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1.
Heart ; 108(23): 1881-1886, 2022 11 10.
Artículo en Inglés | MEDLINE | ID: mdl-35851319

RESUMEN

OBJECTIVES: To determine the long-term and transplantation-free survival of all patients after atrial correction of transposition of the great arteries (TGA) in the Czech and Slovak republics, including its preoperative and perioperative determinants. METHODS: Retrospective analysis of all 454 consecutive patients after atrial correction of TGA was performed. Of these, 126 (27.8%) were female, median age at operation was 7.4 months (Q1 5.3; Q3 13.3) and 164 (36.1%) underwent the Mustard procedure. The relationships between age, weight, the complexity of TGA, operative technique, additional surgical procedures, immediate postoperative presence of tricuspid regurgitation and revision procedures during follow-up to major composite outcome, as such defined as long-term and transplantation-free survival, were tested. RESULTS: Early 30-day mortality did not differ between the Mustard (9.76%) and Senning (8.97%) cohorts (p=0.866). The long-term and transplantation-free survival, which differed between the Mustard and Senning cohorts in favour of the Senning procedure (HR 0.43; 95% CI 0.21 to 0.87), was shorter in complex TGA (HR 2.4; 95% CI 1.59 to 3.78) and in complex surgical interventions (HR 3.51; 95% CI 2.31 to 5.56). The immediate presence of at least moderate tricuspid regurgitation after correction was associated with a shorter long-term and transplantation-free survival in the univariate but not in the multivariable model. CONCLUSIONS: The lower long-term survival of patients after an atrial switch operation of TGA in the Czech and Slovak republics is associated with greater complexity of TGA, complex surgical interventions and application of the the Mustard operative procedure.


Asunto(s)
Operación de Switch Arterial , Transposición de los Grandes Vasos , Insuficiencia de la Válvula Tricúspide , Humanos , Femenino , Lactante , Masculino , Insuficiencia de la Válvula Tricúspide/cirugía , Estudios Retrospectivos , Operación de Switch Arterial/efectos adversos , Arterias , Estudios de Seguimiento , Resultado del Tratamiento
2.
Front Cardiovasc Med ; 9: 876755, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35757340

RESUMEN

Pulmonary hypertension (PH) is an established risk factor in patients with atrial septal defect (ASD), and its persistence after ASD closure is associated with increased mortality. Therefore, predictors for PH normalization after defect closure are needed. Multiple hemodynamic types of PH exist, but little is known about their prevalence and prognostic value for PH normalization after ASD closure. We carried out a retrospective study on 97 patients (76% female, median age at ASD closure 58 years) with four types of PH determined predominantly by right heart catheterization: hyperkinetic, pulmonary arterial hypertension, isolated post-capillary, and combined pre- and post-capillary. We investigated the frequency of the PH types and their prognostic significance for PH normalization after ASD closure. Frequency of PH types before ASD closure in our study was: hyperkinetic 55%, pulmonary arterial hypertension 10%, isolated post-capillary PH 24%, and combined PH 11%. Hyperkinetic PH type was positively associated with PH normalization after ASD closure (78% patients normalized), remaining a significant independent predictor when adjusted for age at closure, sex, heart failure, and NYHA. Hyperkinetic PH patients also had significantly better survival prognosis versus patients with other PH types (p = 0.04). Combined PH was negatively associated with PH normalization, with no patients normalizing. Pulmonary arterial hypertension and isolated post-capillary PH had intermediate rates of normalization (60 and 52%, respectively). In summary, all four hemodynamic types of PH are found in adult patients with ASD, and they can be used to stratify patients by their likelihood of PH normalization and survival after ASD closure.

3.
Front Cardiovasc Med ; 9: 867012, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35571174

RESUMEN

Background: Atrial septal defect (ASD) is the most common congenital heart disease (CHD) in adults and pulmonary hypertension (PH) is an established risk factor. A decision whether to perform ASD closure, especially in elderly patients with PH, is a complex dilemma. The aim of our study was to compare long-term survival in patients with closed and open ASD. Methods: A retrospective cohort study was performed on 427 patients with ASD (median age at diagnosis 38 years, IQR 18-56) out of which 186 patients (44%) manifested PH. ASD closure in patients with PH was only considered in patients without Eisenmenger syndrome with pulmonary vascular resistance < 5 WU. Median follow-up duration was 18 years (IQR 9-31 years). Kaplan-Meier and Cox proportional hazards survival analyses were performed to evaluate 12 potential predictors of survival. Results: Defect closure was associated with improved long-term survival in ASD patients both with (P < 0.001) and without PH (P = 0.01) and this association was present also in patients over 40 years. The 20-year survival since diagnosis was significantly higher in patients with PH and closed ASD compared to those with PH and open ASD (65% vs. 41%). ASD closure was a significant independent predictor of long-term survival (P = 0.003) after accounting for age at diagnosis, PH, NYHA class, Eisenmenger syndrome, and mitral regurgitation. Significant negative independent predictors of survival were older age at diagnosis (P < 0.001), Eisenmenger syndrome (P < 0.001), and PH (P = 0.03). Conclusion: ASD closure appears to be associated with improved long-term survival independently of age, PH, and other clinical variables.

4.
J Am Heart Assoc ; 10(6): e018302, 2021 03 16.
Artículo en Inglés | MEDLINE | ID: mdl-33719495

RESUMEN

Background Cardiac resynchronization therapy (CRT) is rarely used in patients with congenital heart disease, and reported follow-up is short. We sought to evaluate long-term impact of CRT in a single-center cohort of patients with congenital heart disease. Methods and Results Thirty-two consecutive patients with structural congenital heart disease (N=30) or congenital atrioventricular block (N=2), aged median of 12.9 years at CRT with pacing capability device implantation, were followed up for a median of 8.7 years. CRT response was defined as an increase in systemic ventricular ejection fraction or fractional area of change by >10 units and improved or unchanged New York Heart Association class. Freedom from cardiovascular death, heart failure hospitalization, or new transplant listing was 92.6% and 83.2% at 5 and 10 years, respectively. Freedom from CRT complications, leading to surgical system revision (elective generator replacement excluded) or therapy termination, was 82.7% and 72.2% at 5 and 10 years, respectively. The overall probability of an uneventful therapy continuation was 76.3% and 58.8% at 5 and 10 years, respectively. There was a significant increase in ejection fraction/fractional area of change (P<0.001) mainly attributable to patients with systemic left ventricle (P=0.002) and decrease in systemic ventricular end-diastolic dimensions (P<0.05) after CRT. New York Heart Association functional class improved from a median 2.0 to 1.25 (P<0.001). Long-term CRT response was present in 54.8% of patients at last follow-up and was more frequent in systemic left ventricle (P<0.001). Conclusions CRT in patients with congenital heart disease was associated with acceptable survival and long-term response in ≈50% of patients. Probability of an uneventful CRT continuation was modest.


Asunto(s)
Terapia de Resincronización Cardíaca/métodos , Cardiopatías Congénitas/terapia , Ventrículos Cardíacos/fisiopatología , Remodelación Ventricular/fisiología , Adolescente , Niño , Preescolar , Electrocardiografía , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/fisiopatología , Ventrículos Cardíacos/diagnóstico por imagen , Humanos , Masculino , Volumen Sistólico , Factores de Tiempo , Resultado del Tratamiento
5.
Int J Cardiol ; 276: 87-92, 2019 Feb 01.
Artículo en Inglés | MEDLINE | ID: mdl-30172474

RESUMEN

AIMS: To develop and validate a clinically useful risk prediction tool for patients with adult congenital heart disease (ACHD). METHODS AND RESULTS: A risk model was developed in a prospective cohort of 602 patients with moderate/complex ACHD who routinely visited the outpatient clinic of a tertiary care centre in the Netherlands (2011-2013). This model was externally validated in a retrospective cohort of 402 ACHD patients (Czech Republic, 2004-2013). The primary endpoint was the 4-year risk of death, heart failure, or arrhythmia, which occurred in 135 of 602 patients (22%). Model development was performed using multivariable logistic regression. Model performance was assessed with C-statistics and calibration plots. Of the 14 variables that were selected by an expert panel, the final prediction model included age (OR 1.02, 95%CI 1.00-1.03, p = 0.031), congenital diagnosis (OR 1.52, 95%CI 1.03-2.23, p = 0.034), NYHA class (OR 1.74, 95%CI 1.07-2.84, p = 0.026), cardiac medication (OR 2.27, 95%CI 1.56-3.31, p < 0.001), re-intervention (OR 1.41, 95%CI 0.99-2.01, p = 0.060), BMI (OR 1.03, 95%CI 0.99-1.07, p = 0.123), and NT-proBNP (OR 1.63, 95%CI 1.45-1.84, p < 0.001). Calibration-in-the-large was suboptimal, reflected by a lower observed event rate in the validation cohort (17%) than predicted (36%), likely explained by heterogeneity and different treatment strategies. The externally validated C-statistic was 0.78 (95%CI 0.72-0.83), indicating good discriminative ability. CONCLUSION: The proposed ACHD risk score combines six readily available clinical characteristics and NT-proBNP. This tool is easy to use and can aid in distinguishing high- and low-risk patients, which could further streamline counselling, location of care, and treatment in ACHD.


Asunto(s)
Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/epidemiología , Modelos Teóricos , Adulto , Estudios de Cohortes , Femenino , Estudios de Seguimiento , Humanos , Masculino , Países Bajos/epidemiología , Valor Predictivo de las Pruebas , Estudios Prospectivos , Reproducibilidad de los Resultados , Medición de Riesgo/métodos , Medición de Riesgo/normas
6.
Congenit Heart Dis ; 12(4): 448-457, 2017 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-28419713

RESUMEN

OBJECTIVE: The patients after Mustard and Senning corrections of transposition of the great arteries (TGA) are at an increased risk of unexpected death. The aim of this study was to identify markers allowing risk stratification of patients after atrial switch correction of TGA to provide them with optimum care. METHODS AND RESULTS: In this study, 87 patients were retrospectively evaluated after atrial switch correction of TGA followed-up between 2005 and 2015. The mortality during the follow-up was 9% (8 cardiac deaths). Markers significantly predictive of death using univariable Cox proportional hazard ratio survival analysis were: N-terminal pro-B-type natriuretic peptide (NT-proBNP), ejection fraction and end-diastolic dimension of the systemic right ventricle, mitral E, e', and s'. Surprisingly, the Doppler parameters of mitral valve in subpulmonary ventricle were more important for prognosis than those of systemic tricuspid valve. In multivariable analysis, the only independent predictors of mortality were NT-proBNP (P = .00048; AUC 0.97) and the velocity of early diastolic filling (mitral E) in subpulmonary ventricle (P = .01815; AUC 0.81). According to Kaplan-Meier survival analysis, patients with NT-proBNP > 1000 pg/ml are at high risk of death. Patients with mitral E < 68 cm/s are also at an increased risk of death. CONCLUSIONS: NT-proBNP is the most reliable prognostic mortality factor and should be measured regularly in TGA patients after Mustard or Senning correction. Diastolic filling velocity of the subpulmonary left ventricle (mitral E) may be more important for prognosis than systolic function of the systemic right ventricle.


Asunto(s)
Procedimientos Quirúrgicos Cardíacos/métodos , Péptido Natriurético Encefálico/sangre , Fragmentos de Péptidos/sangre , Medición de Riesgo/métodos , Transposición de los Grandes Vasos/mortalidad , Adulto , Biomarcadores/sangre , República Checa/epidemiología , Ecocardiografía Doppler , Femenino , Estudios de Seguimiento , Humanos , Masculino , Periodo Posoperatorio , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Tasa de Supervivencia/tendencias , Factores de Tiempo , Transposición de los Grandes Vasos/sangre , Transposición de los Grandes Vasos/cirugía , Adulto Joven
8.
Int J Cardiol ; 220: 131-6, 2016 Oct 01.
Artículo en Inglés | MEDLINE | ID: mdl-27376569

RESUMEN

OBJECTIVES: To describe the incidence, onset, predictors and outcome of ventricular tachyarrhythmia (VTA) in pregnant women with heart disease. BACKGROUND: VTA during pregnancy will cause maternal morbidity and even mortality and will have impact on fetal outcome. Insufficient data exist on the incidence and outcome of VTA in pregnancy. METHODS AND RESULTS: From January 2007 up to October 2013, 99 hospitals in 39 countries enrolled 2966 pregnancies in women with structural heart disease. Forty-two women (1.4%) developed clinically relevant VTA during pregnancy, which occurred mainly in the third trimester (48%). NYHA class >1 before pregnancy was an independent predictor for VTA. Heart failure during pregnancy was more common in women with VTA than in women without VTA (24% vs. 12%, p=0.03) and maternal mortality was respectively 2.4% and 0.3% (p=0.15). More women with VTA delivered by Cesarean section than women without VTA (68% vs. 47%, p=0.01). Neonatal death, preterm birth (<37weeks), low birthweight (<2500g) and Apgar score <7 occurred more often in women with VTA (4.8% vs. 0.3%, p=0.01; 36% vs. 16%, p=0.001; 33% vs. 15%, p=0.001 and 25% vs. 7.3%, p=0.001, respectively). CONCLUSIONS: VTA occurred in 1.4% of pregnant women with cardiovascular disease, mainly in the third trimester, and was associated with heart failure during pregnancy. NYHA class before pregnancy was predictive. VTA during pregnancy had clear impact on fetal outcome.


Asunto(s)
Insuficiencia Cardíaca , Complicaciones Cardiovasculares del Embarazo , Taquicardia Ventricular , Adulto , Cesárea/estadística & datos numéricos , Europa (Continente)/epidemiología , Femenino , Insuficiencia Cardíaca/complicaciones , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/epidemiología , Humanos , Recién Nacido , Cooperación Internacional , Embarazo , Complicaciones Cardiovasculares del Embarazo/diagnóstico , Complicaciones Cardiovasculares del Embarazo/mortalidad , Resultado del Embarazo/epidemiología , Tercer Trimestre del Embarazo , Nacimiento Prematuro/epidemiología , Nacimiento Prematuro/etiología , Sistema de Registros/estadística & datos numéricos , Medición de Riesgo , Factores de Riesgo , Índice de Severidad de la Enfermedad , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiología , Taquicardia Ventricular/mortalidad
10.
Am J Cardiol ; 116(9): 1425-30, 2015 Nov 01.
Artículo en Inglés | MEDLINE | ID: mdl-26404943

RESUMEN

Natriuretic peptides are often elevated in congenital heart disease (CHD); however, the clinical impact on mortality is unclear. The aim of our study was to evaluate the prognostic value of N-terminal pro-brain natriuretic peptide (NT-proBNP) in the prediction of all-cause mortality in adults with different CHD. In this prospective longitudinal mortality study, we evaluated NT-proBNP in 1,242 blood samples from 646 outpatient adults with stable CHD (mean age 35 ± 12 years; 345 women). Patients were followed up for 6 ± 3 (1 to 10) years. The mortality rate was 5% (35 patients, mean age 40 ± 14 years, 17 women). Median NT-proBNP (pg/ml) was 220 in the whole cohort, 203 in survivors, and 1,548 in deceased patients. The best discrimination value for mortality prediction was 630 pg/ml with 74% sensitivity and 84% specificity. During the follow-up, the survival rate was 65% for those with median NT-proBNP ≥630 pg/ml and 94% for NT-proBNP <630 pg/ml; p <0.0001. There was only 1% mortality among 388 patients with at least 1 NT-proBNP value ≤220 pg/ml compared with 41% mortality among 54 patients with at least 1 NT-proBNP value >1,548 pg/ml. Even the first (baseline) measurements of NT-proBNP were strongly associated with a high risk of death (log10 NT-proBNP had hazard ratio 7, p <0.0001). In conclusion, NT-proBNP assessment is a useful and simple tool for the prediction of mortality in long-term follow-up of adults with CHD.


Asunto(s)
Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/mortalidad , Péptido Natriurético Encefálico/sangre , Fragmentos de Péptidos/sangre , Adolescente , Adulto , Anciano , Biomarcadores/sangre , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/sangre , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Pronóstico , Estudios Prospectivos , Medición de Riesgo , Factores de Riesgo , Sensibilidad y Especificidad
11.
Vnitr Lek ; 61(5): 466-9, 2015 May.
Artículo en Checo | MEDLINE | ID: mdl-26075857

RESUMEN

Care of patients with congenital heart diseases in Czech Republic does not have only important history, falling to the prewar period, but especially systematic and long-term work with excellent results in pediatric patients. With improvements in the care of these patients also improves their survival with the need for continuous care in adulthood including reoperation, catheter ablations incisional tachycardias and other specialized procedures in difficult heart morphology. The article is a reflection and an appeal to all responsible professionals, organizers and payers of health care, how to proceed and provide specialized training, continuity of care for these patients, including proposed organizational changes and outlines the need for further development in this important medical field.


Asunto(s)
Cardiología/tendencias , Atención a la Salud/tendencias , Cardiopatías Congénitas/terapia , República Checa , Humanos
12.
Clin Chem Lab Med ; 53(8): 1291-6, 2015 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-25781544

RESUMEN

BACKGROUND: The aim of the study was to investigate serum NT-proBNP levels in adult patients with transposition of the great arteries (d-TGA) corrected by atrial switch procedures (Mustard or Senning) operation and to assess the relationship with ventricular impairment and NYHA class. METHODS: Serum NT-proBNP levels were measured in a group of 81 consecutive adult patients (59 males, mean age 27 years and 22 females, mean age 28 years) with transposition of the great arteries (TGA) after surgical correction in childhood, and in a control group of 25 healthy individuals (16 males, mean age 32 years, and 9 females, mean age 29 years). Age-matched correlation of NT-proBNP concentrations in TGA patients after Mustard or Senning correction was performed, but this correlation was considered not significant (p=0.08). RESULTS: Concentrations of NT-proBNP in patients with TGA were significantly elevated compared to the control group of healthy individuals (203 ng/L vs. 41 ng/L, p<0.0001). Patients after the Mustard repair had significantly higher NT-proBNP values than patients after the Senning operation (234 ng/L vs. 148 ng/L, p=0.0023). NT-proBNP correlated negatively with the systemic right ventricular ejection fraction with the greatest significance in patients after Mustard correction (r=-0.32, p<0.0001). The concentration of NT-proBNP was also associated with NYHA functional class (p=0.0035) with the greatest significance in patients with Mustard correction (p=0.028). CONCLUSIONS: Elevated levels of NT-proBNP appear to be a useful tool in assessing heart failure in patients with transposition of the great arteries after atrial switch correction.


Asunto(s)
Operación de Switch Arterial/efectos adversos , Péptido Natriurético Encefálico/sangre , Fragmentos de Péptidos/sangre , Transposición de los Grandes Vasos/sangre , Disfunción Ventricular/sangre , Adulto , Femenino , Humanos , Masculino , Transposición de los Grandes Vasos/cirugía , Disfunción Ventricular/cirugía
14.
Vnitr Lek ; 60(4): 335-40, 2014 Apr.
Artículo en Checo | MEDLINE | ID: mdl-24985995

RESUMEN

INTRODUCTION: The outcome of the operation of the Ebstein anomaly in adulthood depends on the experience of the surgical team. METHODS AND RESULTS: We operated 38 adult patients with Ebstein anomaly at the age of 34.8 ± 12.7 (19-63) years at the Department of Cardiac Surgery, Hospital Na Homolce, Prague, in the period of 2005-2013. The majority of patients (71 %) had Ebstein anomaly type C or D. The 30-days postoperative mortality was 5.2 %. In the long-term follow-up 2 other patients died. Tricuspid valve repair was performed in 45 %, bioprosthesis was implanted in 53 %. Concomitant bi-directional cavo-pulmonary anastomosis was performed in 31.5 %, mitral valve repair in 8 %, closure of atrial communication in 83 %, right-sided MAZE or istmus cryo-ablation in 39 %. After the operation we found improvement of the functional NYHA class (from 2.2 ± 0.7 to 1.7 ± 0.6; p < 0.0001) as well as decrease of tricuspid regurgitation (from grade 3.8 ± 0.4 to 0.9 ± 1; p < 0.0001). The ejection fraction of the right ventricle (RVEF) improved in 86 % of patients, in 14 % RVEF decreased or did not change (RVEF before operation 36 ± 10.5 %, after operation 42 ± 9.5 %, p = 0.001). In the long-term follow-up we found dysfunction of the tricuspid valve repair in 12 % and degeneration of the bioprosthesis in 15 %. Among 86 adult patients with Ebstein anomaly from our database the mortality was significantly higher in unoperated compared to operated patients (26 % vs 12 %, p = 0.006). Patients with cyanosis had high mortality regardless of surgery (40 % with and 83 % without operation). Reoperation after surgery in childhood or adulthood was necessary in 20 % of adults. CONCLUSION: The adult patients with Ebstein anomaly should be examined in a specialized center even if the symptoms are mild. The operation should be performed by a team with good results and experience in surgery and post-operative care in Ebstein anomaly. According to our knowledge Hospital Na Homolce represents such specialized center for Czech Republic.


Asunto(s)
Anomalía de Ebstein/cirugía , Adulto , Anciano , Procedimientos Quirúrgicos Cardíacos , República Checa , Anomalía de Ebstein/mortalidad , Femenino , Humanos , Masculino , Reoperación , Análisis de Supervivencia , Resultado del Tratamiento
15.
BMC Pulm Med ; 14: 45, 2014 Mar 15.
Artículo en Inglés | MEDLINE | ID: mdl-24629043

RESUMEN

BACKGROUND: Pulmonary arterial hypertension (PAH) is a severe and progressive disease characterized by increased pulmonary vascular resistance, ultimately leading to right heart failure and death. Epidemiological data from national registries are growing worldwide, but are still unavailable in Eastern Europe. METHODS: A PAH registry was initiated in January 2007 using a nationwide network of echocardiographic centers and four diagnostic centers that specialize in PAH. All patients aged above 18 years, diagnosed with PAH and monitored between January 2000 and December 2007 were included. Patients diagnosed with PAH between January and December 2007 were classified as incident. The survival analyses were performed up to the end of 2010. Prognostic factors at the time of diagnosis were identified using uni- and multivariable Cox proportional hazard models. RESULTS: Overall, 191 patients were included (100 prevalent cases, 91 incident cases). Patients were predominantly female (n = 125) and had a mean age of 51.9 ± 16.9 years. Incident patients were significantly older at the time of diagnosis than prevalent patients (p < 0.001). Most patients (60.7%) had idiopathic PAH; 20.4% had PAH associated with congenital heart disease and 11.4% had PAH associated with connective tissue disease. Estimates of prevalence and incidence of PAH in adults were 22.4 cases per million and 10.7 cases per million per year, respectively. The 1-, 2- and 3-year survival rates in the incident PAH cohort were 89% (95% confidence intervals [CI] 83-95%), 78% (95% CI 70-87%) and 74% (95% CI 65-83%), respectively. Lower survival rates were significantly associated with higher age (hazard ratio [HR] 6.6 95% CI 1.4-30.9) and lower creatinine clearance (HR 3.3 95% CI 1.1-9.7). CONCLUSION: This is the first study in Eastern Europe to describe the prevalence, incidence and survival of patients with PAH from a national representative registry. This registry from the Czech Republic highlights that diagnosis of PAH is frequently made late in the disease continuum when patients have significant functional impairment.


Asunto(s)
Hipertensión Pulmonar/epidemiología , Sistema de Registros , Adulto , Anciano , República Checa/epidemiología , Hipertensión Pulmonar Primaria Familiar , Femenino , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Prevalencia , Estudios Retrospectivos , Tasa de Supervivencia , Factores de Tiempo , Adulto Joven
16.
Can J Cardiol ; 28(4): 471-6, 2012.
Artículo en Inglés | MEDLINE | ID: mdl-22482396

RESUMEN

BACKGROUND: Elevated levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) are an expected finding in many adults with congenital heart disease (ACHD) but no reports have described the range of values within different diagnostic groups. METHODS: Between the years 2003 and 2011 we measured NT-proBNP in consecutive ACHD patients attending the outpatient department. Further NT-proBNP analysis was performed on 705 blood samples from 394 stable patients with the defect corrected. The results were compared among different diagnostic groups and with a control group of healthy volunteers. RESULTS: The median value of NT-proBNP in the whole cohort was signifinatly higher than in the control group (211 vs 42 pg/mL; P < 0.0001). The median value of NT-proBNP in pg/mL and the percentage of normal NT-proBNP values in the listed diagnostic groups were as follows: coarctation of the aorta (COA), 97 (64%); pulmonary stenosis (PS),160 (48%); secundum atrial septal defect (ASD), 254 (44%); incomplete atrioventricular septal defect (AVSD), 211 (32%); ventricular septal defect (VSD), 154 (58%); tetralogy of Fallot (TOF), 177 (38%); transposition of the great arteries (TGA) after Mustard, 237 (20%) or Senning correction, 143 (39%); Ebstein anomaly 287, (26%); pulmonary atresia (PA), 327 (18%); and complex defects after Fontan procedure, 184 (31%). CONCLUSIONS: Normal levels of NT-proBNP were found most often in patients after the correction of coarctation of the aorta and ventricular septal defect with the highest levels found in pulmonary atresia and Ebstein anomaly. Our results may serve as reference values for different ACHD groups.


Asunto(s)
Cardiopatías Congénitas/sangre , Cardiopatías Congénitas/cirugía , Péptido Natriurético Encefálico/sangre , Fragmentos de Péptidos/sangre , Adulto , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/diagnóstico , Humanos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/sangre , Valores de Referencia , Adulto Joven
17.
Cas Lek Cesk ; 150(4-5): 293-6, 2011.
Artículo en Checo | MEDLINE | ID: mdl-21634211

RESUMEN

BACKGROUND: Increasing number of adults with congenital heart disease (ACHD) require reoperation or primary repair. To define risk and outcome of operations of ACHD performed in adult cardiac surgery. METHODS: Between 2005-2010 we operated on 459 patients with ACHD, aged 40.4 +/- 15 years. Operations of ACHD represented 9.5% of 4854 cardiac operations in our department. RESULTS: The 30-days mortality was 1.1%, hospital mortality was 1.52%, long-term mortality among discharged patients was 1.3%. Reoperations after surgery in childhood represented 39%, repeated reoperations 9%, operations of 3 or 4 valves 2.6%. Robotic surgery or minithoracotomy was performed in 9%. Pulmonary hypertension was present in 11.3%, NYHA III-IV in 30%. The risk factors of mortality were: NYHA class III-IV (p < 0.001), cyanosis (p < 0.001), severe systolic dysfunction of left (p = 0.006) or right ventricle (p < 0.001).We did not confirm increased risk in older patients, women, pulmonary hypertension, reoperations and operations of 3-4 valves. CONCLUSIONS: Excellent surgical results among older adults with CHD were achieved by the co-operation of paediatric cardiac surgeon within the experienced centre for adult cardiac surgery. The highest risk was found in patients with NYHA class Ill-IV, cyanosis and ventricular dysfunction.


Asunto(s)
Cardiopatías Congénitas/cirugía , Adulto , Femenino , Cardiopatías Congénitas/mortalidad , Humanos , Masculino , Procedimientos Quirúrgicos Mínimamente Invasivos , Factores de Riesgo , Robótica , Tasa de Supervivencia
18.
Circ Arrhythm Electrophysiol ; 4(2): 235-41, 2011 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-21270102

RESUMEN

BACKGROUND: Catheter ablation of left-sided atrial arrhythmias generally is performed using a transfemoral venous approach through the inferior vena cava (IVC). In this report, we assessed the feasibility of a percutaneous transhepatic approach to ablation of left-sided atrial arrhythmias in 2 patients with interruption of the IVC. METHODS AND RESULTS: Patient 1 had atrial flutter in the setting of complex congenital heart disease and prior Fontan for univentricular physiology and a single atrium. Patient 2 had atrial fibrillation. Percutaneous hepatic vein access was obtained with ultrasound and fluoroscopic guidance. Transseptal catheterization was performed in patient 2. After the procedure, the hepatic tract in patient 1 was cauterized using a bipolar radiofrequency catheter, and an Amplatzer vascular plug was used in patient 2 to obtain hemostasis. Percutaneous hepatic vein access was achieved without complications. After electroanatomical mapping, a linear lesion was placed between the single atrioventricular valve and the confluence of the hepatic veins in patient 1; this terminated the flutter, and bidirectional block was achieved. In patient 2, the pulmonary veins were electrically isolated using an extraostial approach, isolating the ipsilateral veins in pairs. Additionally, ablation of right-side atrial flutter was achieved by obtaining bidirectional block across a linear lesion between the tricuspid valve and confluence of the hepatic veins. Hemostasis of the transhepatic tract was attained in both patients. CONCLUSIONS: In patients with interrupted IVCs, a percutaneous transhepatic approach is a feasible alternative for performing catheter ablation of complex left-sided arrhythmias.


Asunto(s)
Fibrilación Atrial/cirugía , Aleteo Atrial/cirugía , Ablación por Catéter/métodos , Cardiopatías Congénitas/complicaciones , Venas Hepáticas , Malformaciones Vasculares/complicaciones , Vena Cava Inferior/anomalías , Adulto , Fibrilación Atrial/complicaciones , Fibrilación Atrial/diagnóstico , Aleteo Atrial/complicaciones , Aleteo Atrial/diagnóstico , Técnicas Electrofisiológicas Cardíacas , Femenino , Técnicas Hemostáticas/instrumentación , Venas Hepáticas/diagnóstico por imagen , Humanos , Persona de Mediana Edad , Punciones , Radiografía Intervencional , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Ultrasonografía , Vena Cava Inferior/diagnóstico por imagen
19.
Eur J Pediatr ; 169(10): 1283-6, 2010 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-20396914

RESUMEN

Aortic dissection occurs in Turner syndrome with substantially higher frequency in comparison to the general population, and its prevention is one of the main aims of cardiologic follow-up. Findings of cystic medial necrosis in the aortic wall and a high prevalence of aortic dilation suggest that a form of aortopathy exists in Turner syndrome. However, little is known about natural development of aortic dilation prior to dissection. We present a 16-year-old girl with Turner syndrome with a bicuspid aortic valve, aortic stenosis, and dilation of ascending aorta, who underwent annual echocardiographic examinations from early childhood. Significant progressions of proximal aortic dilation occurred twice at the age of 10 and 15 years. Thereafter, another rapid progression was observed during 8 months and within 3 weeks preceding dissection. Acute aortic dissection was diagnosed while the girl was waiting for elective surgery. She was successfully operated. Frequent estimations of aortic diameter in Turner patients with abnormal findings may help to anticipate this life-threatening event. Additionally, we learned that rapid progression of aortic dilation should lead to immediate surgery to prevent more risky urgent intervention following the dissection.


Asunto(s)
Aneurisma Roto/etiología , Aneurisma de la Aorta/etiología , Disección Aórtica/etiología , Síndrome de Turner/complicaciones , Adolescente , Disección Aórtica/diagnóstico , Disección Aórtica/cirugía , Aneurisma Roto/diagnóstico , Aneurisma Roto/cirugía , Aneurisma de la Aorta/diagnóstico , Aneurisma de la Aorta/cirugía , Dilatación Patológica , Progresión de la Enfermedad , Ecocardiografía , Femenino , Humanos , Imagen por Resonancia Magnética
20.
Artículo en Inglés | MEDLINE | ID: mdl-16936915

RESUMEN

We investigated the performance of brain natriuretic peptides (BNP and NT-proBNP) in detecting various degrees of left ventricular systolic dysfunction. The NT-proBNP assay (Roche) and the BNP assay (Bayer Shionoria) were performed in 46 patients (mean age 50 years; range 20-79 years) with various types of heart disease (chronic heart failure due to coronary artery disease, cardiomyopathy, acquired valve disease, congenital heart diseases) and different impairment of left ventricular systolic dysfunction was assessed by echocardiography. Patients were divided into four groups according to the left ventricular ejection fraction (LVEF) correlated with clinical severity. Significant differences in medians of NT-proBNP and BNP values between all groups were determined (P= 0.0161 for NT-proBNP and P=0.0180 for BNP). For identifying patients with severe systolic dysfunction (LVEF<40%), receiver operating characteristic (ROC) analysis for both BNP and NT-proBNP was performed. The diagnostic performances expressed as areas under the curve were of 0.69 for NT-proBNP (cut off value 367 pg/ml) and 0.60 for BNP (cut off value 172 pg/ml). However, the BNP showed higher sensitivity (85 % vs. 63 %) and a higher positive predictive value (69 % vs 55 %) than the NT-proBNP. The negative predictive values of BNP and NT-proBNP were similar (70 % and 71 % respectively). Brain natriuretic peptides are promising markers for the diagnosis of severe left ventricular systolic dysfunction.


Asunto(s)
Péptido Natriurético Encefálico/sangre , Fragmentos de Péptidos/sangre , Disfunción Ventricular Izquierda/diagnóstico , Adulto , Anciano , Biomarcadores/sangre , Femenino , Humanos , Masculino , Persona de Mediana Edad , Volumen Sistólico , Disfunción Ventricular Izquierda/sangre , Disfunción Ventricular Izquierda/fisiopatología
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