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1.
BMC Med Genet ; 21(1): 37, 2020 02 19.
Artigo em Inglês | MEDLINE | ID: mdl-32075597

RESUMO

BACKGROUND: The Mucopolysaccharidosis type VI (MPS VI), also known as Maroteaux-Lamy syndrome (OMIM 253200) is an autosomal recessive lysosomal disorder, caused by the deficiency of the enzyme N-acetylgalactosamine 4-sulfatase (also known as arylsulfatase B) due to mutations of the ARSB gene. Cardiologic features are well recognized, and are always present in MPS VI patients. Generally, the onset and the progression of the cardiologic symptoms are insidious, and just a few patients have developed a rapidly progressive disease. Cardiac involvement in MPS VI is a common and progressive feature. For MPS patients, cardiac evaluations are recommended every 1 to 2 years, including blood pressure measurement, electrocardiography and echocardiography. However, congestive heart failure and valvular surgical repair are not frequently seen, and if so, they are performed in adults. Here we report on an atypical MPS VI case with ascites fetalis and a rapidly progressive cardiac disease. CASE PRESENTATION: A 6-month-old Brazilian male, only child of a Brazilian healthy non-consanguineous couple. During pregnancy, second trimester ultrasonography observed fetal ascites and bilateral hydrocele. Physical exam at 6 months-old revealed a typical gibbus deformity and MPS was suspected. Biochemical investigation revealed a diagnosis of MPS type VI, confirmed by molecular test. Baseline echocardiogram revealed discrete tricuspid regurgitation and a thickened mitral valve with posterior leaflet prolapse, causing moderate to severe regurgitation. The patient evolved with mitral insufficiency and congestive heart failure, eventually requiring surgical repair by the first year of age. CONCLUSIONS: We report the first case of MPS VI whose manifestations started in the prenatal period with fetal ascites, with severe cardiac valvular disease that eventually required early surgical repair. Moreover, in MPS with neonatal presentation, including fetal hydrops, besides MPS I, IVA and VII, clinicians should include MPS VI in the differential diagnosis.


Assuntos
Insuficiência Cardíaca/genética , Coração/fisiopatologia , Mucopolissacaridose VI/genética , N-Acetilgalactosamina-4-Sulfatase/genética , Ascite , Brasil/epidemiologia , Progressão da Doença , Coração/diagnóstico por imagem , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/fisiopatologia , Humanos , Lactente , Masculino , Mucopolissacaridose VI/diagnóstico por imagem , Mucopolissacaridose VI/fisiopatologia , Mutação , Fenótipo
2.
Bernoche, Claudia; Timerman, Sergio; Polastri, Thatiane Facholi; Giannetti, Natali Schiavo; Siqueira, Adailson Wagner da Silva; Piscopo, Agnaldo; Soeiro, Alexandre de Matos; Reis, Amélia Gorete Afonso da Costa; Tanaka, Ana Cristina Sayuri; Thomaz, Ana Maria; Quilici, Ana Paula; Catarino, Andrei Hilário; Ribeiro, Anna Christina de Lima; Barreto, Antonio Carlos Pereira; Azevedo, Antonio Fernando Barros de Filho; Pazin, Antonio Filho; Timerman, Ari; Scarpa, Bruna Romanelli; Timerman, Bruno; Tavares, Caio de Assis Moura; Martins, Cantidio Soares Lemos; Serrano, Carlos Vicente Junior; Malaque, Ceila Maria Sant'Ana; Pisani, Cristiano Faria; Batista, Daniel Valente; Leandro, Daniela Luana Fernandes; Szpilman, David; Gonçalves, Diego Manoel; Paiva, Edison Ferreira de; Osawa, Eduardo Atsushi; Lima, Eduardo Gomes; Adam, Eduardo Leal; Peixoto, Elaine; Evaristo, Eli Faria; Azeka, Estela; Silva, Fabio Bruno da; Wen, Fan Hui; Ferreira, Fatima Gil; Lima, Felipe Gallego; Fernandes, Felipe Lourenço; Ganem, Fernando; Galas, Filomena Regina Barbosa Gomes; Tarasoutchi, Flavio; Souza, Germano Emilio Conceição; Feitosa, Gilson Soares Filho; Foronda, Gustavo; Guimarães, Helio Penna; Abud, Isabela Cristina Kirnew; Leite, Ivanhoé Stuart Lima; Linhares, Jaime Paula Pessoa Filho; Moraes, Junior João Batista de Moura Xavier; Falcão, João Luiz Alencar de Araripe; Ramires, Jose Antônio Franchini; Cavalini, José Fernando; Saraiva, José Francisco Kerr; Abrão, Karen Cristine; Pinto, Lecio Figueira; Bianchi, Leonardo Luís Torres; Lopes, Leonardo Nícolau Geisler Daud; Piegas, Leopoldo Soares; Kopel, Liliane; Godoy, Lucas Colombo; Tobase, Lucia; Hajjar, Ludhmila Abrahão; Dallan, Luís Augusto Palma; Caneo, Luiz Fernando; Cardoso, Luiz Francisco; Canesin, Manoel Fernandes; Park, Marcelo; Rabelo, Marcia Maria Noya; Malachias, Marcus Vinícius Bolívar; Gonçalves, Maria Aparecida Batistão; Almeida, Maria Fernanda Branco de; Souza, Maria Francilene Silva; Favarato, Maria Helena Sampaio; Carrion, Maria Julia Machline; Gonzalez, Maria Margarita; Bortolotto, Maria Rita de Figueiredo Lemos; Macatrão-Costa, Milena Frota; Shimoda, Mônica Satsuki; Oliveira-Junior, Mucio Tavares de; Ikari, Nana Miura; Dutra, Oscar Pereira; Berwanger, Otávio; Pinheiro, Patricia Ana Paiva Corrêa; Reis, Patrícia Feitosa Frota dos; Cellia, Pedro Henrique Moraes; Santos Filho, Raul Dias dos; Gianotto-Oliveira, Renan; Kalil Filho, Roberto; Guinsburg, Ruth; Managini, Sandrigo; Lage, Silvia Helena Gelas; Yeu, So Pei; Franchi, Sonia Meiken; Shimoda-Sakano, Tania; Accorsi, Tarso Duenhas; Leal, Tatiana de Carvalho Andreucci; Guimarães, Vanessa; Sallai, Vanessa Santos; Ávila, Walkiria Samuel; Sako, Yara Kimiko.
Arq. bras. cardiol ; 113(3): 449-663, Sept. 2019. tab, graf
Artigo em Português | LILACS-Express | LILACS, Sec. Est. Saúde SP, SESSP-IDPCPROD, Sec. Est. Saúde SP | ID: biblio-1038561
3.
AJR Am J Roentgenol ; 211(6): 1212-1220, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30247977

RESUMO

OBJECTIVE: Currently, there is no consensus in the literature regarding the screening of hepatic nodules in patients who have undergone the Fontan procedure. The objectives of this study are to evaluate in this population the frequency of hepatic nodules at ultrasound (US), CT, and MRI; to measure liver stiffness using acoustic radiation force impulse (ARFI) elastography; and to investigate predictive factors for hepatic nodules. SUBJECTS AND METHODS: In this cross-sectional study, 49 patients who underwent the Fontan procedure were prospectively recruited from August 2014 through June 2016. These patients underwent clinical evaluation for hepatic disorders, ARFI elastography, US, CT, and MRI. RESULTS: Most of the patients had no symptoms, and hepatic nodules were detected in three of 49 (6.1%) patients at US, 14 of 44 (31.8%) patients at CT, and 19 of 48 (39.6%) patients at MRI. Liver stiffness at ARFI elastography was significantly higher in patients with hepatic nodules than in patients without such nodules (2.64 ± 0.81 m/s vs 1.94 ± 0.49 m/s; p = 0.002) and was a significant predictor of hepatic nodule (AUC, 0.767; p = 0.002). No clinical or laboratory data had any significant correlation with the existence of hepatic nodules, including time since Fontan procedure. CONCLUSION: In our study, more than one-third of patients had hepatic nodules at CT or MRI, but US did not detect most hepatic nodules. Liver stiffness at ARFI elastography was significantly higher in patients with hepatic nodules, and it may help guiding which patient should be further imaged with CT or MRI.


Assuntos
Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Hepatopatias/diagnóstico por imagem , Imagem por Ressonância Magnética , Tomografia Computadorizada por Raios X , Ultrassonografia , Adulto , Estudos Transversais , Técnicas de Imagem por Elasticidade , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico por imagem , Humanos , Hepatopatias/complicações , Masculino , Imagem Multimodal , Valor Preditivo dos Testes , Adulto Jovem
4.
Braz J Cardiovasc Surg ; 32(4): 260-269, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28977197

RESUMO

OBJECTIVE: ASSIST is the first Brazilian initiative in building a collaborative quality improvement program in pediatric cardiology and congenital heart disease. The purposes of this manuscript are: (a) to describe the development of the ASSIST project, including the historical, philosophical, organizational, and infrastructural components that will facilitate collaborative quality improvement in congenital heart disease care; (b) to report past and ongoing challenges faced; and (c) to report the first preliminary data analysis. METHODS: A total of 614 operations were prospectively included in a comprehensive online database between September 2014 and December 2015 in two participating centers. Risk Adjustment for Congenital Heart Surgery (RACHS) 1 and Aristotle Basic Complexity (ABC) scores were obtained. Descriptive statistics were provided, and the predictive values of the two scores for mortality were calculated by multivariate logistic regression models. RESULTS: Many barriers and challenges were faced and overcome. Overall mortality was 13.4%. Independent predictors of in-hospital death were: RACHS-1 categories (3, 4, and 5/6), ABC level 4, and age group (≤ 30 days, and 30 days - 1 year). CONCLUSION: The ASSIST project was successfully created over a solid base of collaborative work. The main challenges faced, and overcome, were lack of institutional support, funding, computational infrastructure, dedicated staff, and trust. RACHS-1 and ABC scores performed well in our case mix. Our preliminary outcome analysis shows opportunities for improvement.


Assuntos
Cardiopatias Congênitas/cirurgia , Avaliação de Processos e Resultados em Cuidados de Saúde/organização & administração , Melhoria de Qualidade/organização & administração , Adolescente , Brasil , Criança , Pré-Escolar , Grupos Diagnósticos Relacionados/estatística & dados numéricos , Feminino , Cardiopatias Congênitas/mortalidade , Mortalidade Hospitalar , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Multicêntricos como Assunto/métodos , Valor Preditivo dos Testes , Avaliação de Programas e Projetos de Saúde , Estudos Prospectivos , Risco Ajustado/métodos
5.
Rev. bras. cir. cardiovasc ; 32(4): 260-269, July-Aug. 2017. tab, graf
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-897919

RESUMO

Abstract Objective: ASSIST is the first Brazilian initiative in building a collaborative quality improvement program in pediatric cardiology and congenital heart disease. The purposes of this manuscript are: (a) to describe the development of the ASSIST project, including the historical, philosophical, organizational, and infrastructural components that will facilitate collaborative quality improvement in congenital heart disease care; (b) to report past and ongoing challenges faced; and (c) to report the first preliminary data analysis. Methods: A total of 614 operations were prospectively included in a comprehensive online database between September 2014 and December 2015 in two participating centers. Risk Adjustment for Congenital Heart Surgery (RACHS) 1 and Aristotle Basic Complexity (ABC) scores were obtained. Descriptive statistics were provided, and the predictive values of the two scores for mortality were calculated by multivariate logistic regression models. Results: Many barriers and challenges were faced and overcome. Overall mortality was 13.4%. Independent predictors of in-hospital death were: RACHS-1 categories (3, 4, and 5/6), ABC level 4, and age group (≤ 30 days, and 30 days - 1 year). Conclusion: The ASSIST project was successfully created over a solid base of collaborative work. The main challenges faced, and overcome, were lack of institutional support, funding, computational infrastructure, dedicated staff, and trust. RACHS-1 and ABC scores performed well in our case mix. Our preliminary outcome analysis shows opportunities for improvement.

6.
In. Soeiro, Alexandre de Matos; Leal, Tatiana de Carvalho Andreucci; Accorsi, Tarso augusto Duenhas; Gualandro, Danielle Menosi; Oliveira Junior, Múcio Tavares de; Kalil Filho, Roberto. Manual da residência em cardiologia / Manual residence in cardiology. São Paulo, Manole, 2016. p.32-39.
Monografia em Português | LILACS | ID: biblio-971583
7.
In. Soeiro, Alexandre de Matos; Leal, Tatiana de Carvalho Andreucci; Accorsi, Tarso augusto Duenhas; Gualandro, Danielle Menosi; Oliveira Junior, Múcio Tavares de; Kalil Filho, Roberto. Manual da residência em cardiologia / Manual residence in cardiology. São Paulo, Manole, 2016. p.40-46.
Monografia em Português | LILACS | ID: biblio-971584
8.
Arq Bras Cardiol ; 94(4): e109-12, 2010 Apr.
Artigo em Português | MEDLINE | ID: mdl-20498923

RESUMO

We report an unusual case of association of plastic bronchitis (PB) to protein-losing enteropathy (PLE) in a girl of 4 years and 9 months of age with double inlet single left ventricle and ventriculoarterial concordance. submitted to total cavopulmonary surgery. with an intracardiac lateral tunnel at the age of three. The elimination of the 10 cm fibrin bronchial mold (PB) and the alpha-1-antitrypsin elevation of 52 mg/g in feces had both become outstanding. Using sildenafil. the thoracic duct ligature and the cardiac transplant were programmed in case of continuity of the process.


Assuntos
Bronquite/etiologia , Técnica de Fontan/efeitos adversos , Enteropatias Perdedoras de Proteínas/etiologia , Pré-Escolar , Feminino , Ventrículos do Coração/anormalidades , Humanos
9.
Arq. bras. cardiol ; 94(4): e109-e112, abr. 2010. ilus
Artigo em Português | LILACS | ID: lil-546706

RESUMO

Relatamos um caso incomum de associação de bronquite plástica (BP) com enteropatia perdedora de proteínas (EPP) em menina de 4 anos e 9 meses de idade. com dupla via de entrada de ventrículo único tipo esquerdo e concordância ventrículo-arterial submetida à cirurgia cavopulmonar total. com túnel lateral intracardíaco aos três anos. Tornaram-se chamativas a eliminação de molde brônquico de fibrina de 10 cm (BP) e a elevação de alfa-1-antitripsina de 52 mg/g de fezes. Em uso de sildenafila. programou-se. em caso de continuidade do processo. a ligadura do ducto torácico e transplante cardíaco.


We report an unusual case of association of plastic bronchitis (PB) to protein-losing enteropathy (PLE) in a girl of 4 years and 9 months of age with double inlet single left ventricle and ventriculoarterial concordance. submitted to total cavopulmonary surgery. with an intracardiac lateral tunnel at the age of three. The elimination of the 10 cm fibrin bronchial mold (PB) and the alpha-1-antitrypsin elevation of 52 mg/g in feces had both become outstanding. Using sildenafil. the thoracic duct ligature and the cardiac transplant were programmed in case of continuity of the process.


Assuntos
Pré-Escolar , Feminino , Humanos , Bronquite/etiologia , Técnica de Fontan/efeitos adversos , Enteropatias Perdedoras de Proteínas/etiologia , Ventrículos do Coração/anormalidades
10.
Arq Bras Cardiol ; 81(5): 462-73, 2003 Nov.
Artigo em Inglês, Português | MEDLINE | ID: mdl-14666267

RESUMO

OBJECTIVE: To evaluate the cardiovascular findings and clinical follow-up of patients with Williams-Beuren syndrome. METHODS: We studied 20 patients (11 males, mean age at diagnosis: 5.9 years old), assessed for cardiovascular abnormalities with electrocardiography and Doppler echocardiography. Fluorescence in situ hybridization (FISH) was used to confirm the diagnosis of the syndrome. RESULTS: Elastin gene locus microdeletion was detected in 17 patients (85%) (positive FISH), and in 3 patients deletion was not detected (negative FISH). Sixteen patients with a positive FISH (94%) had congenital cardiovascular disease (mean age at diagnosis: 2,3 years old). We observed isolated (2/16) supravalvular aortic stenosis and supravalvular aortic stenosis associated (11/16) with pulmonary artery stenosis (4/11); mitral valve prolapse (3/11); bicuspid aortic valve (3/11); aortic coarctation (2/11), thickened pulmonary valve (2/11); pulmonary valvular stenosis (1/11); supravalvular pulmonary stenosis (1/11); valvular aortic stenosis (1/11); fixed subaortic stenosis (1/11); pulmonary artery stenosis (2/16) associated with pulmonary valvar stenosis (1/2) and with mitral valve prolapse (1/2); and isolated mitral valve prolapse (1/16). Four patients with severe supravalvular aortic stenosis underwent surgery (mean age: 5.7 years old), and 2 patients had normal pressure gradients (mean follow-up: 8.4 years). CONCLUSION: A detailed cardiac evaluation must be performed in all patients with Williams-Beuren syndrome due to the high frequency of cardiovascular abnormalities.


Assuntos
Cardiopatias Congênitas/diagnóstico , Hibridização in Situ Fluorescente , Síndrome de Williams/diagnóstico , Criança , Pré-Escolar , Feminino , Seguimentos , Cardiopatias Congênitas/etiologia , Cardiopatias Congênitas/genética , Humanos , Lactente , Masculino , Estudos Prospectivos , Estudos Retrospectivos , Síndrome de Williams/complicações , Síndrome de Williams/genética
11.
Arq. bras. cardiol ; 81(5): 462-473, nov. 2003. ilus, tab
Artigo em Inglês, Português | LILACS | ID: lil-351141

RESUMO

OBJECTIVE: To evaluate the cardiovascular findings and clinical follow-up of patients with Williams-Beuren syndrome. METHODS: We studied 20 patients (11 males, mean age at diagnosis: 5.9 years old), assessed for cardiovascular abnormalities with electrocardiography and Doppler echocardiography. Fluorescence in situ hybridization (FISH) was used to confirm the diagnosis of the syndrome. RESULTS: Elastin gene locus microdeletion was detected in 17 patients (85 percent) (positive FISH), and in 3 patients deletion was not detected (negative FISH). Sixteen patients with a positive FISH (94 percent) had congenital cardiovascular disease (mean age at diagnosis: 2,3 years old). We observed isolated (2/16) supravalvular aortic stenosis and supravalvular aortic stenosis associated (11/16) with pulmonary artery stenosis (4/11); mitral valve prolapse (3/11); bicuspid aortic valve (3/11); aortic coarctation (2/11), thickened pulmonary valve (2/11); pulmonary valvular stenosis (1/11); supravalvular pulmonary stenosis (1/11); valvular aortic stenosis (1/11); fixed subaortic stenosis (1/11); pulmonary artery stenosis (2/16) associated with pulmonary valvar stenosis (1/2) and with mitral valve prolapse (1/2); and isolated mitral valve prolapse (1/16). Four patients with severe supravalvular aortic stenosis underwent surgery (mean age: 5.7 years old), and 2 patients had normal pressure gradients (mean follow-up: 8.4 years). CONCLUSION: A detailed cardiac evaluation must be performed in all patients with Williams-Beuren syndrome due to the high frequency of cardiovascular abnormalities


Assuntos
Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/genética , Hibridização in Situ Fluorescente , Síndrome de Williams/genética , Seguimentos , Cardiopatias Congênitas/etiologia , Estudos Prospectivos , Estudos Retrospectivos , Síndrome de Williams/complicações
12.
Arq Bras Cardiol ; 78(2): 162-6, 2002 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-11887191

RESUMO

OBJECTIVE: To compare immediate and late results in patients with or without fenestration who underwent cavopulmonary anastomosis so that we could assess the efficiency of the technique. METHODS: Sixty-two patients underwent surgery between 1988 and 1999, 41 with fenestration (group I -G I) and 21 without fenestration (group II -G II). Tricuspid atresia was prevalent in group I (23-56%) and single ventricle was prevalent in group II (14-66%). Mean ages at the time of operation were 7.3 years in group I and 7.6 in group II. At late follow-up, mean ages were 10.6 years in group I and 12.8 years in group II. RESULTS: Immediate and late mortality were 7.3% in G-I and 4.7% in G-II. Significant pleural effusion occurred in 41.4% of G-I patients and in 23.8% of G-II patients. Significant pericardial effusion occurred in 29.2% and 14.2%, respectively, in groups I and II. Central venous pressure was greater in G-II, 17.7 cm in H2O, as opposed to 15 cm in G-I. Hospital stay was similar between the groups, 26.3 and 21.8 days, respectively. Cyanosis and arterial insaturation occurred in 5 patients, and 4 patients were in functional class II, all from G-I. At late follow-up, 58 (93.5%) were in functional class I. Sinus rhythm was present in 94%, and pulmonary perfusion was similar in both groups. Eleven patients who underwent spirometry had good tolerance to physical effort. CONCLUSION: Atrial fenestration did not improve the immediate or late follow-up of patients who underwent cavopulmonary anastomosis, and is, therefore, dispensable.


Assuntos
Átrios do Coração/anormalidades , Derivação Cardíaca Direita/métodos , Cardiopatias Congênitas/cirurgia , Criança , Feminino , Seguimentos , Técnica de Fontan/métodos , Técnica de Fontan/mortalidade , Átrios do Coração/cirurgia , Derivação Cardíaca Direita/mortalidade , Cardiopatias Congênitas/mortalidade , Humanos , Masculino , Derrame Pericárdico/etiologia , Derrame Pleural/etiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/mortalidade , Resultado do Tratamento
13.
Arq. bras. cardiol ; 78(2): 162-166, Feb. 2002. tab, graf
Artigo em Inglês | LILACS | ID: lil-303900

RESUMO

OBJECTIVE: To compare immediate and late results in patients with or without fenestration who underwent cavopulmonary anastomosis so that we could assess the efficiency of the technique. METHODS: Sixty-two patients underwent surgery between 1988 and 1999, 41 with fenestration (group I -G I) and 21 without fenestration (group II -G II). Tricuspid atresia was prevalent in group I (23-56 percent) and single ventricle was prevalent in group II (14-66 percent). Mean ages at the time of operation were 7.3 years in group I and 7.6 in group II. At late follow-up, mean ages were 10.6 years in group I and 12.8 years in group II. RESULTS: Immediate and late mortality were 7.3 percent in G-I and 4.7 percent in G-II. Significant pleural effusion occurred in 41.4 percent of G-I patients and in 23.8 percent of G-II patients. Significant pericardial effusion occurred in 29.2 percent and 14.2 percent, respectively, in groups I and II. Central venous pressure was greater in G-II, 17.7 cm in H2O, as opposed to 15 cm in G-I. Hospital stay was similar between the groups, 26.3 and 21.8 days, respectively. Cyanosis and arterial insaturation occurred in 5 patients, and 4 patients were in functional class II, all from G-I. At late follow-up, 58 (93.5 percent) were in functional class I. Sinus rhythm was present in 94 percent, and pulmonary perfusion was similar in both groups. Eleven patients who underwent spirometry had good tolerance to physical effort. CONCLUSION: Atrial fenestration did not improve the immediate or late follow-up of patients who underwent cavopulmonary anastomosis, and is, therefore, dispensable


Assuntos
Humanos , Masculino , Feminino , Criança , Átrios do Coração , Derivação Cardíaca Direita , Cardiopatias Congênitas , Seguimentos , Técnica de Fontan , Átrios do Coração , Derrame Pericárdico , Derrame Pleural , Complicações Pós-Operatórias , Resultado do Tratamento
14.
Arq. bras. cardiol ; 77(1): 69-76, July 2001. ilus
Artigo em Português, Inglês | LILACS | ID: lil-288992

RESUMO

We report the case of an 8-month-old female infant with Uhl's anomaly, who underwent successful cardiac transplantation. The clinical findings, complementary laboratory tests, anatomic findings, and differential diagnosis of the anomaly are discussed


Assuntos
Humanos , Feminino , Lactente , Cardiopatias Congênitas/diagnóstico , Transplante de Coração/métodos , Ventrículos do Coração/anormalidades , Diagnóstico Diferencial , Cardiopatias Congênitas/patologia , Cardiopatias Congênitas/cirurgia
17.
Arq. bras. cardiol ; 73(2): 219-24, ago. 1999. ilus
Artigo em Português, Inglês | LILACS | ID: lil-252841

RESUMO

Ebstein's anomaly with coarctation of the aorta is an extremely unusual condition. In this report, the clinical and surgical features of 3 male patients, aged 7 months, 4 years and 14 years, are discussed. All patients were in situs solittus. The first 2 patients had atrioventricular and ventriculoarterial discordance and progressed to heart failure in the neonatal period. The third had atrioventricular and ventriculoarterial concordance, as well as Wolf-Parkinson-White syndrome, with frequent episodes of paroxysmal tachycardia. The 3 patients underwent surgery for correction of the coarctation of the aorta. The patient with atrioventricular and ventriculoarterial concordance underwent tricuspid valvuloplasty using a De Vega-like technique. In addition, ablation of 2 anomalous pathways (Kent bundle), which were detected by the electrophysiologic study, was also subsequently performed. The 3 patients showed a good postoperative outcome for 2 years, although, in those with discordance, the surgical procedure did not influence the dysplasia of the tricuspid valve, because this valve showed light to moderate dysfunction.


Assuntos
Humanos , Masculino , Lactente , Pré-Escolar , Adolescente , Coartação Aórtica/complicações , Anomalia de Ebstein/complicações , Coartação Aórtica/diagnóstico , Coartação Aórtica/cirurgia , Anomalia de Ebstein/diagnóstico , Doenças das Valvas Cardíacas/diagnóstico , Doenças das Valvas Cardíacas/cirurgia , Valva Tricúspide/cirurgia
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