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1.
Pediatr Cardiol ; 2024 Mar 08.
Artigo em Inglês | MEDLINE | ID: mdl-38456890

RESUMO

Anthracyclines are effective chemotherapeutics used in approximately 60% of pediatric cancer cases but have a well-documented risk of cardiotoxicity. Existing cardiotoxicity risk calculators do not include cardiovascular risk factors present at the time of diagnosis. The goal of this study is to leverage the advanced sensitivity of strain echocardiography to identify pre-existing risk factors for early subclinical cardiac dysfunction among anthracycline-exposed pediatric patients. We identified 115 pediatric patients with cancer who were treated with an anthracycline between 2013 and 2019. Peak longitudinal left ventricular strain was retroactively calculated on 495 surveillance echocardiograms via the TOMTEC AutoSTRAIN software. Cox proportional hazards models were employed to identify risk factors for abnormal longitudinal strain (> - 16%) following anthracycline treatment. High anthracycline dose (≥ 250 mg/m2 doxorubicin equivalents) and obesity at the time of diagnosis (BMI > 95th percentile-for-age) were both significant predictors of abnormal strain with hazard ratios of 2.79, 95% CI (1.07-7.25), and 3.85, 95% CI (1.42-10.48), respectively. Among pediatric cancer survivors, patients who are obese at the time of diagnosis are at an increased risk of sub-clinical cardiac dysfunction following anthracycline exposure. Future studies should explore the incidence of symptomatic cardiomyopathy 10-15 years post-treatment among patients with early subclinical cardiac dysfunction.

2.
Am J Perinatol ; 2024 May 02.
Artigo em Inglês | MEDLINE | ID: mdl-38698596

RESUMO

OBJECTIVE: Bronchopulmonary dysplasia (BPD) is the most common complication of preterm birth. Infants with BPD are at increased risk for pulmonary hypertension (PH). Cardiac catheterization is the gold standard for diagnosing PH, but cardiac catheterization is challenging to perform in small, sick, premature infants. The utility of echocardiography for diagnosing PH and predicting outcomes in extremely premature infants has not been clearly defined. Therefore, we sought to use predefined criteria to diagnose PH by echocardiogram and relate PH severity to mortality in extremely premature infants with BPD. STUDY DESIGN: Echocardiograms from 46 infants born ≤28 weeks' postmenstrual age with a diagnosis of BPD were assessed for PH by three pediatric cardiologists using predefined criteria, and survival times among categories of PH patients were compared. A total of 458 echocardiograms were reviewed, and 15 (33%) patients were found to have at least moderate PH. Patients with at least moderate PH had similar demographic characteristics to those with no/mild PH. RESULTS: Ninety percent of infants without moderate to severe PH survived to hospital discharge, compared with 67% of infants with at least moderate PH (p = 0.048). Patients with severe PH had decreased survival to hospital discharge (38%) compared with moderate (100%) and no/mild PH (90%) groups. Kaplan-Meier survival curves also differed among PH severity groups (Wilcoxon p < 0.001). CONCLUSION: Using predefined criteria for PH, premature infants with BPD can be stratified into PH severity categories. Patients diagnosed with severe PH by echocardiogram have significantly reduced survival. KEY POINTS: · A composite score definition of PH by echocardiogram showed high inter- and intrarater reliability.. · Infants with severe PH by echocardiogram had decreased survival rates.. · Early diagnosis of PH by echocardiogram dictates treatment which may improve outcomes..

3.
Cardiol Young ; : 1-6, 2024 Mar 20.
Artigo em Inglês | MEDLINE | ID: mdl-38506050

RESUMO

OBJECTIVE: Identifying thrombus formation in Fontan circulation has been highly variable, with reports between 17 and 33%. Initially, thrombus detection was mainly done through echocardiograms. Delayed-enhancement cardiac MRI is emerging as a more effective imaging technique for thrombus identification. This study aims to determine the prevalence of occult cardiac thrombosis in patients undergoing clinically indicated cardiac MRI. METHODS: A retrospective chart review of children and adults in the Duke University Hospital Fontan registry who underwent delayed-enhancement cardiac MRI. Individuals were excluded if they never received a delayed-enhancement cardiac MRI or had insufficient data. Demographic characteristics, native heart anatomy, cardiac MRI measurements, and thromboembolic events were collected for all patients. RESULTS: In total, 119 unique individuals met inclusion criteria with a total of 171 scans. The median age at Fontan procedure was 3 (interquartile range 1, 4) years. The majority of patients had dominant systemic right ventricle. Cardiac function was relatively unchanged from the first cardiac MRI to the third cardiac MRI. While 36.4% had a thrombotic event by history, only 0.5% (1 patient) had an intracardiac thrombus detected by delayed-enhancement cardiac MRI. CONCLUSIONS: Despite previous echocardiographic reports of high prevalence of occult thrombosis in patients with Fontan circulation, we found very low prevalence using delayed-enhancement cardiac MRI. As more individuals are reaching adulthood after requiring early Fontan procedures in childhood, further work is needed to develop thrombus-screening protocols as a part of anticoagulation management.

4.
Echocardiography ; 38(11): 1978-1983, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-34719050

RESUMO

Abnormal atrioventricular valve present great challenges to the surgeon in achieving a successful repair, and thus present a great opportunity for enhanced 3D imaging to guide pre- and intra-operative management. Spatial and temporal resolution of 3D echocardiography enables 3D printing of valve morphology. However, non-linearity, angle dependence, speckle, blur, and resampling complicate segmentation compared to computed tomography (CT) and magnetic resonance imaging (MRI). A case of complex mitral valve disease in a pediatric patient is therefore presented to illustrate the technical challenges of segmentation and 3D printing from echocardiographic data.


Assuntos
Ecocardiografia Tridimensional , Doenças das Valvas Cardíacas , Insuficiência da Valva Mitral , Criança , Ecocardiografia , Ecocardiografia Transesofagiana , Humanos , Valva Mitral/diagnóstico por imagem , Valva Mitral/cirurgia , Insuficiência da Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Mitral/cirurgia , Impressão Tridimensional
5.
Catheter Cardiovasc Interv ; 95(6): 1141-1148, 2020 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-31854085

RESUMO

OBJECTIVES: Describe the use of three-dimensional (3D) patent ductus arteriosus (PDA) modeling to better define ductal anatomy to improve preprocedural planning for ductal stent placement. BACKGROUND: Ductal stenting is an alternative to surgical shunting in patients with ductal dependent pulmonary blood flow. Ductal anatomy is often complex with extreme tortuosity and risk of pulmonary artery isolation, thus increasing procedural risks. METHODS: CT angiograms were segmented to produce 3D PDA models. Ductal morphology was characterized with attention to access approach, degree of pulmonary artery offset/risk of isolation and ductal tortuosity. 3D models were retrospectively compared with biplane angiography. RESULTS: 3D modeling was performed in 12 patients with adequate image quality for complete analysis in 11; median (interquartile range) age/weight 17 days (8-20 days) and 3.1 kg (2.4-3.9 kg). The PDA was reverse oriented in nine with average length of 17.2 ± 2.5 mm and high tortuosity (mean tortuosity index 52, range 3-108). From 3D modeling, two patients were excluded from ductal stenting-extreme ductal tortuosity and threatened pulmonary artery discontinuity, respectively. Ductal stenting was successful in the remaining nine with no major procedural complications. 3D modeling predicted a successful access approach based on the aortic orientation of the ductus in all patients (five carotid, two axillary, two femoral). When comparing 2D angiography with 3D models, angiography consistently underestimated ductal length (-3.2 mm ± 1.6 mm) and tortuosity (-14.8 ± 7.2). CONCLUSIONS: 3D modeling prior to ductal stent placement for ductal dependent pulmonary blood flow is useful in procedural planning, specifically for eligibility, access approach, and accurate ductal measurements. Further studies are needed to determine if 3D planning improves procedural outcomes.


Assuntos
Cateterismo Cardíaco/instrumentação , Angiografia por Tomografia Computadorizada , Angiografia Coronária , Permeabilidade do Canal Arterial/diagnóstico por imagem , Permeabilidade do Canal Arterial/terapia , Imageamento Tridimensional , Modelos Cardiovasculares , Modelagem Computacional Específica para o Paciente , Interpretação de Imagem Radiográfica Assistida por Computador , Stents , Cateterismo Cardíaco/efeitos adversos , Permeabilidade do Canal Arterial/fisiopatologia , Feminino , Humanos , Recém-Nascido , Masculino , North Carolina , Valor Preditivo dos Testes , Circulação Pulmonar , Estudos Retrospectivos , Resultado do Tratamento
6.
Pediatr Crit Care Med ; 21(9): e795-e803, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32639468

RESUMO

OBJECTIVES: To determine the pharmacokinetics, pharmacodynamics, and safety of the hepatically metabolized endothelin receptor antagonist, ambrisentan in children after Fontan surgery. DESIGN: Prospective, randomized, double-blind, placebo-controlled pharmacokinetic/pharmacodynamics and safety trial. SETTING: Single-center, postoperative cardiac ICU. PATIENTS: Children undergoing elective Fontan surgery. INTERVENTIONS: Subjects randomized on postoperative day number 1 to short-term (3 d) treatment with oral ambrisentan (2.5 mg in suspension, daily) versus placebo (4:1 randomization). MEASUREMENTS AND MAIN RESULTS: Plasma drug concentrations were measured at 0.5, 1, 2, 4, and 18-36 hours after the first dose. We developed a population pharmacokinetic model in NONMEM 7.2 (Icon Solutions, Ellicott City, MD) and applied the model to dose-exposure simulations. Pharmacodynamics endpoints were assessed at baseline and 3 hours after study drug administration, using postoperative hemodynamic monitoring lines. The analysis included 16 patients, 13 on ambrisentan (77 plasma samples); median age 36 months (range, 26-72 mo), weight 13.3 kg (11.1-17.6 kg), and nine males. There were no differences in baseline characteristics between ambrisentan and controls. A one-compartment model with first-order absorption and lag-time characterized the data well. Allometrically scaled weight was the only covariate retained in the final model. Typical values for clearance and volume of distribution were lower than previously reported in adults, 1 L/hr/70 kg and 13.7 L/70 kg, respectively. Simulated exposures with doses of 0.1-0.2 mg/kg approximated therapeutic exposures in adults with pulmonary arterial hypertension receiving 5 mg or 10 mg doses. Ambrisentan lowered plasma brain natriuretic peptide concentrations (452 ± 479 to 413 ± 462; p = 0.046), Fontan pressures (16.8 ± 2.9 to 15.6 ± 2.9; p = 0.01), and indexed pulmonary vascular resistance (2.3 ± 0.9 to 1.8 ± 0.6; p = 0.01) with no drug-related adverse events. CONCLUSIONS: Ambrisentan clearance is reduced following Fontan surgery, perhaps reflecting abnormal hepatic metabolism in this population. The observed safety profile appears favorable and hemodynamic effects of ambrisentan may be beneficial for Fontan patients.


Assuntos
Técnica de Fontan , Fenilpropionatos , Piridazinas , Adulto , Criança , Pré-Escolar , Humanos , Masculino , Estudos Prospectivos
7.
Echocardiography ; 37(7): 1101-1104, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32652546

RESUMO

Left-sided unguarded tricuspid valve disease with congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac malformation, only reported a few times in the literature. Two-dimensional echocardiography (2DE) uses standard views to diagnose tricuspid valve disease. Advanced imaging techniques, such as three-dimensional echocardiography, allow for simultaneous visualization of the tricuspid valve annulus and all leaflets. Three-dimensional echocardiography (3DE) may be useful in distinguishing unguarded tricuspid valve orifice from other forms of tricuspid valve disease.


Assuntos
Ecocardiografia Tridimensional , Transposição dos Grandes Vasos , Insuficiência da Valva Tricúspide , Transposição das Grandes Artérias Corrigida Congenitamente , Humanos , Transposição dos Grandes Vasos/diagnóstico por imagem , Valva Tricúspide/diagnóstico por imagem , Insuficiência da Valva Tricúspide/diagnóstico por imagem
8.
Cardiol Young ; 28(11): 1306-1315, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30079851

RESUMO

BACKGROUND: Infants with prenatally diagnosed CHD are at high risk for adverse outcomes owing to multiple physiologic and psychosocial factors. Lack of immediate physical postnatal contact because of rapid initiation of medical therapy impairs maternal-infant bonding. On the basis of expected physiology, maternal-infant bonding may be safe for select cardiac diagnoses. METHODS: This is a single-centre study to assess safety of maternal-infant bonding in prenatal CHD. RESULTS: In total, 157 fetuses with prenatally diagnosed CHD were reviewed. On the basis of cardiac diagnosis, 91 fetuses (58%) were prenatally approved for bonding and successfully bonded, 38 fetuses (24%) were prenatally approved but deemed not suitable for bonding at delivery, and 28 (18%) were not prenatally approved to bond. There were no complications attributable to bonding. Those who successfully bonded were larger in weight (3.26 versus 2.6 kg, p<0.001) and at later gestation (39 versus 38 weeks, p<0.001). Those unsuccessful at bonding were more likely to have been delivered via Caesarean section (74 versus 49%, p=0.011) and have additional non-cardiac diagnoses (53 versus 29%, p=0.014). There was no significant difference regarding the need for cardiac intervention before hospital discharge. Infants who bonded had shorter hospital (7 versus 26 days, p=0.02) and ICU lengths of stay (5 versus 23 days, p=0.002) and higher survival (98 versus 76%, p<0.001). CONCLUSION: Fetal echocardiography combined with a structured bonding programme can permit mothers and infants with select types of CHD to successfully bond before ICU admission and intervention.


Assuntos
Ecocardiografia/métodos , Coração Fetal/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico , Ultrassonografia Pré-Natal , Adulto , Cesárea , Feminino , Humanos , Recém-Nascido , Gravidez , Reprodutibilidade dos Testes , Adulto Jovem
9.
Cardiol Young ; 25(7): 1348-57, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-25602135

RESUMO

BACKGROUND: Aortic arch obstruction can be evaluated by catheter peak-to-peak gradient or by Doppler peak instantaneous pressure gradient. Previous studies have shown moderate correlation in discrete coarctation, but few have assessed correlation in patients with more complex aortic reconstruction. METHODS: We carried out retrospective comparison of cardiac catheterisations and pre- and post-catheterisation echocardiograms in 60 patients with native/recurrent coarctation or aortic reconstruction. Aortic arch obstruction was defined as peak-to-peak gradient ⩾25 mmHg in patients with native/recurrent coarctation and ⩾10 mmHg in aortic reconstruction. RESULTS: Diastolic continuation of flow was not associated with aortic arch obstruction in either group. Doppler peak instantaneous pressure gradient, with and without the expanded Bernoulli equation, weakly correlated with peak-to-peak gradient even in patients with a normal cardiac index (r=0.36, p=0.016, and r=0.49, p=0.001, respectively). Receiver operating characteristic curve analysis identified an area under the curve of 0.61 for patients with all types of obstruction, with a cut-off point of 45 mmHg correctly classifying 64% of patients with arch obstruction (sensitivity 39%, specificity 89%). In patients with aortic arch reconstruction who had a cardiac index ⩾3 L/min/m², a cut-off point of 23 mmHg correctly classified 69% of patients (71% sensitivity, 50% specificity) with an area under the curve of 0.82. CONCLUSION: The non-invasive assessment of aortic obstruction remains challenging. The greatest correlation of Doppler indices was noted in patients with aortic reconstruction and a normal cardiac index.


Assuntos
Aorta Torácica/cirurgia , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/cirurgia , Ecocardiografia Doppler/métodos , Pressão Arterial , Cateterismo Cardíaco , Feminino , Humanos , Lactente , Masculino , Curva ROC , Estudos Retrospectivos , Sensibilidade e Especificidade , Procedimentos Cirúrgicos Vasculares
10.
Pediatr Crit Care Med ; 15(1): 28-34, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24201857

RESUMO

OBJECTIVE: Determine sildenafil exposure and hemodynamic effect in children after Fontan single-ventricle surgery. DESIGN: Prospective dose-escalation trial. SETTING: Single-center pediatric catheterization laboratory. PATIENTS: Nine children post Fontan single-ventricle surgical palliation and undergoing elective cardiac catheterization: median (range) age and weight, 5.2 years (2.5-9.4 yr) and 16.3 kg (9.5-28.1 kg). Five children (55%) were boys, and six of nine (67%) had a systemic right ventricle. INTERVENTIONS: Catheterization and echocardiography performed before and immediately after single-dose IV sildenafil (0.25, 0.35, or 0.45 mg/kg over 20 min). MEASUREMENTS AND MAIN RESULTS: Peak sildenafil and desmethyl sildenafil concentration, change in hemodynamic variables measured by cardiac catheterization and echocardiography. Maximum sildenafil concentrations ranged from 124 to 646 ng/mL and were above the in vitro threshold needed for 77% phosphodiesterase type-5 inhibition in eight of nine children and 90% inhibition in seven of seven children with doses more than or equal to 0.35 mg/kg. Sildenafil improved stroke volume (+22%, p = 0.05) and cardiac output (+10%, p = 0.01) with no significant change in heart rate in eight of nine children. Sildenafil also lowered systemic (-16%, p = 0.01) and pulmonary vascular resistance index in all nine children (median baseline pulmonary vascular resistance index 2.4 [range, 1.3-3.7]; decreased to 1.9 [0.8-2.7] Wood Units × m; p = 0.01) with no dose-response effect. Pulmonary arterial pressures decreased (-10%, p = 0.02) and pulmonary blood flow increased (9%, p = 0.02). There was no change in myocardial performance index and no adverse events. CONCLUSIONS: After Fontan surgery, sildenafil infusion acutely improves cardiopulmonary hemodynamics, increasing cardiac index. For the range of doses studied, exposure was within the acute safety range reported in adult subjects.


Assuntos
Técnica de Fontan , Hemodinâmica/efeitos dos fármacos , Inibidores da Fosfodiesterase 5/farmacologia , Piperazinas/farmacologia , Sulfonas/farmacologia , Pressão Arterial/efeitos dos fármacos , Cateterismo Cardíaco , Criança , Pré-Escolar , Ecocardiografia , Feminino , Cardiopatias Congênitas/cirurgia , Frequência Cardíaca/efeitos dos fármacos , Humanos , Masculino , Inibidores da Fosfodiesterase 5/farmacocinética , Piperazinas/farmacocinética , Cuidados Pós-Operatórios , Estudos Prospectivos , Artéria Pulmonar/fisiologia , Purinas/farmacocinética , Purinas/farmacologia , Citrato de Sildenafila , Volume Sistólico/efeitos dos fármacos , Sulfonas/farmacocinética , Resistência Vascular/efeitos dos fármacos
11.
Artigo em Inglês | MEDLINE | ID: mdl-38459240

RESUMO

PURPOSR: This study created 3D CFD models of the Norwood procedure for hypoplastic left heart syndrome (HLHS) using standard angiography and echocardiogram data to investigate the impact of shunt characteristics on pulmonary artery (PA) hemodynamics. Leveraging routine clinical data offers advantages such as availability and cost-effectiveness without subjecting patients to additional invasive procedures. METHODS: Patient-specific geometries of the intrathoracic arteries of two Norwood patients were generated from biplane cineangiograms. "Virtual surgery" was then performed to simulate the hemodynamics of alternative PA shunt configurations, including shunt type (modified Blalock-Thomas-Taussig shunt (mBTTS) vs. right ventricle-to-pulmonary artery shunt (RVPAS)), shunt diameter, and pulmonary artery anastomosis angle. Left-right pulmonary flow differential, Qp/Qs, time-averaged wall shear stress (TAWSS), and oscillatory shear index (OSI) were evaluated. RESULTS: There was strong agreement between clinically measured data and CFD model output throughout the patient-specific models. Geometries with a RVPAS tended toward more balanced left-right pulmonary flow, lower Qp/Qs, and greater TAWSS and OSI than models with a mBTTS. For both shunt types, larger shunts resulted in a higher Qp/Qs and higher TAWSS, with minimal effect on OSI. Low TAWSS areas correlated with regions of low flow and changing the PA-shunt anastomosis angle to face toward low TAWSS regions increased TAWSS. CONCLUSION: Excellent correlation between clinically measured and CFD model data shows that 3D CFD models of HLHS Norwood can be developed using standard angiography and echocardiographic data. The CFD analysis also revealed consistent changes in PA TAWSS, flow differential, and OSI as a function of shunt characteristics.

12.
AIDS ; 38(7): 1090-1093, 2024 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-38691053

RESUMO

People with HIV are at increased risk of cardiac dysfunction; however, limited tools are available to identify patients at highest risk for future cardiac disease. We performed proteomic profiling using plasma samples from children and young adults with perinatally acquired HIV without clinical cardiac disease, comparing samples from participants with and without an abnormal myocardial performance index (MPI). We identified four proteins independently associated with subclinical cardiac dysfunction: ST2, CA1, EN-RAGE, and VSIG2.


Assuntos
Biomarcadores , Infecções por HIV , Proteômica , Humanos , Infecções por HIV/complicações , Biomarcadores/sangue , Masculino , Feminino , Criança , Adolescente , Adulto Jovem , Adulto , Fibrose , Cardiopatias/sangue
13.
J Am Soc Echocardiogr ; 37(2): 119-170, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38309834

RESUMO

Echocardiography is a fundamental component of pediatric cardiology, and appropriate indications have been established for its use in the setting of suspected, congenital, or acquired heart disease in children. Since the publication of guidelines for pediatric transthoracic echocardiography in 2006 and 2010, advances in knowledge and technology have expanded the scope of practice beyond the use of traditional modalities such as two-dimensional, M-mode, and Doppler echocardiography to evaluate the cardiac segmental structures and their function. Adjunct modalities such as contrast, three-dimensional, and speckle-tracking echocardiography are now used routinely at many pediatric centers. Guidelines and recommendations for the use of traditional and newer adjunct modalities in children are described in detail in this document. In addition, suggested protocols related to standard operations, infection control, sedation, and quality assurance and improvement are included to provide an organizational structure for centers performing pediatric transthoracic echocardiograms.


Assuntos
Cardiologia , Cardiopatias , Criança , Humanos , Estados Unidos , Ecocardiografia/métodos , Ecocardiografia Doppler/métodos
14.
Am J Obstet Gynecol ; 208(1): 64.e1-7, 2013 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-23063019

RESUMO

OBJECTIVE: The importance of maternal autoantibody levels in congenital heart block and elucidation of maternal factors that may reduce disease burden require further clarification. STUDY DESIGN: Pregnancies complicated by maternal anti-Ro antibodies from 2007 through 2011 were retrospectively reviewed. RESULTS: In all, 33 women were followed up throughout pregnancy. Semiquantitative maternal anti-La levels were significantly higher in pregnancies complicated by fetal heart block of any degree (median difference, 227.5; P = .04), but there was no difference in maternal anti-Ro levels. In all, 94% of fetuses maintained normal conduction when the mother was treated with hydroxychloroquine or daily prednisone therapy throughout pregnancy, compared to 59% in the untreated group (odds ratio, 0.1; P = .04). CONCLUSION: Pregnancies complicated by fetal heart block did not have higher levels of maternal anti-Ro antibodies. Maternal anti-La level may be a useful predictor of fetal heart block. Maternal treatment with either hydroxychloroquine or daily low-dose prednisone throughout pregnancy may provide a protective effect.


Assuntos
Anti-Inflamatórios/uso terapêutico , Anticorpos Antinucleares/sangue , Bloqueio Cardíaco/congênito , Bloqueio Cardíaco/tratamento farmacológico , Hidroxicloroquina/uso terapêutico , Prednisona/uso terapêutico , Adolescente , Adulto , Anticorpos Antinucleares/imunologia , Feminino , Bloqueio Cardíaco/imunologia , Humanos , Troca Materno-Fetal/imunologia , Gravidez , Estudos Retrospectivos , Resultado do Tratamento
15.
Pediatr Crit Care Med ; 14(6): 593-600, 2013 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-23823195

RESUMO

OBJECTIVES: To determine sildenafil exposure and hemodynamic effect in children after stage II single-ventricle surgery. DESIGN: Prospective, dose escalation trial. SETTING: Single-center, pediatric catheterization laboratory. PATIENTS: Twelve children poststage II single-ventricle surgical palliation and undergoing elective cardiac catheterization: median age 1.9 years (range, 0.8, 4.0), weight 11 kg (8, 13), nine females, and 10 with a single right ventricle. INTERVENTIONS: Catheterization and echocardiography performed before and immediately after single-dose IV sildenafil (0.125, 0.25, 0.35, or 0.45 mg/kg over 20 min). MEASUREMENTS: Peak sildenafil and desmethyl sildenafil concentration, change in hemodynamic parameters measured by cardiac catheterization and echocardiography including indexed pulmonary vascular resistance, and myocardial performance. MAIN RESULTS: Maximum sildenafil concentrations ranged from 92 to 775 ng/mL and were above the in vitro threshold needed for 77% phosphodiesterase type 5 (PDE-5) inhibition in 80% of subjects and 90% inhibition in 80% of subjects with doses ≥0.35 mg/kg. Sildenafil lowered pulmonary vascular resistance index in all 12 subjects (median pulmonary vascular resistance index 2.2 [range, 1.6, 7.9]; decreased to 1.7 [1.2, 5.4] WU × m; p < 0.01) with no dose-response effect. Sildenafil improved pulmonary blood flow (+8% [0, 20], p = 0.04) and saturations (+2% [0, 16], p = 0.04) in those with baseline pulmonary vascular resistance index ≥ 2 WU × m (n = 7). Change in saturations correlated inversely with change in pulmonary vascular resistance index (r = 0.74, p < 0.01). Sildenafil also lowered mean blood pressure (-12% [-20, +10]; p = 0.04). There was no change in cardiac index and no effect on myocardial performance. There were no adverse events. CONCLUSIONS: Sildenafil demonstrated nonlinear exposure with high interindividual variability but was well tolerated and effectively lowered pulmonary vascular resistance index in all subjects. Sildenafil did not acutely improve myocardial performance or increase cardiac index.


Assuntos
Ventrículos do Coração/anormalidades , Hemodinâmica/efeitos dos fármacos , Síndrome do Coração Esquerdo Hipoplásico/tratamento farmacológico , Inibidores da Fosfodiesterase 5/uso terapêutico , Piperazinas/uso terapêutico , Sulfonas/uso terapêutico , Cateterismo Cardíaco , Quimioterapia Adjuvante , Criança , Pré-Escolar , Relação Dose-Resposta a Droga , Esquema de Medicação , Feminino , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/cirurgia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Lactente , Injeções Intravenosas , Modelos Lineares , Masculino , Cuidados Paliativos/métodos , Inibidores da Fosfodiesterase 5/farmacocinética , Inibidores da Fosfodiesterase 5/farmacologia , Piperazinas/farmacocinética , Piperazinas/farmacologia , Estudos Prospectivos , Purinas/farmacocinética , Purinas/farmacologia , Purinas/uso terapêutico , Citrato de Sildenafila , Sulfonas/farmacocinética , Sulfonas/farmacologia , Resultado do Tratamento , Ultrassonografia , Resistência Vascular/efeitos dos fármacos
16.
Echocardiography ; 30(10): E307-9, 2013 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-24102990

RESUMO

Residual intracardiac or extracardiac shunting is frequently seen in patients with repaired congenital heart disease and can cause systemic hypoxemia. We present the case of an adult with tetralogy of Fallot who underwent two corrective surgical procedures as a child with subsequent pulmonary valve replacement due to residual pulmonary insufficiency. Further details of her operative history were otherwise unknown. After being lost to follow-up for many years, she presented with unexplained cyanosis and a markedly abnormal agitated saline study on transthoracic echocardiography in which only the left heart filled after contrast administration. We review the differential diagnosis for such a presentation and discuss the utility of cardiac magnetic resonance imaging (MRI) to define the etiology. Cardiac MRI, a frequently used imaging modality to longitudinally assess adult patients with congenital heart disease, provides excellent image quality of cardiac structures and the ability to perform angiography in a variety of imaging planes. These advantages render cardiac MRI, a useful modality to determine the etiology of unexplained cyanosis in these patients as both intracardiac and extracardiac shunts can be detected.


Assuntos
Malformações Arteriovenosas/diagnóstico , Cianose/etiologia , Complicações Pós-Operatórias/diagnóstico , Artéria Pulmonar/anormalidades , Insuficiência da Valva Pulmonar/diagnóstico , Tetralogia de Fallot/cirurgia , Veia Cava Superior/anormalidades , Malformações Arteriovenosas/etiologia , Diagnóstico Diferencial , Ecocardiografia , Feminino , Humanos , Hipóxia/etiologia , Imageamento Tridimensional , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Insuficiência da Valva Pulmonar/complicações , Insuficiência da Valva Pulmonar/cirurgia , Tetralogia de Fallot/complicações , Veia Cava Superior/patologia
17.
World J Pediatr Congenit Heart Surg ; 14(1): 95-97, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36266955

RESUMO

The double switch operation for congenitally corrected transposition of the great arteries (CC-TGA) has been associated with high rates of reintervention, including the need for pulmonary valve replacement. Hybrid interventional approaches can avoid bypass when complex anatomy complicates traditional catheter-based approaches. We present a case of successful transcatheter pulmonary valve replacement via hybrid per-ventricular approach with pre-procedural planning aided by 3D segmentation of skeletal and cardiac anatomy in a patient with surgically corrected CC-GTA.


Assuntos
Transposição das Grandes Artérias , Procedimentos Cirúrgicos Cardíacos , Valva Pulmonar , Transposição dos Grandes Vasos , Humanos , Transposição das Grandes Artérias/métodos , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Transposição dos Grandes Vasos/complicações , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Resultado do Tratamento
18.
Cardiol Young ; 22(2): 216-8, 2012 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-22380847

RESUMO

We present a patient with DiGeorge syndrome and an isolated right subclavian artery arising from the right pulmonary artery via a right-sided ductus arteriosus. The patient showed a subclavian and pulmonary steal with perfusion of the right arm and right lung via retrograde circulation in the right vertebral artery. The patient underwent successful surgical repair.


Assuntos
Síndrome de DiGeorge/complicações , Permeabilidade do Canal Arterial/complicações , Permeabilidade do Canal Arterial/diagnóstico , Artéria Pulmonar/anormalidades , Síndrome do Roubo Subclávio/congênito , Síndrome do Roubo Subclávio/complicações , Feminino , Humanos , Lactente , Artéria Pulmonar/cirurgia , Artéria Subclávia/anormalidades , Artéria Subclávia/cirurgia , Síndrome do Roubo Subclávio/diagnóstico , Síndrome do Roubo Subclávio/cirurgia
19.
Am Heart J ; 161(1): 138-44, 2011 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-21167346

RESUMO

BACKGROUND: Mortality between stage I and II palliation for hypoplastic left heart syndrome (HLHS) has been associated with arrhythmias. The stage-related proportion, associations, and clinical impact of arrhythmias in patients with HLHS have not been evaluated. In addition, arrhythmia subtypes have not been described in this patient group. METHODS: We performed a retrospective analysis of all patients at Duke University Medical Center who received one or more palliative stages for HLHS from September 2000 to October 2008. RESULTS: Overall, 49 (57%) of 86 patients had 63 arrhythmias. The majority of arrhythmias occurred between stage I and II, with 44 (51%) of 86 patients manifesting a new arrhythmia. Arrhythmias occurring in this interval tended to be associated with a higher mortality compared with arrhythmias occurring after stage II (odds ratio = 3.2 [95% CI 0.84-12.0], P = .09). Overall mortality was similar in patients with and without arrhythmias (P = .99). Supraventricular tachycardia was the most common arrhythmia (16/63; 25%), but persistent bradycardias (sinus node dysfunction or high-grade atrioventricular block) had the worst clinical outcome with 73% mortality (8/11). There was no association between arrhythmia occurrence and degree of tricuspid regurgitation, left ventricular hypertension, genetic syndrome, type of stage I operation, or need for extracorporeal membrane oxygenation. CONCLUSIONS: A large proportion of patients with HLHS experience serious arrhythmias requiring therapy, especially between stage I and II. Persistent bradycardia following stage I is associated with a high mortality rate. Considering all arrhythmia patients, overall mortality was not different compared with the arrhythmia-free group.


Assuntos
Arritmias Cardíacas/etiologia , Procedimentos Cirúrgicos Cardíacos/métodos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/fisiopatologia , Pré-Escolar , Progressão da Doença , Eletrocardiografia , Feminino , Seguimentos , Frequência Cardíaca , Humanos , Síndrome do Coração Esquerdo Hipoplásico/complicações , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Lactente , Recém-Nascido , Masculino , Prognóstico , Estudos Retrospectivos , Fatores de Tempo
20.
J Am Soc Echocardiogr ; 34(5): 553-561, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33516940

RESUMO

Over the 12 months since the start of the coronavirus disease 2019 pandemic, an explosion of investigation and an increase in experience have led to vast improvement in our knowledge about this disease. However, coronavirus disease 2019 remains a huge public health threat.


Assuntos
COVID-19/diagnóstico por imagem , Atenção à Saúde , Ecocardiografia/métodos , Cardiopatias Congênitas/diagnóstico por imagem , Sociedades Médicas , Criança , Atenção à Saúde/métodos , Feminino , Humanos , Recém-Nascido , Gravidez , Estados Unidos
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