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2.
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.
Am Heart J ; 225: 78-87, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32474207

RESUMO

BACKGROUND: The severity of aortic coarctation (CoA) may be underestimated during cardiac catheterization. We aimed to investigate whether epinephrine stress testing improves clinical decision making and outcome in CoA. METHODS: We retrospectively evaluated CoA patients >50 kg with a peak systolic gradient (PSG) ≤20 mm Hg during cardiac catheterization who underwent epinephrine stress testing. Subsequent interventional management (stenting or balloon dilatation), complications, and medium-term clinical outcome were assessed. RESULTS: Fifty CoA patients underwent cardiac catheterization with epinephrine stress testing. Patients with a high epinephrine PSG (>20 mm Hg; n = 24) were younger and more likely to have a hypertensive response to exercise compared to patients with a low epinephrine PSG (≤20 mm Hg; n = 26). In total, 21 patients (88%) with a high epinephrine PSG underwent intervention, and 20 patients (77%) with a low epinephrine PSG were treated conservatively. After a mean follow-up of 25 ±â€¯18 months, there was a lower prevalence of hypertension in patients with a high epinephrine PSG who underwent intervention compared to patients with a low epinephrine PSG treated conservatively (19% vs. 76%; P = .001). In a multivariate model, intervention was independently associated with a 14.3-mm Hg reduction in systolic blood pressure (P = .001) and a decrease in the use of antihypertensive agents. CONCLUSIONS: In CoA patients with a low baseline PSG but high epinephrine PSG, percutaneous intervention is associated with a substantial reduction in systemic blood pressure and the use of antihypertensive medication. Accordingly, epinephrine stress testing may be a useful addition in the evaluation of CoA.


Assuntos
Agonistas Adrenérgicos beta/farmacologia , Coartação Aórtica/cirurgia , Pressão Sanguínea/efeitos dos fármacos , Cateterismo Cardíaco , Epinefrina/farmacologia , Adolescente , Adulto , Coartação Aórtica/diagnóstico , Coartação Aórtica/fisiopatologia , Tomada de Decisão Clínica , Teste de Esforço/métodos , Feminino , Humanos , Hipertensão/etiologia , Masculino , Estudos Retrospectivos , Sístole/fisiologia , Adulto Jovem
5.
Am J Cardiol ; 129: 87-94, 2020 08 15.
Artigo em Inglês | MEDLINE | ID: mdl-32593432

RESUMO

Several studies have reported aortic dilation and increased stiffness of the ascending aorta in patients after repair of congenital heart disease (CHD), which may be a predominant cardiovascular risk. However, the clinical significance has not been described in detail. In this retrospective study, 175 repaired patients with complex CHD achieving biventricular circulation and age-matched 39 control subjects were reviewed (median age: 14.9 and 15.7 years, respectively). We measured the diameters of the ascending aorta and descending aorta from catheterization angiograms to yield Z-scores and stiffness indexes (ß) using diameter fluctuations corresponding to pulsatile pressures. Clinical profile, peak oxygen uptake during the cardiopulmonary exercise test, and incidence of unscheduled hospitalization during follow-up was also reviewed. Compared with controls, patients with complex CHD, except for those with aortic coarctation, exhibited significant dilation and increased stiffness of the aortic root and ascending aorta, but not of the descending aorta. In this CHD population (n = 147, including 112 conotruncal anomalies), exercise capacities correlated independently with the diameter Z-score and stiffness index of the ascending aorta along with the history of repetitive thoracotomies, reduced forced vital capacity, and right ventricular hypertension. During a follow-up period (median 15.6 years), either dilation (Z-score >3.5) or increased stiffness (ß >6.0) of the ascending aorta stratified morbidity, but no synergistic impact was detected. In conclusion, in repaired patients with complex CHD, a stiffened and dilated ascending aorta was frequently found, exerting significant adverse impacts on diminished exercise capacity and morbidity.


Assuntos
Aorta/fisiopatologia , Doenças da Aorta/fisiopatologia , Tolerância ao Exercício/fisiologia , Cardiopatias Congênitas/fisiopatologia , Rigidez Vascular/fisiologia , Adolescente , Coartação Aórtica/fisiopatologia , Coartação Aórtica/cirurgia , Insuficiência da Valva Aórtica/fisiopatologia , Estenose da Valva Aórtica/fisiopatologia , Transposição das Grandes Artérias , Procedimentos Cirúrgicos Cardíacos , Estudos de Casos e Controles , Criança , Dilatação Patológica/fisiopatologia , Dupla Via de Saída do Ventrículo Direito/fisiopatologia , Dupla Via de Saída do Ventrículo Direito/cirurgia , Feminino , Cardiopatias Congênitas/cirurgia , Humanos , Masculino , Estudos Retrospectivos , Tetralogia de Fallot/fisiopatologia , Tetralogia de Fallot/cirurgia , Transposição dos Grandes Vasos/fisiopatologia , Transposição dos Grandes Vasos/cirurgia , Persistência do Tronco Arterial/fisiopatologia , Persistência do Tronco Arterial/cirurgia
6.
BMC Cardiovasc Disord ; 20(1): 216, 2020 05 11.
Artigo em Inglês | MEDLINE | ID: mdl-32393237

RESUMO

BACKGROUND: Jehovah's Witnesses refuse allogeneic blood transfusions, which makes cardiovascular surgery challenging. Surgeons must minimize blood and fluid loss within one procedure. CASE PRESENTATION: We herein describe a 17-year-old male Jehovah's Witness with bicuspid aortic valve regurgitation and coarctation of the aorta. The procedures were performed in the following order: aortic valve replacement combined with Nick's aortic root enlargement, right axillary artery-bilateral external iliac artery bypass, and distal arch-descending aorta bypass. CONCLUSIONS: Axillary artery-bilateral external iliac artery bypass maintained distal perfusion and reduced the amount of heparin during distal arch-descending aorta bypass surgery.


Assuntos
Coartação Aórtica/cirurgia , Insuficiência da Valva Aórtica/cirurgia , Valva Aórtica/anormalidades , Transposição das Grandes Artérias , Perda Sanguínea Cirúrgica/prevenção & controle , Doenças das Valvas Cardíacas/cirurgia , Implante de Prótese de Valva Cardíaca , Testemunhas de Jeová , Recuperação de Sangue Operatório , Religião e Medicina , Adolescente , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/fisiopatologia , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/fisiopatologia , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/fisiopatologia , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/fisiopatologia , Humanos , Masculino , Resultado do Tratamento
7.
Vasc Endovascular Surg ; 54(5): 463-466, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32338186

RESUMO

Aortic isthmic atresia is a severe form of aortic coarctation where there is loss of luminal communication at the aortic isthmus. The primary approach for correcting aortic isthmic atresia has been surgical repair of the coarctation. A small number of case series have shown that percutaneous correction of aortic isthmic atresia is possible. We describe 3 cases of aortic isthmic atresia that was successfully treated using a percutaneous approach. Our cases ranged in age between 42 and 51 years, and they all had hypertension. In our case series, 2 patients were successfully treated with radiofrequency perforation and 1 patient had anterograde recanalization performed using a stiff wire. Our patients have been followed up for between 2 and 4 years post-procedure, and they continue to do well. The success of percutaneous management in this case series adds to the small but increasing amount of data available in support of endovascular management of aortic isthmic atresia in adult patients.


Assuntos
Coartação Aórtica/terapia , Procedimentos Endovasculares , Adulto , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/fisiopatologia , Procedimentos Endovasculares/instrumentação , Humanos , Masculino , Pessoa de Meia-Idade , Stents , Resultado do Tratamento , Dispositivos de Acesso Vascular
8.
Medicine (Baltimore) ; 99(9): e19297, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32118745

RESUMO

To evaluate the utility of echocardiography (echo) in the diagnosis of persistent fifth aortic arch (PFAA), a very rare congenital aortic arch anomaly, and to compare echo and computed tomography angiography (CTA) imaging findings to improve our understanding of this anomaly.Data on the clinical diagnosis, imaging findings, and clinical management of PFAA were retrospectively analyzed in 10 suspected cases of PFAA admitted to our hospital between January 2012 and February 2017. We compared echo as a first line examination modality, and CTA and surgery results as the gold standard. Weinberg's classification was used to classify the type of PFAA.All patients (100%) received echo examination, eight patients (80%) received CTA examination, and four patients (40%) received sternotomy surgery; all recovered well after surgery. According to Weinberg's classification, 2, 6, and 2 cases (20%, 60%, and 20%) were classified as Type A, B, and C, respectively. Echo was able to diagnose 5 cases of PFAA (1 Type A case and 4 Type B cases) in the first instance. The diagnostic conformance rate of echo was 62.5% after comparisons with CTA and surgery results.The clinical manifestation of PFAA was atypical, and its diagnosis depended primarily on medical imaging. Echo has a relatively high diagnostic accuracy for PFAA, which is very valuable for its early detection.


Assuntos
Coartação Aórtica/diagnóstico , Angiografia por Tomografia Computadorizada/normas , Ecocardiografia/normas , Coartação Aórtica/fisiopatologia , Criança , Pré-Escolar , Angiografia por Tomografia Computadorizada/métodos , Angiografia por Tomografia Computadorizada/estatística & dados numéricos , Ecocardiografia/métodos , Ecocardiografia/estatística & dados numéricos , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos
9.
Circ Cardiovasc Imaging ; 13(2): e009672, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-32069118

RESUMO

BACKGROUND: Patients with vascular stiffening may display increased arterial afterload that is out of proportion to systolic blood pressure (SBP). Since vascular and endothelial dysfunction develop in patients with coarctation of aorta (COA), we hypothesized that for any SBP, patients with mild COA (COA peak velocity <2 m/s) will have a higher arterial afterload and increased left ventricular mass index (LVMI) compared with controls, and that Doppler-derived arterial load indices would be a better predictor of LVMI compared with SBP alone. METHODS: We studied 204 COA patients (age 35±12 y) and 204 matched controls. Doppler-derived arterial afterload was assessed using effective arterial elastance index and total arterial compliance index. RESULTS: Despite similar SBP, the mild COA group displayed higher arterial afterload as evidenced by a higher elastance index (3.3±0.9 versus 2.9±0.7 mm Hg/mL·m2; P<0.001) and lower total arterial compliance index (0.8±0.3 versus 1.2±0.5 mL/mm Hg·m2; P<0.001). This was associated with higher LVMI in COA (109±35 versus 93±32, g/m2; P<0.001). Compared with SBP (ß=0.24 [95% CI, 0.02-0.45]), elastance index (ß=20.2 [95% CI, 15.8-44.1]) and total arterial compliance index (ß=-32.5 [95% CI, -43.8 to -123.6]) were better predictors of LVMI. Elastance index (but not SBP) was predictive of longitudinal increases in LVMI (r=0.43, P<0.001). CONCLUSIONS: COA patients had higher arterial afterload compared with controls with similar SBP. In comparison to SBP, Doppler-derived arterial load indices correlate more strongly with LV hypertrophy. These data suggest that SBP may underestimate LV afterload in this population. This has important clinical implications since titration of antihypertensive therapy is currently based on SBP.


Assuntos
Coartação Aórtica/diagnóstico , Pressão Sanguínea/fisiologia , Ecocardiografia Doppler/métodos , Ventrículos do Coração/diagnóstico por imagem , Rigidez Vascular/fisiologia , Função Ventricular Esquerda/fisiologia , Adulto , Coartação Aórtica/fisiopatologia , Feminino , Seguimentos , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Reprodutibilidade dos Testes , Estudos Retrospectivos , Sístole
10.
Circ Heart Fail ; 13(2): e006651, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-32059629

RESUMO

BACKGROUND: Coarctation of aorta (COA) results in chronic left ventricular (LV) pressure overload and subsequently leads to LV diastolic dysfunction and heart failure over time. The goal of COA intervention is to prevent these complications. The timing of COA interventions is based on the presence of these COA severity indices: doppler mean COA gradient, systolic blood pressure, upper-to-lower-extremity SBP gradient, aortic isthmus ratio, presence of collaterals, and exercise-induced hypertension. Although these indices are physiologically intuitive, the relationship between these indices and LV diastolic dysfunction and exertional symptoms has not been studied. The purpose of this study was to evaluate the association between the indices of COA severity and LV diastolic function and symptoms. METHODS: In this cross-sectional study, multivariate linear and logistic regression analyses were used to assess the correlation between indices of COA severity, LV diastolic function (average e' and E/e'), and exertional symptoms (NYHA II-IV and peak oxygen consumption). RESULTS: Of all the COA indices analyzed in 546 adult COA patients, aortic isthmus ratio had the strongest correlation with e' (ß [95% CI]: 3.11 [2.02-4.31]; P=0.014) per 1 cm/second; E/e' (-13.4 [-22.3 to -4.81]; P=0.009) per 1 unit; peak oxygen consumption (4.05 [1.97-6.59] per 1% change, P=0.019), and NYHA II to IV symptoms (odds ratio, 2.16 [1.65-3.18]; P=0.006). CONCLUSIONS: Of all the COA severity indices stipulated in the guidelines, aortic isthmus ratio had the strongest correlation with LV diastolic function and exertional symptoms. As LV diastolic dysfunction typically precede heart failure symptoms, we anticipate that the results of this study will improve and simplify patient selection for COA intervention and potentially improve long-term outcomes.


Assuntos
Coartação Aórtica/complicações , Tolerância ao Exercício , Disfunção Ventricular Esquerda/etiologia , Função Ventricular Esquerda , Adulto , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/fisiopatologia , Estudos Transversais , Diástole , Ecocardiografia Doppler , Feminino , Humanos , Masculino , Consumo de Oxigênio , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/fisiopatologia , Adulto Jovem
11.
Catheter Cardiovasc Interv ; 95(2): 294-299, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31609061

RESUMO

Computational fluid dynamics (CFD) can be used to analyze blood flow and to predict hemodynamic outcomes after interventions for coarctation of the aorta and other cardiovascular diseases. We report the first use of cardiac 3-dimensional rotational angiography for CFD and show not only feasibility but also validation of its hemodynamic computations with catheter-based measurements in three patients.


Assuntos
Angioplastia com Balão , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/terapia , Aortografia , Hemodinâmica , Imageamento Tridimensional , Modelos Cardiovasculares , Modelagem Computacional Específica para o Paciente , Adolescente , Angioplastia com Balão/instrumentação , Coartação Aórtica/fisiopatologia , Criança , Estudos de Viabilidade , Feminino , Humanos , Hidrodinâmica , Masculino , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Stents , Resultado do Tratamento
12.
Ann Vasc Surg ; 63: 336-381, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31344467

RESUMO

BACKGROUND: The analysis of the correlation between blood flow and aortic pathology through computational fluid dynamics (CFD) shows promise in predicting disease progression, the effect of operative intervention, and guiding patient treatment. However, to date, there has not been a comprehensive systematic review of the published literature describing CFD in aortic diseases and their treatment. METHODS: This review includes 136 published articles which have investigated the application of CFD in all types of aortic disease (aneurysms, dissections, and coarctation). We took into account case studies of both, treated or untreated pathology, investigated with CFD. We also graded all studies using an author-defined Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach based on the validation method used for the CFD results. RESULTS: There are no randomized controlled trials assessing the efficacy of CFD as applied to aortic pathology, treated or untreated. Although a large number of observational studies are available, those using clinical imaging tools as independent validation of the calculated CFD results exist in far smaller numbers. Only 21% of all studies used clinical imaging as a tool to validate the CFD results and these were graded as high-quality studies. CONCLUSIONS: Contemporary evidence shows that CFD can provide additional hemodynamic parameters such as wall shear stress, vorticity, disturbed laminar flow, and recirculation regions in untreated and treated aortic disease. These have the potential to predict the progression of aortic disease, the effect of operative intervention, and ultimately help guide the choice and timing of treatment to the benefit of patients and clinicians alike.


Assuntos
Aneurisma Dissecante/fisiopatologia , Aorta/fisiopatologia , Aneurisma Aórtico/fisiopatologia , Coartação Aórtica/fisiopatologia , Hemodinâmica , Modelos Cardiovasculares , Modelagem Computacional Específica para o Paciente , Aneurisma Dissecante/diagnóstico por imagem , Aneurisma Dissecante/terapia , Aorta/anormalidades , Aorta/diagnóstico por imagem , Aneurisma Aórtico/diagnóstico por imagem , Aneurisma Aórtico/terapia , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/terapia , Humanos , Hidrodinâmica , Valor Preditivo dos Testes , Prognóstico , Fluxo Sanguíneo Regional
13.
J Vasc Surg ; 71(2): 505-516.e4, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31153701

RESUMO

OBJECTIVE: Suprarenal abdominal aortic coarctation (SAAC) alters flow and pressure patterns to the kidneys and is often associated with severe angiotensin-mediated hypertension refractory to drug therapy. SAAC is most often treated by a thoracoabdominal bypass (TAB) or patch aortoplasty (PA). It is currently unclear what effect these interventions have on renal flow and pressure waveforms. This study, using retrospective data from a patient with SAAC subjected to a TAB, undertook computational modeling to analyze aortorenal blood flow preoperatively as well as postoperatively after a variety of TAB and PA interventions. METHODS: Patient-specific anatomic models were constructed from preoperative computed tomography angiograms of a 9-year-old child with an isolated SAAC. Fluid-structure interaction (FSI) simulations of hemodynamics were performed to analyze preoperative renal flow and pressure waveforms. A parametric study was then performed to examine the hemodynamic impact of different bypass diameters and patch oversizing. RESULTS: Preoperative FSI results documented diastole-dominated renal perfusion with considerable high-frequency disturbances in blood flow and pressure. The postoperative TAB right and left kidney volumes increased by 58% and 79%, respectively, reflecting the increased renal artery blood flows calculated by the FSI analysis. Postoperative increases in systolic flow accompanied decreases in high-frequency disturbances, aortic pressure, and collateral flow after all surgical interventions. In general, lesser degrees of high-frequency disturbances followed PA interventions. High-frequency disturbances were eliminated with the 0% PA in contrast to the 30% and 50% PA oversizing and TAB interventions, in which these flow disturbances remained. CONCLUSIONS: Both TAB and PA dramatically improved renal artery flow and pressure waveforms, although disturbed renal waveforms remained in many of the surgical scenarios. Importantly, only the 0% PA oversizing scenario eliminated all high-frequency disturbances, resulting in nearly normal aortorenal blood flow. The study also establishes the relevance of patient-specific computational modeling in planning interventions for the midaortic syndrome.


Assuntos
Aorta Abdominal/cirurgia , Coartação Aórtica/fisiopatologia , Coartação Aórtica/cirurgia , Modelagem Computacional Específica para o Paciente , Artéria Renal/fisiopatologia , Criança , Feminino , Humanos , Fluxo Sanguíneo Regional , Circulação Renal , Estudos Retrospectivos , Procedimentos Cirúrgicos Vasculares/métodos
14.
JAMA Dermatol ; 156(2): 186-190, 2020 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-31825455

RESUMO

Importance: Oral propranolol is widely considered to be first-line therapy for complicated infantile hemangioma, but its use in patients with PHACE (posterior fossa malformations, hemangioma, arterial anomalies, cardiac defects, eye anomalies) syndrome has been debated owing to concerns that the cardiovascular effects of the drug may increase the risk for arterial ischemic stroke. Objective: To assess the incidence of adverse events among patients with PHACE syndrome receiving oral propranolol for infantile hemangioma. Design, Setting, and Participants: This multicenter retrospective cohort study assessed the incidence of adverse events among 76 patients with PHACE syndrome receiving oral propranolol for infantile hemangioma at 11 tertiary care, academic pediatric dermatology practices. Medical records from January 1, 2010, through April 25, 2017, were reviewed. Exposures: Patients received oral propranolol, 0.3 mg/kg/dose or more. Main Outcomes and Measures: The main outcome was the rate and severity of adverse events occurring throughout the course of treatment with oral propranolol, as documented in the medical records. Adverse events were graded from 1 to 5 using a scale derived from the Common Terminology Criteria for Adverse Events and were considered to be serious if they were grade 3 or higher. Results: A total of 76 patients (59 girls and 17 boys; median age at propranolol initiation, 56 days [range, 0-396 days]) met the inclusion criteria. There were no reports of serious adverse events (ie, stroke, transient ischemic attack, or cardiovascular events) during treatment with oral propranolol. A total of 46 nonserious adverse events were reported among 29 patients (38.2%); the most commonly reported nonserious adverse events were sleep disturbances and minor gastrointestinal tract and respiratory tract symptoms. In a comparison with 726 infants who received oral propranolol for hemangioma but did not meet criteria for PHACE syndrome, there was no significant difference in the rate of serious adverse events experienced during treatment (0 of 76 patients with PHACE syndrome and 3 of 726 patients without PHACE syndrome [0.4%]). Conclusions and Relevance: This study found that oral propranolol was used to treat infantile hemangioma in 76 patients with PHACE syndrome and that no serious adverse events were experienced. These data provide support for the safety of oral propranolol in this patient population.


Assuntos
Antagonistas Adrenérgicos beta/administração & dosagem , Coartação Aórtica/fisiopatologia , Anormalidades do Olho/fisiopatologia , Hemangioma/tratamento farmacológico , Síndromes Neurocutâneas/fisiopatologia , Propranolol/administração & dosagem , Administração Oral , Antagonistas Adrenérgicos beta/efeitos adversos , Estudos de Coortes , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Propranolol/efeitos adversos , Estudos Retrospectivos , Resultado do Tratamento
15.
Am J Cardiol ; 125(1): 153-156, 2020 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-31699361

RESUMO

Aortic coarctation presents in adult life in a significant number of patients. The preferred treatment in this age group is percutaneous stent implantation without which life expectancy is reduced due to the complications of uncontrolled hypertension. We present the first case of an "uncrossable" aortic coarctation transversed using the BEACON (Brief Electrification of a guidewire to assist in crossing an Aortic CoarctatiON) technique, a novel approach incorporating the electrification of a guidewire. It requires standard transcaval equipment and techniques but crosses from aortic lumen-to-lumen instead of cava-to-aorta, thus creating a rail for successful stent delivery.


Assuntos
Coartação Aórtica/cirurgia , Implante de Prótese Vascular/instrumentação , Cateterismo Cardíaco/métodos , Stents , Coartação Aórtica/diagnóstico , Coartação Aórtica/fisiopatologia , Desenho de Equipamento , Humanos , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X
16.
BMC Cardiovasc Disord ; 19(1): 286, 2019 12 12.
Artigo em Inglês | MEDLINE | ID: mdl-31830907

RESUMO

BACKGROUND: Patients after aortic coarctation (CoA) repair show impaired aortic bioelasticity and altered left ventricular (LV) mechanics, predisposing diastolic dysfunction. Our purpose was to assess aortic bioelasticity and LV properties in CoA patients who underwent endovascular stenting or surgery using cardiovascular magnetic resonance (CMR) imaging. METHODS: Fifty CoA patients (20.5 ± 9.5 years) were examined by 3-Tesla CMR. Eighteen patients had previous stent implantation and 32 had surgical repair. We performed volumetric analysis of both ventricles (LV, RV) and left atrium (LA) to measure biventricular volumes, ejection fractions, left atrial (LA) volumes, and functional parameters (LAEFPassive, LAEFContractile, LAEFReservoir). Aortic distensibility and pulse wave velocity (PWV) were assessed. Native T1 mapping was applied to examine LV tissue properties. In twelve patients post-contrast T1 mapping was performed. RESULTS: LV, RV and LA parameters did not differ between the surgical and stent group. There was also no significant difference for aortic distensibility, PWV and T1 relaxation times. Aortic root distensibility correlated negatively with age, BMI, BSA and weight (p < 0.001). Native T1 values correlated negatively with age, weight, BSA and BMI (p < 0.001). Lower post-contrast T1 values were associated with lower aortic arch distensibility and higher aortic arch PWV (p < 0.001). CONCLUSIONS: CoA patients after surgery or stent implantation did not show significant difference of aortic elasticity. Thus, presumably other factors like intrinsic aortic abnormalities might have a greater impact on aortic elasticity than the approach of repair. Interestingly, our data suggest that native T1 values are influenced by demographic characteristics.


Assuntos
Angioplastia com Balão , Aorta/cirurgia , Coartação Aórtica/terapia , Imagem Cinética por Ressonância Magnética , Rigidez Vascular , Procedimentos Cirúrgicos Vasculares , Adolescente , Adulto , Angioplastia com Balão/efeitos adversos , Angioplastia com Balão/instrumentação , Aorta/anormalidades , Aorta/diagnóstico por imagem , Aorta/fisiopatologia , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/fisiopatologia , Função do Átrio Esquerdo , Criança , Pré-Escolar , Elasticidade , Feminino , Humanos , Masculino , Valor Preditivo dos Testes , Estudos Prospectivos , Recuperação de Função Fisiológica , Stents , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Função Ventricular Esquerda , Adulto Jovem
17.
Thorac Cardiovasc Surg ; 67(S 04): e1-e10, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31675756

RESUMO

BACKGROUND: Even after successful aortic coarctation (CoA) repair, hypertension causes premature morbidity and mortality. The mechanisms are not clear. The aim was to evaluate elastic wall properties and aortic morphology and to correlate these results with severity of restenosis, hypertension, aortic arch geometry, noninvasive pressure gradients, and time and kind of surgical procedure. METHODS: Eighty-nine patients (17 ± 6.3 years) and 20 controls (18 ± 4.9 years) were examined using magnetic resonance imaging (MRI). In addition to contrast-enhanced MR angiography and flow measurements, CINE MRI was performed to assess the relative change of aortic cross-sectional areas at diaphragm level to calculate aortic compliance (C). RESULTS: Fifty-four percent of all patients showed hypertension (> 95th percentile), but more than half of them had no significant stenosis (defined as ≥30%). C was lower in CoA than in controls (3.30 ± 2.43 vs. 4.67 ± 2.21 [10-5 Pa-1 m-2]; p = 0.024). Significant differences in compliance were found between hyper- and normotensive patients (2.61 ± 1.60 vs. 4.11 ± 2.95; p = 0.01), and gothic and Romanesque arch geometry (2.64 ± 1.58 vs. 3.78 ± 2.81; p = 0.027). There was a good correlation between C and hypertension (r = 0.671; p < 0.01), but no correlation between C (and hypertension) and time or kind of repair, restenosis, or pressure gradients. CONCLUSION: The decreased compliance, a high rate of hypertension without restenosis, and independency of time and kind of repair confirm the hypothesis that CoA may not be limited to isthmus region but rather be a widespread (systemic) vascular anomaly at least in some of the CoA patients. Therefore, aortic compliance should be assessed in these patients to individually tailor treatment of CoA patients with restenosis and/or hypertension.


Assuntos
Aorta/diagnóstico por imagem , Coartação Aórtica/diagnóstico por imagem , Pressão Arterial , Hipertensão/diagnóstico por imagem , Angiografia por Ressonância Magnética , Imagem Cinética por Ressonância Magnética , Rigidez Vascular , Adolescente , Adulto , Anti-Hipertensivos/uso terapêutico , Aorta/anormalidades , Aorta/fisiopatologia , Aorta/cirurgia , Coartação Aórtica/complicações , Coartação Aórtica/fisiopatologia , Coartação Aórtica/cirurgia , Pressão Arterial/efeitos dos fármacos , Criança , Pré-Escolar , Feminino , Humanos , Hipertensão/tratamento farmacológico , Hipertensão/etiologia , Hipertensão/fisiopatologia , Masculino , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Fatores de Risco , Rigidez Vascular/efeitos dos fármacos , Adulto Jovem
18.
J Cardiovasc Magn Reson ; 21(1): 68, 2019 11 07.
Artigo em Inglês | MEDLINE | ID: mdl-31703697

RESUMO

BACKGROUND: It has been estimated that 20-30% of repaired aortic coarctation (CoA) patients develop hypertension, with significant cardiovascular morbidity and mortality. Vertebral artery hypoplasia (VAH) with an incomplete posterior circle of Willis (ipCoW; VAH + ipCoW) is associated with increased cerebrovascular resistance before the onset of increased sympathetic nerve activity in borderline hypertensive humans, suggesting brainstem hypoperfusion may evoke hypertension to maintain cerebral blood flow: the "selfish brain" hypothesis. We now assess the "selfish brain" in hypertension post-CoA repair. METHODS: Time-of-flight cardiovascular magnetic resonance angiography from 127 repaired CoA patients (34 ± 14 years, 61% male, systolic blood pressure (SBP) 138 ± 19 mmHg, diastolic blood pressure (DBP) 76 ± 11 mmHg) was compared with 33 normotensive controls (42 ± 14 years, 48% male, SBP 124 ± 10 mmHg, DBP 76 ± 8 mmHg). VAH was defined as < 2 mm and ipCoW as hypoplasia of one or both posterior communicating arteries. RESULTS: VAH + ipCoW was more prevalent in repaired CoA than controls (odds ratio: 5.8 [1.6-20.8], p = 0.007), after controlling for age, sex and body mass index (BMI). VAH + ipCoW was an independent predictor of hypertension (odds ratio: 2.5 [1.2-5.2], p = 0.017), after controlling for age, gender and BMI. Repaired CoA subjects with VAH + ipCoW were more likely to have difficult to treat hypertension (odds ratio: 3.3 [1.01-10.7], p = 0.049). Neither age at time of CoA repair nor any specific repair type were significant predictors of VAH + ipCoW in univariate regression analysis. CONCLUSIONS: VAH + ipCoW predicts arterial hypertension and difficult to treat hypertension in repaired CoA. It is unrelated to age at time of repair or repair type. CoA appears to be a marker of wider congenital cerebrovascular problems. Understanding the "selfish brain" in post-CoA repair may help guide management. JOURNAL SUBJECT CODES: High Blood Pressure; Hypertension; Magnetic Resonance Imaging (MRI); Cardiovascular Surgery; Cerebrovascular Malformations.


Assuntos
Coartação Aórtica/cirurgia , Pressão Arterial , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Malformações Vasculares do Sistema Nervoso Central/complicações , Circulação Cerebrovascular , Círculo Arterial do Cérebro/fisiopatologia , Hipertensão/etiologia , Artéria Vertebral/fisiopatologia , Adulto , Coartação Aórtica/complicações , Coartação Aórtica/fisiopatologia , Malformações Vasculares do Sistema Nervoso Central/diagnóstico por imagem , Malformações Vasculares do Sistema Nervoso Central/fisiopatologia , Círculo Arterial do Cérebro/anormalidades , Círculo Arterial do Cérebro/diagnóstico por imagem , Bases de Dados Factuais , Feminino , Humanos , Hipertensão/diagnóstico , Hipertensão/fisiopatologia , Angiografia por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Resultado do Tratamento , Artéria Vertebral/anormalidades , Artéria Vertebral/diagnóstico por imagem , Adulto Jovem
19.
Hypertension ; 74(6): 1484-1489, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31630577

RESUMO

Exercise-induced hypertension is a predictor of cardiovascular events in patients with coarctation of aorta (COA). However, it is unclear whether mild COA diagnosis is an independent risk factor of exercise-induced hypertension. We hypothesized that for every unit increase in exercise, patients with COA (without hemodynamically significant coarctation) will have a higher rise in systolic blood pressure (SBP) compared with matched controls. One hundred forty-nine patients with COA (aortic coarctation peak velocity <2 m/s) who underwent exercise testing were matched 1:1 to controls using propensity score method based on age, sex, body mass index, hypertension diagnosis, and SBP at rest. We compared exercise-induced change in SBP between patients with COA and controls and also assessed the correlation between Doppler-derived aortic vascular function indices (effective arterial elastance index and total arterial compliance index) and exercise-induced changes in SBP. Compared with controls, patients with COA had a greater change in SBP per unit metabolic equivalent (ß=2.86; 95% CI, 1.96-4.77 versus 1.07, 95% CI, -0.15 to 1.75; P=0.018) and per unit oxygen pulse (ß=4.57; 95% CI, 2.97-7.12 versus 1.45, 95% CI, -0.79 to 2.09, P<0.001). There was a correlation between SBPpeak-SBPrest and elastance index (r=0.38, P=0.032) and between SBPpeak-SBPrest and total arterial compliance index (r=-0.51, P=0.001), suggesting an association between vascular dysfunction and exercise-induced BP changes. Patients with COA, without significant obstruction, had higher exercise-induced changes in SBP after adjustment for other risk factors for hypertension. Considering the already known prognostic importance of exercise-induced hypertension, the current study highlights the potential role of exercise testing for risk stratification of patients with mild COA.


Assuntos
Coartação Aórtica/diagnóstico por imagem , Teste de Esforço/efeitos adversos , Hipertensão/etiologia , Centros Médicos Acadêmicos , Adolescente , Adulto , Coartação Aórtica/epidemiologia , Coartação Aórtica/fisiopatologia , Determinação da Pressão Arterial , Estudos de Casos e Controles , Bases de Dados Factuais , Ecocardiografia Doppler/métodos , Teste de Esforço/métodos , Feminino , Humanos , Hipertensão/epidemiologia , Hipertensão/fisiopatologia , Incidência , Masculino , Prognóstico , Pontuação de Propensão , Valores de Referência , Estudos Retrospectivos , Comportamento de Redução do Risco , Índice de Gravidade de Doença , Estados Unidos , Adulto Jovem
20.
BMJ Case Rep ; 12(9)2019 Sep 18.
Artigo em Inglês | MEDLINE | ID: mdl-31537594

RESUMO

Prostaglandins are widely used in aortic coarctation to maintain ductal patency and preserve systemic perfusion until surgical intervention can be performed. Although the short-term use of prostaglandins to ameliorate aortic narrowing in neonates with a closed ductus has been reported, it has not been described as a longer term therapy in extremely preterm neonates. A 27-week gestation baby weighing 560 g presented at 40 days of age with coarctation and a closed ductus arteriosus. He was successfully treated with a 7-week course of prostaglandin E2 therapy because surgical intervention was not deemed feasible in view of his size. Treatment resulted in a relaxation of the aortic constriction and improvement in aortic blood flow velocity profile, highlighting the value of long-term prostaglandin therapy in this population and supporting the hypothesis that the presence of ductal tissue contributes to the development of juxtaductal aortic constriction in some extremely preterm infants.


Assuntos
Coartação Aórtica/tratamento farmacológico , Dinoprostona/administração & dosagem , Ocitócicos/administração & dosagem , Administração Intravenosa , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/fisiopatologia , Velocidade do Fluxo Sanguíneo/efeitos dos fármacos , Dinoprostona/uso terapêutico , Permeabilidade do Canal Arterial/diagnóstico por imagem , Ecocardiografia , Humanos , Lactente , Lactente Extremamente Prematuro , Recém-Nascido , Doenças do Recém-Nascido , Masculino , Ocitócicos/uso terapêutico , Resultado do Tratamento
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