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1.
Rev Med Liege ; 75(1): 43-48, 2020 Jan.
Artigo em Francês | MEDLINE | ID: mdl-31920043

RESUMO

Ebstein's anomaly is a rare and complex congenital heart disease involving abnormal embryological development of the tricuspid valve. Its exact prevalence remains unknown given the under-diagnosis of minor forms and the broad clinical spectrum of this pathology resulting from the highly variable tricuspid morphology. Echocardiography plays an important role in diagnosis, assessment and prognostic evaluation of this condition. We present the case of a 6-year-old boy born from a twin pregnancy with a severe neonatal EA diagnosed by ultrasound at 29 weeks of gestation and we discuss the management on the light of the recent literature.


Assuntos
Anomalia de Ebstein , Criança , Anomalia de Ebstein/diagnóstico , Anomalia de Ebstein/cirurgia , Ecocardiografia , Feminino , Humanos , Masculino , Gravidez , Diagnóstico Pré-Natal , Valva Tricúspide
2.
Am J Forensic Med Pathol ; 41(1): 40-41, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31929320

RESUMO

The deceased was a 44-year-old male who was treated for a suspected Ebstein's anomaly observed using transthoracic echocardiogram. He was found dead in his bed at home. Autopsy revealed that the septal tricuspid leaflet was intact; however, a large anterior tricuspid leaflet cleft and right atrioventricular cavity dilation were observed. Pathological examination revealed a normal tricuspid valve, except for the presence of a cleft with local fibrosis of the left ventricle papillary muscle and hemosiderin-containing macrophages at both lungs. There were no other abnormalities that may have led to death. It was concluded that he died a cardiac death based on the right heart overload associated with the anterior tricuspid leaflet cleft. This case indicates the possibility that the anterior tricuspid leaflet cleft can cause death and also highlights the necessity of a detailed autopsy to accurately diagnose the cause of death.


Assuntos
Valva Tricúspide/anormalidades , Valva Tricúspide/patologia , Adulto , Proteína C-Reativa/análise , Diagnóstico Diferencial , Anomalia de Ebstein/diagnóstico , Fibrose , Patologia Legal , Insuficiência Cardíaca/etiologia , Ventrículos do Coração/patologia , Hemossiderina/metabolismo , Humanos , Pulmão/metabolismo , Macrófagos/metabolismo , Masculino , Peptídeo Natriurético Encefálico/sangue , Músculos Papilares/patologia , Fragmentos de Peptídeos/sangue , Insuficiência da Valva Tricúspide/complicações
3.
BMC Oral Health ; 19(1): 200, 2019 08 30.
Artigo em Inglês | MEDLINE | ID: mdl-31470835

RESUMO

BACKGROUND: Antibiotic prophylaxis before invasive treatments, including dental extractions, is still recommended for patients at high risk of infective endocarditis. However, the risk from self-extraction of teeth in daily life of patients with intellectual disabilities is uncertain. CASE PRESENTATION: A 6-year-old patient with Ebstein's anomaly developed cerebral abscess, which appeared associated with infective endocarditis of dental origin. Two weeks after self-extraction of his deciduous teeth, he began to experience pain in his ear and developed continuous fever, followed by vomiting, facial spasm, and a loss of consciousness. He was admitted into a hospital for 2 months, during which he received intravenously administered antibiotics and a drainage tube in his brain. CONCLUSIONS: Deciduous teeth can be self-extracted before root resorption and natural shedding in patients with intellectual disabilities. When they are at high risk of infective endocarditis and frequently touch mobile deciduous teeth, it seems to be an option to extract the teeth early with antibiotic prophylaxis, rather than to wait natural fall.


Assuntos
Abscesso Encefálico/diagnóstico , Anomalia de Ebstein/complicações , Endocardite/microbiologia , Deficiência Intelectual/complicações , Extração Dentária/efeitos adversos , Antibacterianos/uso terapêutico , Abscesso Encefálico/tratamento farmacológico , Abscesso Encefálico/microbiologia , Criança , Pré-Escolar , Feminino , Febre/etiologia , Humanos , Lactente , Masculino , Infecções Estafilocócicas
4.
Methodist Debakey Cardiovasc J ; 15(2): 138-144, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31384377

RESUMO

Ebstein's anomaly is a malformation of the tricuspid valve with myopathy of the right ventricle (RV) that presents with variable anatomic and pathophysiologic characteristics, leading to equally variable clinical scenarios. Medical management and observation is often recommended for asymptomatic patients and may be successful for many years. Tricuspid valve repair is the goal of operative intervention; repair also typically includes RV plication, right atrial reduction, and atrial septal closure or subtotal closure. Postoperative functional assessments generally demonstrate an improvement or relative stability related to degree of RV enlargement, RV dysfunction, RV fractional area change, and tricuspid valve regurgitation.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Fármacos Cardiovasculares/uso terapêutico , Anomalia de Ebstein/terapia , Insuficiência da Valva Tricúspide/terapia , Valva Tricúspide/efeitos dos fármacos , Valva Tricúspide/cirurgia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Fármacos Cardiovasculares/efeitos adversos , Anomalia de Ebstein/complicações , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Hemodinâmica/efeitos dos fármacos , Humanos , Recuperação de Função Fisiológica , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/fisiopatologia , Insuficiência da Valva Tricúspide/tratamento farmacológico , Insuficiência da Valva Tricúspide/etiologia , Insuficiência da Valva Tricúspide/fisiopatologia , Função Ventricular Direita/efeitos dos fármacos
5.
Braz J Cardiovasc Surg ; 34(5): 511-516, 2019 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-31364344

RESUMO

OBJECTIVE: This study aimed to evaluate Ebstein's anomaly surgical correction and its early and long-term outcomes. METHODS: A retrospective analysis of 62 consecutive patients who underwent surgical repair of Ebstein's anomaly in our institution from January 2000 to July 2016. The following long-term outcomes were evaluated: survival, reoperations, tricuspid regurgitation, and postoperative right ventricular dysfunction. RESULTS: Valve repair was performed in 46 (74.2%) patients - 12 of them using the Da Silva cone reconstruction; tricuspid valve replacement was performed in 11 (17.7%) patients; univentricular palliation in one (1.6%) patient; and the one and a half ventricle repair in four (6.5%) patients. The patients' mean age at the time of surgery was 20.5±14.9 years, and 46.8% of them were male. The mean follow-up time was 8.8±6 years. The 30-day mortality rate was 8.06% and the one and 10-year survival rates were 91.9% both. Eleven (17.7%) of the 62 patients required late reoperation due to tricuspid regurgitation, in an average time of 7.1±4.9 years after the first procedure. CONCLUSION: In our experience, the long-term results of the surgical treatment of Ebstein's anomaly demonstrate an acceptable survival rate and a low incidence of reinterventions.


Assuntos
Anomalia de Ebstein/cirurgia , Valva Tricúspide/cirurgia , Adolescente , Adulto , Idoso , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/métodos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Criança , Pré-Escolar , Anomalia de Ebstein/complicações , Anomalia de Ebstein/mortalidade , Feminino , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do Tratamento , Insuficiência da Valva Tricúspide/etiologia , Disfunção Ventricular Direita/etiologia , Adulto Jovem
6.
Kyobu Geka ; 72(4): 283-289, 2019 Apr.
Artigo em Japonês | MEDLINE | ID: mdl-31266914

RESUMO

Ebstein's anomaly (EA) is a rare congenital cardiac malformation and this anomaly is a disorder of a tricuspid valve development in which the valve leaflets fail to delaminate properly from the ventricular wall. Clinical symptoms are age dependent and include cyanosis (size of inter-atrial communication), right-sided heart failure, arrhythmias, and general fatigue on exercise. Optimal timing of surgical intervention is often difficult and must be individualized. Patients have good long-term survival and functional outcomes after undergoing surgery for Ebstein's anomaly in adult population. Operation includes tricuspid valve repair or replacement, closure of any inter-atrial communications, and appropriate antiarrhythmia procedures.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Anomalia de Ebstein , Cardiopatias Congênitas , Adulto , Átrios do Coração , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração , Humanos
7.
Kyobu Geka ; 72(4): 290-295, 2019 Apr.
Artigo em Japonês | MEDLINE | ID: mdl-31266915

RESUMO

BACKGROUND: As for the medical management including surgery for the patients with adult congenital heart disease(ACHD), it may be difficult for most hospitals other than that have both adult/congenital cardiologists/cardiovascular surgeons. Between Shizuoka Prefectural General Hospital and Mt. Fuji Shizuoka Children's Hospital, medical stuff and information have been shared for these 4 years. And joint cardiovascular surgeries have started since 2015 autumn at Shizuoka Prefectural General Hospital. PURPOSE: The contents and the results of these joint operations were evaluated. PATIENTS AND METHODS: Thirteen joint operations were performed and median age at operation was 55 years old( male 3, female 10). The original diagnosis was tetralogy of Fallot/pulmonary atresia with ventricular septal defect 6, ventricular septal defect( VSD)±pulmonary stenosis 4, atrioventicular septal defect/two chamber right ventricle/Ebstein's anomaly 1 ( each). The procedures were pulmonary valve replacement/right ventricle out flow tract reconstruction 7, mitral valve plasty/tricuspid annuloplasty 4, Bentall 2, VSD closure 2 etc.(included multiple choices). RESULTS: There was no early mortality. One late mortality was occurred 17 months after the surgery due to acute myeloid leukemia. General conditions in other patients have been feasible and most of them were followed in Shizuoka Prefectural General Hospital. CONCLUSIONS: The results of the joint operations were feasible in the present study. Our joint project may become more important in the future.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Anomalia de Ebstein , Cardiopatias Congênitas , Comunicação Interventricular , Feminino , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração , Humanos , Masculino , Pessoa de Meia-Idade
8.
Rev Port Cardiol ; 38(5): 385.e1-385.e4, 2019 05.
Artigo em Inglês, Português | MEDLINE | ID: mdl-31256796

RESUMO

Ripple mapping is a novel, three-dimensional, electroanatomic mapping tool that displays each electrogram at its corresponding 3-dimensional coordinate as a dynamic moving bar, which changes in length according to the electrogram voltage-time relationship. We present the case of a 43-year-old male patient with surgically repaired Ebstein's anomaly who previously underwent two unsuccessful ablation procedures for right atrial flutter (cavotricuspid isthmus and intercaval lines). Ripple mapping was decisive, enabling the arrhythmia mechanism to be appropriately recognized, and a distinction to be made between critical areas of the circuit and delayed activated bystander regions.


Assuntos
Mapeamento Potencial de Superfície Corporal/métodos , Procedimentos Cirúrgicos Cardíacos/métodos , Anomalia de Ebstein/complicações , Imagem Tridimensional , Taquicardia por Reentrada no Nó Sinoatrial/diagnóstico , Adulto , Ablação por Cateter/métodos , Anomalia de Ebstein/diagnóstico , Anomalia de Ebstein/cirurgia , Humanos , Masculino , Taquicardia por Reentrada no Nó Sinoatrial/etiologia , Taquicardia por Reentrada no Nó Sinoatrial/cirurgia
9.
Cardiol Young ; 29(6): 819-827, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31159903

RESUMO

BACKGROUND: Ebstein anomaly is a rare congenital heart defect (CHD) that, when severe, requires corrective surgery or other catheter-based intervention in the first year of life. Due to its rarity, risk factors for Ebstein anomaly remain largely unknown. Using national data, we examined 18 potential risk factors for Ebstein anomaly. METHODS: Using 1997-2011 data from the National Birth Defects Prevention Study, a population-based case-control study, we calculated crude and adjusted odds ratios and 95% confidence intervals for paternal age, maternal socio-demographics, reproductive history, and modifiable risk factors, and infant characteristics reported by mothers of 135 Ebstein anomaly cases and 11,829 controls. RESULTS: Mothers of Ebstein anomaly cases had 4.1 (95% confidence interval: 1.8, 9.5) times the odds of reporting a family history of CHD compared with mothers of controls. Ebstein anomaly was associated with maternal second-hand cigarette smoke exposure at home (odds ratio = 2.2 [95% confidence interval: 1.1, 4.4]), but not maternal cigarette smoking (odds ratio = 1.3 [95% confidence interval: 0.8, 2.1]). Odds were elevated, but the 95% confidence interval included 1.0, for maternal marijuana use (odds ratio = 1.8 [95% confidence interval: 0.9, 3.8]) and paternal age ≥40 years at delivery (odds ratio = 1.9 [95% confidence interval: 1.0, 3.5]). CONCLUSIONS: Maternal exposure to second-hand cigarette smoke at home and a family history of CHD were associated with elevated odds of Ebstein anomaly. Genetic analyses could clarify the potential heritability of Ebstein anomaly.


Assuntos
Anomalia de Ebstein/epidemiologia , Exposição Materna/efeitos adversos , Sistema de Registros , Medição de Risco , Adulto , Anomalia de Ebstein/etiologia , Anomalia de Ebstein/prevenção & controle , Feminino , Humanos , Incidência , Recém-Nascido , Masculino , Gravidez , Estudos Retrospectivos , Fatores de Risco , Estados Unidos/epidemiologia , Adulto Jovem
10.
Cardiol Young ; 29(6): 800-807, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31159904

RESUMO

BACKGROUND: Tricuspid valve regurgitation is an inherent part of Ebstein's anomaly, yet whether the severity of the regurgitation further impairs exercise capacity and contributes to long-term morbidity on top of the lesion severity per se is unknown. METHODS: To evaluate for this potential effect, we included 30 patients with Ebstein's anomaly who did not undergo any form of surgical interventions and had a cardiopulmonary exercise test and echocardiographic studies in this retrospective analysis. Echocardiographic studies and cardiopulmonary exercise tests were critically reviewed for lesion severity grade, tricuspid regurgitation degree, and exercise parameters. Cardiac-related hospitalisations were recorded from computerised medical records and during clinic visits. RESULTS: Fourteen patients (47%) had moderate and 8 (27%) had severe regurgitation. Patients with ≥ moderate regurgitation exhibited significantly lower exercise capacity (median % predicted maximal oxygen consumption, 62 versus 79%, p = 0.03) and venilatory efficiency at exercise. When stratifying exercise results by regurgitation degree, a stepwise decrease in oxygen consumption and ventilatory efficiency with increasing regurgitation severity was observed, regardless of the anatomic lesion severity. During a median follow-up of 4.6 years, > moderate tricuspid regurgitation was associated with significantly lower cumulative probability of freedom from cardiac hospitalisations. CONCLUSIONS: We report that among non-operated Ebstein's anomaly patients, greater tricuspid regurgitation severity was associated with worse exercise capacity and with overall higher probability of cardiac-related hospitalisations independent from the underlying lesion severity.


Assuntos
Anuloplastia da Valva Cardíaca/métodos , Anomalia de Ebstein/complicações , Tolerância ao Exercício/fisiologia , Hospitalização/tendências , Insuficiência da Valva Tricúspide/cirurgia , Valva Tricúspide/cirurgia , Adulto , Anomalia de Ebstein/diagnóstico , Ecocardiografia , Teste de Esforço , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Consumo de Oxigênio , Estudos Retrospectivos , Índice de Gravidade de Doença , Fatores de Tempo , Valva Tricúspide/diagnóstico por imagem , Insuficiência da Valva Tricúspide/diagnóstico , Insuficiência da Valva Tricúspide/fisiopatologia , Função Ventricular Direita/fisiologia , Adulto Jovem
11.
J Cardiovasc Magn Reson ; 21(1): 34, 2019 06 27.
Artigo em Inglês | MEDLINE | ID: mdl-31242903

RESUMO

BACKGROUND: Once surgical management is indicated, variation of Ebstein valve morphology affects surgical strategy. This study explored practical, easily measureable, cardiovascular magnetic resonance (CMR)-derived attributes that may contribute to the complexity and risk of cone reconstruction. METHODS: A retrospective assessment was performed of Ebstein anomaly patients older than 12 years age, with pre-operative CMR, undergoing cone surgical reconstruction by one surgeon. In addition to clinical data, the CMR-derived Ebstein valve rotation angle (EVRA), area ratios of chamber size, indexed functional RV (RVEDVi) and left ventricular (LV) volumes, tricuspid valve regurgitant fraction (TR%) and other valve attributes were related to early surgical outcome; including death, significant residual TR% or breakdown of repair. RESULTS: Of 26 operated patients older than 12 years age, since program start, 20 had pre-op CMR and underwent surgery at median (range) age 20 (14-57) years. TR% was improved in all patients. Four of the 20 CMR patients (20%) experienced early surgical dehiscence of the paravalve tissue, with cone-shaped tricuspid valve intact; one of whom died. A larger EVRA correlated with Carpentier category and was significantly related to dehiscence. If EVRA >60o, relative risk of dehiscence was 3.2 (CI 1.3-4.9, p = 0.03). Those with dehiscence had thickened, more tethered anterior leaflet edges (RR 17, CI 3-100, p < 0.01), smaller pre-operative functional RVEDVi; (132 vs 177 mL/m2, p = 0.04), and were older (median 38 vs 19 years, p = 0.01). TR %, chamber area ratios and LV parameters were not different. CONCLUSIONS: Comprehensive CMR assessment characterizes patients prior to cone surgical reconstruction of Ebstein anomaly. Pragmatic observation of larger EVRA, smaller RVEDVi and leaflet thickening, suggests risk of repair tension and dehiscence, and may require specific modification of cone surgical technique, such as leaflet augmentation.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/cirurgia , Imagem Cinética por Ressonância Magnética , Procedimentos Cirúrgicos Reconstrutivos , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/cirurgia , Adolescente , Adulto , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Tomada de Decisão Clínica , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/fisiopatologia , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/mortalidade , Valor Preditivo dos Testes , Procedimentos Cirúrgicos Reconstrutivos/efeitos adversos , Procedimentos Cirúrgicos Reconstrutivos/mortalidade , Recuperação de Função Fisiológica , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/fisiopatologia , Adulto Jovem
12.
Cardiol Young ; 29(6): 756-760, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31198119

RESUMO

OBJECTIVE: To evaluate the health status and quality of life of young patients who had cone reconstruction for Ebstein anomaly. METHODS: We reviewed all patients who had cone reconstruction from 2007 to 2016 at our institution. Prospective surveys were mailed to all eligible patients. Quality of life was assessed using the PedsQL 4.0 Generic Core Scales, including four domains: physical, emotional, social, and school functioning. RESULTS: Of 116 eligible patients, 72 (62%) responded. About 96% reported their health as excellent or good, and 52% were symptom-free. Only 37% of patients were taking any medications, the most common of which was aspirin (30%). Only 19% had been hospitalised for cardiac reasons following cone reconstruction. The average self-reported quality of life was 85.3/100, whereas the average parent proxy-reported quality of life was 81.8/100. There was no difference by self or parent proxy-report in quality of life between cone reconstruction patients and healthy children; however, quality of life was significantly better compared with children with other chronic health conditions. By self-report and parent proxy-report, 15.1 and 16.7% of patients were deemed "at risk" for reduced quality of life, respectively. Socially, 63/64 (98%) patients over 5 years old were either full-time students or working full-time. CONCLUSION: Children with Ebstein anomaly following cone reconstruction have excellent quality of life comparable with healthy peers and significantly better than other children with chronic health conditions. Families of children with Ebstein anomaly can expect excellent quality of life, long-term health status, and social functioning following cone reconstruction.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Anomalia de Ebstein/psicologia , Nível de Saúde , Qualidade de Vida , Adolescente , Adulto , Criança , Pré-Escolar , Anomalia de Ebstein/cirurgia , Feminino , Seguimentos , Humanos , Masculino , Período Pós-Operatório , Estudos Retrospectivos , Adulto Jovem
13.
Zhonghua Er Ke Za Zhi ; 57(6): 465-470, 2019 Jun 02.
Artigo em Chinês | MEDLINE | ID: mdl-31216805

RESUMO

Objective: To investigate the echocardiographic features of fetal Ebstein's anomaly (EA) and to analyze its clinical outcome and prognosis. Methods: A retrospective case-control study was conducted to analyze the echocardiographic features in fetus with EA. Thirty-five EA fetuses (EA group) and 35 normal fetuses matched for gestational age (control group) were enrolled. The main echocardiographic parameters of the two groups were collected and compared. According to the direction of blood flow in the ductus arteriosus (DA),fetuses in EA group were divided into DA reverse perfusion subgroup (n=11) and normal DA blood flow subgroup (n=24). The echocardiographic parameters and GOSE scores were compared between the two subgroups. The echocardiographic features of EA and the difference of fetal hemodynamics were summarized,and the clinical outcome of EA fetus was evaluated by GOSE score. Chi-square test Rank sum test or t test were used for comparison between groups. Results: Nineteen (54%) of the 35 patients terminated the pregnancy and 16 (46%) continued pregnancy until delivery during follow-up. Compared with the normal fetus group,the cardiothoracic ratio was significantly higher (0.47±0.11 vs. 0.34±0.01, t=6.640, P<0.01) and the transverse diameter ratio of right atrium to left atrium was significantly greater (1.42±0.38 vs. 1.08±0.11, t=5.030, P<0.01) in the EA group, and the ratio of pulmonary artery diameter to aortic diameter was significantly lower in the EA group (1.04±0.21 vs. 1.20±0.15, t=-3.770, P<0.01). Compared with the normal DA blood flow subgroup,the GOSE scores ≥ 1.5 was more frequently seen (7/11 vs. 3/24, P=0.004) and the ratio of pulmonary artery diameter to aortic diameter was significantly lower (0.91±0.18 vs. 1.24±0.20, t=-4.696, P=0.002) in the DA reverse perfusion subgroup. Six of the 16 delivery cases underwent EA corrective surgery after birth with 100% successful rate of operation. Among the 6 cases,two had preoperative GOSE score of ≥1.5 who were considered as severe cases,and four had low GOSE score of<1.5. The remaining ten cases who had not undergone the corrective surgery were followed up routinely. Conclusion: Prenatal hemodynamics of EA combined with GOSE score can more accurately evaluate the severity and prognosis of fetal EA, reduce unnecessary labor induction, and improve postpartum cure rate and clinical outcomes.


Assuntos
Anomalia de Ebstein/diagnóstico por imagem , Diagnóstico Pré-Natal , Ultrassonografia Pré-Natal/métodos , Ultrassonografia/métodos , Estudos de Casos e Controles , Feminino , Humanos , Cuidado Pós-Natal , Gravidez , Cuidado Pré-Natal , Prognóstico , Estudos Retrospectivos
14.
Semin Thorac Cardiovasc Surg ; 31(3): 569-570, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30981739
15.
Heart Vessels ; 34(8): 1332-1339, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30848337

RESUMO

The variable anatomy of Ebstein's anomaly leads to its various surgical procedures. The long-term outcomes of different operations were not well established. Thirty-five patients with Ebstein's anomaly who underwent operations from 2006 to 2018 in our department were retrospectively reviewed. Individualized surgical plans were performed according to the preoperative echocardiography and surgeons' preference. Tricuspid repair, either Danielson's or Carpentier's technique, was the primary choice in patients who had sufficient tricuspid leaflets and adequate right ventricle, while tricuspid replacement was used when a reliable repair is not achievable. Additional bidirectional cavopulmonary shunt was performed in those who had unstable hemodynamics despite of high central venous pressure after separation from cardiopulmonary bypass. The perioperative and follow-up data were collected. The age was 26.9 (0.6-54) years [16 children (age < 14, and 19 adults (age ≥ 14)]. Preoperative tricuspid regurgitation was severe in 30, moderate in 4, and mild in the remaining 1 patient. Preoperative cardiac-associated malformations include 20 atrial septal defects, 2 ventricular septal defects, 2 pulmonary stenosis, and 1 sub aortic ridge, and these were operated simultaneously. Among all the surgical patients, 2 needed additional reoperation during the same admission, and ultimately, 29 patients had biventricular repair, including 21 tricuspid repair and 8 replacements. The other 6 patients had cavopulmonary connection and achieved 1.5 ventricular repair (3 tricuspid repair and 3 replacements). In all the 24 tricuspid repair patients, Danielson's procedure was used in 17, while Carpentier's technique was used in the other 7 patients. The average cardiopulmonary bypass time was 90 ± 28 min and cross-clamp time was 48 ± 24min. There were 2 perioperative deaths (5.7%) and no third-degree atrioventricular block. The postoperative in hospital stay was 13.7 ± 9.6 days. In the 33 survivors who were followed up at a median of 29.2 months, 6 patients had severe tricuspid regurgitation, and 2 of them underwent tricuspid replacement. The 5-year freedom from severe tricuspid dysfunction or reoperation was 78.5%, and no difference was found between children and adults, neither between different surgical choices. The surgeries of Ebstein's anomaly were variable, and individualized operation achieved reasonable short- and mid-term results. However, severe tricuspid regurgitation during the follow-up was not neglectable, and reoperation in such cases also achieved good outcomes. New repair strategy such as cone repair may be considered.


Assuntos
Anuloplastia da Valva Cardíaca , Anomalia de Ebstein/cirurgia , Técnica de Fontan , Adolescente , Adulto , Ponte Cardiopulmonar , Criança , Pré-Escolar , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/fisiopatologia , Ecocardiografia , Feminino , Humanos , Masculino , Reoperação , Estudos Retrospectivos , Valva Tricúspide/cirurgia , Insuficiência da Valva Tricúspide/fisiopatologia , Função Ventricular Direita , Adulto Jovem
16.
Semin Thorac Cardiovasc Surg ; 31(3): 566-568, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30735711

RESUMO

We describe a change in our technique for plication of the atrialized right ventricle during a Cone repair. The plication was performed from the "Outside"-on a beating heart. Advantages expected from this change are-all major coronaries are better seen when full of blood and can be avoided, if a major vessel is compromised-electrocardiography changes should point it out immediately, there is a small but significant saving in terms of arrest time and if the plication needs to be taken down because of an adverse coronary event-this can be done without the need of another cardioplegia.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Anomalia de Ebstein/cirurgia , Átrios do Coração/cirurgia , Ventrículos do Coração/cirurgia , Valva Tricúspide/cirurgia , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/fisiopatologia
17.
Pediatr Cardiol ; 40(4): 792-798, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30726509

RESUMO

BACKGROUND: Exercise capacity is a well-defined marker of outcome in congenital heart disease. We analyzed seventeen cardiovascular magnetic resonance (CMR) derived parameters and their correlation to exercise capacity in patients with Ebstein's anomaly (EA). METHODS: Fifty-four surgery free patients, age 5 to 69 years (median 30 years) prospectively underwent CMR examination and cardiopulmonary exercise testing (CPET). The following volume/flow parameters were compared with peak oxygen uptake as the percentage of normal (peakVO2%) using univariate and multivariate analysis: right and left ventricular ejection fraction (RVEF and LVEF), the indexed end-diastolic and end-systolic volumes (RVEDVi, RVESVi, LVEDVi, and LVESVi), the indexed stroke volumes (RVSVi and LVSVi), the total normalized right and left heart volumes; the total right to left heart volume ratio (R/L-ratio). The indexed antegrade flow (ante), indexed net flow (net) as well as cardiac index (CI) in the aorta (Ao) and pulmonary artery (PA) were used. RESULTS: RVEF (R2 0.2788), indexed flow PA net (R2 0.2330), and PA ante (R2 0.1912) showed the best correlation with peakVO2% (all p < 0.001) in the univariate model. Further significant correlation could also be demonstrated with CI-PA, LVEF, LVSVi, Aorta net, RVESVi, and Aorta ante. Multivariate analysis for RVEF and indexed net flow PA revealed a R2 of 0.4350. CONCLUSION: Functional CMR parameters as RVEF and LVEF and flow data of cardiac forward flow correlate to peakVO2%. Evaluation of the indexed net flow in the pulmonary artery and the overall function of the right ventricle best predicts the maximal exercise capacity in patients with EA.


Assuntos
Anomalia de Ebstein/diagnóstico por imagem , Tolerância ao Exercício/fisiologia , Coração/diagnóstico por imagem , Hemodinâmica/fisiologia , Imagem Cinética por Ressonância Magnética/métodos , Adolescente , Adulto , Idoso , Biomarcadores , Criança , Pré-Escolar , Anomalia de Ebstein/fisiopatologia , Teste de Esforço/métodos , Feminino , Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Consumo de Oxigênio/fisiologia , Estudos Prospectivos , Adulto Jovem
18.
Pediatr Cardiol ; 40(4): 726-732, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30701277

RESUMO

Repair of Ebstein anomaly has evolved over the last decade, and timing of repair remains variable. There have been no studies of exercise or functional capacity in patients who have had tricuspid valve surgery for Ebstein anomaly in adulthood. We aimed to compare exercise capacity before and after tricuspid valve repair or replacement for Ebstein anomaly in adults at Mayo Clinic. We performed a retrospective chart review of all patients with Ebstein anomaly who underwent tricuspid valve surgery at Mayo Clinic between June 2007 and January 2015. We compared pre- and postoperative echocardiograms, exercise tests, and clinic visits. Tricuspid valve surgery was done for 322 patients, and 32 patients met criteria of native tricuspid valve repair or replacement at age 18 or older and had maximal pre- and postoperative exercise tests. Nineteen patients had valve repair, and 13 had valve replacement. Surgery for Ebstein anomaly resulted in significant reduction in tricuspid regurgitation and right ventricular size. There was a significant improvement in NYHA functional class after surgery; however, there was no significant improvement in functional aerobic capacity (FAC), metabolic equivalents (METs), exercise time, or [Formula: see text] after surgery. Patients who had an atrial shunt closed during surgery had improved minimum blood oxygen saturations during exercise, though no improvement in exercise capacity. In our cohort, patients who had tricuspid valve repair or replacement for Ebstein anomaly reported an improvement in functional capacity; however, this did not reflect improvement in measured exercise capacity, despite excellent surgical results by echocardiography.


Assuntos
Anomalia de Ebstein/reabilitação , Teste de Esforço/métodos , Tolerância ao Exercício/fisiologia , Adolescente , Antagonistas Adrenérgicos beta/uso terapêutico , Adulto , Anomalia de Ebstein/tratamento farmacológico , Anomalia de Ebstein/cirurgia , Ecocardiografia , Exercício/fisiologia , Tolerância ao Exercício/efeitos dos fármacos , Feminino , Frequência Cardíaca/efeitos dos fármacos , Humanos , Masculino , Pessoa de Meia-Idade , Oximetria , Período Pós-Operatório , Período Pré-Operatório , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
19.
Congenit Heart Dis ; 14(4): 619-627, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30791188

RESUMO

BACKGROUND: The increasing number of patients with Ebstein anomaly (EA) surviving into adulthood implies improvements in the treatments for the complex lesion. We revisited the clinical outcomes of patients with EA to demonstrate their "real world" survival. OBJECTIVES: To identify the survival and predictors of mortality in patients with EA who underwent medical or surgical management in the present era. METHODS: All patients who had EA with atrioventricular concordance between 1994 and 2016 were retrospectively reviewed. Baseline characteristics, initial echocardiographic findings, treatments, and outcomes were explored. The survival analysis was performed at the end of 2017. A multivariate analysis was used to assess mortality risks. RESULTS: A total of 153 patients (25.4 ± 20.4 years, 60% female) were analyzed. Of these, 89 patients had been diagnosed with EA in childhood. During the follow-up [median time of 5.2 years (3 days-23.5 years)], 32 patients (20.9%) died due to major cardiac adverse events. The overall survival at 1, 5, and 10 years were 89%, 82.2%, and 79%, respectively. Of the total 153 patients, 64 patients underwent at least one surgical intervention [median age of 17 years (1 day-64.4 years)]. The survival at 1, 5, and 10 years were 87.5%, 82.4%, and 77.7%, respectively, in patients with EA surgery. This survival is comparable to the survival of 89 nonoperated patients with EA: 89.9%, 87.5%, and 81.8%, at 1, 5, and 10 years, respectively. The significant predictors of mortality were: age at diagnosis ≤2 years, tricuspid valve (TV) z-score >3.80, TV displacement >19.5 mm/m2 , presence of severe tricuspid regurgitation, and absence of forward flow across the pulmonic valve at the initial diagnosis. CONCLUSION: Patients with EA had a moderately good survival in this era. In this paper, we report five simple predictors of death in this patient population.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Anomalia de Ebstein/cirurgia , Ecocardiografia/métodos , Eletrocardiografia/métodos , Medição de Risco/métodos , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Anomalia de Ebstein/diagnóstico , Anomalia de Ebstein/mortalidade , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Tailândia/epidemiologia , Adulto Jovem
20.
Int J Cardiol ; 278: 76-83, 2019 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-30686335

RESUMO

BACKGROUND: Accurate risk stratification of patients with Ebstein's anomaly (EA) is crucial. Aim of the study was to assess the prognostic value of echocardiography, including 2D speckle tracking (STE) derived myocardial deformation indices, for predicting outcome in pediatric and young adult unrepaired EA patients. METHODS: Fifty consecutive EA patients (1 day-18 years, 52% males) underwent echocardiography and were followed for a mean follow-up of 60 ±â€¯41 months for clinical outcome (ventricular tachyarrhythmia, heart failure, need for surgery and/or death). Clinical and instrumental features of EA patients with stable disease were compared with those of EA patients with progressive disease. RESULTS: Twenty-four (48%) EA patients had progressive disease. A more severe grade of tricuspid valve (TV) displacement [59.7 mm/m2 (IQR 27.5-83) vs 28.4 mm/m2 (IQR 17.5-47); p = 0.002], a lower functional right ventricle (RV) fractional area change (FAC) (29.2 ±â€¯7.7% vs 36.7 ±â€¯9.6%; p = 0.004), a higher Celermajer index [0.8 (IQR 0.7-0.98) vs 0.55 (IQR 0.4-0.7); p = 0.000], a lower functional RV-longitudinal strain (-10.2 ±â€¯6.2% vs -16.2 ±â€¯7.3%; p = 0.003) and a lower right atrium peak systolic strain (RA-PALS) (25.2 ±â€¯13.5% vs 36.3 ±â€¯12.5%; p = 0.004) were detected in progressive disease group compared to stable one, respectively. Functional RV-FAC and RA-PALS were independent predictors of progressive disease at multivariate analysis. CONCLUSION: Our study demonstrated for the first time the prognostic role of RV-FAC and RA-PALS in a long-term follow-up of EA young patients. A complete echocardiographic evaluation should be regular part in the evaluation and risk-stratification of EA children.


Assuntos
Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Ecocardiografia/normas , Adolescente , Criança , Pré-Escolar , Anomalia de Ebstein/epidemiologia , Ecocardiografia/métodos , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Prognóstico , Estudos Retrospectivos
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