prostin) and mechanical ventilation at presentation, pulmonary valve defects (defined as moderate-to-severe pulmonary stenosis and pulmonary atresia), patent arterial duct, and ventricular septal defect were significantly associated with death. CONCLUSION: The overall survival of patients with EA during childhood and adolescence has dramatically improved when compared with earlier reports.


Assuntos
Anomalia de Ebstein/mortalidade , Adolescente , Fatores Etários , Causas de Morte , Criança , Pré-Escolar , Anomalia de Ebstein/complicações , Métodos Epidemiológicos , Feminino , Humanos , Lactente , Recém-Nascido , Masculino
16.
Ann Thorac Surg ; 84(2): 599-605, 2007 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-17643642

RESUMO

BACKGROUND: We analyzed the results in two centers of using bovine jugular vein graft for right ventricular outflow tract reconstruction. METHODS: From April 1999 to July 2005, 133 children with a median age of 30.9 months (range, 4 days to 19 years) underwent graft implantation. Echocardiography was performed during follow-up and retrospectively reviewed. RESULTS: Nongraft-related early mortality occurred in 8 patients. Late mortality occurred in 11 patients, 2 late deaths were graft related (endocarditis). Median follow-up was 31.6 months (range, 1 to 73). Twelve patients received a new graft, because of endocarditis (3), distal pulmonary artery branch stenosis (4), graft obstruction caused by fibrosis (4), or thrombosis (1). Echocardiography Doppler studies showed good conduit function, with 92% of the patients having absent, trivial, or only mild valve regurgitation at last follow-up. A moderate degree of conduit stenosis due to external compression was observed in 2 patients. Twenty-five patients with otherwise intact conduits had hemodynamically significant distal stenosis. In most cases, the pulmonary branch stenosis was related to preoperative small pulmonary arteries and young age at operation. At 31.6 months, significant graft dilatation was observed in 4 grafts and was related to pulmonary artery branch obstruction or pulmonary hypertension. Calcification did not occur in 5 years time. Survival was 85.7%, freedom from conduit explantation was 91%, and freedom from intervention for pulmonary artery branch stenosis was 80% after 5 years. CONCLUSIONS: The bovine jugular vein graft is a valuable right ventricular outflow tract conduit, but younger age and small pulmonary arteries increase the risk of distal conduit stenosis.


Assuntos
Ventrículos do Coração/cirurgia , Veias Jugulares/transplante , Atresia Pulmonar/cirurgia , Tronco Arterial/cirurgia , Adolescente , Animais , Bovinos , Criança , Pré-Escolar , Ecocardiografia , Ventrículos do Coração/diagnóstico por imagem , Humanos , Lactente , Recém-Nascido , Procedimentos de Cirurgia Plástica , Estudos Retrospectivos , Análise de Sobrevida , Transplante Heterólogo/mortalidade , Resultado do Tratamento , Disfunção Ventricular Direita
17.
Eur J Cardiothorac Surg ; 32(1): 143-8, 2007 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-17478093

RESUMO

OBJECTIVE: To evaluate pediatric atrioventricular valve repair with artificial chordae. METHODS: Between February 2001 and January 2006, artificial chords were used in 21 children with severe mitral or tricuspid valve regurgitation. Patients with AVSD were excluded. Median age was 84 (1-194) months. Five patients had isolated tricuspid valve anomalies, 16 had mitral valve anomalies (associated tricuspid annular dilatation in 4). Tricuspid neochordae were placed to anterior (three patients) and septal (two patients) leaflets. Mitral neochordae were placed to anterior (15 patients) and posterior (1 patient) leaflets. Additional ring annuloplasties were performed in 12 (mitral 11, tricuspid 1), as well as 2 de Vega tricuspid annuloplasties. Patch insertion was used in acute endocarditis (tricuspid one). All echocardiographic studies were reviewed and analyzed by a single cardiologist. RESULTS: No mortality occurred. Follow-up was complete (mean 28+/-18 months). Two patients were reoperated, one for mitral ring dehiscence and one for recurring mitral valve insufficiency. Both valves were replaced by mechanical valve prosthesis. At last follow-up tricuspid insufficiency was mild (three) or moderate (two). Moderate insufficiency occurred due to remaining restriction of the septal leaflet after repair in endocarditis (one) and remaining prolapse of the anterior leaflet (one). Mitral insufficiency was absent (five), mild (seven), or moderate (two). Moderate insufficiency was caused by recurrent anterior leaflet shortening after valve repair in rheumatic valve disease (two). Valve restriction caused by artificial chordae was not found. CONCLUSIONS: Mitral and tricuspid valve repair with artificial chordae in children demonstrated acceptable results. Despite patient growth, valvular restriction by the artificial chordae was not observed ad mid-term follow-up.


Assuntos
Cordas Tendinosas/cirurgia , Próteses Valvulares Cardíacas , Insuficiência da Valva Mitral/cirurgia , Insuficiência da Valva Tricúspide/cirurgia , Adolescente , Criança , Pré-Escolar , Métodos Epidemiológicos , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Valva Mitral/anormalidades , Valva Mitral/cirurgia , Insuficiência da Valva Mitral/diagnóstico por imagem , Politetrafluoretileno , Reoperação , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/cirurgia , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Ultrassonografia
18.
Congenit Heart Dis ; 2(1): 38-43, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-18377515

RESUMO

OBJECTIVE: To determine the incidence of congenital heart disease (CHD) and right ventricular outflow tract obstruction (RVOTO) in twin-to-twin transfusion syndrome (TTTS) treated with fetoscopic laser surgery and evaluate the role of increased afterload by determining the difference in blood pressure and endothelin-1 at birth between donor and recipient twins. DESIGN: Prospective study. SETTING: Tertiary medical center, serving as the national referral center for fetoscopic laser surgery for TTTS in The Netherlands. PATIENTS: All consecutive cases of monochorionic twins with TTTS treated with laser (n = 46 twin pairs) and monochorionic twins without TTTS (n = 55 twin pairs) delivered at our center between June 2002 and June 2005 were included in the study. INTERVENTIONS: Echocardiography was performed within 1 week after delivery. At birth, blood pressure was measured in all survivors and endothelin-1 was determined in umbilical cord blood. Data on RVOTO in TTTS treated with laser surgery at our center but delivered elsewhere were reviewed retrospectively from medical records. RESULTS: The incidence of CHD in the TTTS group and non-TTTS group was 5.4% (4/74) and 2.3% (2/87) (P = .42), respectively. RVOTO was diagnosed in 1 recipient twin delivered at our center and 2 recipient twins delivered elsewhere. The incidence of RVOTO in recipients was 4% (3/75). Mean systolic blood pressure at birth was similar in donor and recipient twins, respectively, 53 mm Hg vs. 56 mm Hg (P = .42). Mean endothelin-1 level at birth was also similar between donors and recipients, respectively, 14.3 ng/L and 13.2 ng/L (P = .64). CONCLUSION: The incidence of CHD in TTTS treated with fetoscopic laser surgery is higher than in the general population (5.4% vs. 0.5%). We found no difference in afterload parameters between donors and recipients after laser treatment.


Assuntos
Transfusão Feto-Fetal/complicações , Fetoscopia , Cardiopatias Congênitas/cirurgia , Terapia a Laser/métodos , Obstrução do Fluxo Ventricular Externo/cirurgia , Pressão Sanguínea , Doenças em Gêmeos , Ecocardiografia , Endotelina-1/sangue , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/fisiopatologia , Humanos , Recém-Nascido , Masculino , Gravidez , Estudos Prospectivos , Resultado do Tratamento , Obstrução do Fluxo Ventricular Externo/complicações , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem
19.
Pacing Clin Electrophysiol ; 26(10): 2042-4, 2003 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-14516349

RESUMO

This report describes the mid-term beneficial hemodynamic effect of biventricular pacing in an infant with congestive heart failure after congenital heart surgery, due to resynchronization of the left and right ventricle, optimization of the AV delay, and (partial) correction of the LV dyssynchrony.


Assuntos
Bloqueio Cardíaco/terapia , Cardiopatias Congênitas/cirurgia , Marca-Passo Artificial , Disfunção Ventricular Esquerda/cirurgia , Feminino , Bloqueio Cardíaco/etiologia , Humanos , Recém-Nascido
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