RESUMO
BACKGROUND: Morphological features including interarterial course, intramural course, high ostial location and slit-like ostium are presumed risk factors for sudden cardiac death in children with anomalous aortic origin of the coronary artery (AAOCA). To facilitate clinical risk stratification, the diagnostic accuracy of CT angiography for individual risk factors in the setting of AAOCA must be established. OBJECTIVE: We assessed diagnostic accuracy of standardized CT angiography interpretation for morphological characteristics that might determine risk in children with AAOCA by comparing them to surgical findings. MATERIALS AND METHODS: We created a standardized protocol for CT angiography of AAOCA and retrospectively evaluated diagnostic performance in 25 consecutive surgical patients. Relevant morphological variables in AAOCA were assessed by three independent blinded readers, with surgery as the reference standard. We used Cohen kappa coefficients and accuracies to assess agreement between readers and surgical findings, and we calculated intraclass correlation coefficients to compare length of the intramural course. RESULTS: CT angiography correctly identified AAOCA in all patients. For the three readers, accuracies for detecting ostial stenosis were 84%, 94% and 96%; for high ostial origin, accuracies were 76%, 78% 82%; for intramurality using the peri-coronary fat sign, accuracies were 98%, 96% and 92%; and for intramurality using oval shape of coronary artery, accuracies were 98%, 94% and 92%. The intraclass correlation coefficients (ICCs) for predicting intramural length among the three readers were 0.67, 0.75 and 0.81 using peri-coronary fat, and 0.69, 0.50 and 0.81 using oval shape, respectively. CONCLUSION: CT angiography reliably identified AAOCA in all children and detected the presence of intramurality with high accuracy.
Assuntos
Angiografia por Tomografia Computadorizada , Anomalias dos Vasos Coronários , Aorta , Criança , Angiografia Coronária , Anomalias dos Vasos Coronários/diagnóstico por imagem , Humanos , Estudos RetrospectivosRESUMO
Isolated pulmonary artery (PA) of ductal origin (IPADO) is a rare cardiac defect which requires surgical repair, with or without preceding palliation. We sought to determine the impact of treatment strategy on outcomes. Retrospective study of consecutive patients with IPADO that underwent staged or primary repair from 1/05 to 9/16 at 6 Congenital Cardiac Research Collaborative centers. Patients with single ventricle physiology, major aortopulmonary collaterals, or bilateral IPADO were excluded. Primary outcome was isolated PA z-score at late follow-up. Secondary outcomes included PA symmetry index (isolated:confluent PA diameter) and reintervention burden. Propensity score adjustment was used to account for baseline differences. Of 60 patients in the study cohort, 26 (43%) underwent staged and 34 (57%) primary repair. The staged and primary repair groups differed in weight at diagnosis and presence of other heart disease but not in baseline PA dimensions. Staged patients underwent ductal stent (n = 16) or surgical shunt (n = 10) placement followed by repair at 210 vs. 21 days in the primary repair group (p < 0.001). At median follow-up of 4.5 years post-repair, after adjustment, isolated PA z-score (- 0.74 [- 1.75, - 0.26] vs. - 1.95 [- 2.91, - 1.59], p = 0.012) and PA symmetry index (0.81 [0.49, 1.0] vs. 0.55 [0.48, 0.69], p = 0.042) significantly favored the staged repair group. Freedom from PA reintervention was not different between groups (adjusted HR 0.78 [0.41, 1.48]; p = 0.445). A staged approach to repair of IPADO is associated with superior isolated PA size and symmetry at late follow-up. Consideration should be given to initial palliation in IPADO patients, when feasible.
Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Cardiopatias Congênitas/cirurgia , Artéria Pulmonar/anormalidades , Artéria Pulmonar/cirurgia , Cateterismo Cardíaco/métodos , Permeabilidade do Canal Arterial/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Cuidados Paliativos/métodos , Reoperação/métodos , Estudos Retrospectivos , Stents , Resultado do TratamentoRESUMO
OBJECTIVE: To describe a monthly outreach pediatric cardiology clinic established to better understand the cardiac needs of immigrant/resettled refugee children. STUDY DESIGN: Data obtained between 2014 and 2017 from a monthly pediatric cardiology clinic at a Federally Qualified Health Center were analyzed using descriptive statistics. RESULTS: A total of 366 patients (222 male, 61%) were evaluated. Indications for referral included murmur (242, 66%), nonexertional symptoms (31, 9%), exertional symptoms (16, 4%), history of cardiac surgery/transcatheter interventions (15, 4%), previous diagnosis of heart conditions without intervention (13, 4%), arrhythmia/bradycardia (13, 4%), and others (36, 10%). Echocardiograms were performed on 136 patients (67 were abnormal, 49%). The most common final diagnoses include innocent murmur in 201 (55%), simple congenital heart disease in 61 (16%), complex congenital heart disease in 3 (1%), and acquired heart disease in 3 (1%). A total of 15 patients (4%) were ultimately determined to require surgical or cardiac catherization as an intervention. Patients have been followed for a median of 0.7 years (range 0-3.3 years). CONCLUSIONS: Rates of abnormal echocardiograms suggest a greater likelihood of congenital or acquired heart disease at time of initial consultation compared with nonimmigrant/refugee populations. The most common indication for referral to the outreach pediatric cardiology clinic was a murmur. Collaborative efforts between physicians and support services are essential in assisting this vulnerable population access pediatric subspecialty care.
Assuntos
Instituições de Assistência Ambulatorial/estatística & dados numéricos , Emigrantes e Imigrantes/estatística & dados numéricos , Cardiopatias Congênitas/diagnóstico , Sopros Cardíacos/diagnóstico , Refugiados/estatística & dados numéricos , Adolescente , Criança , Pré-Escolar , Ecocardiografia/estatística & dados numéricos , Feminino , Cardiopatias Congênitas/epidemiologia , Sopros Cardíacos/epidemiologia , Humanos , Lactente , Masculino , Área Carente de Assistência Médica , Estudos Retrospectivos , Texas/epidemiologiaRESUMO
BACKGROUND: Infants with ductal-dependent pulmonary blood flow may undergo palliation with either a patent ductus arteriosus (PDA) stent or a modified Blalock-Taussig (BT) shunt. A balanced multicenter comparison of these 2 approaches is lacking. METHODS: Infants with ductal-dependent pulmonary blood flow palliated with either a PDA stent or a BT shunt from January 2008 to November 2015 were reviewed from the 4 member centers of the Congenital Catheterization Research Collaborative. Outcomes were compared by use of propensity score adjustment to account for baseline differences between groups. RESULTS: One hundred six patients with a PDA stent and 251 patients with a BT shunt were included. The groups differed in underlying anatomy (expected 2-ventricle circulation in 60% of PDA stents versus 45% of BT shunts; P=0.001) and presence of antegrade pulmonary blood flow (61% of PDA stents versus 38% of BT shunts; P<0.001). After propensity score adjustment, there was no difference in the hazard of the primary composite outcome of death or unplanned reintervention to treat cyanosis (hazard ratio, 0.8; 95% confidence interval [CI], 0.52-1.23; P=0.31). Other reinterventions were more common in the PDA stent group (hazard ratio, 29.8; 95% CI, 9.8-91.1; P<0.001). However, the PDA stent group had a lower adjusted intensive care unit length of stay (5.3 days [95% CI, 4.2-6.7] versus 9.19 days [95% CI, 7.9-10.6]; P<0.001), a lower risk of diuretic use at discharge (odds ratio, 0.4; 95% CI, 0.25-0.64; P<0.001) and procedural complications (odds ratio, 0.4; 95% CI, 0.2-0.77; P=0.006), and larger (152 mm2/m2 [95% CI, 132-176] versus 125 mm2/m2 [95% CI, 113-138]; P=0.029) and more symmetrical (symmetry index, 0.84 [95% CI, 0.8-0.89] versus 0.77 [95% CI, 0.75-0.8]; P=0.008] pulmonary arteries at the time of subsequent surgical repair or last follow-up. CONCLUSIONS: In this multicenter comparison of palliative PDA stent and BT shunt for infants with ductal-dependent pulmonary blood flow adjusted for differences in patient factors, there was no difference in the primary end point, death or unplanned reintervention to treat cyanosis. However, other markers of morbidity and pulmonary artery size favored the PDA stent group, supporting PDA stent as a reasonable alternative to BT shunt in select patients.
Assuntos
Procedimento de Blalock-Taussig , Cateterismo Cardíaco/instrumentação , Permeabilidade do Canal Arterial/cirurgia , Cuidados Paliativos/métodos , Circulação Pulmonar , Stents , Procedimento de Blalock-Taussig/efeitos adversos , Procedimento de Blalock-Taussig/mortalidade , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/mortalidade , Permeabilidade do Canal Arterial/diagnóstico por imagem , Permeabilidade do Canal Arterial/mortalidade , Permeabilidade do Canal Arterial/fisiopatologia , Feminino , Mortalidade Hospitalar , Humanos , Lactente , Mortalidade Infantil , Recém-Nascido , Masculino , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Estados UnidosRESUMO
OBJECTIVES: To devise a classification scheme for ductal morphology in patients with ductal dependent pulmonary blood flow (PBF) that can be used to assess outcomes. BACKGROUND: The impact of ductal morphology on outcomes following patent ductus arteriosus (PDA) stenting is not well defined. METHODS: Patients <1 year of age who underwent PDA stenting for ductal dependent PBF at the four centers comprising the Congenital Catheterization Research Collaborative (CCRC) were included. A classification scheme for PDA morphology was devised based on a tortuosity index (TI)-Type I (straight), Type II (one turn), and Type III (multiple turns). A subtype classification was used based upon the ductal origin. RESULTS: One hundred and five patients underwent PDA stenting. TI was Type I in 58, Type II in 24, and Type III in 23 PDAs, respectively. There was a significant association between ductal origin and vascular access site (p < 0.001). Procedure times and need for >1 stent did not differ based on TI. Greater TI was associated with pulmonary artery (PA) jailing (p = 0.003). Twelve (11.4%) patients underwent unplanned reintervention, more commonly with greater TI (p = 0.022) and PA jailing (p < 0.001). At the time of subsequent surgical repair/palliative staging, PA arterioplasty was performed in 32 patients, more commonly when a PA was jailed (p = 0.048). PA jailing did not affect PA size at follow up. CONCLUSIONS: The proposed qualitative and quantitative PDA morphology classification scheme may be helpful in anticipating outcomes in patients with ductal dependent PBF undergoing PDA stenting.
Assuntos
Cateterismo Cardíaco/instrumentação , Angiografia Coronária , Cianose/etiologia , Permeabilidade do Canal Arterial/terapia , Canal Arterial/diagnóstico por imagem , Circulação Pulmonar , Stents , Cateterismo Cardíaco/efeitos adversos , Canal Arterial/fisiopatologia , Permeabilidade do Canal Arterial/complicações , Permeabilidade do Canal Arterial/diagnóstico por imagem , Permeabilidade do Canal Arterial/fisiopatologia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Valor Preditivo dos Testes , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Estados UnidosRESUMO
BACKGROUND: Use of small diameter stents in young children and jailing of side branches pose significant challenges to future re-interventions. We sought to assess the capacity to induce longitudinal fractures in undersized stents to increase vessel diameter, and side cell fractures to enlarge stenotic jailed branches. METHODS: Retrospective review of patients who underwent attempted intentional stent fractures (ISF) from 01/06-02/17. RESULTS: Twenty-two patients, median age 4.4 (1.1-47.8) years, weighing 14.3 (6.9-102) kg underwent attempted ISF in 32 vessels. Initial stent implant occurred at a median age of 1.0 (0.1-34.1) years, at the following sites: pulmonary arteries (11), pulmonary veins (14), systemic veins (6), and aorta (1). Initial diameters of the stents were 3.5-12 mm. Thirteen vessels had overlapping stents. Using high-pressure balloons, longitudinal ISF was achieved in 23 and side cell ISF in eight vessels. One longitudinal ISF attempt was unsuccessful. Three lesions were immediately re-stented after longitudinal ISF, and three lesions treated with angioplasty (1) or stenting (2) after side cell ISF. Only one complication occurred (pseudoaneurysm in a pulmonary artery after longitudinal ISF requiring placement of a covered stent). At a median follow up of 2.0 years (2 days - 10.8 years), eight patients had 16 additional interventions for restenosis at site of ISF. CONCLUSIONS: ISF can be induced safely in a variety of vascular beds using high-pressure balloons both longitudinally or through side cells. Longitudinal ISF only rarely requires immediate placement of a new stent; however, late restenosis may occur, requiring re-stenting.
Assuntos
Angioplastia com Balão/instrumentação , Stents , Doenças Vasculares/cirurgia , Adolescente , Adulto , Angioplastia com Balão/efeitos adversos , Criança , Pré-Escolar , Angiografia Coronária , Estudos de Viabilidade , Feminino , Seguimentos , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Desenho de Prótese , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Doenças Vasculares/diagnóstico por imagem , Adulto JovemRESUMO
In isolated unilateral ductal origin of a pulmonary artery (DOPA), intervention to establish reperfusion of the affected lung without direct re-anastomosis may lead to pulmonary hypertension (PH) in the contralateral lung. Multicenter retrospective review of patients with unilateral DOPA, who underwent palliation with a ductal stent (DS) or Blalock-Taussig (BT) shunt with subsequent development of PH in the contralateral lung, was conducted. Ten patients (4 females; median weight 3.2 kg, range 2.1-5.2) who underwent DS or BT shunt and developed contralateral PH were identified. Eight infants had right-sided DOPA. Stents/shunt used had a median diameter of 3.5 mm (range 2.5-5.2). After DS in 9 patients, 7 patients were exclusively treated with medical therapies, whereas 2 patients received intermediate procedures: one received an additional contralateral DS and other underwent surgical banding of the DS prior to PA reimplantation. Seven patients who underwent DS and one patient with BT shunt underwent PA reimplantation at median of 3.3 (0.6-18) months. PA pressure was documented to be normal in 5 patients immediately following PA reimplantation, 1 year later in 2 patients, and 1 patient is on Tadalafil with elevated PVR of 5.5 indexed Wood units. One patient died and one patient is awaiting surgery with normal PA pressure. We describe the development of severe contralateral PH following DS or BT shunt as the initial intervention for unilateral DOPA. Pulmonary hypertension resolved in 7/8 patients who underwent surgical PA reimplantation. The cause of PH in the normally connected lung in these cases remains unclear.
Assuntos
Procedimento de Blalock-Taussig/efeitos adversos , Cateterismo Cardíaco/métodos , Hipertensão Pulmonar/etiologia , Artéria Pulmonar/cirurgia , Stents/efeitos adversos , Angiografia/métodos , Cateterismo Cardíaco/efeitos adversos , Ecocardiografia/métodos , Feminino , Humanos , Hipertensão Pulmonar/cirurgia , Lactente , Recém-Nascido , Pulmão/cirurgia , Masculino , Cuidados Paliativos/métodos , Artéria Pulmonar/anormalidades , Estudos Retrospectivos , Resultado do TratamentoRESUMO
BACKGROUND: Anomalous aortic origin of a coronary artery is the second leading cause of sudden cardiac arrest/death in young athletes in the United States of America. Limited data are available regarding family history in this patient population. METHODS: Patients were evaluated prospectively from 12/2012 to 02/2017 in the Coronary Anomalies Program at Texas Children's Hospital. Relevant family history included the presence of CHD, sudden cardiac arrest/death, arrhythmia/pacemaker use, cardiomyopathy, and atherosclerotic coronary artery disease before the age of 50 years. The presence of one or more of these in 1st- or 2nd-degree relatives was considered significant. RESULTS: Of 168 unrelated probands (171 patients total) included, 36 (21%) had significant family history involving 19 (53%) 1st-degree and 17 (47%) 2nd-degree relatives. Positive family history led to cardiology referral in nine (5%) patients and the presence of abnormal tests/symptoms in the remaining patients. Coronary anomalies in probands with positive family history were anomalous right (27), anomalous left (five), single right coronary artery (two), myocardial bridge (one), and anomalous circumflex coronary artery (one). Conditions present in their family members included sudden cardiac arrest/death (15, 42%), atherosclerotic coronary artery disease (14, 39%), cardiomyopathy (12, 33%), CHD (11, 31%), coronary anomalies (3, 8%), myocardial bridge (1, 3%), long-QT syndrome (2, 6%), and Wolff-Parkinson-White (1, 3%). CONCLUSION: In patients with anomalous aortic origin of a coronary artery and/or myocardial bridges, there appears to be familial clustering of cardiac diseases in approximately 20% of patients, half of these with early occurrence of sudden cardiac arrest/death in the family.
Assuntos
Anomalias dos Vasos Coronários/diagnóstico , Vasos Coronários/diagnóstico por imagem , Morte Súbita Cardíaca/epidemiologia , Medição de Risco/métodos , Seio Aórtico/anormalidades , Adolescente , Criança , Angiografia Coronária , Anomalias dos Vasos Coronários/complicações , Anomalias dos Vasos Coronários/epidemiologia , Morte Súbita Cardíaca/etiologia , Feminino , Seguimentos , Humanos , Incidência , Masculino , Estudos Prospectivos , Fatores de Risco , Seio Aórtico/diagnóstico por imagem , Estados Unidos/epidemiologiaRESUMO
The evaluation of the vast majority of children with anomalous aortic origin of a coronary artery (AAOCA) and/or myocardial bridges is performed with non-invasive testing. However, a subset of these patients may benefit from invasive testing for risk stratification. All patients included in the Coronary Anomalies Program (CAP) at Texas Children's Hospital who underwent cardiac catheterization were included. Techniques included selective coronary angiograms (SCA), intravascular ultrasound (IVUS), and fractional flow reserve (FFR) measurements with provocative testing using adenosine and/or dobutamine infusions. Out of the 131 patients followed by the CAP between 12/12-4/16, 8 (6%) patients underwent 9 cath investigations at median age 13.1 (2.6-18.7) years and median weight 49.5 (11.4-142.7) kg. Six patients presented with cardiac signs/symptoms. Four patients had myocardial bridges of the left anterior descending (LAD) coronary artery, 2 patients had isolated AAOCA, and 2 patients had an anomalous left coronary artery (LCA) with an intramyocardial course of the LAD. SCA was performed in all patients. FFR was positive in 4/6 patients: IVUS showed >70% intraluminal narrowing in 3/5 patients. One patient had hemodynamic instability that reversed with catheter removal from the coronary ostium. Based on the catheterization data obtained, findings were reassuring in three patients, surgery was performed in three patients, and two patients are being medically managed/restricted from competitive sports. In our small cohort of patients, we demonstrated that IVUS and FFR can safely be performed in children and may help to risk stratify some patients with AAOCA and myocardial bridges.
Assuntos
Aorta Torácica/diagnóstico por imagem , Cateterismo Cardíaco , Angiografia Coronária , Vasos Coronários/diagnóstico por imagem , Ponte Miocárdica/terapia , Ultrassonografia de Intervenção , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Prospectivos , Medição de Risco , TexasRESUMO
OBJECTIVES: Patients with dextro-transposition of the great arteries (d-TGA) frequently undergo balloon atrial septostomy (BAS) prior to the arterial switch operation (ASO) to promote atrial-level mixing. Balloon atrial septostomy has inherent risks as an invasive procedure and may not always be necessary. This study revisits the routine utilization of BAS prior to ASO. METHODS: Single-center, retrospective review of d-TGA patients undergoing the ASO from July 2018 to March 2023. Preoperative patient characteristics, pulse oximetry oxygen saturations (SpO2), cerebral/renal near-infrared spectroscopy (NIRS) readings along with prostaglandin status at the time of the ASO were analyzed with descriptive and univariate statistics. RESULTS: Thirty patients underwent the ASO. Of these, 7 (23%) were female, 25 (83%) were white, and median weight at ASO was 3.2 kg (range 0.8-4.2). Twenty-two (73%) patients underwent BAS. There were no demographic differences between BAS and no-BAS patients. Of those who underwent BAS, there was a significant increase in SpO2 (median 83% [range 54-92] to median 87% [range 72-95], P = .007); however, there was no change in NIRS from pre-to-post BAS. Six (27%) patients in the BAS group were prostaglandin-free at ASO. Balloon atrial septostomy patients underwent the ASO later compared with no-BAS patients (median 8 [range 3-32] vs 4 [range 2-10] days old, P = .016) and had a longer hospital length of stay (median 13 [range 7-43] vs 10 [range 8-131] days, P = .108). CONCLUSIONS: While BAS is an accepted preoperative procedure in d-TGA patients to improve oxygen saturations, it is also an additional invasive procedure, does not guarantee prostaglandin-free status at the time of ASO, and may increase the interval to ASO. Birth to direct early ASO, with prostaglandin support, should be revisited as an alternative, potentially more expeditious strategy.
RESUMO
After more than a decade of extensive experimentation, the promise of stem cells to revolutionize the field of medicine has negotiated their entry into clinical trial. Adipose tissue specifically holds potential as an attainable and abundant source of stem cells. Currently undergoing investigation are adipose stem cell (ASC) therapies for diabetes and critical limb ischemia, among others. In the enthusiastic pursuit of regenerative therapies, however, questions remain regarding ASC persistence and migration, and, importantly, their safety and potential for neoplasia. To date, assays of in vivo ASC activity have been limited by early end points. We hypothesized that with time, ASCs injected subcutaneously undergo removal by normal tissue turnover and homeostasis, and by the host's immune system. In this study, a high dose of culture expanded ASCs was formulated and implanted as multicellular aggregates into immunocompromised mice, which were maintained for over one year. Animals were monitored for toxicity, and surviving cells quantified at study endpoint. No difference in growth/weight or lifespan was found between cell-treated and vehicle treated animals, and no malignancies were detected in treated animals. Moreover, real-time PCR for a human specific sequence, ERV-3, detected no persistent ASCs. With the advent of clinical application, clarification of currently enigmatic stem cell properties has become imperative. Our study represents the longest duration determination of stem cell activity in vivo, and contributes strong evidence in support of the safety of adipose derived stem cell applications.
Assuntos
Tecido Adiposo/citologia , Células-Tronco Adultas/fisiologia , Transformação Celular Neoplásica , Células Estromais/fisiologia , Células-Tronco Adultas/citologia , Células-Tronco Adultas/patologia , Animais , Transformação Celular Neoplásica/patologia , Células Cultivadas , Criopreservação/métodos , Humanos , Camundongos , Camundongos Nus , Cultura Primária de Células/métodos , Células Estromais/citologia , Células Estromais/patologia , Análise de Sobrevida , Fatores de Tempo , Preservação de Tecido/métodos , Transplante Heterólogo , Ensaio Tumoral de Célula-TroncoRESUMO
Kearns-Sayre syndrome is a rare mitochondrial disorder characterized by large-scale deletion or rearrangement of mitochondrial DNA, which is usually not inherited but occur spontaneously probably at the germ cell level or very early in embryonic development by Mehndiratta et al. (Neurol India 50:162-167, 2002). Neuromuscular and cardiac conduction abnormalities are most commonly involved in these patients, which may have subtle presenting signs.
Assuntos
Bloqueio Cardíaco/etiologia , Bloqueio Cardíaco/fisiopatologia , Sistema de Condução Cardíaco/fisiopatologia , Síndrome de Kearns-Sayre/complicações , Síndrome de Kearns-Sayre/fisiopatologia , Adolescente , Diagnóstico Diferencial , Ecocardiografia , Eletrocardiografia , Bloqueio Cardíaco/diagnóstico , Humanos , MasculinoRESUMO
Myriad electrocardiographic changes, such as ST-segment elevation/depression, altered T-wave morphology, and QT prolongation, have been described with hyperkalemia in the setting of diabetic ketoacidosis (DKA) [2, 3]. We present an adolescent with DKA in whom T-wave inversions was seen despite his having normal serum potassium level.
Assuntos
Cetoacidose Diabética/fisiopatologia , Eletrocardiografia , Sistema de Condução Cardíaco/fisiopatologia , Infarto do Miocárdio/etiologia , Potássio/sangue , Adolescente , Cetoacidose Diabética/sangue , Cetoacidose Diabética/complicações , Diagnóstico Diferencial , Humanos , Masculino , Infarto do Miocárdio/diagnóstico , Infarto do Miocárdio/fisiopatologiaRESUMO
INTRODUCTION: There are many uncertainties surrounding anomalous aortic origin of a coronary artery (AAOCA) including the pathophysiology of sudden cardiac death, how to best risk stratify patients, how to best evaluate patients, who would benefit from exercise restriction, who should undergo surgical intervention, and which operation to perform. AREAS COVERED: The goal of this review is to provide a comprehensive but succinct overview of AAOCA to help clinicians with the difficult task of navigating optimal evaluation and treatment of an individual patient with AAOCA. EXPERT OPINION: Beginning in year 2012, some of our authors proposed an integrated, multi-disciplinary working group which has become the standard management strategy for patients diagnosed with AAOCA. A multi-disciplinary team with a focus on shared decision-making with the patients/families is likely necessary to optimize outcomes. Long-term follow-up and research are needed to improve our understanding of AAOCA.
Assuntos
Anomalias dos Vasos Coronários , Vasos Coronários , Humanos , Anomalias dos Vasos Coronários/complicações , Anomalias dos Vasos Coronários/terapia , Anomalias dos Vasos Coronários/diagnóstico , Aorta , Morte Súbita Cardíaca/etiologia , Morte Súbita Cardíaca/prevenção & controle , Tomada de DecisõesRESUMO
OBJECTIVE: To estimate the incidence and risk factors of visual impairment and complications in eyes with macular neovascularization (MNV) because of angioid streaks (ASs). DESIGN: Longitudinal multicenter retrospective cohort study. SUBJECTS: Patients with AS-associated MNV treated with anti-VEGF agents and a follow-up of > 3 months. METHODS: Clinical and MNV characteristics were collected at baseline. Visual acuity (VA) values and the presence of atrophy or fibrosis were collected at each visit. MAIN OUTCOME MEASURES: Rate of VA change over time and associated factors; the incidence rate of moderate-to-severe visual impairment (MSVI) and blindness and hazard ratio (HR) of candidate risk factors for MSVI; the incidence rate of fibrosis and macular atrophy. RESULTS: Overall, 84 eyes of 66 patients (39 men, 58%) with a mean (standard deviation) age of 55.7 (13.8) years were followed for a mean (standard deviation) of 67.7 (48.5) months. The median number of anti-VEGF doses per eye was 13. The average rate (95% confidence interval [CI]) of visual loss was +0.04 (0.02-0.06) logarithm of the minimum angle of resolution/year (P < 0.001); the visual loss was faster in nonnaive eyes (P = 0.007) and those with better baseline VA (P < 0.001); it was slower in eyes with pattern dystrophy-like features (P = 0.04). The incidence rates (95% CI) of MSVI and blindness were 10.4 (6.88-15)/100-eye-years and 2.33 (1.12-4.29)/100-eye-years. A higher number of injections (HR [95% CI] = 0.45 [0.19-0.94] for receiving ≥ 13 injections vs. < 13; P = 0.03) was protective against MSVI. The incidence rates (95% CI) of fibrosis and macular atrophy were 24.1 (17.5-32.3)/100-eye-years and 14.3 (10.1-19.6)/100-eye-years. CONCLUSIONS: Eyes with MNV-related AS had a high rate of visual impairment and propensity to macular fibrosis and atrophy. A higher number of injections yielded better chances of maintaining good VA, suggesting the need for intensive treatment. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.
Assuntos
Estrias Angioides , Degeneração Macular , Baixa Visão , Masculino , Humanos , Pessoa de Meia-Idade , Estrias Angioides/complicações , Estrias Angioides/diagnóstico , Estrias Angioides/epidemiologia , Incidência , Estudos Retrospectivos , Neovascularização Patológica , Degeneração Macular/complicações , Cegueira/epidemiologia , Cegueira/etiologia , Fatores de Risco , FibroseRESUMO
Idiopathic atrial fibrillation (AF) in adolescents is extremely rare and has usually been associated with structural heart disease. We present two cases of symptomatic AF in adolescents without any identifiable etiology. No definitive guidelines are available for management of such patients.
Assuntos
Fibrilação Atrial/diagnóstico , Adolescente , Diagnóstico Diferencial , Eletrocardiografia , Feminino , Humanos , Masculino , Fatores de RiscoRESUMO
Acellular dermal matrices are used in a variety of reconstructive and cosmetic procedures. There seems to be host tissue integration, revascularization, and recellularization into these products, but the exact timing and differences among these remain unknown. The purpose of this study is to determine and compare these properties of 4 different acellular dermal matrices (AlloDerm, DermACELL, DermaMatrix, and Integra) in an in vivo rat model. Tissue specimens were obtained at various time points. Histology and immunohistologic assays were used to quantify the extent of cellular infiltration and revascularization within the various matrices. A bimodal cellular response was observed in all products except for DermACELL. Cellular infiltration was highest in DermACELL and lowest in AlloDerm, and angiogenesis was evident by day 7. There were clear differences within the various products. It is undetermined whether these differences are advantageous or clinically significant. Future work is needed to define the specific roles for each.
Assuntos
Materiais Biocompatíveis , Movimento Celular , Sulfatos de Condroitina/fisiologia , Colágeno/fisiologia , Regeneração Tecidual Guiada/instrumentação , Neovascularização Fisiológica , Alicerces Teciduais , Animais , Biomarcadores/metabolismo , Molécula-1 de Adesão Celular Endotelial a Plaquetas/metabolismo , Distribuição Aleatória , Ratos , Ratos Sprague-DawleyRESUMO
The current technique for implanting flangedintraocular lens (IOL) suffers from complications like haptic exposure and tilting of the implanted IOL. We describe a modification of the currently described technique to obviate its shortcomings. Five eyes of five patients with a minimum of 1 year of follow-up were included. In this technique, two scleral pockets were made nasal and temporal to embed the flanged haptics. The primary outcome measure was the improvement in visual acuity (VA) postoperatively and the secondary outcome measures were postoperative complications. The primary objective of this current modification is to simplify the surgical technique for secondary IOL implantation and make it more replicable and predictive. The mean age of the patients was 19.44 years. The mean preoperative VA was 0.44 logMAR which improved to 0.26 logMAR at the 6-week postoperative visit. The mean follow-up was 496+/- 80 days. The maximum follow-up was 647 days. There were no postoperative complications such as haptic exposure, hypotony, or IOL tilt in any cases. The new E-flanged IOL technique has good visual outcomes and does not have postoperative complications. It has less intraoperative manipulation and complications.
Assuntos
Lentes Intraoculares , Adulto , Humanos , Implante de Lente Intraocular/métodos , Complicações Pós-Operatórias/cirurgia , Estudos Retrospectivos , Esclera/cirurgia , Técnicas de Sutura , Acuidade Visual , Adulto JovemRESUMO
Newer anticancer drugs have revolutionized cancer treatment in the last decade, but conventional chemotherapy still occupies a central position in many cancers, with combination therapy and newer methods of delivery increasing their efficacy while minimizing toxicities. We discuss the retinal toxicities of anticancer drugs with an emphasis on the mechanism of toxicity. Uveitis is seen with the use of v-raf murine sarcoma viral oncogene homolog B editing anticancer inhibitors as well as immunotherapy. Most of the cases are mild with only anterior uveitis, but severe cases of posterior uveitis, panuveitis, and Vogt-Koyanagi-Harada-like disease may also occur. In the retina, a transient neurosensory detachment is observed in almost all patients on mitogen-activated protein kinase kinase (MEK) inhibitors. Microvasculopathy is often seen with interferon α, but vascular occlusion is a more serious toxicity caused by interferon α and MEK inhibitors. Crystalline retinopathy with or without macular edema may occur with tamoxifen; however, even asymptomatic patients may develop cavitatory spaces seen on optical coherence tomography. A unique macular edema with angiographic silence is characteristic of taxanes. Delayed dark adaptation has been observed with fenretinide. Interestingly, this drug is finding potential application in Stargardt disease and age-related macular degeneration.