Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 567
Filtrar
1.
Medicine (Baltimore) ; 98(42): e17492, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31626103

RESUMO

Fetal pulmonary atresia with intact ventricular septum (PA/IVS) is a rare congenital heart disease. The present study aimed to classify PA/IVS and determine the relationship between prenatal echocardiographic characteristics and postnatal biventricular or univentricular repair strategies.A total of 51 fetuses with PA/IVS were examined from 2012 to 2019. Data on prenatal echocardiography, associated anomaly, karyotype, and outcome were collected. Two-dimensional measurements included tricuspid valve (TV) z-score, mitral valve (MV) z-score, TV/MV ratio, and ratio of right to left ventricle (RV/LV) length, whereas color Doppler measurements included degree of tricuspid regurgitation (TR), ventriculo-coronary artery communication (VCAC), tricuspid inflow duration (TID), cardiac cycle duration (CCD), middle cerebral artery pulsatility index (MCA PI), and umbilical artery pulsatility index (UA PI). Diagnostic classification was based on the development of RV and the presence or absence of VCAC. Postnatal evaluation was divided according biventricular or univentricular repair.Of the 51 fetuses with PA/IVS, 20 were type I, 17 were type II, and 14 were type III. Only one fetus exhibited right aortic arch. The karyotype of all the fetuses was normal. Of the 28 patients who underwent postnatal surgery, 13 (46%) underwent biventricular repair and 15 (54%) underwent univentricular repair. TV z-score was significantly higher for the biventricular repair group compared with univentricular repair group (-1.20 ±â€Š0.98 vs -4.33 ±â€Š0.80, P = .000). TV/MV, RV/LV length, and TID/CCD were significantly higher for the biventricular repair group than the univentricular repair group (0.81 ±â€Š0.14 vs 0.54 ±â€Š0.09, 0.71 ±â€Š0.11 vs 0.49 ±â€Š0.09, 39.20 ±â€Š3.84 vs 29.16 ±â€Š4.58, P = .000). Moderate or severe TR and VCAC were significantly different between the 2 groups (P = .000). Gestational age, MCA PI, and UA PI did not differ between the 2 groups (P = .72, P = .36, P = .06). The cutoff values for the biventricular repair characteristic curves were TV z-score >-3.28, TV/MV ratio >0.71, RV/LV length >0.62, and TID/CCD >33.95%. The sensitivities of the TV z-score, TV/MV, RV/LV length, and TID/CCD were 100%, 77%, 85%, and 92%, respectively. The specificities of the TV z-score, TV/MV, RV/LV length, and TID/CCD were 94%, 100%, 100%, and 94%, respectively.Fetal echocardiography was able to classify PA/IVS according to variable degree of RV and VCAC. In fetal PA/IVS, TV z-score >-3.28, TV/MV >0.71, RV/LV length >0.62, TID/CCD >33.95%, moderate and severe TR, and the absence of VCAC were associated with postnatal biventricular repair strategy. These findings may have implications for prenatal counseling and prediction of fetal outcome.


Assuntos
Ecocardiografia/estatística & dados numéricos , Coração Fetal/diagnóstico por imagem , Cardiopatias Congênitas/classificação , Cardiopatias Congênitas/diagnóstico por imagem , Atresia Pulmonar/classificação , Atresia Pulmonar/diagnóstico por imagem , Ultrassonografia Pré-Natal/estatística & dados numéricos , Adulto , Ecocardiografia/métodos , Feminino , Idade Gestacional , Cardiopatias Congênitas/embriologia , Humanos , Valor Preditivo dos Testes , Gravidez , Prognóstico , Atresia Pulmonar/embriologia , Valores de Referência , Sensibilidade e Especificidade , Ultrassonografia Pré-Natal/métodos , Adulto Jovem
2.
Cardiol Young ; 29(5): 689-694, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31133083

RESUMO

OBJECTIVES: The aim of the study was to report the outcome of cardiac catheterisation in low-weight patients. BACKGROUND: Data regarding cardiac catheterisation in infants weighing <2500 g are scarce. METHODS: We reviewed all cardiac catheterisations performed in infants weighing <2500 g between January 2000 and May 2016. An analysis with respect to the type of procedure, the complexity of procedure (procedure type risk), and haemodynamic vulnerability index was finally carried out. We report the occurrence of deaths and complications using the adverse event severity score. RESULTS: A total of 218 procedures were performed on 211 patients. The mean age and weight were, respectively, 15 ± 26 days (range, 0-152) and 2111 ± 338 g (range, 1000-2500). Procedures were interventional and diagnostic, respectively, in 174 (80%) and 44 (20%) patients. Out of 218, 205 (94%) were successful. Eleven complications (5%) occurred - six with an adverse event severity score of 4 and five with an adverse event severity score of 3. Ten patients (91%) showed a favourable outcome, and one died (stent thrombosis few hours after patent ductus arteriosus stenting). No correlation was found between lower weight and occurrence of death (p = 0.68) or complications (p = 0.23). The gravity scores (procedure type risk and haemodynamic vulnerability index) were not predictive of complications. CONCLUSIONS: Cardiac catheterisation in infants weighing <2500 g appears feasible and effective with low risk. The weight should not discourage from performing cardiac catheterisation in this population.


Assuntos
Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/métodos , Cardiopatias Congênitas/terapia , Recém-Nascido de Baixo Peso , Cateterismo Cardíaco/mortalidade , Feminino , França/epidemiologia , Cardiopatias Congênitas/classificação , Hemodinâmica , Humanos , Lactente , Recém-Nascido , Masculino , Morbidade , Estudos Retrospectivos , Resultado do Tratamento
3.
Am J Cardiol ; 123(11): 1868-1873, 2019 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-30954207

RESUMO

We aimed to compare New York Heart Association (NYHA) functional class in adult congenital heart disease (ACHD) patients with objectively measured cardiopulmonary exercise testing (CPET) parameters. This study included retrospective review of ACHD patients who underwent a CPET between August 2014 and April 2018 at our center. Patients were grouped according to severity of CHD, and NYHA class as recorded in their medical record or estimated from the clinical narrative. A total of 175 ACHD patients (mean age 30 ± 11 years) with NYHA class I-III enrolled in the study. The NYHA functional class was II or III in most complex CHD. There was a strong inverse relation between NYHA class and peak oxygen consumption, oxygen uptake efficiency slope, and the double product at peak exercise (product of heart rate and systolic blood pressure) (p<0.0001). There was no relation between NYHA class and ventilation efficiency slope (p = 0.37). In conclusion, NYHA functional class correlates with objective measures of CPET, however there is wide variability in measured exercise capacity in each NYHA classification. Therefore, whereas NYHA class of patients is a simple measure for assessment of functional status, CPET is an important tool to identify the source of exercise limitation in ACHD patients.


Assuntos
Teste de Esforço , Cardiopatias Congênitas/fisiopatologia , Adulto , Feminino , Cardiopatias Congênitas/classificação , Humanos , Masculino , Estudos Retrospectivos , Adulto Jovem
4.
Arch Cardiovasc Dis ; 112(1): 31-43, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30612895

RESUMO

BACKGROUND: The content of electronic medical records (EMRs) encompasses both structured data, such as billing codes, and unstructured data, including free-text reports. Epidemiological and clinical research into adult congenital heart disease (ACHD) increasingly relies on administrative claim data using the International Classification of Diseases (9th revision) (ICD-9). In France, administrative databases use ICD-10, the reliability of which is largely unknown in this context. AIMS: To assess the accuracy of ICD-10 codes retrieved from administrative claim data in the identification and classification of ACHD. METHODS: We randomly included 6000 patients hospitalized at least once in 2000-2014 in a cardiology department with a dedicated specialized ACHD Unit. For each patient, the clinical diagnosis extracted from the EMR was compared with the assigned ICD-10 codes. Performance of ICD-10 codes in the identification and classification of ACHD was assessed by estimating sensitivity, specificity and positive predictive value. RESULTS: Among the 6000 patients included, 780 (13%) patients with ACHD were manually identified from EMRs (107,092 documents). ICD-10 codes correctly categorized 629 as having ACHD (sensitivity 0.81, 95% confidence interval 0.78-0.83), with a specificity of 0.99 (95% confidence interval 0.99-1). The performance of ICD-10 codes in correctly categorizing the ACHD defect subtype depended on the defect, with sensitivity ranging from 0 (e.g. unspecified congenital malformation of tricuspid valve) to 1 (e.g. common arterial trunk), and specificity ranging from 0.99 to 1. CONCLUSIONS: Administrative data using ICD-10 codes is a precise tool for detecting ACHD, and may be used to establish a national cohort. Mining free-text reports in addition to coded administrative data may offset the lack of sensitivity and accuracy when describing the spectrum of congenital heart disease using ICD-10 codes.


Assuntos
Demandas Administrativas em Assistência à Saúde , Confiabilidade dos Dados , Mineração de Dados/métodos , Registros Eletrônicos de Saúde , Cardiopatias Congênitas/classificação , Cardiopatias Congênitas/diagnóstico , Classificação Internacional de Doenças , Adulto , Bases de Dados Factuais , Cardiopatias Congênitas/epidemiologia , Humanos , Paris/epidemiologia , Admissão do Paciente , Prevalência , Reprodutibilidade dos Testes , Terminologia como Assunto
5.
Cardiovasc Pathol ; 39: 38-50, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30623879

RESUMO

The traditional classification of congenital aortic arch abnormalities was described by James Stewart and colleagues in 1964. Since that time, advances in diagnostic imaging technology have led to better delineation of the vasculature anatomy and the identification of previously unrecognized and unclassified anomalies. In this manuscript, we review the existing literature and propose a series of modifications to the original Stewart classification of congenital aortic arch abnormalities to incorporate this new knowledge. In brief, we propose the following modifications: (1) In Group I, we further divide subgroup B into left arch atretic and right arch atretic; (2) In Group II, we add three more subgroups, including aberrant right innominate artery, "isolated" right innominate artery (RIA), "isolated" right carotid artery with aberrant right subclavian artery; (3) In Groups I, II, and III, we add a subgroup of absence of both ductus arteriosus; and (4) In Group IV, we add three subgroups, including circumflex retro-esophageal aorta arch, persistent V aortic arch, and anomalous origin of pulmonary artery from ascending aorta.


Assuntos
Aorta Torácica/anormalidades , Cardiopatias Congênitas/classificação , Terminologia como Assunto , Malformações Vasculares/classificação , Aorta Torácica/diagnóstico por imagem , Tomada de Decisão Clínica , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/terapia , Humanos , Valor Preditivo dos Testes , Prognóstico , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/terapia
8.
An. pediatr. (2003. Ed. impr.) ; 89(5): 294-301, nov. 2018. tab, graf
Artigo em Espanhol | IBECS | ID: ibc-177119

RESUMO

Introducción y objetivos: Las cardiopatías congénitas (CC) son las malformaciones congénitas más frecuentes. En España no hay datos de su incidencia a nivel nacional. El objetivo del estudio es analizar la incidencia y evolución de las CC en España. Métodos: Estudio observacional retrospectivo utilizando el conjunto mínimo básico de datos durante 10 años (2003-2012), en menores de un año, seleccionando las altas hospitalarias con códigos de CC (Clasificación Internacional de Enfermedades, 9.a revisión, modificación clínica). Se describió la evolución anual de la incidencia acumulada y su distribución geográfica, y se analizó mediante riesgos relativos de incidencia y razón de incidencias estandarizadas por comunidad autónoma. Resultados: Durante el periodo analizado 64.831 menores de un año fueron diagnosticados de CC al alta hospitalaria sobre 4.766.325 nacimientos con una incidencia del 13,6‰. La incidencia excluyendo la comunicación interauricular fue del 7,29‰. Los códigos más frecuentes fueron: comunicación interauricular (6,31‰), comunicación interventricular (3,48‰), ductus arteriosus persistente (2,71‰), coartación de aorta (0,55‰), estenosis pulmonar (0,50‰), trasposición de grandes vasos (0,49‰), canal auriculoventricular (0,45‰) y tetralogía de Fallot (0,41‰). La distribución geográfica de las cardiopatías graves y muy graves no resultó uniforme presentando Castilla y León junto con Extremadura la mayor incidencia, y Madrid y Cantabria la menor. Conclusiones: Durante el tiempo de estudio se observa un aumento de cardiopatías leves que puede estar influido por la mejora de las técnicas diagnósticas, el uso extendido de la ecocardiografía y la propia codificación Clasificación Internacional de Enfermedades, 9.a revisión, modificación clínica y una disminución de las cardiopatías muy graves cuyo análisis presenta mayor validez al estar menos influido por factores externos. La incidencia de cardiopatías graves y muy graves no fue uniforme en España


Introduction and objectives: Congenital heart disease (CHD) represents the most common congenital malformation. The objective of this study was to analyse the incidence of CHD in Spain, and it is the first nationwide study so far. Methods: A retrospective observational study was performed in order to evaluate the incidence of CHD in Spain. The administrative database (minimum basic data set) from 2003 to 2012 was analysed in children less than one year old admitted to hospital with codes of CHD (International Classification of Diseases, 9 th Revision, clinical modification). Cumulative incidence, Incidence relative risk, and standardised incidence ratio were calculated to study geographic variations. Results: There were 64,831 infants with CHD among the 4,766,325 births analysed during the period studied, with an incidence of 13.6‰. The incidence excluding atrial septal defect was 7.29 ‰.The most frequent CHD were atrial septal defect (6.31‰), ventricular septal defect (3.48‰), patent ductus arteriosus (2.71‰), coarctation of the aorta (0.55‰), pulmonary stenosis (0.50‰), transposition of the great arteries (0.49‰), atrioventricular septal defect (0.45‰), and tetralogy of Fallot (0.41‰). Castilla and Leon, together with Extremadura, showed the highest risks for severe and very severe CHD, while Madrid and Cantabria showed the lowest. Conclusions: An increase of mild CHD was observed during the period analysed. This could have been influenced by improvements in diagnostic techniques, extended use of echocardiography, and the International Classification of Diseases, 9 th Revision, clinical modification coding system, and to a decrease in very severe CHD, which is less influenced by external factors. Significant geographical differences were found in the incidence of severe and very severe CHD


Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/classificação , Espanha/epidemiologia , Estudo Observacional , Estudos Retrospectivos , Estenose da Valva Pulmonar/complicações , Ecocardiografia , Classificação Internacional de Doenças
9.
Comput Methods Programs Biomed ; 165: 107-116, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30337065

RESUMO

BACKGROUND: Qualitative features of aortic and mitral valvar pathology have traditionally been used to classify congenital cardiac anomalies for which the left heart structures are unable to sustain adequate systemic cardiac output. We aimed to determine if novel groups of patients with greater clinical relevance could be defined within this population of patients with critical left heart obstruction (CLHO) using a data-driven approach based on both qualitative and quantitative echocardiographic measures. METHODS: An independent standardized review of recordings from pre-intervention transthoracic echocardiograms for 651 neonates with CLHO was performed. An unsupervised cluster analysis, incorporating 136 echocardiographic measures, was used to group patients with similar characteristics. Key measures differentiating the groups were then identified. RESULTS: Based on all measures, cluster analysis linked the 651 neonates into groups of 215 (Group 1), 338 (Group 2), and 98 (Group 3) patients. Aortic valve atresia and left ventricular (LV) end diastolic volume were identified as significant variables differentiating the groups. The median LV end diastolic area was 1.35, 0.69, and 2.47 cm2 in Groups 1, 2, and 3, respectively (p < 0.0001). Aortic atresia was present in 11% (24/215), 87% (294/338), and 8% (8/98), in Groups 1, 2, and 3, respectively (p < 0.0001). Balloon aortic valvotomy was the first intervention for 9% (19/215), 2% (6/338), and 61% (60/98), respectively (p < 0.0001). For those with an initial operation, single ventricle palliation was performed in 90% (176/215), 98% (326/338), and 58% (22/38) (p < 0.0001). Overall mortality in each group was 27% (59/215), 41% (138/338), and 12% (12/98) (p < 0.0001). CONCLUSIONS: Using a data-driven approach, we conceptualized three distinct patient groups, primarily based quantitatively on baseline LV size and qualitatively by the presence of aortic valve atresia. Management strategy and overall mortality differed significantly by group. These groups roughly correspond anatomically and are analogous to multi-level LV hypoplasia, hypoplastic left heart syndrome, and critical aortic stenosis, respectively. Our analysis suggests that quantitative and qualitative assessment of left heart structures, particularly LV size and type of aortic valve pathology, may yield conceptually more internally consistent groups than a simplistic scheme limited to valvar pathology alone.


Assuntos
Diagnóstico por Computador/métodos , Cardiopatias Congênitas/classificação , Cardiopatias Congênitas/diagnóstico por imagem , Valva Aórtica/anormalidades , Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/classificação , Estenose da Valva Aórtica/congênito , Estenose da Valva Aórtica/diagnóstico por imagem , Análise por Conglomerados , Estudos de Coortes , Diagnóstico por Computador/estatística & dados numéricos , Ecocardiografia , Feminino , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/diagnóstico por imagem , Humanos , Síndrome do Coração Esquerdo Hipoplásico/classificação , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Recém-Nascido , Masculino , Modelos Cardiovasculares , Estudos Prospectivos , Aprendizado de Máquina não Supervisionado , Disfunção Ventricular Esquerda/classificação , Disfunção Ventricular Esquerda/diagnóstico por imagem
10.
J Pediatr ; 202: 63-69, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30243535

RESUMO

OBJECTIVE: To test a model to predict psychosocial quality of life (QOL) in children with congenital heart disease (CHD) via executive dysfunction. STUDY DESIGN: Parents of 91 children with CHD requiring surgery in the first year of life completed questionnaires by mail or as part of their cardiology clinic visit. Latent class analysis identified 2 groups of patients with different likelihoods of executive dysfunction. Select medical and demographic characteristics and executive dysfunction group membership were evaluated as predictors of QOL using structural equation modeling. RESULTS: In children with CHD, aortic obstruction, male sex, and premature birth predicted worse executive function, explaining 59% of the variance. Structural equation modeling results indicated that executive dysfunction plays an important mediating role, through which CHD with aortic obstruction, male sex, and premature birth indirectly affect psychosocial QOL. Neurologic abnormalities and single-ventricle CHD did not significantly predict executive dysfunction or QOL. CONCLUSIONS: Executive dysfunction is a strong predictor of psychosocial QOL at school age. Select medical and demographic risk factors did not directly predict QOL at school age in CHD; however, aortic obstruction, premature birth, and male sex impacted QOL indirectly by contributing to executive dysfunction. These findings suggest important risk factors for executive dysfunction that can be monitored, allowing for provision of early supports for executive skills development in an effort to improve long term psychosocial QOL in at-risk children with CHD.


Assuntos
Função Executiva/fisiologia , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/psicologia , Qualidade de Vida , Adolescente , Arteriopatias Oclusivas/fisiopatologia , Criança , Feminino , Cardiopatias Congênitas/classificação , Humanos , Masculino , Modelos Estatísticos , Pais/psicologia , Gravidez , Nascimento Prematuro , Fatores Sexuais , Estresse Psicológico , Inquéritos e Questionários
11.
J Thorac Cardiovasc Surg ; 156(6): 2260-2269, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30243713

RESUMO

OBJECTIVES: Variations in coronary anatomy are common and may relate to the position of the coronary ostium relative to the aortic sinus, the angle of coronary take-off, or the course of the coronary arterial branches. Several classification systems have been proposed. However, they all lack a simple rationale that is applicable irrespective of the relative position of the great arteries, as well as in bicuspid aortic valves. We present a modification of a relatively simple system introduced in the early 1980s, designated the "Leiden Convention." METHODS: The first step of the Leiden Convention is that the clinician takes position in the nonfacing sinus of the aorta looking toward the pulmonary orifice. The right-hand facing sinus is sinus 1, and the left-hand facing sinus is sinus 2. The coronary branches arising from sinus 1 are annotated proceeding in a counterclockwise fashion toward sinus 2. "Usual" (normal) coronary anatomy would be 1R-2LCx. Given their clinical relevance, single sinus coronary arteries are discussed separately. RESULTS: This system was originally designed and highly applicable in hearts with an altered great artery relationship, such as in the variable and complicated patterns seen in transposition of the great arteries and double outlet right ventricle. The modified system also can be used in cases with normally related great arteries, cases with single sinus coronary arteries, and cases with bicuspid aortic valves. CONCLUSIONS: The modified Leiden Convention is not a strict classification but a simple coronary coding system that is broadly applicable.


Assuntos
Seio Coronário/anormalidades , Anomalias dos Vasos Coronários/classificação , Cardiopatias Congênitas/classificação , Terminologia como Assunto , Pontos de Referência Anatômicos , Angiografia por Tomografia Computadorizada , Angiografia Coronária/métodos , Seio Coronário/diagnóstico por imagem , Anomalias dos Vasos Coronários/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico por imagem , Humanos
12.
Medicine (Baltimore) ; 97(31): e11770, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30075604

RESUMO

Congenital heart defects (CHDs) are the most common birth defects (BDs) and account for nearly one-third of all BDs. The aim of this article was to investigate the epidemiology and major subtypes of CHDs in Hunan Province, China in the last 5 years.CHD surveillance data from 2012 to 2016 were collected from 52 registered hospitals in Hunan. The prevalence rates of CHDs, incidence rates of CHDs combined with other BDs, and rates of termination of pregnancy (TOP) for CHDs among different regions, infant sexes, and maternal ages were calculated for both early fetuses (<28 weeks of gestation) and perinatal infants (PIs) (between 28 weeks of gestation and 7 days after birth). Both the constituent ratio and prevalence rates were computed among subtypes.CHDs were found in 6289 out of 673,060 births. The overall prevalence was 93.44 per 10,000 PIs, with 19.27 and 74.17 per 10,000 in early fetuses and PIs, respectively. The risks of CHDs were higher in infants from urban areas than those from rural areas during the whole gestation and were higher in male infants than in female infants during the perinatal period. The total prevalence of CHDs increased significantly with maternal age (χ trend = 141.84, P < .05). Among fetuses in early gestation, there were 288 cases (22.21%) of CHDs combined with other BDs and 1292 cases (99.61%) of TOP for CHD. The 3 major subtypes of CHDs were ventricular septal defect (VSD) (22.06%), Tetralogy of Fallot (TOF) (9.43%), and atrioventricular septal defect (AVSD) (6.69%). Among PIs, there were 1541 cases (30.87%) of CHD diagnosed before delivery and 1184 cases (76.83%) were TOP. The 3 major subtypes were atrial septal defect (ASD) (42.81%), patent ductus arteriosus (PDA) (16.07%), and VSDs (15.21%).The total prevalence of CHD in Hunan Province and the rate of TOP for CHD was high, especially for early-gestation fetuses. Pregnancies in urban women, male PIs, and maternal age were the risk factors for CHDs. Among early-gestation fetuses, the most common types were VSD, TOF, and ASD, and among PIs, the most common types were ASD, PDA, and VSD.


Assuntos
Cardiopatias Congênitas/classificação , Cardiopatias Congênitas/epidemiologia , Aborto Induzido/estatística & dados numéricos , Adulto , China/epidemiologia , Estudos Transversais , Feminino , Idade Gestacional , Humanos , Incidência , Recém-Nascido , Masculino , Idade Materna , Prevalência , Características de Residência , Distribuição por Sexo , Natimorto/epidemiologia
13.
Ann Thorac Surg ; 106(5): 1578-1589, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30031844

RESUMO

The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past 9 years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases. The scheme to categorize a ventricular septal defect uses both its location and the structures along its borders, thereby bridging the two most popular and disparate classification approaches and providing a common language for describing each phenotype. Although the first-order terms are based on the geographic categories of central perimembranous, inlet, trabecular muscular, and outlet defects, inlet and outlet defects are further characterized by descriptors that incorporate the borders of the defect, namely the perimembranous, muscular, and juxta-arterial types. The Society recognizes that it is equally valid to classify these defects by geography or borders, so the emphasis in this system is on the second-order terms that incorporate both geography and borders to describe each phenotype. The unified terminology should help the medical community describe with better precision all types of ventricular septal defects.


Assuntos
Cardiopatias Congênitas/classificação , Comunicação Interventricular/classificação , Melhoria de Qualidade , Terminologia como Assunto , Pré-Escolar , Consenso , Feminino , Cardiopatias Congênitas/cirurgia , Comunicação Interventricular/cirurgia , Humanos , Lactente , Recém-Nascido , Classificação Internacional de Doenças , Masculino , Pediatria , Sociedades Médicas
14.
Heart ; 104(19): 1568-1574, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29720395

RESUMO

Pulmonary arterial hypertension (PAH) is commonly associated with congenital heart disease (CHD) and relates to type of the underlying cardiac defects and repair history. Large systemic to pulmonary shunts may develop PAH if untreated or repaired late. PAH, when present, markedly increases morbidity and mortality in patients with CHD. Significant progress has been made for patients with Eisenmenger syndrome in pathophysiology, prognostication and disease-targeting therapy (DTT), which needs to be applied to routine patient care. Patients with PAH-CHD and systemic to pulmonary shunting may benefit from late defect closure if pulmonary vascular resistance (PVR) is still normal or near normal. Patients with PAH and coincidental defects, or previous repair of CHD should be managed as those with idiopathic PAH. Patients with a Fontan circulation, despite not strictly fulfilling criteria for PAH, may have elevated PVR; recent evidence suggests that they may also benefit from DTT, but more data are required before general recommendations can be made. CHD-PAH is a lifelong, progressive disease; patients should receive tertiary care and benefit from a proactive DTT approach. Novel biomarkers and genetic advances may identify patients with CHD who should be referred for late defect closure and/or patients at high risk of developing PAH despite early closure in childhood. Ongoing vigilance for PAH and further controlled studies are clearly warranted in CHD.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Cardiopatias Congênitas , Hipertensão Pulmonar , Adulto , Cardiopatias Congênitas/classificação , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/cirurgia , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Hipertensão Pulmonar/terapia , Prognóstico , Tempo para o Tratamento , Resultado do Tratamento , Resistência Vascular
15.
Cardiovasc Pathol ; 35: 8-11, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29729634

RESUMO

We report a 52-year-old male patient who had a quadricuspid aortic valve (QAV) associated with aortic regurgitation (AR) and left ventricular hypertrophy (LVH). A new accessory cusp (ACC) with maximum thickness than other cusps was located between right coronary cusp (RCC) and left coronary cusp (LCC). The histopathological features revealed markedly thickened and distorted cusp architecture with fibrosis and/or myxomatous degeneration in both non-coronary cusp (NCC) and ACC. Two equal sizes for larger cusps (RCC and NCC) and two equal sizes for smaller cusps (LCC and ACC) were obtained. This QAV belonged to type C QAV of Hurwitz's classification, but also suggested as a modified type III of Jagannath's classification or a new type V of Nakamura's classification by locating ACC between RCC and LCC.


Assuntos
Insuficiência da Valva Aórtica/patologia , Valva Aórtica/anormalidades , Cardiopatias Congênitas/patologia , Hipertrofia Ventricular Esquerda/patologia , Valva Aórtica/fisiopatologia , Valva Aórtica/transplante , Insuficiência da Valva Aórtica/fisiopatologia , Insuficiência da Valva Aórtica/cirurgia , Biópsia , Ecocardiografia Doppler em Cores , Ecocardiografia Transesofagiana , Fibrose , Cardiopatias Congênitas/classificação , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/cirurgia , Implante de Prótese de Valva Cardíaca , Humanos , Hipertrofia Ventricular Esquerda/fisiopatologia , Hipertrofia Ventricular Esquerda/cirurgia , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
17.
J Am Heart Assoc ; 7(2)2018 01 12.
Artigo em Inglês | MEDLINE | ID: mdl-29330259

RESUMO

BACKGROUND: Administrative data sets utilize billing codes for research and quality assessment. Previous data suggest that such codes can accurately identify adults with congenital heart disease (CHD) in the cardiology clinic, but their use has yet to be validated in a larger population. METHODS AND RESULTS: All administrative codes from an entire health system were queried for a single year. Adults with a CHD diagnosis code (International Classification of Diseases, Ninth Revision, (ICD-9) codes 745-747) defined the cohort. A previously validated hierarchical algorithm was used to identify diagnoses and classify patients. All charts were reviewed to determine a gold standard diagnosis, and comparisons were made to determine accuracy. Of 2399 individuals identified, 206 had no CHD by the algorithm or were deemed to have an uncertain diagnosis after provider review. Of the remaining 2193, only 1069 had a confirmed CHD diagnosis, yielding overall accuracy of 48.7% (95% confidence interval, 47-51%). When limited to those with moderate or complex disease (n=484), accuracy was 77% (95% confidence interval, 74-81%). Among those with CHD, misclassification occurred in 23%. The discriminative ability of the hierarchical algorithm (C statistic: 0.79; 95% confidence interval, 0.77-0.80) improved further with the addition of age, encounter type, and provider (C statistic: 0.89; 95% confidence interval, 0.88-0.90). CONCLUSIONS: ICD codes from an entire healthcare system were frequently erroneous in detecting and classifying CHD patients. Accuracy was higher for those with moderate or complex disease or when coupled with other data. These findings should be taken into account in future studies utilizing administrative data sets in CHD.


Assuntos
Demandas Administrativas em Assistência à Saúde , Algoritmos , Mineração de Dados/métodos , Cardiopatias Congênitas/diagnóstico , Classificação Internacional de Doenças , Adulto , Idoso , Confiabilidade dos Dados , Bases de Dados Factuais , Registros Eletrônicos de Saúde , Feminino , Cardiopatias Congênitas/classificação , Humanos , Masculino , Pessoa de Meia-Idade , Oregon , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Centros de Atenção Terciária
18.
Eur Heart J Qual Care Clin Outcomes ; 4(1): 51-58, 2018 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-28950356

RESUMO

Aims: The New York Heart Association functional classification (NYHA class) is often used to describe the functional capacity of adults with congenital heart disease (ACHD), albeit with limited evidence on its validity in this heterogeneous population. We aimed to validate the NYHA functional classification in ACHD by examining its relation to objective measures of limitation using cardiopulmonary exercise testing (CPET) and mortality. Methods and results: This study included all ACHD patients who underwent a CPET between 2005 and 2015 at the Royal Brompton, in whom functional capacity was graded according to the NYHA classification. Congenital heart diagnoses were classified according to the Bethesda score. Time to all-cause mortality from CPET was recorded in all 2781 ACHD patients (mean age 33.8 ± 14.2 years) enrolled in the study. There was a strong relation between NYHA class and peak oxygen consumption (peak VO2), ventilation per unit in carbon dioxide production (VE/VCO2) slope and the Bethesda classification (P < 0.0001). Although a large number of 'asymptomatic' (NYHA class 1) patients did not achieve a 'normal' peak VO2, the NYHA class was a strong predictor of mortality, with an 8.7-fold increased mortality risk in class 3 compared with class 1 (hazard ratio 8.68, 95% confidence interval: 5.26-14.35, P < 0.0001). Conclusion: Despite underestimating the degree of limitation in some ACHD patients, NYHA classification remains a valuable clinical tool. It correlates with objective measures of exercise and the severity of underlying cardiac disease, as well as mid- to long-term mortality and should, thus, be into incorporated the routine assessment and risk stratification of these patients.


Assuntos
American Heart Association , Tolerância ao Exercício/fisiologia , Cardiopatias Congênitas/classificação , Adulto , Teste de Esforço , Feminino , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Humanos , Masculino , New York , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Reino Unido/epidemiologia , Estados Unidos
19.
Hellenic J Cardiol ; 59(3): 155-159, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-28873334

RESUMO

OBJECTIVE: Pregnancies in patients with adult congenital heart disease (ACHD) are often complicated. We aimed to highlight the nature and the rate of these complications in a single-center patient population. METHODS: We retrospectively studied all the pregnancies of women who presented on an outpatient basis, and all pregnancies were reviewed for maternal and fetal outcomes. RESULTS: Of 117 pregnancies from 52 ACHD patients (age at pregnancy 28.3 ± 6.6 years), 10 were therapeutically aborted. A proportion of 41.1% of the remaining 107 pregnancies were complicated either with cardiac (3.7%), obstetric (15.0%), or fetal/neonatal (11.2%) adverse events or with spontaneous abortion (17.8%). Hospitalization during pregnancy was required in 10 patients. Elective cesarean sections were initially planned for 31% of the 87 pregnancies, which were finally completed, while 8% required an emergency cesarean section, mostly for obstetric reasons. NYHA class deterioration after pregnancy was detected in 9.3% of our cohort. Modified WHO class III/IV, prior medication use, and higher body mass index (BMI) were related to cardiac complications during pregnancy or NYHA deterioration. CONCLUSION: Pregnancies in ACHD patients are at high risk for complications. Advanced modified WHO class, prior medication use, and high BMI were related to adverse cardiac events.


Assuntos
Aborto Induzido/estatística & dados numéricos , Aborto Espontâneo , Cesárea/estatística & dados numéricos , Cardiopatias Congênitas , Doenças do Recém-Nascido , Complicações Cardiovasculares na Gravidez/epidemiologia , Aborto Espontâneo/epidemiologia , Aborto Espontâneo/etiologia , Adulto , Índice de Massa Corporal , Procedimentos Cirúrgicos Eletivos/métodos , Procedimentos Cirúrgicos Eletivos/estatística & dados numéricos , Feminino , Grécia/epidemiologia , Cardiopatias Congênitas/classificação , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/epidemiologia , Humanos , Recém-Nascido , Doenças do Recém-Nascido/classificação , Doenças do Recém-Nascido/epidemiologia , Doenças do Recém-Nascido/etiologia , Gravidez , Gravidez de Alto Risco , Estudos Retrospectivos , Medição de Risco/métodos
20.
Cardiol Young ; 28(1): 134-141, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-28889824

RESUMO

BACKGROUND: This study evaluates the morbidity, mortality, and cost differences between patients who underwent either a simple or a complex arterial switch operation. METHODS: A retrospective study of patients undergoing an arterial switch operation at a single institution was performed. Simple cases were defined as patients with d-transposition of the great arteries with usual coronary anatomy or circumflex artery originating from the right with either intact ventricular septum or ventricular septal defect. Complex cases included all other forms of coronary anatomy, aortic coarctation or arch hypoplasia, and Taussig-Bing anomalies. Costs were acquired using an institutional activity-based accounting system. RESULTS: A total of 98 patients were identified, 68 patients in the simple group and 30 in the complex group. The mortality rate was 2% for the simple and 7% for the complex group, p=0.23. Major morbidities including cardiac arrest, extracorporeal membrane oxygenation, a major coronary event, surgical or catheter-based re-intervention, stroke, or permanent pacemaker placement, non-cardiac surgical procedures, mediastinitis, and sepsis did not differ between the simple and complex groups (16 versus 27%, p=0.16). The complex group had increased bleeding requiring re-exploration (0 versus 10%, p=0.04). Hospital and ICU length of stay did not differ. Complex patients had higher overall hospital costs (simple $80,749 versus complex $97,387, p=0.01) and higher postoperative costs (simple $60,192 versus complex $70,132, p=0.02). The operating room and supplies accounted for the majority of the cost difference. CONCLUSION: Complex arterial switches can be safely performed with low rates of morbidity and mortality but at an increased cost.


Assuntos
Transposição das Grandes Artérias/mortalidade , Transposição das Grandes Artérias/métodos , Anomalias dos Vasos Coronários/cirurgia , Cardiopatias Congênitas/cirurgia , Custos Hospitalares/estatística & dados numéricos , Transposição das Grandes Artérias/efeitos adversos , Ponte Cardiopulmonar/efeitos adversos , Pré-Escolar , Feminino , Seguimentos , Cardiopatias Congênitas/classificação , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Complicações Pós-Operatórias , Estudos Retrospectivos , Resultado do Tratamento
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA