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1.
J Card Surg ; 2019 Aug 26.
Artigo em Inglês | MEDLINE | ID: mdl-31449687

RESUMO

Surgical management of complex congenital heart disease (CHD) is challenging. Three-dimensional (3D) printing can improve multidisciplinary team decision-making, patient and family understanding, and education of medical professionals. We describe 3D printing for surgical management of five patients with complex CHD. The anatomical details of the 3D printed models were instrumental in planning surgical techniques especially in determining between single ventricle, 1.5 ventricle, and biventricular repair.

2.
Catheter Cardiovasc Interv ; 93(7): E381-E384, 2019 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-30702202

RESUMO

An infant with hypoplastic left heart syndrome (HLHS) presented with complete heart block and severe myocardial dysfunction requiring ECMO support due to complete left main coronary artery (LMCA) thrombosis. Current guidelines for managing coronary artery thrombosis in infants with single ventricle physiology are inadequate. We describe successful LMCA and branch recanalization via intra coronary infusion of recombinant tissue plasminogen activator and discuss management of acute coronary thrombosis in children with single ventricle physiology.

4.
Pediatr Cardiol ; 39(8): 1669-1675, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30105466

RESUMO

Patients with repaired Tetralogy of Fallot (rToF) can have significant right and left ventricular dysfunction which can impact clinical outcome. Conventional echocardiographic evaluation using ejection fraction can be limited in detecting early stages of deteriorating left ventricular (LV) systolic function. Speckle-tracking echocardiography (STE) provides a promising emerging tool for early detection of LV systolic dysfunction. The aim of this cross-sectional study is to evaluate the left ventricular global and regional function using STE in rToF patients with preserved left ventricular ejection fraction to detect early signs of LV dysfunction. The study is a retrospective review of the medical records and transthoracic echocardiograms of rTOF patients who were seen in the pediatric cardiology clinic at our institution from August 2016 till May 2018. The rTOF group was compared with a matched healthy control group. Offline strain analysis of TTE studies was completed in each subject according to mode set by the American Society of Echocardiography. Twenty-two patients with rTOF and 22 age-matched controls (rTOF 18.7 ± 6 years and control 14.6 ± 9.6 years, P value 0.19) were included during the study period. The rTOF group consisted of 12 males and 10 females, whereas the control group consisted of 15 males and 7 females. There was no significant difference in terms of LV ejection fraction using both 2-chamber as well as 4-chamber views. Global LV longitudinal strain was significantly lower in the rTOF group compared with the control group (rTOF - 15.92 ± 3.16 vs control - 22.79 ± 2.45, P value 0.00). All 17 segments showed significantly lower longitudinal strains in rTOF group compared with the control group. Global circumferential left ventricle strain was significantly lower in the rTOF patients compared with the control group (- 17.24 ± 5.86 vs - 22.74 ± 3.99, P value 0.001). 13 out of 16 segments had lower circumferential strains in the rTOF patients compared with controls. Asymptomatic surgically repaired TOF patients exhibited abnormal segmental and global left ventricle longitudinal and circumferential strains despite having normal left ventricle ejection fraction. Our findings suggest subclinical damage to the left ventricle systolic function post-surgical repair of TOF. 2-D speckle-tracking echocardiography may be used as a valuable method in the early recognition of left myocardial systolic function impairment in patients after TOF repair. The application of this method to guide clinical decision and management of rTOF patients such as earlier pulmonary valve replacement or risk stratification for sudden cardiac death needs further investigation.

5.
Pediatr Cardiol ; 39(8): 1657-1662, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30105467

RESUMO

Embolization of systemic to pulmonary artery collaterals to regulate pulmonary arterial flow or pressure of the cavopulmonary circulation in patients with single ventricle is a common practice. The relative incidence and impact of this practice on future interventions like coronary artery bypass grafting is poorly understood. This study aims to evaluate the frequency and implications of internal mammary artery (IMA) embolization in the single ventricle (SV) population. A retrospective chart review was performed of SV patients who underwent cardiac catheterization before and after Fontan procedure between February 2007 and 2017. Data were collected from two tertiary care centers in the Midwest. Of the 304 SV patients, 62 (20.4%) underwent embolization of one or more IMAs, whereas 242 (79.6%) did not. The rate of embolization of IMA was 40.5% in one center and 14.5% in the second center. Among patients who received IMA embolization, left internal mammary artery (LIMA) embolization was seen in 6 (9.7%) patients. Majority of patients underwent either right internal mammary artery (RIMA) embolization (n = 25; 40.3%) or RIMA and LIMA embolization (n = 27; 43.5%). IMA embolization in SV patients is common. Embolizing IMAs early in life will likely eliminate a valuable graft option for coronary artery bypass grafting should it be required in the future care of these patients. Multi-center, prospective, nation-wide studies are warranted to examine coronary artery disease in the SV population and true frequency of IMA embolization. Delineation of which IMAs were embolized is a necessary in surgical and cardiac intervention national data, such as Society of Thoracic Surgeons (STS) database. All measures should be taken to preserve IMAs patency, if deemed feasible and safe.

6.
Pediatr Cardiol ; 39(4): 818-823, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29396581

RESUMO

Prior to the Fontan procedure, patients with single ventricle physiology with Glenn shunt are typically referred for cardiac catheterization to assess hemodynamics and potentially provide interventional measures. Currently, echocardiography provides detailed information which together with other non-invasive imaging such as CT scan and MRI may obviate the need for routine cardiac catheterization prior to the Fontan procedure. In this study, we examine the findings in cardiac catheterization in this population to determine: (a) the accuracy of echocardiography in providing adequate information prior to the Fontan procedure, particularly in identifying those in need of per-catheter intervention, and (b) the percentage of patients requiring interventional procedures during cardiac catheterization. We performed a retrospective chart review of echocardiographic and cardiac catheterization data for patients who underwent pre-Fontan cardiac catheterization at our center in the period from 02/01/2008 to 02/28/2017. We aimed to re-examine the necessity of routine cardiac catheterization in all single ventricle patients. This was performed through examining pre-catheterization echocardiography reports and comparing them to findings of the subsequent cardiac catheterization reports. Echocardiography reports were evaluated for accuracy in identifying significant anatomical or hemodynamic findings, which may impact success of Fontan procedure as well as the ability of echocardiography to predict findings important to know prior to the Fontan procedure. In this cohort of 40 children, 3 patients were found to have significant hemodynamic findings through cardiac catheterization which were not previously known by echocardiography. In addition, 28 out of 40 patients (70%) required interventional procedures to address significant abnormalities (systemic to pulmonary arterial collaterals, pulmonary artery stenosis, aortic arch stenosis, etc.). All cases of aortic arch stenosis were detected by echocardiography, however, all patients who required systemic to pulmonary arterial or left SVC embolization were not detected by echocardiography. Furthermore, echocardiography did not detect the need for branch pulmonary artery stenosis in 50% of cases. Cardiac catheterization appears to be an essential part of patient assessment prior to Fontan completion in patients with single ventricle physiology. This current practice may change in the future if a non-invasive screening tool is found to have high positive and negative predictive values in identifying the subset of patients who require potential intervention in pre-Fontan cardiac catheterization.


Assuntos
Cateterismo Cardíaco/métodos , Ecocardiografia/métodos , Cardiopatias Congênitas/diagnóstico , Cuidados Pré-Operatórios/métodos , Pré-Escolar , Feminino , Técnica de Fontan/métodos , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/cirurgia , Hemodinâmica/fisiologia , Humanos , Lactente , Masculino , Estudos Retrospectivos
7.
Pediatr Cardiol ; 38(7): 1434-1440, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28702716

RESUMO

The correlation between mean pulmonary artery wedge pressure (PAWP) and left atrial mean pressure (LAMP) has been poorly studied in patients with single ventricle (SV) physiology (Bernstein et al. in Pediatr Cardiol 33: 15-20 2012). The aim of this study is to determine if the PAWP can be used safely as a surrogate to the LAMP to calculate the pulmonary vascular resistance (PVR) during the pre-Fontan evaluation. Also, we aimed to understand if the presence of significant systemic-to-pulmonary collaterals (SPCs) is a confounding factor for accurate estimation of the LAMP. From February 2007 to February 2017, forty-one patients were eligible for inclusion in the study. These patients were varied in terms of underlying cardiac malformation. Sex distribution was equal with 20 males and 21 females. Median weight was 11.8 kg, median body surface area was 0.51 m2, median age at catheterization was 2 years, and the median age at Glenn surgery was 5 months. We found the left and right PAWP and LAMP correlated strongly and the differences in the measurements were negligible. Similar findings were observed when calculating PVRs using PAWP and LAMP. These findings were more pronounced in the absence of significant SPCs. PAWP and LAMP correlation was still valid in the presence of significant SPCs; however, the correlation among the calculated PVRs was more attenuated.


Assuntos
Pressão Atrial/fisiologia , Átrios do Coração/fisiopatologia , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Pressão Propulsora Pulmonar/fisiologia , Resistência Vascular/fisiologia , Adolescente , Biomarcadores , Cateterismo Cardíaco/métodos , Criança , Pré-Escolar , Feminino , Ventrículos do Coração/fisiopatologia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Lactente , Masculino , Artéria Pulmonar/fisiopatologia , Artéria Pulmonar/cirurgia , Estudos Retrospectivos , Adulto Jovem
8.
Pediatr Cardiol ; 38(6): 1305-1308, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28512719

RESUMO

Congenital complete atrioventricular block (CCAVB) is a rare condition with an incidence of 1 of 20,000 live births. Hypoplastic left heart syndrome (HLHS) occurs more frequently than CCAVB and occurs in 1 of 5000 live births. HLHS in association with CCAVB is exceedingly rare. In this report, we describe a rare case of HLHS and CCAVB diagnosed in utero. Postnatal diagnosis, management and outcome are presented as well as review of the medical literature.


Assuntos
Bloqueio Cardíaco/congênito , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Adulto , Procedimentos Cirúrgicos Cardiovasculares , Feminino , Bloqueio Cardíaco/complicações , Bloqueio Cardíaco/diagnóstico por imagem , Bloqueio Cardíaco/cirurgia , Transplante de Coração , Humanos , Síndrome do Coração Esquerdo Hipoplásico/complicações , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Cuidados Paliativos , Ultrassonografia Pré-Natal
9.
Pediatr Cardiol ; 38(5): 1084-1086, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28396933

RESUMO

We present a case of small coronary sinus defect detected after transcatheter device closure of a large secundum atrial septal defect. Although device erosion of the dilated coronary sinus is suspected, the defect in the coronary sinus may have been present prior to ASD device closure. Dilated coronary sinus may be a risk factor when closing a secundum ASD with a device. To the best of our knowledge, coronary sinus erosion by an ASD device has not yet been reported in the medical literature.


Assuntos
Seio Coronário/lesões , Comunicação Interatrial/cirurgia , Dispositivo para Oclusão Septal/efeitos adversos , Lesões do Sistema Vascular/diagnóstico por imagem , Cateterismo Cardíaco , Pré-Escolar , Seio Coronário/diagnóstico por imagem , Ecocardiografia , Feminino , Humanos , Implantação de Prótese/efeitos adversos , Lesões do Sistema Vascular/etiologia
10.
Catheter Cardiovasc Interv ; 90(7): 1154-1157, 2017 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-28296217

RESUMO

We present a rare case of unroofed coronary sinus in a patient who underwent supra-annular tricuspid valve replacement with consequent drainage of the coronary sinus to the right ventricle. It is unclear whether the coronary sinus was unroofed congenitally or iatrogenically. This rare setup resulted in significant cyanosis. The abnormal drainage was successfully closed via trans-catheter delivery of covered stents with resolution of the cyanosis. © 2017 Wiley Periodicals, Inc.


Assuntos
Cateterismo Cardíaco/instrumentação , Seio Coronário , Comunicação Interatrial/terapia , Implante de Prótese de Valva Cardíaca , Ventrículos do Coração , Stents , Valva Tricúspide/cirurgia , Adulto , Angiografia Coronária , Seio Coronário/diagnóstico por imagem , Seio Coronário/fisiopatologia , Cianose/diagnóstico , Cianose/etiologia , Cianose/fisiopatologia , Ecocardiografia Doppler em Cores , Ecocardiografia Transesofagiana , Feminino , Comunicação Interatrial/diagnóstico por imagem , Comunicação Interatrial/fisiopatologia , Implante de Prótese de Valva Cardíaca/efeitos adversos , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Hemodinâmica , Humanos , Desenho de Prótese , Resultado do Tratamento , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/fisiopatologia , Função Ventricular Direita
11.
13.
BMJ Case Rep ; 20142014 May 02.
Artigo em Inglês | MEDLINE | ID: mdl-24792020

RESUMO

A 14-week-old boy who is known to have a single right kidney presented to our emergency department with history of fever for 1 day. A sepsis work up was performed (complete blood count, blood culture, urine culture and lumbar puncture) in the emergency room. On the second day of admission he developed swelling in the parieto-occipital area. Head CT showed crescent-shaped extra cranial area of homogeneous low attenuation. He remained asymptomatic with a stable haemoglobin and haematocrit. Swelling subsequently resolved within 5 weeks.


Assuntos
Edema/diagnóstico por imagem , Couro Cabeludo/diagnóstico por imagem , Edema/complicações , Febre/etiologia , Humanos , Lactente , Masculino , Pielonefrite/complicações , Pielonefrite/diagnóstico , Punção Espinal , Tomografia Computadorizada por Raios X
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