Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 1.635
Filtrar
2.
Cardiovasc Pathol ; 50: 107277, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-32882373

RESUMO

Mitral valve is a complex cardiac structure composed of several components to work in synchrony to allow blood flow into left ventricle during diastole and not to allow blood flow into left atrium during systole. Accessory mitral valve tissue (AMVT) was defined as existence of any additional part and parcel of valvular structure which has an attachment to normal mitral valve apparatus in left-sided cardiac chambers. AMVT may present itself in different clinical circumstances ranging from a silent clinical course to thromboembolic events, heart failure, left ventricular outflow tract obstruction, and severe arrhythmia. This article reviews the clinical perspectives of AMVT in terms of symptoms, diagnosis, and treatment, providing a new anatomical classification regarding the location of AMVT. Briefly type I refers to AMVT having attachments on the supra leaflets level, type II refers to attachments on the mitral leaflets, and type III refers to attachment below the mitral leaflets. Increased awareness and widespread use of echocardiographic techniques would increase recognition of AMVT in patients with heart murmurs but otherwise healthy and in those with left ventricular outflow tract obstruction or tissue which causes subaortic stenosis and with unexplained cerebrovascular events.


Assuntos
Cardiopatias Congênitas , Valva Mitral/anormalidades , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/terapia , Humanos , Valva Mitral/diagnóstico por imagem , Valva Mitral/fisiopatologia , Valva Mitral/cirurgia , Valor Preditivo dos Testes , Prognóstico , Fatores de Risco , Tromboembolia/etiologia , Obstrução do Fluxo Ventricular Externo/etiologia
3.
Am J Cardiol ; 130: 137-142, 2020 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-32703525

RESUMO

Patients with Shone complex (SC) have multiple left-sided obstructive lesions and thus are at risk for left ventricular (LV) remodeling, LV diastolic dysfunction and pulmonary hypertension. Yet, to date, there has been no description of hemodynamics in adults with SC. Retrospective chart review of 25 patients with SC who underwent cardiac catheterization at Mayo Clinic, MN between 2002 and 2019 was performed. SC was defined as multiple left-sided obstructive lesions in the presence of an anatomically abnormal mitral valve. Median age was 32 years (22.5, 42) and 15 patients (60%) were female. The majority of patients (84%) had history of coarctation of the aorta, 10 (40%) had subaortic stenosis, 11 (44%) had prior aortic valve replacement, and 10 (40%) had prior mitral valve replacement. Structural disease at the time of catheterization which warranted intervention within the next year was present in 13 patients (52%). The mean LV end-diastolic pressure was 21.3 ± 9.0 mm Hg (>15 mm Hg in 71%), pulmonary artery peak systolic pressure was 55.4 ± 13.4 mm Hg, and the pulmonary artery mean pressure was 37.0 ± 9.4 mm Hg (>20 mm Hg in 96%). During a mean follow-up of 8.3 ± 4.4 years, there were 7 deaths (28%) and 3 additional patients (12%) underwent cardiac transplantation. In conclusion, adults with SC who underwent catheterization showed significant left-sided heart and pulmonary vascular remodeling. Elevated LV end-diastolic pressure and pulmonary artery pressures were highly prevalent. There were high mortality and cardiac transplant rates in our cohort.


Assuntos
Coartação Aórtica/fisiopatologia , Estenose da Valva Aórtica/fisiopatologia , Hemodinâmica , Valva Mitral/anormalidades , Valva Mitral/fisiopatologia , Adulto , Coartação Aórtica/complicações , Estenose da Valva Aórtica/complicações , Feminino , Humanos , Masculino , Estudos Retrospectivos , Adulto Jovem
4.
Prog Cardiovasc Dis ; 63(3): 308-326, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32201287

RESUMO

Floppy mitral valve/mitral valve prolapse (FMV/MVP) is a common valvular abnormality affecting 2% to 3% of the general population. It occurs in a heterogeneous group of patients with varying and age dependent expressions. FMV/MVP can be familial or sporadic, isolated (called non-syndromic) or as a part of a well-defined syndrome of heritable connective tissue disorders or other diseases. A wide range of phenotypic expression exists ranging from asymptomatic to non-specific symptoms related to neuroendocrine or autonomic nervous system functional abnormalities, varying degrees of mitral regurgitation that may require interventional therapy, heart failure, infective endocarditis, cardiac arrhythmias and/or sudden cardiac death. FMV/MVP is predominantly considered a heritable disorder with clinical manifestations not present at birth, but appearing later in life. Though a variant gene may initiate the development of FMV/MVP, precise phenotypic expression may be related to multiple other molecular, genetic and epigenetic factors that modify the final expression of the disease. A better understanding of these mechanisms will help to better define the natural history of the disease, inhibit disease progression and even prevent the phenotypic expression of FMV/MVP.


Assuntos
Prolapso da Valva Mitral/genética , Valva Mitral/anormalidades , Progressão da Doença , Predisposição Genética para Doença , Hereditariedade , Humanos , Valva Mitral/fisiopatologia , Prolapso da Valva Mitral/epidemiologia , Prolapso da Valva Mitral/fisiopatologia , Prolapso da Valva Mitral/terapia , Linhagem , Fenótipo , Prevalência , Fatores de Risco
5.
Semin Thorac Cardiovasc Surg ; 32(3): 531-538, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32060012

RESUMO

Palliation of patients with hypoplastic left heart syndrome remains challenging. Although coronary ischemia can be catastrophic, the prevalence and pathologies of anomalies of the coronary arteries remains unknown. We reviewed 119 specimens with the features of hypoplastic left heart syndrome, focusing our attention on the aortic root and the coronary arteries. We found 36 (30%) specimens with the combination of mitral and aortic atresia, 26 (22%) with mitral and aortic stenosis, and 57 (48%) with mitral stenosis combined with aortic atresia. In 29 specimens (24%), the coronary arteries were not located in the center of any sinuses, while in 24 specimens (21%) at least 1 coronary artery was located very proximal to a raphe or commissure, with potential for obstruction. The specimens with combined stenosis were more likely to have eccentric positions of the coronary arteries (11 specimens, 42%), compared to the 3 specimens with combined atresia (9%, P = 0.009). The specimens with combined stenosis were also more likely to have positioning at risk for obstruction (9 specimens, 35%), compared to those with combined atresia (3 specimens, 9%, P = 0.05). Coronary arterial fistulous communications were found in 11 specimens (9%), significantly more frequently in specimens with mitral stenosis and aortic atresia (9 specimens, 16%, P = 0.041). The origins of the coronary arteries in patients with hypoplastic left heart syndrome place them at potential risk for ischemia, with fistulous communications being a particular risk in those with mitral stenosis combined with aortic atresia.


Assuntos
Anormalidades Múltiplas , Anomalias dos Vasos Coronários/patologia , Vasos Coronários/patologia , Síndrome do Coração Esquerdo Hipoplásico/patologia , Fístula Vascular/patologia , Valva Aórtica/anormalidades , Estenose da Valva Aórtica/complicações , Estenose da Valva Aórtica/patologia , Chicago , Anomalias dos Vasos Coronários/complicações , Florida , Humanos , Síndrome do Coração Esquerdo Hipoplásico/complicações , Valva Mitral/anormalidades , Estenose da Valva Mitral/complicações , Estenose da Valva Mitral/patologia , Isquemia Miocárdica/etiologia , Fístula Vascular/complicações
7.
Cardiol Young ; 30(1): 1-11, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31910934

RESUMO

We reviewed the recent literature for echocardiographic assessment of mitral valve abnormalities in children. A literature search was performed within the National Library of Medicine using the keywords "mitral regurgitation and/or stenosis, children." The search was refined by adding the keywords "echocardiographic definition, classification, and evaluation." Thirty-one studies were finally included. Significant advances in echocardiographic imaging of mitral valve defects, mainly due to the implementation of three-dimensional technology, contribute to a better understanding of the underlying anatomy. However, heterogeneity between classification systems of mitral valve disease severity is a serious problem. For regurgitant lesions, there is only very limited evidence from small studies that support the adoption of quantitative/semi-quantitative indexes commonly employed in adults. Despite the lack of evidence base, qualitative evaluation of regurgitation severity is often employed. For stenotic lesions, no clear categorisation based on trans-valvular echocardiography-derived "gradients" has been consistently applied to define mild, moderate, or severe obstruction across different paediatric age ranges. Quantitative parameters such as valve area have also been poorly validated in children. Adult recommendations are frequently applied without validation for the paediatric age. In conclusion, significant advances in the anatomical evaluation of mitral valve diseases have been made, thanks to three-dimensional echocardiography; however, limitations remain in the quantitative/semi-quantitative estimation of disease severity, both with respect to valvular regurgitation and stenosis. Because adult echocardiographic recommendations should not be simply translated to the paediatric age, more specific paediatric guidelines and standards for the assessment of mitral valve diseases are needed.


Assuntos
Ecocardiografia Doppler/métodos , Insuficiência da Valva Mitral/diagnóstico , Estenose da Valva Mitral/diagnóstico , Valva Mitral/diagnóstico por imagem , Criança , Humanos , Valva Mitral/anormalidades , Insuficiência da Valva Mitral/congênito , Estenose da Valva Mitral/congênito
9.
Pediatr Cardiol ; 41(3): 607-614, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31894395

RESUMO

Despite improvements in valve repair techniques, conditions in which infants and children need for mechanical valve replacement (MVR) are still present. We analyzed supra-annular MVR outcomes in infants and children with small annulus and compared them with conventional annular MVR outcomes. Data were collected retrospectively from medical records of infants and children (weighing < 20 kg) who underwent atrioventricular valve replacement with mechanical valve in Seoul National University Children's Hospital between December 1984 and January 2019. We identified 8 patients (median age 20 months, median weight 10.2 kg) who underwent supra-annular MVR with polytetrafluoroethylene graft (supra-annular group). The patients were diagnosed with congenital mitral valve malformation (5 patients), complete atrioventricular septal defect (2 patients), and functional single ventricle (1 patient). The implanted mechanical valve size ranged from 16 to 23 mm. Thirty-three patients (median age 40 months, median weight 13 kg) underwent conventional annular MVR (annular group). The survival rate was not significantly different between the supra-annular and annular groups (75.0 vs 78.8%, P = 0.816). In patients with biventricular repair (7 patients with supra-annular MVR and 28 patients with annular MVR), mechanical valve-to-mitral valve annulus size ratio was higher in the supra-annular group than in the annular group (1.24 ± 0.30 vs 0.96 ± 0.22, P = 0.035). No coronary complication or heart block were observed in the supra-annular group. Supra-annular MVR with polytetrafluoroethylene graft may be a feasible surgical option in children with a small annulus when valve repair is unsuccessful.


Assuntos
Doenças das Valvas Cardíacas/cirurgia , Implante de Prótese de Valva Cardíaca/métodos , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Valva Mitral/anormalidades , Politetrafluoretileno/uso terapêutico , Estudos Retrospectivos , Seul , Resultado do Tratamento
10.
Dev Biol ; 458(1): 88-97, 2020 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-31669335

RESUMO

Atrioventricular valve development requires endothelial-to-mesenchymal transition (EndMT) that induces cushion endocardial cells to give rise to mesenchymal cells crucial to valve formation. In the adult endothelium, deletion of the docking protein FRS2α induces EndMT by activating TGFß signaling in a miRNA let-7-dependent manner. To study the role of endothelial FRS2α during embryonic development, we generated mice with an inducible endothelial-specific deletion of Frs2α (FRS2αiECKO). Analysis of the FRS2αiECKO embryos uncovered a combination of impaired EndMT in AV cushions and defective maturation of AV valves leading to development of thickened, abnormal valves when Frs2α was deleted early (E7.5) in development. At the same time, no AV valve developmental abnormalities were observed after late (E10.5) deletion. These observations identify FRS2α as a pivotal controller of cell fate transition during both EndMT and post-EndMT valvulogenesis.


Assuntos
Coxins Endocárdicos/embriologia , Regulação da Expressão Gênica no Desenvolvimento , Proteínas de Membrana/fisiologia , Animais , Contagem de Células , Linhagem da Célula , Comunicação Atrioventricular/embriologia , Comunicação Atrioventricular/genética , Coxins Endocárdicos/citologia , Coxins Endocárdicos/patologia , Células Endoteliais/citologia , Deleção de Genes , Proteínas de Membrana/deficiência , Proteínas de Membrana/genética , Mesoderma/citologia , Mesoderma/embriologia , Camundongos , Camundongos Endogâmicos C57BL , MicroRNAs/fisiologia , Valva Mitral/anormalidades , Valva Mitral/embriologia , Morfogênese/genética , Fenótipo , Valva Tricúspide/anormalidades , Valva Tricúspide/embriologia
11.
Gen Thorac Cardiovasc Surg ; 68(3): 287-289, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30565035

RESUMO

A 65 year-old man was diagnosed with congestive heart failure secondary to severe mitral regurgitation resulting from a parachute-like asymmetrical mitral valve. We performed mitral annuloplasty and triangular resection of the thick tissue. The postoperative course was uneventful with no recurrence of mitral regurgitation.


Assuntos
Insuficiência Cardíaca/cirurgia , Anuloplastia da Valva Mitral , Insuficiência da Valva Mitral/cirurgia , Valva Mitral/cirurgia , Idoso , Ecocardiografia , Implante de Prótese de Valva Cardíaca , Humanos , Masculino , Valva Mitral/anormalidades , Resultado do Tratamento
16.
Echocardiography ; 36(11): 2122-2125, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31573705

RESUMO

We report the case of a 74-year-old male, with a medical history of cor triatriatum, admitted with a 10-day history of intermittent fever. Three sets of blood cultures were positive for Providencia rettgeri. Transthoracic and transesophageal echocardiogram excluded infective endocarditis, but revealed a congenital accessory tissue adhering to the mitral valve, causing supravalvular mitral stenosis. Cor triatriatum sinistrum is a rare congenital cardiac anomaly, even more uncommon in adults, and quite exceptional when associated with mitral valve disease. Because the patient had no symptoms related to the heart valve disease, no surgical indication was given and he was managed conservatively.


Assuntos
Anormalidades Múltiplas , Coração Triatriado/diagnóstico , Ecocardiografia Tridimensional/métodos , Ecocardiografia Transesofagiana/métodos , Ventrículos do Coração/diagnóstico por imagem , Estenose da Valva Mitral/diagnóstico , Valva Mitral/diagnóstico por imagem , Idoso , Diagnóstico Diferencial , Humanos , Masculino , Valva Mitral/anormalidades , Estenose da Valva Mitral/congênito , Doenças Raras
17.
Pediatr Cardiol ; 40(8): 1761-1762, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31506732

RESUMO

We here report a neonate with prenatal echocardiographic diagnosis of tricuspid atresia, with normally related great vessels, and large ventricular septal defect. This diagnosis could be confirmed with echocardiography at birth. An additional double mitral orifice was also seen. This is a very rare association.


Assuntos
Valva Mitral/anormalidades , Atresia Tricúspide/complicações , Ecocardiografia , Feminino , Humanos , Recém-Nascido , Valva Mitral/diagnóstico por imagem , Diagnóstico Pré-Natal , Atresia Tricúspide/diagnóstico
18.
Echocardiography ; 36(8): 1549-1558, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31385360

RESUMO

BACKGROUND: Mitral annular disjunction (MAD) is a structural abnormality where there is a separation between the mitral valve annulus and the left atrial wall which is not well understood. METHODS: We conducted a systematic review to evaluate the prevalence of MAD, factors associated with MAD and clinical outcomes among patients with MAD. RESULTS: A total of 19 studies were included in this review, and the number of noncase report studies had between 23 and 1439 patients. The pooled rate of MAD in studies of myxomatous mitral valve patients was 66/130 (50.8%, 3 studies), and among patients with mitral valve prolapse was 95/291 (32.6%, 3 studies). One study suggests that 78% of patients with MAD had mitral valve prolapse, and another suggested it was strongly associated with myxomatous mitral valve disease (HR 5.04 95% CI 1.66-15.31). In terms of clinical significance, it has been reported that MAD with disjunction > 8.5 mm was associated with nonsustained ventricular tachycardia (OR 10 95% CI 1.28-78.1). There is also evidence that gadolinium enhancement in papillary muscle (OR 4.09 95% CI 1.28-13.05) and longitudinal MAD distance in posterolateral wall (OR 1.16 95% CI 1.02-1.33) was predictive of ventricular arrhythmia and late gadolinium enhancement in anterolateral papillary muscle was strongly associated with serious arrhythmic event (OR 7.35 95% CI 1.15-47.02). CONCLUSIONS: Mitral annular disjunction appears to be common in myxomatous mitral valve disease and mitral valve prolapse which can be detected on cardiac imaging and may be important because of its association with ventricular arrhythmias and sudden cardiac death.


Assuntos
Ecocardiografia/métodos , Cardiopatias Congênitas/diagnóstico , Doenças das Valvas Cardíacas/diagnóstico , Valva Mitral/diagnóstico por imagem , Doenças das Valvas Cardíacas/congênito , Humanos , Valva Mitral/anormalidades
19.
Echocardiography ; 36(9): 1769-1770, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31454100

RESUMO

Anterior mitral valve clefts are unusual anomalies particularly associated with endocardial cushion defects. A dysplastic subvalvular apparatus causing left ventricular outflow tract obstruction may result in diagnostic confusion. We present an isolated anterior mitral cleft associated with subvalvular dysplasia.


Assuntos
Cordas Tendinosas/anormalidades , Ecocardiografia Tridimensional , Cardiopatias Congênitas/diagnóstico por imagem , Insuficiência da Valva Mitral/diagnóstico por imagem , Valva Mitral/anormalidades , Adulto , Cordas Tendinosas/diagnóstico por imagem , Diagnóstico Diferencial , Feminino , Humanos , Valva Mitral/diagnóstico por imagem
20.
Pediatr Cardiol ; 40(6): 1304-1313, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31338561

RESUMO

This study aimed to evaluate fetal echocardiographic parameters associated with neonatal intervention and single-ventricle palliation (SVP) in fetuses with suspected left-sided cardiac lesions. Initial fetal echocardiograms (1/2002-1/2017) were interpreted by the contemporary fetal cardiologist as coarctation of the aorta (COA), left heart hypoplasia (LHH), hypoplastic left heart syndrome (HLHS), mitral valve hypoplasia (MVH) ± stenosis, and aortic valve hypoplasia ± stenosis (AS). The cohort comprised 68 fetuses with suspected left-sided cardiac lesions (COA n = 15, LHH n = 9, HLHS n = 39, MVH n = 1, and AS n = 4). Smaller left ventricular (LV) length Z score, aortic valve Z score, ascending aorta Z score, and aorta/pulmonary artery ratio; left-to-right shunting at the foramen ovale; and retrograde flow in the aortic arch were associated with the need for neonatal intervention (p = 0.005-0.04). Smaller mitral valve (MV) Z score, LV length Z score, aortic valve Z score, ascending aorta Z score, aorta/pulmonary artery ratio, and LV ejection fraction, as well as higher tricuspid valve-to-MV (TV/MV) ratio, right ventricular-to-LV (RV/LV) length ratio, left-to-right shunting at the foramen ovale, abnormal pulmonary vein Doppler, absence of prograde aortic flow, and retrograde flow in the aortic arch were associated with SVP (p < 0.001-0.008). The strongest independent variable associated with SVP was RV/LV length ratio (stepwise logistical regression, p = 0.03); an RV/LV length ratio > 1.28 was associated with SVP with a sensitivity of 76% and specificity of 96% (AUC 0.90, p < 0.001). A fetal RV/LV length ratio of > 1.28 may be a useful threshold for identifying fetuses requiring SVP.


Assuntos
Ecocardiografia/métodos , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/cirurgia , Diagnóstico Pré-Natal , Adulto , Aorta Torácica/anormalidades , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/patologia , Feminino , Idade Gestacional , Humanos , Recém-Nascido , Valva Mitral/anormalidades , Valva Mitral/diagnóstico por imagem , Valva Mitral/patologia , Gravidez , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/patologia , Estudos Retrospectivos , Sensibilidade e Especificidade , Resultado do Tratamento
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...