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INTRODUCTION: Total anomalous pulmonary venous connection (TAPVC) has a low prenatal diagnostic rate. Therefore, we investigated whether Doppler waveforms with a low pulsatility in the pulmonary veins can indicate fetal TAPVC. METHODS: This retrospective study included 16 fetuses with TAPVC, including 10 with complex congenital heart disease and 104 healthy fetuses that underwent fetal echocardiography. Pulmonary venous S and D wave flow velocities and the valley (representing the lowest velocity between the S and D waves) were measured. Valley indices I and II were then calculated as (velocity of valley/greater of the S and D wave velocities) and (velocity of valley/lesser of the S and D wave velocities), respectively. RESULTS: Supra/infracardiac TAPVC cases exhibited significantly greater valley indices than that of the healthy group. After adjusting for gestational age at fetal echocardiography, valley indices I (odds ratio [OR] 7.26, p < 0.01) and II (OR: 9.23, p < 0.01) were significant predictors of supra/infracardiac TAPVC. Furthermore, valley indices I and II exhibited a high area under the curve for detecting supra/infracardiac TAPVC, regardless of the presence of pulmonary venous obstruction. CONCLUSION: The valley index may be a useful tool for the detection of fetal TAPVC.
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BACKGROUND: The determinants and prognostic value of albuminuria remain unclear in patients with adult congenital heart disease (ACHD), especially in those with Fontan circulation (FC). METHODS: We retrospectively reviewed 512 consecutive ACHD patients and investigated the determinants of urinary albumin-to-creatinine ratio (ACR) and albuminuria (MAU) and their association with all-cause mortality. Demographic data and laboratory and hemodynamic parameters were collected. Regression analysis and Cox proportional hazard models were used to identify the relationship between log ACR and variables, and clinical factors and all-cause mortality, respectively. RESULTS: Body mass index, aortic systolic blood pressure (ASP), arterial oxygen saturation (SaO2), glycated hemoglobin (HbA1c), B-type natriuretic peptide, and diuretic use were independently associated with log ACR. ASP, SaO2, and HbA1c were independently associated with MAU (P < .05-0.001). The prevalence of MAU was highest in unrepaired patients with low SaO2 (50%; P < .0001). Log ACR and MAU were associated with exercise capacity and all-cause mortality (P < .0001 for both) independent of renal function. Patients with ACHD, MAU, and renal dysfunction (n = 23) had the highest risk of all-cause mortality, while those without MAU or renal dysfunction had the lowest risk (P < .0001). These prognostic values remained significant in separate analyses of Fontan and biventricular circulation (P < .0001). CONCLUSIONS: ASP, SaO2, and HbA1c levels were independently associated with MAU in ACHD patients. MAU and log ACR were associated with all-cause mortality in patients with Fontan and biventricular circulation, independent of renal dysfunction.
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Cardiopatías Congénitas , Enfermedades Renales , Humanos , Adulto , Pronóstico , Factores de Riesgo , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/cirugía , Hemoglobina Glucada , Estudios Retrospectivos , Albuminuria/complicaciones , Albuminuria/epidemiologíaRESUMEN
BACKGROUND: Super-Fontan (SF) is an excellent phenotype of patients with Fontan circulation and normal exercise capacity. This study aimed to clarify the prevalence and clinical correlates and characteristics of SF. METHODS: We reviewed 404 Fontan patients who had undergone cardiopulmonary exercise testing, and the results were compared with clinical profiles. RESULTS: Seventy-seven (19%) patients had SF, and the postoperative prevalence at 5, 10, 15, 20, and ≥ 25 years was 16 (35%), 30 (39%), 18 (19%), 13 (14%), and 0 (0%), respectively. Compared with non-SF, SF patients were younger (P < .001) and were mostly men (P < .05). SF was characterized by a current high arterial blood pressure and oxygen saturation (SaO2), low systemic ventricle (SV) end-diastolic pressure, favorable body composition, superior pulmonary function, preserved hepatorenal and hemostatic functions, and better glucose tolerance (P < .05-.001). Pre-Fontan better SV function, low pulmonary artery resistance, and high SaO2 were associated with current SF (P < .05-.01). Furthermore, positive trajectory of exercise capacity and high daily activity during childhood were associated with current adult SF (P < .05). During the follow-up, 25 patients died, and 74 patients were unexpectedly hospitalized. There was no death in the SF group, and the rate of hospitalization was 67% lower than that of the non-SF group (P < .01-.001). CONCLUSIONS: The prevalence of SF gradually decreased over time. SF was characterized by preserved multi-end-organ function and an excellent prognosis. Pre-Fontan hemodynamics and post-Fontan childhood daily activity were associated with being adult SF.
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Procedimiento de Fontan , Cardiopatías Congénitas , Humanos , Prevalencia , Procedimiento de Fontan/métodos , Arteria Pulmonar , Hemodinámica , Ventrículos Cardíacos , Cardiopatías Congénitas/epidemiología , Cardiopatías Congénitas/cirugíaRESUMEN
The study aimed to investigate the long-term influence of atrial switch on post-Rastelli hemodynamic condition. Of 112 patients with transposition of the great arteries (TGA) or TGA-type double outlet right ventricle, ventricular septal defect (VSD), and pulmonary stenosis (PS) who underwent intra-cardiac repair between 1979 and 2018, 50 patients with levo-TGA underwent atrial switch and Rastelli as an anatomic repair and 62 patients with dextro-TGA underwent Rastelli. Postoperative outcomes were retrospectively compared. The median follow-up durations were 20.1 years (interquartile range: 4.3, 32.4) in the Rastelli group and 15.3 years (7.1, 23.0) in the atrial switch plus Rastelli group (p = 0.19). Sex, age, and weight at operation were similar in both groups. Overall survival rates at 30 years were 69.8% in the Rastelli group and 80.1% in the atrial switch plus Rastelli group (p = 0.18). The atrial switch plus Rastelli group required more frequent catheter interventions (p < 0.001), mainly for caval obstruction (n = 8) and atrial arrhythmia (n = 6). Medication was more frequent in the atrial switch plus Rastelli group (p = 0.009). Exercise capacity was similarly reduced in two groups. Protein-losing enteropathy (PLE) occurred in three long-term survivors in the atrial switch plus Rastelli group (p = 0.07). Concomitantly performed atrial switch operation did not affect long-term survival and exercise capacity after Rastelli procedure. However, the occurrence of PLE, a frequent need for medication, and catheter interventions after atrial switch plus Rastelli may result from atrial switch under the post-Rastelli condition.
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Operación de Switch Arterial , Fibrilación Atrial , Transposición de los Grandes Vasos , Humanos , Lactante , Transposición de los Grandes Vasos/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , HemodinámicaRESUMEN
BACKGROUND: We evaluated the significance of perinatal plasma natriuretic peptide (NP) levels in neonates with congenital heart defects (CHDs) or arrhythmias and determined whether measurement of perinatal plasma NP levels and echocardiographic assessment in utero could predict heart failure after birth. METHODS: The study was conducted between 2012 and 2016 to evaluate the correlation of perinatal atrial NP (ANP) and brain NP (BNP) levels at birth with the modified Ross score after birth and the cardiovascular profile (CVP) score before birth. RESULTS: A total of 122 singletons with CHDs or arrhythmias and 27 controls were analyzed. Neonatal blood sampling was performed at a median of 0.7 h (range, 0.1-1.5) after birth. The neonatal plasma ANP and BNP levels shortly after birth were significantly higher than those in the umbilical artery (UA) plasma. The ANP and BNP levels in UA and neonatal blood were correlated with the modified Ross score. The neonatal plasma ANP and BNP levels and the modified Ross scores were inversely correlated with the CVP score in neonates with CHDs or arrhythmias. The area under the receiver operating characteristic curve of UA ANP levels for predicting neonatal heart failure was highest among those for the CVP score, perinatal plasma ANP and BNP levels, and their combinations. CONCLUSIONS: The plasma ANP and BNP levels increased markedly shortly after birth. Assessment of the UA plasma ANP level at birth and the CVP score in utero may be utilized to predict neonatal heart failure.
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Cardiopatías Congénitas , Insuficiencia Cardíaca , Arritmias Cardíacas , Factor Natriurético Atrial , Femenino , Cardiopatías Congénitas/diagnóstico , Insuficiencia Cardíaca/diagnóstico , Humanos , Recién Nacido , Péptido Natriurético Encefálico , Péptidos Natriuréticos , Embarazo , VasodilatadoresRESUMEN
BACKGROUND: Pregnant women with a Fontan circulation have a high risk of obstetric complications, such as preterm delivery and small for gestational age (SGA), which may be affected by low blood flow to the placenta and hypoxia. This study investigated placental pathology in a Fontan circulation.MethodsâandâResults:Eighteen pregnancies in 11 women with a Fontan circulation were reviewed. Pregnancy outcomes showed 9 miscarriages and 9 live births, with 4 preterm deliveries. Five neonates were SGA (<5th percentile). Eight placentas from live births in 7 women were available for the study. Five placentas had low weight placenta for gestational age, and 7 grossly showed a chronic subchorionic hematoma. Histological examination revealed all placentas had some form of histological hypoxic lesions: maternal vascular malperfusion in 7, fetal vascular malperfusion in 1, and other hypoxia-related lesions in 8. Quantitative analyses, including immunohistochemistry (CD31, CD68, and hypoxia inducible factor-1α antibodies) and Masson's trichrome staining, were also performed and compared with 5 control placentas. Capillary density and the area of fibrosis were significantly greater in placentas from women with a Fontan circulation than in control placentas. CONCLUSIONS: Placentas in a Fontan circulation were characterized by a high frequency of low placental weight, chronic subchorionic hematoma, and constant histological hypoxic changes, which could reflect altered maternal cardiac conditions and lead to poor pregnancy outcomes.
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Procedimiento de Fontan , Niño , Femenino , Retardo del Crecimiento Fetal , Procedimiento de Fontan/efectos adversos , Hematoma , Humanos , Hipoxia/patología , Recién Nacido , Recién Nacido Pequeño para la Edad Gestacional , Placenta/irrigación sanguínea , Placenta/patología , EmbarazoRESUMEN
Heterograft and artificial materials have been used for extracardiac conduit implantation to create right ventricular (RV) to pulmonary artery (PA) continuity for biventricular repair in Japan because of the limited availability of homograft valves. However, few studies have examined morphological changes and number of candidates for transcatheter pulmonary valve implantation (TPVI) in which the conduit includes more than one type of material. Overall, 88 patients who underwent biventricular repair with an external conduit were included in this evaluation. Based on catheterization data and surgical records, we estimated morphological change in the RV outflow tract for each material and the number of candidates for Melody valve implantation based on premarket approval application criteria established by the U.S. Food and Drug Administration. There were 63 candidates for TPVI (72%, 63/88). Median anteroposterior and lateral diameter of the RV outflow tract was 20.4 mm (range 9.0-41.5) and 17.8 mm (range 9.5-34.9), respectively. Bovine pericardium tended to dilate by 11.2%. Polytetrafluoroethylene (ePTFE), homograft, and Dacron polyethylene terephthalate (PET) tended to become stenotic by 11.1%, 28.0%, and 13.4%, respectively. While ePTFE (27/33, 82%) and Dacron PET (2/2, 100%) were highly suitable for TPVI, bovine pericardium (32/48, 67%) was less suitable. In Japan, many patients with hemodynamic indications for TPVI following extracardiac conduit implantation to create RV to PA continuity may also meet the morphological indications.
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Implantación de Prótesis de Válvulas Cardíacas , Prótesis Valvulares Cardíacas , Válvula Pulmonar , Obstrucción del Flujo Ventricular Externo , Animales , Cateterismo Cardíaco , Bovinos , Xenoinjertos , Humanos , Tereftalatos Polietilenos , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/cirugía , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
The study aimed to assess the long-term outcomes after fenestration closure in patients at risk for Fontan failure. Of 119 patients who underwent Fontan operation between 1995 and 2004, fenestration was not created in 89 patients (NF group) and created in 30 patients with hypoplastic left heart syndrome, heterotaxy syndrome, high pulmonary arterial pressure, high systemic ventricular end-diastolic pressure, low ventricular ejection fraction, or atrioventricular valve regurgitation. All fenestrations were closed spontaneously or by catheter/surgical interventions, excepting two patients, and therefore, they were excluded. In fenestration group, patients with pre-Fontan mean pulmonary arterial pressure ≥ 15 mmHg or systemic atrioventricular valve regurgitation ≥ moderate were classified as high-risk Fontan candidates (F-HR group, n = 16), and the remaining patients were as standard-risk (F-SR group, n = 12). Protein-losing enteropathy-free survival rates did not differ among the three groups (p = 0.72). Serial follow-up catheter examinations after Fontan operation were completed in 69 patients in NF group and 11 patients in both F-SR and F-HR groups. Cardiac index and pulmonary vascular resistance significantly and similarly decreased over time in all groups, though the F-HR group showed lowest arterial oxygen saturation, lowest cardiac index, and highest pulmonary vascular resistance. The F-HR group also showed much veno-venous collaterals (p = 0.049), low peak oxygen consumption (p = 0.019), and low anaerobic threshold (p = 0.023) as compared to those in the F-SR group. In F-HR group, cyanosis remained after fenestration closure due to transformation from fenestration to veno-venous collaterals, which resulted in elevation of pulmonary vascular resistance, low cardiac index, and deterioration of exercise tolerance.
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Procedimiento de Fontan/métodos , Cardiopatías Congénitas/cirugía , Hemodinámica/fisiología , Cateterismo Cardíaco/métodos , Niño , Preescolar , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/fisiopatología , Ventrículos Cardíacos/fisiopatología , Humanos , Masculino , Factores de Tiempo , Resultado del TratamientoRESUMEN
Failed Fontan Patients with high cardiac output (CO) heart failure (HF) might have vasodilatory syndrome and markedly high mortality rates. The aim of this study was to review the clinical effects of vasoconstrictor therapy (VCT) for failed Fontan hemodynamics. We retrospectively reviewed 10 consecutive patients with Fontan failure (median age, 33 years) and high CO-HF who had received VCT. The hemodynamics were characterized by high central venous pressure (CVP: median, 16 mm Hg), low systolic blood pressure (median, 83 mm Hg), low systemic vascular resistance (median, 8.8 U·m2), high cardiac index (median, 4.6 L/min/m2), and low arterial oxygen saturation (median, 89%). VCT included intravenous noradrenaline infusion for five unstable patients, oral midodrine administration for nine stable patients, and both for four patients. After VCT introduction with a median interval of 1.7 months, the median systolic blood pressure (102 mm Hg, p = 0.004), arterial oxygen saturation (90%, p = 0.03), and systemic vascular resistance (12.1 U·m2, p = 0.13) increased without significant changes in CVP or cardiac index. After a median follow-up of 21 months, the number of readmissions per year decreased from 4 (1-11) to 1 (0-9) (p = 0.25), and there were no VCT-related complications; however, five patients (50%) developed hepatic encephalopathy, and six patients (60%) eventually died. VCT was safely introduced and could prevent the rapidly deteriorating Fontan hemodynamics. VCT could be an effective therapeutic strategy for failed Fontan patients with high CO-HF.
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Gasto Cardíaco Elevado/tratamiento farmacológico , Procedimiento de Fontan/efectos adversos , Insuficiencia Cardíaca/tratamiento farmacológico , Vasoconstrictores/uso terapéutico , Adulto , Gasto Cardíaco Elevado/etiología , Presión Venosa Central/efectos de los fármacos , Femenino , Cardiopatías Congénitas/cirugía , Insuficiencia Cardíaca/etiología , Humanos , Masculino , Estudios Retrospectivos , Resistencia Vascular/efectos de los fármacosRESUMEN
BACKGROUND: To clarify ventricular function in patients with asymptomatic coronary artery occlusion (ACAO) after Kawasaki disease (KD).MethodsâandâResults:We enrolled 65 patients with coronary artery lesions who had undergone cardiac magnetic resonance (CMR). Median age at CMR was 29 years. CMR was performed to evaluate only the transmural extent of late gadolinium enhancement (LGE) and ejection fraction (EF). Based on the depth of LGE, it was classified into 5 groups: 0% (G0), 1-25% (G1), 26-50% (G2), 51-75% (G3), and 76-100% (G4). We investigated the relationship of the degree of LGE and EF. Further, we also evaluated the EF among 3 groups [ACAO, myocardial infarction (MI), and noncoronary artery occlusion (Non-CO)]. The grade of LGE and the LVEF (mean±SD, %) were as follows: G0 (n=24, 52.6±4.8), G1 (n=13, 50.8±4.4), G2 (n=15, 49.1±5.6), G3 (n=9, 30.9±9.1), and G4 (n=9, 27.7±6.8). LVEF in patients with G3 and G4 was significantly low (P<0.05). LVEF (%) in patients with ACAO, MI, and Non-CO were 50.5±4.8 (n=38), 33.6±10.8 (n=17), and 53.0±5.7 (n=10), respectively. LVEF in the MI group was significantly low (P<0.0001). CONCLUSIONS: LGE >50% can lead to LV dysfunction. The transmural extent of LGE in most of the study patients with ACAO was ≤50% and they had subendocardial infarction, with preserved LV function.
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Oclusión Coronaria/diagnóstico por imagen , Imagen por Resonancia Cinemagnética , Síndrome Mucocutáneo Linfonodular/complicaciones , Infarto del Miocardio/diagnóstico por imagen , Volumen Sistólico , Disfunción Ventricular Izquierda/diagnóstico por imagen , Función Ventricular Izquierda , Adolescente , Adulto , Enfermedades Asintomáticas , Niño , Preescolar , Medios de Contraste/administración & dosificación , Oclusión Coronaria/etiología , Oclusión Coronaria/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Síndrome Mucocutáneo Linfonodular/diagnóstico , Infarto del Miocardio/etiología , Infarto del Miocardio/fisiopatología , Valor Predictivo de las Pruebas , Estudios Retrospectivos , Disfunción Ventricular Izquierda/etiología , Disfunción Ventricular Izquierda/fisiopatología , Remodelación Ventricular , Adulto JovenRESUMEN
BACKGROUND: Congenital heart disease (CHD) is often diagnosed prenatally using fetal echocardiography, but few studies have evaluated the accuracy of these fetal cardiac diagnoses in detail. We investigated the discrepancy between pre- and postnatal diagnoses of CHD and the impact of discrepant diagnoses.MethodsâandâResults:This retrospective study at a tertiary institution included data from the medical records of 207 neonates with prenatally diagnosed CHD admitted to the cardiac neonatal intensive care unit between January 2011 and December 2016. Pre- and postnatal diagnoses of CHD differed in 12% of neonates. Coarctation of the aorta and ventricular septal defects were the most frequent causes of discrepant diagnosis. Unexpected treatments were added to 38% of discrepant diagnostic cases. However, discrepant diagnoses did not adversely affect the clinical course. The 9% of the 207 neonates who required invasive intervention within 24 h of delivery were accurately diagnosed prenatally. CONCLUSIONS: Pre- and postnatal diagnoses differed in only a few neonates, with differences not adversely affecting the clinical course. Neonates who required invasive intervention immediately after delivery were accurately diagnosed prenatally. Prenatal diagnosis thus seems to contribute to improved prognosis in neonates with CHD.
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Cardiopatías Congénitas , Tamizaje Neonatal , Diagnóstico Prenatal , Ecocardiografía , Femenino , Cardiopatías Congénitas/diagnóstico por imagen , Humanos , Recién Nacido , Japón/epidemiología , Embarazo , Estudios RetrospectivosRESUMEN
BACKGROUND: Fontan patients exhibit a high prevalence of abnormal glucose metabolism (AGM). We aimed to characterize AGM and clarify its association with Fontan pathophysiology. METHODS: We prospectively evaluated AGM with plasma glucose dynamics [mg/dL; fasting glucose (FPG), and maximum glucose increase (PG-spike)] during oral glucose tolerance test and hemoglobin A1c (HbA1c) in 276 consecutive Fontan patients (aged 19⯱â¯7â¯years). Of these, 176 patients had serial AGM assessments with a mean interval of 6.5â¯years. RESULTS: Initial analysis revealed a high prevalence of impaired glucose tolerance (38.4%) and diabetes mellitus (DM) (4.7%), and positive family history, high HbA1c, and high central venous pressure independently predicted presence of DM. HbA1c was independently determined by hypersplenism and presence of DM (Pâ¯<â¯.05). Serial assessments revealed an increased PG-spike and a decreased HbA1c (Pâ¯<â¯.001 for both). Prevalence of DM increased (6.3% to 10.3%), and positive family history, high liver enzymes, and AGM predicted new onset of DM (Pâ¯<â¯.05 for all). Twenty-one patients died during 7.1-year follow-up. FPG (Pâ¯<â¯.01) and PG-spike (Pâ¯<â¯.05) independently predicted all-cause mortality. Particularly, patients with FPGâ¯≤â¯74 and/or PG-spike ≥85 had a mortality rate 8.7 times higher than those without (Pâ¯=â¯.0129). CONCLUSIONS: AGM progressed even in young adult Fontan patients, and HbA1c showed limited predictive value for progression. Oral glucose tolerance test plays important roles in uncovering unique Fontan AGM as well as predicting all-cause mortality.
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Diabetes Mellitus/metabolismo , Ayuno/sangre , Procedimiento de Fontan , Glucosa/metabolismo , Hemoglobina Glucada/metabolismo , Adolescente , Adulto , Glucemia/metabolismo , Estudios de Casos y Controles , Causas de Muerte , Niño , Estudios Transversales , Diabetes Mellitus/epidemiología , Diabetes Mellitus/mortalidad , Progresión de la Enfermedad , Femenino , Prueba de Tolerancia a la Glucosa , Cardiopatías Congénitas/metabolismo , Humanos , Hiperesplenismo/metabolismo , Hepatopatías/metabolismo , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Prevalencia , Estudios Prospectivos , Adulto JovenRESUMEN
OBJECTIVE: We attempted to evaluate fetal cardiac activity of congenital heart disease (CHD) and normal heart fetuses by magnetic resonance imaging (MRI). METHODS: We evaluated the fetal cardiac functional assessment and the blood flow of descending aorta at 34 weeks' gestation or more by MRI and compared the results with ultrasonography findings. We measured 6 normal heart fetuses and 14 CHD fetuses. RESULTS: The ejection fraction (EF) and descending aorta blood flow in the CHD group were 76.4% ± 11.9%, 687.5 ± 303.8 mL/min by ultrasonography and 48.3% ± 7.1%, 711.4 ± 273.1 mL/min by MRI; those in the normal group were 66.9% ± 12.2%, 898.1 ± 245.9 mL/min by ultrasonography and 51.3% ± 4.2%, 911.9 ± 223.1 mL/min by MRI. CONCLUSIONS: There was no significant difference in descending aorta blood flow measurements between ultrasonography and MRI, but the EF showed a significant between-group difference. In the normal group, the variation of EF and blood flow measured by MRI was significantly smaller compared with those of ultrasonography.
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Corazón Fetal/diagnóstico por imagen , Cardiopatías Congénitas/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Diagnóstico Prenatal/métodos , Adulto , Femenino , Corazón Fetal/embriología , Corazón Fetal/fisiopatología , Cardiopatías Congénitas/embriología , Cardiopatías Congénitas/fisiopatología , Humanos , Japón , Embarazo , Estudios ProspectivosRESUMEN
AIM: To predict the prognosis of infants with congenital heart disease, accurate prenatal diagnosis of structural abnormality and heart failure are both necessary. The aim of this study was to investigate whether cardiovascular profile (CVP) and biophysical profile (BP) scores are useful for predicting prognosis in infants with congenital heart defect (CHD). METHODS: A retrospective review of singletons prenatally diagnosed with CHD at a tertiary pediatric cardiac center between 2011 and 2015 was undertaken. RESULTS: A total of 202 patients with CHD were analyzed. Perinatal and infant deaths occurred in 16 (7.9%) and 10 cases (5.0%), respectively. Infants with the last CVP score ≤ 5 had 18.7-fold higher perinatal mortality than those with a last CVP score > 5 (P < 0.01). Infants with a last BP score ≤ 6 had 18.7-fold higher perinatal mortality than those with a last BP score > 6 (P < 0.01). Infants with a CVP score decrease in utero had 4.5-fold higher infant mortality than those with an increase or no change (P < 0.01). Multivariate analysis showed that single-ventricle physiology, pre-term birth at <37 weeks of gestation, last CVP score ≤ 5, and last BP score ≤ 6 were independent predictors of perinatal mortality. Single-ventricle physiology and a CVP score decrease were independent predictors of infant mortality. CONCLUSION: CVP and BP scores are useful for predicting perinatal prognosis in infants with CHD. A CVP score decrease in utero is associated with infant mortality, suggesting that serial CVP score assessment may be useful for management planning.
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Sistema Cardiovascular/embriología , Corazón Fetal/diagnóstico por imagen , Cardiopatías Congénitas/mortalidad , Diagnóstico Prenatal/estadística & datos numéricos , Índice de Severidad de la Enfermedad , Femenino , Corazón Fetal/embriología , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/embriología , Humanos , Lactante , Mortalidad Infantil , Recién Nacido , Masculino , Diagnóstico Prenatal/métodos , Pronóstico , Estudios Retrospectivos , Medición de Riesgo/métodosRESUMEN
The authors have retracted this article [1] because, contrary to the statement in the article, this research did not obtain ethics approval from the National Cerebral and Cardiovascular Center Institutional Review Board prior to submission of the manuscript to Pediatric Cardiology. All authors agree to this retraction.
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The effect of pregnancy on aortic root in women with repaired conotruncal anomalies (CTA) has not been clarified. This study examined aortic diameters during and after pregnancy in women with repaired CTA. A retrospective review of consecutive pregnant women with repaired CTA was performed for results of echocardiography from 1 year before pregnancy to 3 years after delivery and compared with findings from healthy pregnant volunteers. Participants comprised 42 subjects and 49 deliveries with repaired CTA (CTA group), and 47 healthy pregnant women (control group). Although no maternal aortic events were encountered, aortic diameters during pregnancy increased by 1.0 ± 2.2 mm (maximum, 7.0 mm) in the CTA group and 0.6 ± 1.3 mm (maximum, 3.4 mm) in the control group (p = 0.13). The CTA subgroup with increase in aortic diameter ≥ 3.5 mm during pregnancy showed no reversion to baseline diameter at follow-up > 6 months after delivery. Significant risk factors for increased aortic diameter and no reversal included pulmonary atresia, history of aortopulmonary shunt, older age at repair, and smaller left ventricular end-diastolic diameter pre-pregnancy. Women with repaired CTA tolerated pregnancy and delivery well. However, the aortic root progressively dilated during pregnancy and 1/4 of them, especially those with longstanding high aortic blood flow before repair, showed an increase of aortic root size of ≥ 3.5 mm during the peripartum period; this dilatation tended not to revert to the pre-pregnancy diameter. Therefore, serial measurement of the aortic root during pregnancy and after delivery is recommended in these women.
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Aorta/patología , Válvula Aórtica/patología , Seno Aórtico/patología , Adulto , Factores de Edad , Aorta/anomalías , Aorta/diagnóstico por imagen , Válvula Aórtica/anomalías , Válvula Aórtica/diagnóstico por imagen , Estudios de Casos y Controles , Angiografía por Tomografía Computarizada , Parto Obstétrico/efectos adversos , Dilatación Patológica/etiología , Ecocardiografía , Femenino , Cardiopatías Congénitas/cirugía , Humanos , Imagenología Tridimensional , Imagen por Resonancia Magnética , Masculino , Embarazo , Atresia Pulmonar/etiología , Estudios Retrospectivos , Factores de Riesgo , Seno Aórtico/diagnóstico por imagen , Factores de TiempoRESUMEN
The frequency of newborns with congenital heart disease (CHD) is approximately 1% in the general population; however, the recurrence rate of CHD in mothers with CHD differs in ethnicity and reports. We therefore aimed to determine the prevalence of CHD among neonates born to mothers with CHD in our institute in Japan. We reviewed the medical charts of 803 neonates delivered by 529 women with CHD at the National Cerebral and Cardiovascular Center from 1982 to 2016. They included isolated ventricular septal defect (VSD,31.4%), isolated atrial septal defect (ASD, 23.3%), tetralogy of Fallot (TOF,10.6%). We defined CHD in neonates as being diagnosed within 1 month of birth. We estimated that the average rate of the CHD recurrence was 3.1%. The recurrence ratios in each maternal CHD were 8.6%, 7.1%, 6.2%, 4.8%, 3.6%, and 1.5% for PS, CoA, TOF, atrioventricular septal defect, VSD, and ASD, respectively. The rate of CHD in offsprings whose mothers have CHD was 3 times greater than that of mothers with healthy hearts. Almost half of neonates with CHD had the same phenotype as their mother in our series. Especially, PS and CoA were closely related to the type of maternal CHD.
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Cardiopatías Congénitas/epidemiología , Adolescente , Adulto , Niño , Femenino , Cardiopatías Congénitas/etiología , Humanos , Recién Nacido , Japón , Masculino , Persona de Mediana Edad , Madres/estadística & datos numéricos , Prevalencia , Recurrencia , Estudios Retrospectivos , Adulto JovenRESUMEN
Severe valvulitis owing to acute Kawasaki disease leading to severe mitral regurgitation is a rare event in infants. Further, there is less information about underlying ruptured mitral chordae tendineae causing severe mitral regurgitation. We encountered ruptured mitral chordae tendineae in three female patients after Kawasaki disease. The age at the onset of Kawasaki disease ranged from 3 to 8 months, and detection of ruptured mitral chordae tendineae was from 24 to 90 days. Two patients had acute heart failure, and one was asymptomatic. One patient underwent mitral annuloplasty, and the others responded to medication. These ruptured mitral chordae tendineae occurred after the remission of the initial acute Kawasaki disease, in the early course and the convalescent of acute Kawasaki disease. Further, the recurrent fever was also detected in them. The ruptured mitral chordae tendineae in an infant within 6 months can be detected by systolic heart murmur around the convalescent stage of acute Kawasaki disease, although the prevalence is very low.
Asunto(s)
Insuficiencia Cardíaca/etiología , Enfermedades de las Válvulas Cardíacas/etiología , Insuficiencia de la Válvula Mitral/etiología , Válvula Mitral/cirugía , Síndrome Mucocutáneo Linfonodular/complicaciones , Enfermedad Aguda , Cuerdas Tendinosas , Femenino , Humanos , Lactante , Japón , Anuloplastia de la Válvula MitralRESUMEN
BACKGROUND: Heart failure (HF) is an important complication in adults with congenital heart disease (CHD), but because only a few studies have focused on acute HF hospitalization in adults with CHD, we study aimed to define the clinical characteristics of such patients and examine the differences in acute HF between adults with CHD and acquired heart disease.MethodsâandâResults:We retrospectively evaluated 50 adults with CHD admitted for treatment of acute HF and compared their data with those from Japanese HF registries. Patient mean age was 37±15 years and 58% were male. In total, 86% of the patients had complex forms of CHD and 62% had undergone corrective surgery, including the Fontan procedure; 66% of patients showed right heart hemodynamic abnormality. In-hospital mortality was 4%, which was comparable to the Japanese HF registries. Survival rate was 93% at 1 year and 75% at 3 years, which was similarly poor to the rates of HF secondary to acquired heart disease. CONCLUSIONS: We clarified the clinical characteristics of adults with CHD requiring HF hospitalization. Young adults with complex CHD were hospitalized for management of acute right HF. Short-term and mid-term outcomes were similarly poor compared with acute HF secondary to acquired heart disease.
Asunto(s)
Cardiopatías Congénitas/complicaciones , Insuficiencia Cardíaca/etiología , Hospitalización , Adulto , Femenino , Procedimiento de Fontan , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/cirugía , Mortalidad Hospitalaria , Humanos , Japón , Masculino , Persona de Mediana Edad , Sistema de Registros , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: We have previously demonstrated that umbilical cord plasma natriuretic peptide (NP) levels reflect the severity of heart failure (HF) in fetuses with congenital heart defects (CHD). The aim of this study was to evaluate the significance of amniotic fluid (AF) NP levels in the assessment of HF in fetuses with CHD or arrhythmia. MethodsâandâResults: This was a prospective observational study at a tertiary pediatric cardiac center. A total of 95 singletons with CHD or arrhythmia, and 96 controls from 2012 to 2015 were analyzed. AF concentrations of atrial NP (ANP), B-type NP (BNP) and N-terminal pro-B-type NP (NT-proBNP) at birth were compared with ultrasonographic assessment of fetal HF using the cardiovascular profile (CVP) score. Multivariate analysis showed that a CVP score ≤5 and preterm birth are independently associated with high AF NT-proBNP levels. AF NT-proBNP levels of fetuses with CHD or arrhythmia inversely correlated with CVP score (P for trend <0.01). In contrast, AF concentrations of ANP and BNP were extremely low, and it was difficult to assess the degree of fetal HF based on them. CONCLUSIONS: AF NT-proBNP concentrations increase in stepwise fashion with the severity of HF in fetuses with CHD or arrhythmia; it was the optimal NP for assessing the fetal HF.