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1.
Kyobu Geka ; 73(11): 936-939, 2020 Oct.
Artigo em Japonês | MEDLINE | ID: mdl-33130718

RESUMO

A 45-year-old male developed Stanford type A acute aortic dissection combined with aortic root dilation and congenital bicuspid aortic valve (BAV). He had a Sieveres type 0 BAV, lateral subtype with right and left cusps. Valve-sparing root reimplantation was performed with decalcification of the cusps. Transthoracic echocardiography(TTE) at discharge revealed no aortic regurgitation, and peak velocity of BAV was 2.15 m/second, mean pressure gradient was 9.6 mmHg and aortic valve area was 2.15 cm2. TTE after 6 months revealed only slight elevation of the peak velocity to 2.78 m/second. To perform successful reimplantation in the case of BAV, anatomic orientation of the cusps should be approximately at 180° and the tissue of the cusps should either be normal or have only minor abnormalities. Valve-sparing root reimplantation for BAV needs a careful follow-up for progression of the aortic valve dysfunction.


Assuntos
Aneurisma Dissecante , Valva Aórtica , Aneurisma Dissecante/complicações , Aneurisma Dissecante/diagnóstico por imagem , Aneurisma Dissecante/cirurgia , Valva Aórtica/anormalidades , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Dilatação , Doenças das Valvas Cardíacas , Humanos , Masculino , Pessoa de Meia-Idade , Reimplante , Estudos Retrospectivos
5.
Eur J Endocrinol ; 183(4): 463-470, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32822316

RESUMO

Objective: Turner syndrome (TS) is a rare disorder affecting 1/2500 female newborn. Aortic dilatation (AD) and aortic dissection represent a major concern in TS. The aims of our study were to describe the aortic root growth, potential aortic dilatation (AD) risk factors and cardiovascular outcomes in a cohort of patients with TS. Methods: Among 204 adult patients included, 197 were studied using a standardized 1.5 Tesla MRI protocol. AD was defined as an aortic diameter ≥20 mm/m2 at the Valsalva sinuses and/or at the ascending aorta, when indexed to body surface area. Results: At baseline, AD was present in 81/197 (41.1%) and 32/197 (16.2%) of patients, at the levels of Valsalva and ascending aorta, respectively. The aortic Valsalva diameter was larger in patients treated for thyroiditis (P < 0.001). Potential risk factors of AD were aging (P < 0.001) and the presence of bicuspid aortic valve (BAV) (P = 0.002). The hazard ratio (HR) of AD occurrence in the presence of BAV was 2.2 (95% CI: 1.33-3.71). After a median follow-up period of 5.1 years (n = 143), AD was present in 58/143 (40.6%) and 25/143 (17.5%) of patients at the levels of Valsalva and ascending aorta, respectively. The median aortic growth of the Valsalva sinuses remained stable. At the ascending aorta, it increased by 0.14 ± 0.61 mm/year. Only one aortic-related death was observed. Conclusion: AD is common in adult patients with TS. However, our results are rather reassuring, as the median aortic diameters remained stable after 5.1 years and few aortic events were observed.


Assuntos
Doenças da Aorta/epidemiologia , Síndrome de Turner/epidemiologia , Adulto , Aorta/diagnóstico por imagem , Aorta/patologia , Doenças da Aorta/complicações , Doenças da Aorta/diagnóstico , Valva Aórtica/anormalidades , Valva Aórtica/patologia , Estudos de Coortes , Dilatação Patológica/complicações , Dilatação Patológica/diagnóstico , Dilatação Patológica/epidemiologia , Progressão da Doença , Feminino , França/epidemiologia , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/diagnóstico , Doenças das Valvas Cardíacas/epidemiologia , Doenças das Valvas Cardíacas/patologia , Humanos , Masculino , Prevalência , Síndrome de Turner/complicações , Adulto Jovem
6.
Wiad Lek ; 73(7): 1580-1582, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32759458

RESUMO

Penetrating aortic ulcer (PAU) is ulceration of an aortic atherosclerotic plaque penetrating through the internal lamina into the media. PAU is a rare condition and occurs in 2% - 7% of acute aortic syndromes (AAS); however, the actual incidence is unknown because of asymptomatic patients. One may treat it conservatively as well as surgically. We present a case of a 54-year-old man, who was admitted to hospital due to the exaggeration of exertional chest pain and persistent headaches. During coronary angiography, the suspicion of PAU was raised. Contrast-enhanced computed tomography confirmed the diagnosis. Transesophageal echocardiography showed bicuspid aortic valve with minimal calcification, the dilated ascending aorta, large atherosclerotic plaques in the aortic arch with ulceration (thickness: 5.0 - 5.5mm, diameter: 5 - 6 mm, depth: 3 - 4 mm), without intramural hematoma. Conservative treatment was chosen with uneventful 2-year follow-up. Although surgical management is advocated for patients with PAU type A, we demonstrated that type A PAU can be successfully treated conservatively as well.


Assuntos
Aorta Torácica , Valva Aórtica/anormalidades , Doenças das Valvas Cardíacas , Úlcera , Aorta , Tratamento Conservador , Humanos , Masculino , Pessoa de Meia-Idade
7.
BMC Infect Dis ; 20(1): 583, 2020 Aug 06.
Artigo em Inglês | MEDLINE | ID: mdl-32762676

RESUMO

BACKGROUND: Dengue fever is endemic and a leading health problem in Sri Lanka. Increased incidence of concurrent bacteremia in patients with dengue infection is a recognized complication. However, Staphylococcal endocarditis following dengue fever is uncommon. Quadricuspid aortic valve (QAV) is a rare congenital anomaly and few cases of infective endocarditis have been reported in QAV. CASE PRESENTATION: A 32-year-old Sri Lankan male presented to the National Hospital of Sri Lanka with recurrence of fever and acute left hemiplegia following an uncomplicated recovery of dengue fever. He was diagnosed to have Staphylococcal infective endocarditis of quadricuspid aortic valve, with septic emboli to brain and spleen. He was managed with intravenous vancomycin initially, however, due to inadequate response, intravenous linezolid was added. He developed rhabdomyolysis with very high creatine phosphokinase leading to acute kidney injury, which settled with the cessation of linezolid. The patient succumbed to his illness despite aggressive antimicrobial therapy and maximum supportive care while being assessed for aortic valve replacement. CONCLUSIONS: This case illustrates three clinical issues that a clinician should be aware of. Firstly, the possibility of a serious secondary bacterial infection as a cause for recurrence of fever following dengue infection. Secondly, this case highlights the importance of identifying QAV as a cause for complicated infective endocarditis of increased severity. The report also denotes the value of being vigilant of linezolid induced rhabdomyolysis which had a causal relationship with the commencement of the drug and its cessation.


Assuntos
Valva Aórtica/anormalidades , Dengue/complicações , Endocardite Bacteriana/diagnóstico , Endocardite Bacteriana/etiologia , Doenças das Valvas Cardíacas/diagnóstico , Infecções Estafilocócicas/diagnóstico , Infecções Estafilocócicas/etiologia , Staphylococcus aureus/isolamento & purificação , Lesão Renal Aguda/etiologia , Lesão Renal Aguda/mortalidade , Adulto , Antibacterianos/farmacologia , Antibacterianos/uso terapêutico , Bacteriemia/tratamento farmacológico , Hemocultura , Dengue/tratamento farmacológico , Dengue/virologia , Vírus da Dengue , Endocardite Bacteriana/tratamento farmacológico , Evolução Fatal , Febre/tratamento farmacológico , Humanos , Linezolida/farmacologia , Linezolida/uso terapêutico , Masculino , Rabdomiólise/induzido quimicamente , Rabdomiólise/complicações , Infecções Estafilocócicas/tratamento farmacológico , Infecções Estafilocócicas/microbiologia , Acidente Vascular Cerebral/mortalidade
8.
Prog Cardiovasc Dis ; 63(4): 475-481, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32640281

RESUMO

Patients with a bicuspid aortic valve (BAV) frequently develop aneurysms of the aortic root and tubular ascending aorta. Aneurysms of the aortic arch, in the absence of concomitant aortopathies, are much less common. According to the 2018 American Association of Thoracic Surgery consensus guidelines on BAV-related aortopathy, prophylactic surgical aortic repair / replacement is recommended starting at a maximum aortic diameter of 50 mm in patients with risk factors. Concomitant aortic surgery is also recommended at an aortic diameter of 45 mm in those patients with other indications for cardiac surgery (most commonly aortic valve procedures). The ultimate goal of prophylactic aortic surgery is the prevention of aortic catastrophes, e.g. aortic rupture or acute aortic dissection, which are associated with high morbidity and mortality. The surgical technique used - in elective and emergency cases - depends on the involvement and nature of the aortic valve disease, as well as the extent of aortic aneurysm formation. The current review focusses on the surgical management of the aortic root, tubular ascending aorta, and proximal aortic arch in BAV patients. Despite the abovementioned recommendations, many BAV patients develop acute aortic syndromes below the recommended aortic diameter thresholds. Further research is therefore required in order to identify high-risk BAV subgroups that would benefit from earlier surgical repair.


Assuntos
Aneurisma Aórtico/cirurgia , Valva Aórtica/anormalidades , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/cirurgia , Aneurisma Aórtico/diagnóstico , Aneurisma Aórtico/etiologia , Valva Aórtica/patologia , Valva Aórtica/cirurgia , Implante de Prótese Vascular , Anuloplastia da Valva Cardíaca , Doenças das Valvas Cardíacas/patologia , Humanos , Técnicas de Sutura
10.
Am J Cardiol ; 129: 71-78, 2020 08 15.
Artigo em Inglês | MEDLINE | ID: mdl-32605717

RESUMO

Controversy persists regarding the advisability of anticoagulation for the early period after biological surgical aortic valve replacement (AVR). We aim to examine the impact of various antithrombotic regimens on outcomes in a large cohort of biological AVR patients. Records of 1,111 consecutive adult patients who underwent surgical biological AVR at our institution between 2013 and 2017 were reviewed. Outcomes included stroke, bleeding, and death at 3 and 12 months. Treatment regimens included (1) no therapy, (2) anticoagulants (warfarin or Factor Xa inhibitors), (2) antiplateles (various), and (4) anticoagulants + antiplatelets. Kaplan-Meier analysis was used to track outcomes, and Cox-proportional hazards regression models were conducted to analyze effects of different therapies on adverse events. At 3 months, thromboembolic events were low and not significantly different between the no therapy group (2.2%) and anticoagulation (2.8%) or anticoagulation + antiplatelet (3.6%) or all groups (3.7%). The antiplatelet group was just significantly lower, at 2.2%. However, this was driven by non-stroke cardiovascular events in patients with coronary artery disease. The incidence of death at 3 months was low and not significantly different between all groups. At 12 months, there were no thromboembolic benefits between groups, but bleeding events were significantly higher in the anticoagulation group (no therapy (1.4%), anticoagulation (8.4%), antiplatelet (4.5%), anticoagulation + antiplatelet (7.9%)). In conclusion, none of the antithrombotic regimens showed benefits in stroke or survival at 3 or 12 months after biological AVR. Anticoagulation increased bleeding events. Routine anticoagulation after biological AVR appears to be unnecessary and potentially harmful.


Assuntos
Anticoagulantes/uso terapêutico , Estenose da Valva Aórtica/cirurgia , Bioprótese , Próteses Valvulares Cardíacas , Hemorragia/epidemiologia , Inibidores da Agregação de Plaquetas/uso terapêutico , Acidente Vascular Cerebral/epidemiologia , Tromboembolia/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Valva Aórtica/anormalidades , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/complicações , Insuficiência da Valva Aórtica/cirurgia , Estenose da Valva Aórtica/complicações , Aspirina/uso terapêutico , Fibrilação Atrial/complicações , Doença da Artéria Coronariana/complicações , Doença da Artéria Coronariana/tratamento farmacológico , Inibidores do Fator Xa/uso terapêutico , Feminino , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/cirurgia , Implante de Prótese de Valva Cardíaca , Hemorragia/induzido quimicamente , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Mortalidade , Cuidados Pós-Operatórios , Modelos de Riscos Proporcionais , Antagonistas do Receptor Purinérgico P2Y/uso terapêutico , Varfarina/uso terapêutico , Adulto Jovem
12.
Prog Cardiovasc Dis ; 63(4): 407-418, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32592706

RESUMO

Bicuspid aortic valve (BAV) is the most common congenital cardiac malformation, frequently associated with aortopathies and valvulopathies. The congenital origin of BAV is suspected to impact the development of the disease in the adult life. During the last decade, a number of studies dealing with the embryonic development of congenital heart disease have significantly improved our knowledge on BAV etiology. They describe the developmental defects, at the molecular, cellular and morphological levels, leading to congenital cardiac malformations, including BAV, in animal models. These models consist of a spontaneous hamster and several mouse models with different genetic manipulations in genes belonging to a variety of pathways. In this review paper, we aim to gather information on the developmental defects leading to BAV formation in these animal models, in order to tentatively explain the morphogenetic origin of the spectrum of valve morphologies that characterizes human BAV. BAV may be the only defect resulting from gene manipulation in mice, but usually it appears as the less severe defect of a spectrum of malformations, most frequently affecting the cardiac outflow tract. The genes whose alterations cause BAV belong to different genetic pathways, but many of them are direct or indirectly associated with the NOTCH pathway. These molecular alterations affect three basic cellular mechanisms during heart development, i.e., endocardial-to-mesenchymal transformation, cardiac neural crest (CNC) cell behavior and valve cushion mesenchymal cell differentiation. The defective cellular functions affect three possible morphogenetic mechanisms, i.e., outflow tract endocardial cushion formation, outflow tract septation and valve cushion excavation. While endocardial cushion abnormalities usually lead to latero-lateral BAVs and septation defects to antero-posterior BAVs, alterations in cushion excavation may give rise to both BAV types. The severity of the original defect most probably determines the specific aortic valve phenotype, which includes commissural fusions and raphes. Based on current knowledge on the developmental mechanisms of the cardiac outflow tract, we propose a unified hypothesis of BAV formation, based on the inductive role of CNC cells in the three mechanisms of BAV development. Alterations of CNC cell behavior in three possible alternative key valvulogenic processes may lead to the whole spectrum of BAV.


Assuntos
Valva Aórtica/anormalidades , Doenças das Valvas Cardíacas/embriologia , Animais , Valva Aórtica/embriologia , Valva Aórtica/patologia , Modelos Animais de Doenças , Doenças das Valvas Cardíacas/genética , Doenças das Valvas Cardíacas/patologia , Humanos , Crista Neural
13.
Prog Cardiovasc Dis ; 63(4): 482-487, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32592707

RESUMO

Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality in adults, and symptoms typically develops in adulthood. In the majority of cases, BAV disease progresses with age and surgical aortic valve replacement (AVR) is performed with excellent operative outcomes. Based on a series of randomized trials, transcatheter AVR (TAVR) has evolved from a novel technology to an established therapy for patients with symptomatic severe aortic stenosis (AS) regardless of risk profile. Despite exclusion of bicuspid anatomy from the randomized trials, an increasing number of patients with BAV-AS have been treated with TAVR. Recent observational studies showed the comparable outcomes of TAVR between bicuspid and tricuspid AS. However, worse outcomes in patients with unfavorable bicuspid anatomy such as calcified raphe plus excessive leaflet calcification have raised concerns for TAVR in younger and lower risk population. For the further expansion of TAVR toward BAV-AS population, we need randomized trials of TAVR in this population. Until then, previous registry data combined with computed tomographic-based anatomical assessment may guide the optimal therapy in patients with bicuspid anatomy.


Assuntos
Estenose da Valva Aórtica/cirurgia , Valva Aórtica/anormalidades , Doenças das Valvas Cardíacas/cirurgia , Substituição da Valva Aórtica Transcateter , Valva Aórtica/cirurgia , Estenose da Valva Aórtica/diagnóstico , Estenose da Valva Aórtica/mortalidade , Doenças das Valvas Cardíacas/diagnóstico , Doenças das Valvas Cardíacas/mortalidade , Humanos
14.
Am J Cardiol ; 128: 210-215, 2020 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-32534732

RESUMO

The clinical and imaging differences between bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV) patients with medically managed asymptomatic moderate-to-severe aortic stenosis (AS) have not been studied previously. We aim to characterize these differences and their clinical outcomes in this study. A retrospective observational study was conducted on 836 consecutive cases of isolated asymptomatic moderate-to-severe AS, with median follow-up of 3.4 years. Clinical and echocardiographic characteristics were compared between BAV and TAV patients. Subgroup analysis stratified by AS severity were performed. Survival analysis of all-cause mortality was performed using Kaplan-Meier curves and Cox proportional hazards model. Compared to BAV patients, TAV patients were older (76 ± 11 vs 55 ± 16 years, p <0.001) and had more co-morbidities including hypertension (78% vs 56%; p <0.001), diabetes (41% vs 24%; p <0.001), and chronic kidney disease (20% vs 3%; p = 0.001). TAV patients had less severe aortic valve disease than BAV patients, with a higher aortic valve area index (0.71 ± 0.20 cm2/m2 vs 0.61 ± 0.18 cm2/m2, p <0.001) and less aortic dilation (sinotubular junction: 23.7 ± 4.0 mm vs 26.9 ± 4.8 mm, p <0.001; mid-ascending aorta: 31.4 ± 4.7 mm vs 36.3 ± 6.3 mm, p <0.001). TAV patients were more likely to have eccentric left ventricular hypertrophy and less likely to have a normal geometry (p = 0.003). Competing risk analysis identified increased age (hazard ratio 1.03, 95% confidence interval 1.02 to 1.05, p <0.001) and LVEF (hazard ratio 0.98, 95% confidence interval 0.97 to 0.99, p <0.001) as independent risk factors of all-cause mortality. Valve morphology was not a significant independent risk factor for aortic valve replacement or mortality. In conclusion, asymptomatic TAV patients had more cardiovascular risk factors, less severe aortic valve disease, less sinotubular and mid-ascending aortic dilation, more severe LV remodeling.


Assuntos
Estenose da Valva Aórtica/diagnóstico por imagem , Valva Aórtica/anormalidades , Doenças Assintomáticas , Doenças das Valvas Cardíacas/diagnóstico por imagem , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Doenças da Aorta/diagnóstico por imagem , Doenças da Aorta/epidemiologia , Doenças da Aorta/fisiopatologia , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/fisiopatologia , Estenose da Valva Aórtica/complicações , Estenose da Valva Aórtica/epidemiologia , Estenose da Valva Aórtica/fisiopatologia , Estudos de Casos e Controles , Causas de Morte , Dilatação Patológica/diagnóstico por imagem , Dilatação Patológica/epidemiologia , Dilatação Patológica/fisiopatologia , Ecocardiografia , Feminino , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/epidemiologia , Doenças das Valvas Cardíacas/fisiopatologia , Implante de Prótese de Valva Cardíaca/estatística & dados numéricos , Humanos , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Hipertrofia Ventricular Esquerda/epidemiologia , Hipertrofia Ventricular Esquerda/fisiopatologia , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Mortalidade , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Volume Sistólico
15.
Prog Cardiovasc Dis ; 63(4): 442-451, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32531300

RESUMO

Bicuspid aortic valve (BAV) patients are at increased risk of valve dysfunction and ascending aorta aneurysm. Imaging techniques are essential to establish diagnosis, identify complications and indicate surgical treatment. Transthoracic echocardiography (TTE) is the imaging technique of choice to diagnose BAV, valve morphotype and valvular dysfunction in clinical practice. However, it can be less precise in assessing the aortic root and proximal ascending aorta, and visualization of the mid-distal ascending aorta and the arch may be difficult in some adults where cardiac magnetic resonance (CMR) and computed tomography, using multiplanar reconstructions, are better at assessing aortic diameters. Although valvular dysfunction is very variable, almost half of the patients have more than mild aortic valve disease. TTE is the most effective and accurate test for evaluating the severity of valvular dysfunction and guiding appropriate management decisions. Aorta dilation is a common finding in patients with BAV. The pattern of aortic dimensions has been categorized in three aortic phenotypes: no-dilation phenotype, ascending aorta phenotype and root phenotype. Controversial data exist regarding the relationship between BAV morphology and aorta dilation phenotype. The assessment of aortic stiffness (measuring distensibility or the velocity of propagation of flow) has raised special interest in order to predict progressive aorta dilation. However, current data indicates that BAV aortas do not show altered stiffness compared to those associated with a tricuspid valve with a similar aorta size. Moreover, novel 4D-flow CMR sequences have been crucial in the evaluation of abnormal ascending aorta flow, showing that flow in the aorta of BAV patients is asymmetric and includes the formation of large vortices. Such flow abnormalities are thought to produce changes in wall shear stress which has been associated with extracellular matrix dysregulation. The key points to understand familial screening and the recommendations for establishing the follow-up and therapeutic management of BAV patients are exposed in the review. The main objective of this article is to review the advantages and limitations of the imaging techniques in the diagnosis and management of BAV and the best strategies in the use of multimodality imaging.


Assuntos
Valva Aórtica/anormalidades , Doenças das Valvas Cardíacas/diagnóstico por imagem , Imagem Multimodal , Valva Aórtica/diagnóstico por imagem , Ecocardiografia , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/terapia , Humanos , Imagem por Ressonância Magnética , Tomografia Computadorizada por Raios X
16.
Am J Cardiol ; 129: 53-59, 2020 08 15.
Artigo em Inglês | MEDLINE | ID: mdl-32540169

RESUMO

We compared the long-term outcomes and difference in dilatation rates of the ascending aorta after aortic valve (AV) replacement (AVR) between bicuspid and tricuspid AV patients, and evaluated risk factors associated with ascending aorta dilatation and aortic events during the follow-up. Of 1,127 patients who underwent AVR from 1995 to 2015, 259 patients with a dilated ascending aorta (≥40 mm in diameter) were included. The patients were divided into those with bicuspid (group bicuspid aortic valve [BAV], n = 105) and with tricuspid (group tricuspid aortic valve [TAV], n = 154) AV, and a propensity score-matched analysis was performed to match 98 patients in each group. The differences in the dilation rate of the ascending aorta and long-term outcomes were analyzed. Risk factors for ascending aorta dilatation, mortality, and aortic events were identified. Follow-up was completed in 100% of patients with a median follow-up duration of 106.1 [68.8, 163.0] months. The early clinical outcomes and dilation rate of the ascending aorta were similar between the groups. Overall survivals up to 15 years postoperatively were similar between groups BAV and TAV (p = 0.223). Aortic events occurred in 6 patients (groups BAV vs TAV, 2 vs 4;p = 0.678). Preoperative ascending aorta diameter showed a linear relationship with the dilatation rate of ascending aorta (p <0.001) and was related to progressive aortic dilatation and aortic events (odds ratio: 1.25, p <0.001 and hazard ratio = 1.56, p <0.001, respectively). In conclusion, the long-term outcomes and ascending aorta dilatation rate were similar between the BAV and TAV patients up to 15 years after AVR. Bicuspid AV was not a risk factor of mortality or aortic events.


Assuntos
Aorta/diagnóstico por imagem , Doenças da Aorta/diagnóstico por imagem , Insuficiência da Valva Aórtica/cirurgia , Estenose da Valva Aórtica/cirurgia , Valva Aórtica/anormalidades , Doenças das Valvas Cardíacas/cirurgia , Aneurisma Aórtico/complicações , Aneurisma Aórtico/diagnóstico por imagem , Doenças da Aorta/complicações , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/complicações , Estenose da Valva Aórtica/complicações , Estudos de Casos e Controles , Dilatação Patológica/complicações , Dilatação Patológica/diagnóstico por imagem , Progressão da Doença , Feminino , Doenças das Valvas Cardíacas/complicações , Implante de Prótese de Valva Cardíaca , Humanos , Modelos Logísticos , Masculino , Complicações Pós-Operatórias , Pontuação de Propensão , Modelos de Riscos Proporcionais
17.
Prog Cardiovasc Dis ; 63(4): 425-433, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32497585

RESUMO

Bicuspid aortic valve (BAV) is one of the most common congenital heart defects in children, adolescents and adults. BAV can occur as an isolated lesion or in association with other congenital heart defects, such as coarctation of the aorta or genetic syndromes like Turner syndrome. Although the majority of long term complications associated with BAV manifest later in life, children and adolescents may present with early onset valvular dysfunction or dilation of the aorta. BAV is a heterogeneous disease with a wide array of presentations at various ages, depending on the degree of aortic valve dysfunction, aortic dilation and presence of associated lesions. Aortic valve stenosis and/or regurgitation are the primary indications for intervention in children and adolescents with BAV. Although a majority of young patients with BAV also have some aortic dilation, interventions on the aorta are very rare during this time frame. Children and adolescents with BAV benefit from comprehensive assessment of their risk profile to determine follow-up surveillance intervals, sports recommendations, and timing of surgical intervention. The morphologic phenotype of BAV is important to identify, as it may predict future complications and prognosis.


Assuntos
Valva Aórtica/anormalidades , Doenças das Valvas Cardíacas/diagnóstico , Doenças das Valvas Cardíacas/terapia , Adolescente , Fatores Etários , Criança , Doenças das Valvas Cardíacas/complicações , Humanos
18.
Prog Cardiovasc Dis ; 63(4): 434-441, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32485187

RESUMO

The congenital bicuspid aortic valve (BAV) is a heterogeneous condition that is better understood considering 3 types of valvulo-aortopathy presentations: A typical-presentation valvulo-aortopathy, a complex-presentation valvulo-aortopathy and an uncomplicated/undiagnosed-presentation. The burden of BAV-related complications has been elucidated in patients with the typical-presentation valvulo-aortopathy and is discussed in this review. These patients enjoy an overall normal expected long-term survival as long as complications are identified and treated in a timely manner. This notion and the fact that the most dreaded complications (infective endocarditis and aortic dissection) are infrequent, represent important reassuring points for the BAV patient. Common complications such as valve dysfunction and aorta dilatation must be the subject of focused research in prevention and treatment. Conversely, BAV patients with complex-presentation valvulo-artopathy, as well as typical valvulo-aortopathy BAV patients who are older, with advanced valvular dysfunction, and possible late-referral to specialized care, may incur a survival penalty as compared to the general population. An evidence-based discussion of these concepts is provided in this review.


Assuntos
Valva Aórtica/anormalidades , Doenças das Valvas Cardíacas/diagnóstico , Adulto , Fatores Etários , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/mortalidade , Humanos , Pessoa de Meia-Idade , Taxa de Sobrevida , Adulto Jovem
19.
Prog Cardiovasc Dis ; 63(4): 452-456, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32599025

RESUMO

Bicuspid aortic valve (BAV), the most frequent congenital heart disease, is characterized by an uneven distribution between sexes. BAV is three to four times more frequent in men than in women which could be associated with a reduced dosage of X chromosome genes. In addition, BAV has a multifactorial inheritance, low penetrance and variable phenotypes that may lead to different form of valve degeneration and dysfunction over time as well as different incidence of aortic valve and vascular complications between men and women. Definition of the phenotype is the first step in the evaluation of patients with BAV. Among the various phenotypes, BAV with a fusion raphe between the left and the right coronary cusp is the most frequent phenotype observed in men and women. It has been hypothesized that the valve and vascular related complications vary according to the BAV phenotype and this could explain differences in the clinical outcomes of men versus women. However, the evidence on the distribution of the various BAV phenotypes between sexes in not consistent and while some series have described differences between male and female, others have not confirmed those findings. In terms of valvular complications, women present more frequently with aortic stenosis while aortic regurgitation is more frequently diagnosed in men. Furthermore, endocarditis is more frequently reported in men as compared to women. In terms of vascular complications, men show larger diameters of the various parts of the aortic root and ascending aorta and more frequently present complications in terms of aortic aneurysm and dissection as compared to women. Although there are no survival differences between men and women with BAV, compared to the general population some large series have shown that women have worse prognosis. The present review article summarizes the differences between men and women in terms of BAV phenotype, type and incidence of aortic valve and vascular complications that will determine the differences in clinical outcomes.


Assuntos
Valva Aórtica/anormalidades , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/epidemiologia , Feminino , Doenças das Valvas Cardíacas/diagnóstico , Humanos , Masculino , Fatores Sexuais
20.
Prog Cardiovasc Dis ; 63(4): 419-424, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32599027

RESUMO

There is a growing need to develop a common language when referring to a frequent and heterogeneous condition such as the congenital bicuspid aortic valve and its aortopathy. The following short manuscript serves as an introduction to a standard terminology for the bicuspid aortic valve and its aortopathy.


Assuntos
Valva Aórtica/anormalidades , Doenças das Valvas Cardíacas/congênito , Doenças das Valvas Cardíacas/diagnóstico , Aorta/patologia , Humanos , Terminologia como Assunto
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