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1.
Otolaryngol Clin North Am ; 52(6): 1127-1139, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31540769

RESUMO

Airway narrowing can be idiopathic or can occur as a result of airway tumors, hematomas, infections, and other pathologic conditions. Endoscopic management variously involves balloon dilatation, stent placement, laser vaporization of pathologic tissue, microdebridement, and other interventions, using either a rigid or a flexible bronchoscope. Jet ventilation is frequently used in such settings, especially when the presence of an endotracheal tube would interfere with the procedure. In desperate cases, extracorporeal membrane oxygenation may be used in managing the critical airway.

2.
Am J Med Genet A ; 179(9): 1725-1744, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31222966

RESUMO

Costello syndrome (CS) is a RASopathy caused by activating germline mutations in HRAS. Due to ubiquitous HRAS gene expression, CS affects multiple organ systems and individuals are predisposed to cancer. Individuals with CS may have distinctive craniofacial features, cardiac anomalies, growth and developmental delays, as well as dermatological, orthopedic, ocular, and neurological issues; however, considerable overlap with other RASopathies exists. Medical evaluation requires an understanding of the multifaceted phenotype. Subspecialists may have limited experience in caring for these individuals because of the rarity of CS. Furthermore, the phenotypic presentation may vary with the underlying genotype. These guidelines were developed by an interdisciplinary team of experts in order to encourage timely health care practices and provide medical management guidelines for the primary and specialty care provider, as well as for the families and affected individuals across their lifespan. These guidelines are based on expert opinion and do not represent evidence-based guidelines due to the lack of data for this rare condition.

3.
Echocardiography ; 36(4): 722-731, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30834579

RESUMO

BACKGROUND: Significant mitral regurgitation (MR) is associated with poorer outcomes in patients undergoing transcatheter aortic valve replacement (TAVR). Factors associated with MR improvement have not been studied thoroughly. METHODS: Retrospective analysis of consecutive patients treated with TAVR with more than mild MR at baseline. MR evolution was assessed at 1-3 and 6-12 months after intervention. MR severity and mechanisms were assessed by echocardiography. Mitral annulus calcification (MAC) was quantified using preoperative cardiac CT. RESULTS: From 674 consecutive TAVR recipients, 78 with more than mild MR had a 6-12 months follow-up. Following TAVR, MR improved in 34 patients (43%), remained stable in 38 (49%) and worsened in 6 (8%). Patients with MR improvement had greater tenting area (141 ± 56 vs. 99 ± 40 mm2 , P < 0.01), tenting height (7.2 ± 1.9 vs. 5.6 ± 1.9 mm, P < 0.01) and lower ejection fraction (43 ± 16 vs. 52 ± 14%, P = 0.01). MAC was frequent (87.7% of patients) and a trend in greater MAC was observed in patients without MR improvement (3560 ± 5587 vs. 2053 ± 2800, P = 0.16). In multivariable analysis, tenting area (OR per 10 mm2 increase: 1.012, 95% CI, 1.001-1.024 P = 0.039) and annulus calcifications associated with leaflet restriction (OR = 0.108, 95% CI, 0.012-0.956, P = 0.045) were independently associated with MR outcome after TAVR. CONCLUSION: Larger mitral valve tenting area was associated with more improvement of MR after TAVR whereas extensive MAC associated with leaflet restriction was associated with less improvement. This may help in the clinical decision-making process of TAVR candidates with concomitant MR.

6.
Circ Cardiovasc Interv ; 11(11): e006388, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30571205

RESUMO

Background The optimal access for patients undergoing transcatheter aortic valve replacement (TAVR) who are not candidates for a transfemoral approach has not been elucidated. The purpose of this study was to compare the safety, feasibility, and early clinical outcomes of transcarotid TAVR compared with thoracic approaches. Methods and Results From a multicenter consecutive cohort of 329 alternative-access TAVR patients (2012-2017), we identified 101 patients who underwent transcarotid TAVR and 228 patients who underwent a transapical or transaortic TAVR. Preprocedural success and 30-day clinical outcomes were compared using multivariable propensity score analysis to account for between-group differences in baseline characteristics. All transcarotid cases were performed under general anesthesia, mainly using the left common carotid artery (97%). Propensity-matched groups had similar rates of 30-day all-cause mortality (2.1% versus 4.6%; P=0.37), stroke (2.1% versus 3.5%; P=0.67; transcarotid versus transapical/transaortic, respectively), new pacemaker implantation, and major vascular complications. Transcarotid TAVR was associated with significantly less new-onset atrial fibrillation (3.2% versus 19.0%; P=0.002), major or life-threatening bleeding (4.3% versus 19.9%; P=0.002), acute kidney injury (none versus 12.1%; P=0.002), and shorter median length of hospital stay (6 versus 8 days; P<0.001). Conclusions Transcarotid vascular access for TAVR is safe and feasible and is associated with encouraging short-term clinical outcomes. Our data suggest a clinical benefit of transcarotid TAVR with respect to atrial fibrillation, major bleeding, acute kidney injury, and length of stay compared with the more invasive transapical or transaortic strategies. Randomized studies are required to ascertain whether transcarotid TAVR yields equivalent results to other alternative vascular access routes.


Assuntos
Aorta , Estenose da Valva Aórtica/cirurgia , Valva Aórtica/patologia , Valva Aórtica/cirurgia , Calcinose/cirurgia , Artéria Carótida Primitiva , Substituição da Valva Aórtica Transcateter/métodos , Idoso , Idoso de 80 Anos ou mais , Aorta/diagnóstico por imagem , Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/mortalidade , Calcinose/diagnóstico por imagem , Calcinose/mortalidade , Canadá/epidemiologia , Artéria Carótida Primitiva/diagnóstico por imagem , Feminino , França/epidemiologia , Humanos , Masculino , Complicações Pós-Operatórias/mortalidade , Sistema de Registros , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Fatores de Tempo , Substituição da Valva Aórtica Transcateter/efeitos adversos , Substituição da Valva Aórtica Transcateter/mortalidade , Resultado do Tratamento
7.
Open Heart ; 5(2): e000854, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30018783

RESUMO

Background and objectives: Transcatheter aortic valve-in-valve implantation (ViV) has emerged as a valuable technique to treat failed surgical bioprostheses (BPs) in patients with high risk for redo surgical aortic valve replacement (SAVR). Small BP size (≤21 mm), stenotic pattern of degeneration and pre-existing prosthesis-patient mismatch (PPM) have been associated with worse clinical outcomes after ViV. However, no study has evaluated the actual haemodynamic benefit associated with ViV. This study aims to compare haemodynamic status observed at post-ViV, pre-ViV and early after initial SAVR and to determine the factors associated with worse haemodynamic outcomes following ViV, including the rates of high residual gradient and 'haemodynamic futility'. Methods: Early post-SAVR, pre-ViV and post-ViV echocardiographic data of 79 consecutive patients who underwent aortic ViV at our institution were retrospectively analysed. The primary study endpoint was suboptimal valve haemodynamics (SVH) following ViV defined by the Valve Academic Research Consortium 2 as the presence of high residual aortic mean gradient (≥20 mm Hg) and/or at least moderate aortic regurgitation (AR). Haemodynamic futility of ViV was defined as <10 mm Hg decrease in mean aortic gradient and no improvement in AR compared with pre-ViV. Results: SVH was found in 61% of patients (57% high residual gradient, 4% moderate AR) after ViV versus 24% early after SAVR. Pre-existing PPM and BP mode of failure by stenosis were independently associated with the primary endpoint (OR: 2.87; 95% CI 1.08 to 7.65; p=0.035 and OR: 3.02; 95% CI 1.08 to 8.42; p=0.035, respectively) and with the presence of high residual gradient (OR: 4.38; 95% CI 1.55 to 12.37; p=0.005 and OR: 5.37; 95% CI 1.77 to 16.30; p=0.003, respectively) following ViV. Criteria of ViV haemodynamic futility were met in 7.6% overall and more frequently in patients with pre-existing PPM and stenotic BP (18.5%) compared with other patients (2.0%). ViV restored haemodynamic function to early post-SAVR level in only 34% of patients. Conclusion: Although ViV was associated with significant haemodynamic improvement compared with pre-ViV in >90% of patients, more than half harboured SVH outcome. Furthermore, only one-third of patients had a restoration of valve haemodynamic function to the early post-SAVR level. Pre-existing PPM and stenosis pattern of BP degeneration were the main factors associated with SVH and haemodynamic futility following ViV. These findings provide strong support for the prevention of PPM at the time of initial SAVR and careful preprocedural patient screening.

10.
Pediatrics ; 141(1)2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-29284646

RESUMO

Progressive familial cholestasis type 2 is caused by a genetically determined absence or reduction in the activity of the bile salt export pump (BSEP). Reduction or absence of BSEP activity causes a failure of bile salt excretion, leading to accumulation of bile salts in hepatocytes and subsequent hepatic damage. Clinically, patients are jaundiced, suffer from severe intractable pruritus, and evidence progressive liver dysfunction. A low level of serum γ-glutamyl transpeptidase, when associated with the described signs and symptoms, is often an early identifier of this condition. Treatment options to date include liver transplantation and the use of biliary diversion. We report a multidrug regimen of 4-phenylbutyrate, oxcarbazepine, and maralixibat (an experimental drug owned by Shire Pharmaceuticals, Dublin, Republic of Ireland) that completely controlled symptoms in 2 siblings with partial loss of BSEP activity.


Assuntos
Membro 11 da Subfamília B de Transportadores de Cassetes de Ligação de ATP/genética , Benzotiepinas/uso terapêutico , Colestase Intra-Hepática/tratamento farmacológico , Colestase Intra-Hepática/genética , Predisposição Genética para Doença , Glicosídeos/uso terapêutico , Fenilbutiratos/uso terapêutico , Transporte Biológico/genética , Carbamazepina/análogos & derivados , Carbamazepina/uso terapêutico , Criança , Colestase Intra-Hepática/complicações , Colestase Intra-Hepática/diagnóstico , Drogas em Investigação , Humanos , Masculino , Prurido/tratamento farmacológico , Prurido/etiologia , Prurido/fisiopatologia , Qualidade de Vida , Recidiva , Medição de Risco , Irmãos , Resultado do Tratamento
11.
J Am Coll Cardiol ; 70(15): 1890-1899, 2017 Oct 10.
Artigo em Inglês | MEDLINE | ID: mdl-28982503

RESUMO

BACKGROUND: Very few reports of long-term outcomes of patients who underwent the Ross procedure have been published. OBJECTIVES: The authors reviewed their 25-year experience with the Ross procedure with the aim of defining very-long-term survival and factors associated with Ross-related failure. METHODS: Between January 1990 and December 2014, the Ross procedure was performed in 310 adults (mean age 40.8 years) at a single institution. All patients were prospectively added to a dedicated cardiac surgery registry. Complete post-operative clinical examination and history were obtained, and transthoracic echocardiography was performed according to a standardized protocol. There was no loss to follow-up. Median follow-up was 15.1 years and up to 25 years. RESULTS: Bicuspid aortic valve was diagnosed in 227 patients (73.2%), and the most common indication for surgery was aortic stenosis (n = 225 [72.6%]). Freedom from any Ross-related reintervention was 92.9% and 70.1% at 10 and 20 years, respectively. Independent risk factors for pulmonary autograft degeneration were pre-operative large aortic annulus (hazard ratio: 1.1; p = 0.01), pre-operative aortic insufficiency (hazard ratio: 2.7; p = 0.002), and concomitant replacement of the ascending aorta (hazard ratio: 7.7; p = 0.0003). There were 4 hospital deaths (1.3%), and overall survival at 10 and 20 years was 94.1% and 83.6%, respectively. Long-term survival was not significantly different in patients who required Ross-related reintervention (log-rank p = 0.70). However, compared with the general population, survival was significantly lower in patients following the Ross procedure when matched on age and sex (p < 0.0001). CONCLUSIONS: The Ross procedure was associated with excellent long-term valvular outcomes and survival, regardless of the need for reintervention. Adults presenting with aortic insufficiency or a dilated aortic annulus or ascending aorta were at greater risk for reintervention. Unlike previous reports, long-term survival was lower in Ross patients compared with matched subjects.


Assuntos
Insuficiência da Valva Aórtica , Estenose da Valva Aórtica , Valva Aórtica/anormalidades , Anuloplastia da Valva Cardíaca , Vasos Coronários , Doenças das Valvas Cardíacas , Efeitos Adversos de Longa Duração , Valva Pulmonar , Reimplante , Adulto , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/etiologia , Insuficiência da Valva Aórtica/cirurgia , Estenose da Valva Aórtica/etiologia , Estenose da Valva Aórtica/cirurgia , Canadá/epidemiologia , Anuloplastia da Valva Cardíaca/efeitos adversos , Anuloplastia da Valva Cardíaca/métodos , Vasos Coronários/diagnóstico por imagem , Vasos Coronários/cirurgia , Feminino , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/diagnóstico , Doenças das Valvas Cardíacas/epidemiologia , Doenças das Valvas Cardíacas/cirurgia , Humanos , Efeitos Adversos de Longa Duração/diagnóstico , Efeitos Adversos de Longa Duração/etiologia , Efeitos Adversos de Longa Duração/fisiopatologia , Efeitos Adversos de Longa Duração/terapia , Masculino , Inibidores da Agregação de Plaquetas/uso terapêutico , Período Pós-Operatório , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Sistema de Registros , Reimplante/efeitos adversos , Reimplante/métodos , Tempo
12.
Am J Cardiol ; 120(10): 1863-1868, 2017 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-28886850

RESUMO

Respiratory complications are a major factor contributing to postoperative morbidity and mortality, especially in patients with chronic obstructive pulmonary disease (COPD). Our objective was to compare the rate of respiratory complications in patients with COPD with severe aortic stenosis who underwent transcatheter aortic valve implantation (TAVI) versus surgical aortic valve replacement (SAVR). Low-to-intermediate surgical-risk patients with moderate or severe COPD who underwent TAVI or SAVR at 2 tertiary centers were included in this study. COPD was defined by the Global Initiative for Chronic Lung Disease classification. The primary end point was the 30-day composite of respiratory mortality, prolonged ventilation (>24 hours), the need for reintubation for respiratory causes, tracheostomy, acute respiratory distress syndrome, pneumonia, or pneumothorax. The inverse probability of treatment weighting was determined to reduce baseline imbalance between the 2 groups. A total of 321 patients (mean age 72.4 ± 9.3 years old, 74.5% male, mean Society of Thoracic Surgeons predicted risk of mortality 3.8 ± 1.9%, mean forced expiratory volume 1: 59 ± 13%) were included in the analysis. TAVI was performed in 122 patients, whereas 199 underwent SAVR. There were no differences between the 2 groups regarding the composite respiratory primary end point (SAVR 10.6%, TAVR 7.4%, adjusted odds ratio 0.57, 95% confidence interval 0.20 to 1.65, p = 0.30). Transfemoral TAVI without general anesthesia (28 patients) was associated with the lowest rate of respiratory complications (3.6%). Among patients with moderate or severe COPD at low-to-intermediate surgical risk, TAVI patients had a similar rate of 30-day major pulmonary complications compared with SAVR patients despite a higher baseline risk profile. Future studies should further investigate whether TAVI is associated with reduced respiratory complications, comparing transfemoral TAVI recipients treated with local anesthesia with their SAVR counterparts.


Assuntos
Estenose da Valva Aórtica/cirurgia , Valva Aórtica/cirurgia , Complicações Pós-Operatórias/epidemiologia , Doença Pulmonar Obstrutiva Crônica/complicações , Medição de Risco/métodos , Idoso , Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/complicações , Estenose da Valva Aórtica/mortalidade , Ecocardiografia , Feminino , Seguimentos , França/epidemiologia , Implante de Prótese de Valva Cardíaca/métodos , Humanos , Incidência , Masculino , Razão de Chances , Doença Pulmonar Obstrutiva Crônica/mortalidade , Quebeque/epidemiologia , Fatores de Risco , Índice de Gravidade de Doença , Taxa de Sobrevida/tendências , Fatores de Tempo , Substituição da Valva Aórtica Transcateter , Resultado do Tratamento
13.
Circulation ; 136(7): 632-643, 2017 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-28588077

RESUMO

BACKGROUND: At present, there are no objective data specifically examining the clinical impact of variations in exercise capacity post-transcatheter aortic valve replacement (TAVR). We describe the changes in exercise capacity between baseline and 6 months post-TAVR, and ascertain factors associated with and clinical implications of a lack of improvement in exercise capacity post-TAVR. METHODS: A total of 305 patients (mean age, 79±9 years; 44% men; Society of Thoracic Surgeons predicted risk mortality score, 6.7±4.2%) undergoing TAVR completed both baseline and follow-up exercise capacity assessments at 6 months post-TAVR. Exercise capacity was evaluated by the 6-minute walk test (6MWT). Clinical outcomes were compared between patients displaying greater than (n=152; improving group) versus less than (n=153; nonimproving group) the median percentage change in distance walked between baseline and 6-month follow-up examinations. The primary outcome measure was clinical event rates, measured from the 6-month post-TAVR period onward. Further dichotomization according to baseline 6MWT distance (less than versus more than median walking distance, or slow walker versus fast walker) was also assessed. RESULTS: The mean overall distances walked pre- and post-TAVR (6 months post-TAVR) were 204±119 and 263±116 m, respectively (Δ6MWT=60±106 m), with 219 (72%) patients demonstrating an increase in their walking distance (median percentage increase of the entire population was 20% [interquartile range, 0%-80%]). Factors independently correlated with reduced exercise capacity improvement included a range of baseline clinical characteristics (older age, female sex, chronic obstructive pulmonary disease; P<0.05 for all), periprocedural major or life-threatening bleeding (P=0.009) and new-onset anemia at 6 months post-TAVR (P=0.009). Failure to improve the 6MWT distance by at least 20% was independently associated with all-cause mortality (P=0.002) and cardiovascular death or rehospitalization for cardiovascular causes (P=0.001). Baseline slow walkers who were able to improve the 6MWT distance presented with significantly better outcomes than nonimprovers (P=0.01 for all-cause mortality; P=0.001 for cardiovascular end point). CONCLUSIONS: Approximately one-third of patients undergoing TAVR did not improve their exercise capacity postprocedure. The lack of functional improvement post-TAVR was predicted by a mix of baseline and periprocedural factors translating into poorer clinical outcomes. These results suggest that systematically implementing exercise capacity assessment pre- and post-TAVR may help to improve patient risk stratification.


Assuntos
Estenose da Valva Aórtica/cirurgia , Terapia por Exercício , Idoso , Idoso de 80 Anos ou mais , Estenose da Valva Aórtica/terapia , Doenças Cardiovasculares/diagnóstico , Doenças Cardiovasculares/etiologia , Doenças Cardiovasculares/mortalidade , Feminino , Seguimentos , Humanos , Masculino , Análise Multivariada , Prognóstico , Análise de Sobrevida , Substituição da Valva Aórtica Transcateter , Resultado do Tratamento
14.
Am J Med Genet A ; 173(5): 1309-1318, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28371260

RESUMO

Costello syndrome is part of the RASopathies, a group of neurocardiofaciocutaneous syndromes caused by deregulation of the RAS mitogen-activated protein kinase pathway. Heterozygous mutations in HRAS are responsible for Costello syndrome, with more than 80% of the patients harboring the specific p.Gly12Ser variant. These individuals show a homogeneous phenotype. The clinical characteristics of the Costello syndrome individuals harboring rarer HRAS mutations are less understood, due to the small number of reported cases. Here, we describe the phenotypic spectrum of five additional individuals with HRAS c.38G>A; p.Gly13Asp, including one with somatic mosaicism, and review five previously described cases. The facial and hair abnormalities of the HRAS p.Gly13Asp individuals differ from the typical pattern observed in those showing the common HRAS (p.Gly12Ser) mutation, with less coarse facial features and slow growing, sparse hair with abnormal texture, the latter resembling the pattern observed in Noonan syndrome-like disorder with loose anagen hair and individuals harboring another amino acid substitution in HRAS (p.Gly13Cys). Although some individuals with HRAS p.Gly13Asp developed papillomata and vascular proliferation lesions, no malignant tumors occurred, similar to what was reported for individuals harboring the HRAS p.Gly13Cys. The fact that no malignant tumors were described in these individuals does not allow definitive conclusions about the risk for cancer development. It remains to be determined if substitutions of amino acid 13 in HRAS (p.Gly13Asp and p.Gly13Cys) increase the risk of tumor development.


Assuntos
Anormalidades Múltiplas/genética , Síndrome de Costello/genética , Neoplasias/genética , Proteínas Proto-Oncogênicas p21(ras)/genética , Anormalidades Múltiplas/fisiopatologia , Adolescente , Adulto , Substituição de Aminoácidos/genética , Aminoácidos/genética , Criança , Pré-Escolar , Síndrome de Costello/complicações , Síndrome de Costello/fisiopatologia , Feminino , Mutação em Linhagem Germinativa , Humanos , Masculino , Mosaicismo , Neoplasias/complicações , Neoplasias/fisiopatologia , Fenótipo
15.
J Cardiopulm Rehabil Prev ; 37(4): 295-298, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28306684

RESUMO

PURPOSE: To report on the implementation and clinical outcomes of a community-based pulmonary rehabilitation program in rural Appalachia. METHODS: Three rural health centers and a large referral hospital worked together to establish pulmonary rehabilitation services based on AACVPR guidelines. Each site hired at least 1 respiratory therapist. To measure clinical outcomes, a retrospective medical record study compared pre- and post-program values for the modified Medical Research Council dyspnea level, 6-minute walk test (6MWT), negative inspiratory force (NIF), respiratory disease knowledge, St George Respiratory Questionnaire (SGRQ), BODE index (body mass index, airflow obstruction, dyspnea and exercise capacity), and smoking status. The percentages of persons completing the program and participating in maintenance exercise after the program were recorded. RESULTS: During the first 20 months of the program, 195 unduplicated persons with qualifying chronic lung diseases started the program. Of these, 111 (57%) completed the program. Mean improvements for all 6 measures were highly significant (P < .001) and compared favorably with published results from hospital-based programs: dyspnea level, -1.2; 6MWT, +259 ft; NIF, +11.3 cm H2O; knowledge test, +1.9; SGRQ, -6.2; BODE index, -1.1. Of the 23 smokers, 5 quit by the end of the program. CONCLUSIONS: Community-based pulmonary rehabilitation in rural health centers is feasible and achieves clinical outcomes similar to programs in large hospitals and academic centers. Furthermore, the addition of respiratory therapists to these primary care teams provides important collateral benefits for the evidence-based care of patients with chronic lung diseases.


Assuntos
Serviços de Saúde Comunitária/métodos , Avaliação de Programas e Projetos de Saúde/estatística & dados numéricos , Doença Pulmonar Obstrutiva Crônica/reabilitação , População Rural/estatística & dados numéricos , Região dos Apalaches , Dispneia/fisiopatologia , Dispneia/reabilitação , Teste de Esforço/estatística & dados numéricos , Tolerância ao Exercício/fisiologia , Humanos , Avaliação de Programas e Projetos de Saúde/métodos , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Estudos Retrospectivos , Inquéritos e Questionários , Resultado do Tratamento , West Virginia
16.
J Am Heart Assoc ; 6(2)2017 02 20.
Artigo em Inglês | MEDLINE | ID: mdl-28219920

RESUMO

BACKGROUND: The influence of coronary artery disease (CAD) on clinical and echocardiographic outcomes after transcatheter aortic valve replacement (TAVR) is still controversial. We sought to evaluate the impact of CAD severity as measured by the SYNTAX score (SS) on patients undergoing TAVR. METHODS AND RESULTS: A total of 377 patients who underwent TAVR in 2 high-volume centers in North America were included in our retrospective analysis. A blinded angiographic core laboratory calculated the SS on all available coronary angiograms with the use of quantitative coronary analysis. Patients were stratified into 4 groups: (1) no CAD (SS=0); (2) low SS (SS between 1 and 22); (3) intermediate SS (SS between 23 and 32); and (4) high SS (SS ≥33). Patients who had undergone percutaneous coronary intervention within 6 months prior to TAVR were separated into 2 categories based on their residual SS (<8 and ≥8). Patients with previous coronary artery bypass grafting (CABG) were divided into 2 groups: (1) low CABG SS and (2) high CABG SS. The primary end point was a composite of all-cause mortality, myocardial infarction, and stroke. At 30 days and 1 year, both the presence and the severity of CAD had no impact on the rate of the combined primary end point and on all-cause mortality, cardiovascular mortality, and myocardial infarction. Patients with less complete revascularization (residual SS ≥8 versus residual SS <8 and low CABG SS versus high CABG SS, had similar rates of the combined primary end point, all-cause mortality, cardiovascular mortality, MI, and stroke, at both 30 days and 1 year. CONCLUSIONS: In our core laboratory-validated study, neither the severity of CAD nor completeness of revascularization after percutaneous coronary intervention or CABG were associated with clinical outcomes after TAVR, at both 30 days and 1 year.


Assuntos
Estenose da Valva Aórtica/cirurgia , Doença da Artéria Coronariana/diagnóstico , Intervenção Coronária Percutânea , Substituição da Valva Aórtica Transcateter , Idoso de 80 Anos ou mais , Estenose da Valva Aórtica/complicações , Estenose da Valva Aórtica/diagnóstico , Causas de Morte/tendências , Angiografia Coronária , Doença da Artéria Coronariana/complicações , Doença da Artéria Coronariana/mortalidade , Ecocardiografia , Feminino , Seguimentos , Humanos , Masculino , América do Norte/epidemiologia , Estudos Retrospectivos , Índice de Gravidade de Doença , Taxa de Sobrevida/tendências , Fatores de Tempo , Resultado do Tratamento
17.
EuroIntervention ; 13(1): 44-52, 2017 May 15.
Artigo em Inglês | MEDLINE | ID: mdl-28067195

RESUMO

AIMS: The aim of this study was to evaluate, in anaemic patients, the efficacy of erythropoietin (EPO) in reducing red cell (RC) transfusion rates post TAVI. METHODS AND RESULTS: This was a randomised double-blind trial. Patients with severe symptomatic aortic stenosis and concomitant anaemia with an indication for TAVI were randomised (1:1) to receive two weight-based doses of EPO (darbepoetin alfa)+iron or placebo at days 10 (±4 days) and 1 (±1 day) pre TAVI. The primary outcome was the rate of RC transfusions at 30 days. A total of 100 patients (mean age 81±7 years, male 49%) were included: 48 patients received EPO (+iron) and 52 patients received placebo. Baseline characteristics and procedural findings were well balanced between groups except for baseline haemoglobin levels, which were lower in those patients receiving EPO (10.7±1.2 vs. 11.3±1.1 g/dl, p=0.01). The rate of 30-day RC transfusion was similar in both groups (27.1 vs. 25.0% in the EPO and placebo groups, respectively; adjusted odds ratio 1.05, 95% CI: 0.42-2.64, p=0.92), and no differences were observed in the number of RC units per transfused patient (1 [1-3] vs. 2 [1-2] in the EPO and placebo groups, respectively, adjusted p=0.99). Rates of 30-day mortality, stroke, new-onset atrial fibrillation, acute kidney injury, and troponin peak were also similar between groups (p>0.20 for all). CONCLUSIONS: EPO (+iron) administration failed to reduce RC transfusion rates or the per-patient number of transfusion units in anaemic patients undergoing TAVI.


Assuntos
Anemia/tratamento farmacológico , Eritropoetina/uso terapêutico , Ferro/uso terapêutico , Substituição da Valva Aórtica Transcateter , Idoso , Idoso de 80 Anos ou mais , Método Duplo-Cego , Transfusão de Eritrócitos/métodos , Eritropoetina/metabolismo , Feminino , Humanos , Masculino , Substituição da Valva Aórtica Transcateter/métodos
18.
Pediatrics ; 138(6)2016 12.
Artigo em Inglês | MEDLINE | ID: mdl-27940666

RESUMO

RASopathies, such as Noonan, Costello, and cardio-facio-cutaneous syndromes, are developmental disorders caused by mutations in rat sarcoma-mitogen-activated protein kinase pathway genes. Mutations that cause Noonan syndrome have been associated with delayed puberty. Here we report 4 patients with either Costello or cardio-facio-cutaneous syndrome who developed precocious puberty, suggesting complex regulation of the hypothalamic-pituitary-gonadal axis and the timing of puberty by the rat sarcoma-mitogen-activated protein kinase pathway. Additional study of the timing of puberty among patients with RASopathies is warranted to ascertain the incidence of delayed and precocious puberty in these conditions and to examine genotype-phenotype correlations, which may provide insight into pathways that regulate the timing of puberty.


Assuntos
Predisposição Genética para Doença/epidemiologia , Proteínas Proto-Oncogênicas p21(ras)/genética , Puberdade Tardia/etiologia , Puberdade Precoce/etiologia , Maturidade Sexual/genética , Anormalidades Múltiplas/diagnóstico , Anormalidades Múltiplas/epidemiologia , Adolescente , Criança , Síndrome de Costello/complicações , Síndrome de Costello/genética , Displasia Ectodérmica/complicações , Displasia Ectodérmica/genética , Facies , Insuficiência de Crescimento/complicações , Insuficiência de Crescimento/genética , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/genética , Humanos , Incidência , Masculino , Síndrome de Noonan/complicações , Síndrome de Noonan/genética , Puberdade Tardia/fisiopatologia , Puberdade Precoce/fisiopatologia , Medição de Risco , Amostragem , Sensibilidade e Especificidade , Maturidade Sexual/fisiologia
19.
J Am Coll Cardiol ; 68(20): 2129-2141, 2016 11 15.
Artigo em Inglês | MEDLINE | ID: mdl-27692728

RESUMO

BACKGROUND: Data regarding the mid- to long-term cognitive trajectory of transcatheter aortic valve (TAVR) recipients are scarce. OBJECTIVES: Changes in global cognition and specific cognitive domains up to 1 year post-TAVR were evaluated. METHODS: Fifty-one patients (median age 80.0 [interquartile range: 72.0 to 85.0] years; 37% women) underwent TAVR and prospective assessment of cognitive function using the Montreal Cognitive Assessment (MoCA) at baseline, short-term (30 days), and 1 year post-TAVR. Processing speed and executive cognitive functions were further evaluated with the digit-symbol substitution test (DSST), Trail Making Tests (TMT), and verbal fluency tests at the same time points. Cognitive decline (CD) was determined by changes in mean scores and as a rate using practice-corrected reliable change index (RCI). RESULTS: The baseline mean total MoCA score was 22.71 ± 3.84. Twenty patients (39.2%) were considered cognitively impaired using a cutoff of <23 of 30 points. Mean total MoCA score improved at short-term post-TAVR and remained stable at 1 year (p = 0.022). On the basis of the RCI of total MoCA score, 4 patients (7.8%) presented with short-term CD, which persisted at 1 year in 1 patient (2.0%). Four patients (7.8%) exhibited cognitive improvement at 1 year, increasing to 15% among those with baseline cognitive impairment. No significant changes were observed over time in the mean DSST, TMT, and verbal fluency test scores. On the basis of the RCI, 10 of 40 patients (25%) presented with a reduction in performance of at least 1 test at 30 days that persisted at 1 year in 4 patients (10%). CONCLUSIONS: TAVR was associated with global improvement in cognitive status, more pronounced among those with cognitive impairment pre-TAVR. However, early decline in some complex cognitive functions was observed in one-quarter of TAVR recipients, persisting at 1 year in 10% of patients.


Assuntos
Estenose da Valva Aórtica/cirurgia , Cognição/fisiologia , Disfunção Cognitiva/fisiopatologia , Complicações Pós-Operatórias , Substituição da Valva Aórtica Transcateter/efeitos adversos , Idoso , Idoso de 80 Anos ou mais , Estenose da Valva Aórtica/fisiopatologia , Disfunção Cognitiva/etiologia , Feminino , Seguimentos , Humanos , Masculino , Prognóstico , Estudos Prospectivos , Fatores de Risco , Índice de Gravidade de Doença , Fatores de Tempo
20.
J Card Surg ; 31(10): 617-622, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27577965

RESUMO

BACKGROUND: Stroke remains one of the most worrisome complications following transcatheter aortic valve replacement (TAVR). This pilot study evaluates the safety, feasibility, and exploratory efficacy of the TriGuard HDH embolic deflection device (Keystone Heart Ltd., Caesarea, Israel) in patients undergoing transaortic TAVR. METHODS: A total of 10 patients (median age: 81 years, STS score: 9.6 ± 5.6%) undergoing transaortic TAVR were included. All 30-day events were recorded and defined according to Valve Academic Research Consortium-2 criteria. Cerebral diffusion-weighted magnetic resonance imaging exams were planned preprocedure and within 10 days post-TAVR. The results of the magnetic resonances were analyzed in an independent core laboratory blinded to clinical data. Neurocognitive evaluation tests (Montreal Cognitive Assessment, Cogstate, Digit Symbol Substitution Test, Word Fluency Test, and Trailmaking tests) were performed at baseline, and within 10 and 30 days post-TAVR. RESULTS: The TriGuard HDH device was successfully deployed in all patients without complications. There was one procedural major vascular complication unrelated to the study device, and no clinically apparent stroke events were observed at 30-day follow-up. Cerebral diffusion weighted magnetic resonance imaging exams were performed in six patients at 7.5 ± 1.9 days post-TAVR showing the presence of new ischemic lesions in five patients (83.3%), which were single lesions in 60% of these individuals. Paired neurocognitive evaluation tests demonstrated no significant changes in neurocognitive parameters over time. CONCLUSIONS: This study shows the safety and feasibility of using the TriGuard HDH embolic protection device in transaortic TAVR. Further studies are warranted to determine the efficacy of embolic protection in this population.


Assuntos
Estenose da Valva Aórtica/cirurgia , Valva Aórtica/cirurgia , Dispositivos de Proteção Embólica , Embolia Intracraniana/prevenção & controle , Substituição da Valva Aórtica Transcateter/efeitos adversos , Idoso , Idoso de 80 Anos ou mais , Estenose da Valva Aórtica/diagnóstico , Imagem de Difusão por Ressonância Magnética , Desenho de Equipamento , Estudos de Viabilidade , Feminino , Fluoroscopia , Humanos , Embolia Intracraniana/etiologia , Masculino , Projetos Piloto , Fatores de Tempo
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