Relation of Macrophage Migration Inhibitory Factor to Pulmonary Hemodynamics and Vascular Structure and Carbamyl-Phosphate Synthetase I Genetic Variations in Pediatric Patients with Congenital Cardiac Shunts.

Macrophage migration inhibitory factor (MIF) plays an important pathophysiological role in pulmonary hypertension (PHT). Previously, we demonstrated that serum MIF is increased in pediatric PHT associated with congenital heart disease (CHD). In the present study, we determined possible associations between MIF levels, hemodynamic and histological parameters, and mitochondrial carbamyl-phosphate synthetase I (CPSI) T1405N polymorphism in a similar population. The asparagine 1405 variant (related to A alleles in the C-to-A transversion) has been shown to be advantageous in pediatric PHT compared to the threonine 1405 variant (C alleles). Forty-one patients were enrolled (aged 2-36 months) and subsequently divided into 2 groups after diagnostic evaluation: the high-pulmonary blood flow (high PBF) group (pulmonary-to-systemic blood flow ratio 2.58 (2.21-3.01), geometric mean with 95% CI) and the high-pulmonary vascular resistance (high PVR) group (pulmonary vascular resistance 6.12 (4.78-7.89) Wood units × m2). Serum MIF was measured using a chemiluminescence assay. The CPSI polymorphism was analyzed by polymerase chain reaction followed by high-resolution melting analysis. Medial hypertrophy of pulmonary arteries was assessed by the histological examination of biopsy specimens. Serum MIF was elevated in patients compared to controls (p = 0.045), particularly in the high-PVR group (n = 16) (p = 0.022) and in subjects with the AC CPSI T1405N genotype (n = 16) compared to those with the CC genotype (n = 25) (p = 0.017). Patients with high-PVR/AC-genotype profile (n = 9) had the highest MIF levels (p = 0.030 compared with the high-PBF/CC-genotype subgroup, n = 18). In high-PVR/AC-genotype patients, the medial wall thickness of intra-acinar pulmonary arteries was directly related to MIF levels (p = 0.033). There were no patients with the relatively rare AA genotype in the study population. Thus, in the advantageous scenario of the asparagine 1405 variant (AC heterozygosity in this study), heightened pulmonary vascular resistance in CHD-PHT is associated with medial hypertrophy of pulmonary arteries where MIF chemokine very likely plays a biological role.

Intermittent claudication and severe renal artery stenosis are independently associated in hypertensive patients referred for renal arteriography.

OBJECTIVE:: The purpose of this study was to evaluate the association between the presence of clinical symptoms of peripheral artery disease and severe renal artery stenosis in patients referred for renal angiography. METHOD:: We included 82 patients with clinical suspicion of renovascular hypertension and performed an imaging investigation (renal Doppler ultrasound and/or renal scintigraphy) for possible renal artery stenosis. All patients underwent renal arteriography and were examined for peripheral artery disease based on the presence of intermittent claudication and ankle-brachial index test results. Severe renal artery stenosis was defined as a lesion causing 70% obstruction. RESULTS:: Severe renal artery stenosis was present in 32 of 82 (39%) patients. Patients with severe renal artery stenosis were older (63±12 vs 56±12 years, p=0.006), had more intermittent claudication (55 vs 45%, p=0.027), and had a greater prevalence of an ankle-brachial index <0.9 (44% vs 20%, p=0.021) than patients without severe renal artery stenosis. Multivariate logistic regression analysis showed that the presence of intermittent claudication was independently associated with renal artery stenosis ≥70% (OR: 3.33; 95% CI 1.03-10.82, p=0.04), unlike the ankle-brachial index, which showed no association (OR: 1.44; 95% CI 0.37-5.66, p=0.60). CONCLUSION:: Intermittent claudication is independently associated with severe renal artery stenosis (≥70%) in patients clinically suspected of having renovascular hypertension.

Intermittent claudication and severe renal artery stenosis are independently associated in hypertensive patients referred for renal arteriography

OBJECTIVE: The purpose of this study was to evaluate the association between the presence of clinical symptoms of peripheral artery disease and severe renal artery stenosis in patients referred for renal angiography. METHOD: We included 82 patients with clinical suspicion of renovascular hypertension and performed an imaging investigation (renal Doppler ultrasound and/or renal scintigraphy) for possible renal artery stenosis. All patients underwent renal arteriography and were examined for peripheral artery disease based on the presence of intermittent claudication and ankle-brachial index test results. Severe renal artery stenosis was defined as a lesion causing 70% obstruction. RESULTS: Severe renal artery stenosis was present in 32 of 82 (39%) patients. Patients with severe renal artery stenosis were older (63±12 vs 56±12 years, p=0.006), had more intermittent claudication (55 vs 45%, p=0.027), and had a greater prevalence of an ankle-brachial index <0.9 (44% vs 20%, p=0.021) than patients without severe renal artery stenosis. Multivariate logistic regression analysis showed that the presence of intermittent claudication was independently associated with renal artery stenosis ≥70% (OR: 3.33; 95% CI 1.03-10.82, p=0.04), unlike the ankle-brachial index, which showed no association (OR: 1.44; 95% CI 0.37-5.66, p=0.60). CONCLUSION: Intermittent claudication is independently associated with severe renal artery stenosis (≥70%) in patients clinically suspected of having renovascular hypertension.

Left Atrial Myxoma Hypervascularized from the Right Coronary Artery: An Interesting Cath Lab Finding.

Primary cardiac tumors are rare and approximately half of them are atrial myxomas. They rarely remain asymptomatic, especially if large. The imaging of a myxoma by contrast dye during coronary angiography is an infrequent sign, which clarifies the vascular supply of the tumor. We report herein an interesting and rare case of a left atrial myxoma hypervascularized from the right coronary artery.

Direct communication between the left circumflex and the right coronary arteries: a very rare coronary anomaly circulation.

Coronary artery anomalies (CAA) are congenital changes in their origin, course, and/or structure. Intercoronary communication (ICC) is a very rare subset with uni- or bidirectional blood flow between two or more coronary arteries. We present the case of a 58-year-old man with an acute coronary syndrome whose coronary angiography incidentally showed a surprising and very rare communication between the right coronary and left circumflex arteries.

Clinical performance of a novel ultrathin strut, low-dose, sirolimus-eluting stent with abluminal-only biodegradable polymeric coating for patients undergoing percutaneous coronary intervention in the daily practice.

BACKGROUND: The present study aimed to evaluate the clinical performance, in the daily practice of a busy catheterization laboratory, of a novel drug-eluting stent (DES) built with an ultra-thin-strut metallic platform, eluting sirolimus at low doses, abluminal coated with biodegradable polymers, and mounted in a low-compliant delivery system. METHODS: Prospective, single-arm study, comprising all consecutive patients undergoing percutaneous coronary intervention (PCI) with the Inspiron™ sirolimus-eluting stent (SES) (Scitech, Aparecida de Goiania, Brazil). The primary endpoint was the occurrence of major adverse cardiac events (MACE) [cardiac death, non-PCI related myocardial infarction (MI), or target vessel revascularization (TVR)]. RESULTS: A total of 470 patients were included, from which 51.3% were diabetics, 33.8% had triple-vessel disease, 15.3% had heart failure, 38.9% had at least one bifurcation treated, 19.8% were treated for a bare metal stent restenosis, and 61.9% had at least one type C lesion; one or more of these features were found in 96.0%. At 300 days, the rate target lesion revascularization was 5.4% and the rate of MACE was 8.1%. The incidence of definite or probable stent thrombosis was 0.4%, with no cases between 30 and 300 days. CONCLUSIONS: The novel stent is associated with excellent short and mid-term clinical outcomes in patients treated with PCI in the daily practice.

Does Ad Hoc Coronary Intervention Reduce Radiation Exposure? – Analysis of 568 Patients / Intervenção Coronariana Ad Hoc Reduz a Exposição Radiológica? - Análise em 568 Pacientes

Background:Advantages and disadvantages of ad hoc percutaneous coronary intervention have been described. However little is known about the radiation exposure of that procedure as compared with the staged intervention.Objective:To compare the radiation dose of the ad hoc percutaneous coronary intervention with that of the staged procedureMethods:The dose-area product and total Kerma were measured, and the doses of the diagnostic and therapeutic procedures were added. In addition, total fluoroscopic time and number of acquisitions were evaluated.Results:A total of 568 consecutive patients were treated with ad hoc percutaneous coronary intervention (n = 320) or staged percutaneous coronary intervention (n = 248). On admission, the ad hoc group had less hypertension (74.1% vs 81.9%; p = 0.035), dyslipidemia (57.8% vs. 67.7%; p = 0.02) and three-vessel disease (38.8% vs. 50.4%; p = 0.015). The ad hoc group was exposed to significantly lower radiation doses, even after baseline characteristic adjustment between both groups. The ad hoc group was exposed to a total dose-area product of 119.7 ± 70.7 Gycm2, while the staged group, to 139.2 ± 75.3 Gycm2 (p < 0.001).Conclusion:Ad hoc percutaneous coronary intervention reduced radiation exposure as compared with diagnostic and therapeutic procedures performed at two separate times.

Fundamento:Uma série de vantagens e desvantagens tem sido descrita para a intervenção coronária percutânea ad hoc, como a realizada ao mesmo tempo que o cateterismo diagnóstico, porém pouco se sabe sobre a exposição radiológica desse procedimento, comparado com a intervenção estadiada, ou seja, em dois momentos.Objetivo:Comparar a dose de radiação utilizada na angioplastia ad hoc com a angioplastia estadiada.Métodos:O produto de dose por área e o Kerma total foram mensurados, somando-se ambas as fases de diagnóstico e terapêutica. Além disso, foram computados o tempo total de fluoroscopia e o número de filmagens.Resultados:Um total de 568 pacientes consecutivos foram tratados com intervenção coronária percutânea ad hoc (n = 320) ou estadiada (n = 248). À admissão, o grupo do ad hoc apresentava menos hipertensão (74,1% vs. 81,9%; p = 0,035), dislipidemia (57,8% vs. 67,7%; p = 0,02) e doença triarterial (38,8% vs. 50,4%; p = 0,015). O grupo ad hoc foi exposto a doses significativamente menores de radiação, mesmo após ajuste para as diferenças basais entre os grupos. O grupo ad hoc foi exposto a uma dose de produto de dose por área total de 119,7 ± 70,7 Gycm2 enquanto que os pacientes estadiados foram expostos a uma dose de 139,2 ± 75,3 Gycm2 (p < 0,001).Conclusão:A intervenção coronária percutânea ad hoc reduziu a exposição radiológica, em comparação com procedimentos diagnósticos e terapêuticos realizados em tempos separados.

Does Ad Hoc Coronary Intervention Reduce Radiation Exposure? - Analysis of 568 Patients.

BACKGROUND: Advantages and disadvantages of ad hoc percutaneous coronary intervention have been described. However little is known about the radiation exposure of that procedure as compared with the staged intervention. OBJECTIVE: To compare the radiation dose of the ad hoc percutaneous coronary intervention with that of the staged procedureMethods:The dose-area product and total Kerma were measured, and the doses of the diagnostic and therapeutic procedures were added. In addition, total fluoroscopic time and number of acquisitions were evaluated. RESULTS: A total of 568 consecutive patients were treated with ad hoc percutaneous coronary intervention (n = 320) or staged percutaneous coronary intervention (n = 248). On admission, the ad hoc group had less hypertension (74.1% vs 81.9%; p = 0.035), dyslipidemia (57.8% vs. 67.7%; p = 0.02) and three-vessel disease (38.8% vs. 50.4%; p = 0.015). The ad hoc group was exposed to significantly lower radiation doses, even after baseline characteristic adjustment between both groups. The ad hoc group was exposed to a total dose-area product of 119.7 ± 70.7 Gycm2, while the staged group, to 139.2 ± 75.3 Gycm2 (p < 0.001). CONCLUSION: Ad hoc percutaneous coronary intervention reduced radiation exposure as compared with diagnostic and therapeutic procedures performed at two separate times.

Superdominant Right Coronary Artery with Absence of Left Circumflex and Anomalous Origin of the Left Anterior Descending Coronary from the Right Sinus: An Unheard Coronary Anomaly Circulation.

Coronary artery anomalies are congenital changes in their origin, course, and/or structure. Most of them are discovered as incidental findings during coronary angiographic studies or at autopsies. We present herein the case of a 70-year-old man with symptomatic severe aortic valvar stenosis whose preoperative coronary angiogram revealed a so far unreported coronary anomaly circulation pattern.

Type 4 dual left anterior descending artery: a very rare coronary anomaly circulation.

Coronary artery anomalies are congenital changes in their origin, course, and/or structure. They are the second most frequent cause of sudden death in young athletes. Dual LAD artery is a rare coronary anomaly. We present the case of a 44-year-old man with recent onset exertional angina and documented ischemia whose coronary angiogram and computed tomography (CT) showed type 4 dual LAD artery, the rarest and most interesting variant.

Acurácia e precisão da angiografia coronária quantitativa online com calibração automática: um estudo piloto / Accuracy and precision of online quantitative coronary angiography with automatic calibration: a pilot study

O objetivo deste estudo foi avaliar o grau de acurácia, precisão, correlação e concordância entre as medidas realizadas por um software de angiografia coronária quantitativa (QCA) online de calibração automática. Métodos: Estudo piloto que analisou imagens angiográficas de dez pacientes com um software de QCA online nas calibrações automáticas Auto ISO (calibração automática do isocentro) e Auto TOD (Table-to Object Distance). Foi realizada a medida do calibre do cateter pelos dois métodos, e o diâmetro de referência foi computado. Essas medidas foram comparadas com a medida do diâmetro do cateter quanto à acurácia, precisão e concordância. Resultados: O diâmetro médio real dos cateteres era de 1,75 ± 0,32 mm (variando entre 1,33 e 2,67 mm). A aferição dos cateteres pela QCA TOD e pela QCA ISO resultou em diâmetros médios de 1,78 ± 0,37 mm e 1,88 ± 0,38 mm, respectivamente. A acurácia/precisão da QCA TOD e da QCA ISO foi 0,03 m/0,21 mm e 0,12 mm/0,20 mm, respectivamente. As medidas da QCA TOD e da QCA ISO estiveram entre os limites de concordância em 96,3 e 94,7% dos casos, respectivamente. As medidas da QCA TOD e da QCA ISO correlacionaram-se significativamente (rs = 0,93; p < 0,01). No entanto, apesar da pequena diferença entre os métodos (0,10 ± 0,10 mm), as medidas da QCA ISO foram estatisticamente maiores que aquelas obtidas com a QCA TOD (p < 0,01). Conclusões: A QCA online com calibração automática apresentou boa acurácia, precisão e correlação,podendo representar uma ferramenta promissora no laboratório de hemodinâmica...

The objective of this study was to evaluate the degree of accuracy, precision, correlation, andagreement between the measurements performed by online Quantitative Coronary Angiography (QCA)software with automatic calibration.Methods: Pilot study that analyzed angiographic images of ten patients through online QCA softwareusing Auto ISO (automatic calibration isocenter) and Auto TOD (Table-to-Object Distance) automatic calibration. Catheter size was measured by both methods and the reference diameter was computed. These measurements were compared with the measurement of catheter diameter regarding accuracy, precision,and agreement. Results: The actual average of the catheter diameter was 1.75 ± 0.32 mm (range 1.33 to 2.67 mm). The measurement of catheters by TOD QCA and ISO QCA resulted in mean diameters of 1.78 ± 0.37 mm and 1.88± 0.38 mm, respectively. The accuracy/precision of the TOD QCA and the ISO QCA was 0.03 mm/0.21 mmand 0.12 mm/0.20 mm, respectively. The TOD QCA and ISO QCA measures were among the limits ofagreement in 96.3 and 94.7% of cases, respectively, and were significantly correlated (rs = 0.93, p < 0.01). However, despite the small difference between the methods (0.10 ± 0.10 mm), the ISO QCA measures were significantly higher than those obtained by the TOD QCA (p < 0.01). Conclusions: Online QCA with automatic calibration has good accuracy, precision, and correlation, which may represent a promising tool in the catheterization laboratory...

Hemodynamic Effects of Noninvasive Ventilation in Patients with Venocapillary Pulmonary Hypertension / Efeitos Hemodinâmicos da Ventilação não Invasiva em Pacientes com Hipertensão Pulmonar Venocapilar

Background: The hemodynamic effects of noninvasive ventilation with positive pressure in patients with pulmonary hypertension without left ventricular dysfunction are not clearly established. Objectives: Analyze the impact of increasing airway pressure with continuous positive airway pressure on hemodynamic parameters and, in particular, on cardiac output in patients with variable degrees of pulmonary hypertension. Methods: The study included 38 patients with pulmonary hypertension caused by mitral stenosis without left ventricular dysfunction or other significant valvulopathy. The hemodynamic state of these patients was analyzed in three conditions: baseline, after continuous positive pressure of 7 cmH2O and, finally, after pressure of 14 cmH2O. Results: The population was composed of predominantly young and female individuals with significant elevation in pulmonary arterial pressure (mean systolic pressure of 57 mmHg). Of all variables analyzed, only the right atrial pressure changed across the analyzed moments (from the baseline condition to the pressure of 14 cmH2O there was a change from 8 ± 4 mmHg to 11 ± 3 mmHg, respectively, p = 0.031). Even though there was no variation in mean cardiac output, increased values in pulmonary artery pressure were associated with increased cardiac output. There was no harmful effect or other clinical instability associated with use application of airway pressure. Conclusion: In patients with venocapillary pulmonary hypertension without left ventricular dysfunction, cardiac output response was directly associated with the degree of pulmonary hypertension. The application of noninvasive ventilation did not cause complications directly related to the ventilation systems. .

Fundamento: Os efeitos hemodinâmicos da ventilação não invasiva com pressão positiva em pacientes com hipertensão pulmonar sem disfunção ventricular esquerda não estão bem estabelecidos. Objetivos: Analisar o impacto da pressurização aérea crescente através da pressão positiva contínua das vias aéreas nos parâmetros hemodinâmicos e, principalmente, no débito cardíaco de pacientes com graus variáveis de hipertensão pulmonar. Métodos: Foram incluídos 38 pacientes com hipertensão pulmonar causada por estenose mitral sem disfunção ventricular esquerda ou outra valvopatia significativa. O estado hemodinâmico deses pacientes foi estudado em três condições: basal, após uso de pressão positiva contínua de 7 cmH2O e , finalmente, após pressão de 14 cmH2O. Resultados: A população foi composta por indivíduos predominantemente jovens, do sexo feminino e com elevação significativa da pressão arterial pulmonar (pressão sistólica média de 57 mmHg). De todas as variáveis analisadas, apenas a pressão de átrio direito apresentou variação ao longo dos momentos estudados (da condição basal para a de pressão de 14 cmH2O houve aumento de 8 ± 4 mmHg para 11 ± 3 mmHg, respectivamente, p = 0,031). Apesar de não ter havido variação do valor médio do débito cardíaco, valores mais elevados de pressões na artéria pulmonar estiveram associados ao aumento do débito cardíaco. Não houve efeito deletério ou qualquer instabilidade clínica associada ao uso da pressurização das vias aéreas. Conclusão: Em pacientes com hipertensão venocapilar pulmonar sem disfunção ventricular esquerda, a resposta ...

Hemodynamic Effects of Noninvasive Ventilation in Patients with Venocapillary Pulmonary Hypertension.

Background: The hemodynamic effects of noninvasive ventilation with positive pressure in patients with pulmonary hypertension without left ventricular dysfunction are not clearly established. Objectives: Analyze the impact of increasing airway pressure with continuous positive airway pressure on hemodynamic parameters and, in particular, on cardiac output in patients with variable degrees of pulmonary hypertension. Methods: The study included 38 patients with pulmonary hypertension caused by mitral stenosis without left ventricular dysfunction or other significant valvulopathy. The hemodynamic state of these patients was analyzed in three conditions: baseline, after continuous positive pressure of 7 cmH2O and, finally, after pressure of 14 cmH2O. Results: The population was composed of predominantly young and female individuals with significant elevation in pulmonary arterial pressure (mean systolic pressure of 57 mmHg). Of all variables analyzed, only the right atrial pressure changed across the analyzed moments (from the baseline condition to the pressure of 14 cmH2O there was a change from 8 ± 4 mmHg to 11 ± 3 mmHg, respectively, p = 0.031). Even though there was no variation in mean cardiac output, increased values in pulmonary artery pressure were associated with increased cardiac output. There was no harmful effect or other clinical instability associated with use application of airway pressure. Conclusion: In patients with venocapillary pulmonary hypertension without left ventricular dysfunction, cardiac output response was directly associated with the degree of pulmonary hypertension. The application of noninvasive ventilation did not cause complications directly related to the ventilation systems.

Coarctação da Aorta em Crianças com Menos de 25 kg: Tratamento Percutâneo por Punção da Artéria Axilar / Aortic Coarctation in Children Weighing Less than 25 kg: Percutaneous Axillary Artery Approach

Introdução: O tratamento percutâneo da coarctação da aorta é método de escolha em crianças acima de 6 meses de idade e sem hipoplasia do arco aórtico. No entanto, nos pacientes com menos de 25 kg, a via de acesso clássica (femoral) pode representar um problema, principalmente nos implantes de stents, devido ao tamanho dos introdutores. O objetivo deste estudo foi relatar a experiência com a punção da artéria axilar como via de acesso para o tratamento percutâneo de pacientes com coarctação da aorta e peso < 25 kg. Métodos: A punção foi realizada com agulha 21 G, com o braço abduzido em 90º, sendo introduzido fio-guia 0,014 polegada, posicionado na aorta descendente. Um introdutor 5 F pediátrico de 7 cm foi inicialmente utilizado para realização do procedimento, sendo substituído, quando necessário, por um introdutor maior. Após a intervenção, foi realizada compressão hemostática manual. Resultados: Foram tratadas dez crianças, sendo oito com recoarctação póscirúrgica e duas com coactação nativa, com idades de 51,1 ± 30,8 meses e peso de 15,8 ± 5,8 kg. A punção foi realizada em todos os casos sem dificuldade técnica, e a mediana do calibre do introdutor foi de 7 F. Em oito pacientes, foram implantados oito stents e, em dois, foi realizada apenas angioplastia com balão. Houve sucesso técnico em todos os ...

Background: Percutaneous treatment of aortic coarctation is the method of choice in children over 6 months of age and without aortic arch hypoplasia. However in patients less than 25 kg the classical access route (femoral) may pose a problem, especially in cases of stenting, due to the size of the size of the introducers. The objective of this study was to report our experience with the axillary artery approach for the percutaneous treatmentof patients with aortic coarctation weighing less than 25 kg. Methods: The arterial puncture was performed with a 21 G needle, with the arm abducted at 90º, and a 0.014 inch guidewire was positioned in the descending aorta. A 5 F × 7 cm pediatric introducer was initially used for the procedure and whenever required, it was replaced in a larger one. Manual-compression hemostasis was performed after the intervention. Results: Ten children were treated, eight with residual and two with native coarctation, mean age was 51.1 ± 30.8 months and weight 15.8 ± 5.8 kg. Puncture was performed in all cases without technical difficulty and the median introducer size was 7 F. Eight stents were implanted in eight patients and two patients were only treated by balloon angioplasty. Technical success was observed in all patients. After removal of the introducer, there was no permanent pulse loss and one patient had a small local hematoma. Conclusions: In our experience the axillary artery approach for percutaneous ...