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1.
Microvasc Res ; 149: 104570, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-37315777

RESUMEN

OBJECTIVES: Despite advances in the maintenance of arteriovenous fistulas (AVFs), the patency rates remain suboptimal. Most AVFs fail due to outflow vein stenosis; however, the underlying mechanism of AVF stenosis remains unclear. The present study aimed to identify key factors associated with AVF outflow stenosis. METHODS: We obtained gene expression profiling data for the outflow vein of AVF from three Gene Expression Omnibus database datasets (GSE39488, GSE97377, and GSE116268) and analyzed the common differentially expressed genes (DEGs). We evaluated a common DEG in an aortocaval mouse model and the stenotic outflow veins of AVFs collected from patients. Furthermore, we isolated vascular smooth muscle cells (VSMCs) from the inferior vena cava (IVC) of wild-type (WT) and osteopontin (Opn)-knockout (KO) mice and assessed the proliferation of VSMCs following stimulation with platelet-derived growth factors (PDGFs). RESULTS: OPN was the only common upregulated DEG among all datasets. OPN was expressed in the medial layer of the outflow vein of AVF in aortocaval mouse models and co-stained with the VSMC marker (α-smooth muscle actin). OPN expression was markedly increased in the VSMCs of stenotic outflow veins of AVF collected from patients undergoing hemodialysis compared to presurgical veins acquired during AVF formation surgery. PDGF-induced VSMC proliferation was significantly increased in the VSMCs isolated from the IVC of WT mice but not in those isolated from the IVC of Opn-KO mice. CONCLUSIONS: OPN may be a key gene involved in VSMC proliferation in the AVF outflow veins and a therapeutic target to improve the AVF patency rate.


Asunto(s)
Fístula Arteriovenosa , Derivación Arteriovenosa Quirúrgica , Ratones , Animales , Músculo Liso Vascular/metabolismo , Derivación Arteriovenosa Quirúrgica/efectos adversos , Osteopontina/genética , Osteopontina/metabolismo , Constricción Patológica/metabolismo , Factor de Crecimiento Derivado de Plaquetas , Proliferación Celular , Fístula Arteriovenosa/metabolismo
2.
Clin Transplant ; 31(2)2017 02.
Artículo en Inglés | MEDLINE | ID: mdl-27935648

RESUMEN

OBJECTIVES: Cardiac allograft vasculopathy (CAV) remains a major impediment to long-term survival after heart transplantation (HT). We investigated the incidence, disease course, and risk factors for CAV. METHODS: Among 399 patients who underwent HT between November 1992 and July 2014, 297 survivors were reviewed. Endpoints were CAV development and the composite outcome of death or re-HT. RESULTS: During 5.6±5.2 years, CAV was detected in 54 patients: 45 (83.3%), 8 (14.8%), and 1 (1.8%) patients for CAV 1, 2, and 3, respectively. At 1, 5, and 10 years, 99.0%, 82.4%, and 60.3% of patients were free of CAV, respectively. Only four patients (7.4%) showed progression over 4.8±2.1 years' follow-up. The presence of CAV did not affect the composite outcome (P=.89). Predictors of CAV included donor age (HR1.06, 95% CI: 1.03-1.10: P<.001), recipient age (1.03 [1.003-1.06]; P=.03), ischemic time >240 minutes (3.15 [1.36-7.28], P=.007), postoperative renal replacement therapy (RRT) (7.1 [2.3-21.8]; P=.001), and triglyceride level at 1 year post-HT (1.005 [1.002-1.008], P=.003). CONCLUSIONS: CAV incidence after HT appears acceptable, with most cases being stationary and inconsequential for survival. Development of CAV seems to be influenced by donor and recipient age, ischemic time, postoperative RRT, and high triglyceride level.


Asunto(s)
Enfermedades Cardiovasculares/epidemiología , Trasplante de Corazón/efectos adversos , Complicaciones Posoperatorias , Adulto , Aloinjertos , Enfermedades Cardiovasculares/etiología , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Pronóstico , República de Corea , Estudios Retrospectivos , Factores de Riesgo
3.
Pediatr Radiol ; 47(5): 556-564, 2017 May.
Artículo en Inglés | MEDLINE | ID: mdl-28243677

RESUMEN

BACKGROUND: There are limited data regarding the influence of commissural malalignment of the aortic-pulmonary sinus on the arterial switch operation. OBJECTIVE: To compare diagnostic accuracy between cardiac CT and echocardiography for evaluating commissural malalignment of aortic-pulmonary sinus in children with complete transposition of the great arteries and to seek potential clinical implication of commissural malalignment on the arterial switch operation. MATERIALS AND METHODS: In 37 patients (35 boys; median age: 8 days, range: 3-80 days) with complete transposition of the great arteries who had tricuspid semilunar valves and underwent an arterial switch operation, the degree of the commissural rotation of the aortic-pulmonary sinus was assessed on cardiac CT (n=37) and echocardiography (n=35). With surgical finding as a reference, cardiac CT was compared with echocardiography in identifying commissural malalignment in 35 patients. The influence of the height difference between the semilunar valves measured by cardiac CT on the identification of commissural malalignment with cardiac CT and echocardiography was evaluated. The impact of commissural malalignment on coronary transfer techniques was evaluated. RESULTS: In operative findings, the commissures of the semilunar valves were aligned in 24 patients and malaligned in 13. With surgical findings as a reference, cardiac CT showed higher, but not statistically significant (P>0.05), sensitivity (91.7% vs. 75.0%), specificity (87.0% vs. 78.3%) and accuracy (88.6% vs. 77.1%) for the diagnosis of the malalignment than echocardiography. The measured height difference between the semilunar valves did not affect the identification of the malalignment with cardiac CT and echocardiography. The surgical malalignment group showed a higher requirement of modified coronary transfer techniques than the surgical aligned group (11/13 vs. 11/24, P=0.03). CONCLUSION: Cardiac CT and echocardiography appear useful for evaluating commissural malalignment of the semilunar valves in patients with complete transposition of the great arteries frequently requiring modified coronary transfer techniques at during an arterial switch operation.


Asunto(s)
Aorta/anomalías , Operación de Switch Arterial , Ecocardiografía/métodos , Arteria Pulmonar/anomalías , Tomografía Computarizada por Rayos X/métodos , Transposición de los Grandes Vasos/cirugía , Aorta/diagnóstico por imagen , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Arteria Pulmonar/diagnóstico por imagen , Reproducibilidad de los Resultados , Estudios Retrospectivos , Sensibilidad y Especificidad
4.
J Korean Med Sci ; 31(5): 817-21, 2016 May.
Artículo en Inglés | MEDLINE | ID: mdl-27134508

RESUMEN

From 2006 to 2011, an outbreak of a particular type of childhood interstitial lung disease occurred in Korea. The condition was intractable and progressed to severe respiratory failure, with a high mortality rate. Moreover, in several familial cases, the disease affected young women and children simultaneously. Epidemiologic, animal, and post-interventional studies identified the cause as inhalation of humidifier disinfectants. Here, we report a 4-year-old girl who suffered from severe progressive respiratory failure. She could survive by 100 days of extracorporeal membrane oxygenation support and finally, underwent heart-lung transplantation. This is the first successful pediatric heart-lung transplantation carried out in Korea.


Asunto(s)
Humidificadores , Enfermedades Pulmonares Intersticiales/inducido químicamente , Enfermedades Pulmonares Intersticiales/terapia , Trasplante de Pulmón , Preescolar , Desinfectantes/toxicidad , Oxigenación por Membrana Extracorpórea , Femenino , Humanos , Pulmón/efectos de los fármacos , Pulmón/patología , Enfermedades Pulmonares Intersticiales/patología , República de Corea , Frecuencia Respiratoria , Estudios Retrospectivos , Tórax/diagnóstico por imagen , Tomografía Computarizada por Rayos X
5.
J Card Surg ; 31(3): 168-73, 2016 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-26805702

RESUMEN

BACKGROUND: Surgical techniques currently used for the repair of Ebstein's anomaly comprise reconstruction of the tricuspid valve mechanism at the level of the true annulus with or without plication of the atrialized right ventricle. However, performing this procedure for patients with a dysmorphic anterior leaflet (i.e., insufficient leaflet tissue and decreased mobility due to tethering) may necessitate technical modifications. METHODS: A retrospective review was performed of 31 patients (seven males and 24 females, median age at operation 31 years) with Ebstein's anomaly, who underwent tricuspid valve repair between March 2002 and December 2014. The original Hetzer technique (annulus to annulus approximation) was employed for six patients with a well-formed anterior leaflet. In 25 patients, the tricuspid valve mechanism was restored at the displaced septal leaflet by approximating the anterior leaflet attachment in the true annulus to the displaced septal leaflet attachment in the mid-septum. A bidirectional superior cavopulmonary anastomosis was added in 27 of 31 (87%) patients. RESULTS: No early or late death occurred during the median follow-up of 66 months (1-138 months). Immediate postoperative tricuspid regurgitation was trivial to mild in 22 patients, and the median preoperative, immediate postoperative, and last follow-up tricuspid regurgitation jet areas in 21 adult patients were 23.3 cm2, 10.4 cm2, and 7.0 cm2, respectively. Two patients underwent reoperation at 81 and 119 months postoperatively. Five-year freedom from severe tricuspid regurgitation or reoperation was 93.2%. CONCLUSION: Restoration of the tricuspid valve mechanism at the level of displaced septal leaflet leads to excellent long-term outcomes. The addition of the bidirectional superior cavopulmonary anastomosis has contributed to the success of this technique.


Asunto(s)
Procedimientos Quirúrgicos Cardíacos/métodos , Anomalía de Ebstein/cirugía , Procedimientos de Cirugía Plástica/métodos , Válvula Tricúspide/cirugía , Adolescente , Adulto , Niño , Preescolar , Femenino , Estudios de Seguimiento , Puente Cardíaco Derecho , Humanos , Lactante , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento , Insuficiencia de la Válvula Tricúspide , Adulto Joven
6.
Pediatr Cardiol ; 37(2): 290-4, 2016 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-26433938

RESUMEN

Indications of sutureless repair (SR) for pulmonary vein anomalies have evolved from re-operational SR for pulmonary vein stenosis after the repair of total anomalous pulmonary venous drainage (TAPVD) to primary SR for TAPVD associated with right atrial isomerism or isolated TAPVD with small individual pulmonary veins (IPVs) and an unfavorable pulmonary vein anatomy. We sought to determine whether small IPVs outgrow somatic growth after primary SR. Between 2004 and 2013, 21 children underwent primary SR for TAPVD: 13 with a functionally single ventricle, 11 with right atrial isomerism, six with isolated TAPVD, and 13 with a pulmonary venous obstruction. TAPVD types were supracardiac in nine, infracardiac in 10, and mixed in two. Utilizing cardiac computed tomography (CT), the maximal diameter of each IPV was measured, and pulmonary vein index (PVI, summation of cross-sectional areas of all four IPVs divided by body surface area) was calculated. There were five early deaths after SR. Among survivors, 10 had both preoperative and postoperative cardiac CT at a 3.6-month median interval. On postoperative cardiac CT, IPVs were patent in all patients except one who developed a left lower pulmonary vein obstruction. There was a 71 ± 48 % postoperative increase in the actual diameter of all four IPVs, and PVI increased significantly from 215 ± 55 to 402 ± 117 mm(2)/m(2) (P value = 0.005). IPVs outgrew somatic growth after primary SR of TAPVD. Primary SR may be a useful measure in TAPVD patients whose IPVs are small.


Asunto(s)
Síndrome de Heterotaxia/cirugía , Venas Pulmonares/anomalías , Enfermedad Veno-Oclusiva Pulmonar/cirugía , Síndrome de Cimitarra/complicaciones , Síndrome de Cimitarra/cirugía , Procedimientos Quirúrgicos sin Sutura , Femenino , Humanos , Lactante , Recién Nacido , Modelos Lineales , Masculino , Complicaciones Posoperatorias , Circulación Pulmonar , Venas Pulmonares/cirugía , Reoperación , República de Corea , Resultado del Tratamiento , Cicatrización de Heridas
7.
Pediatr Radiol ; 44(9): 1169-74, 2014 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-24687619

RESUMEN

Most thoracopagus twins present with cardiac fusion and associated congenital cardiac defects, and assessment of this anatomy is of critical importance in determining patient care and outcome. Cardiac CT with electrocardiographic triggering provides an accurate and quick morphological assessment of both intracardiac and extracardiac structures in newborns, making it the best imaging modality to assess thoracopagus twins during the neonatal period. In this case report, we highlight the diagnostic value of cardiac CT in thoracopagus twins with an interatrial channel and complex congenital cardiac defects.


Asunto(s)
Cardiopatías Congénitas/diagnóstico por imagen , Tomografía Computarizada por Rayos X/métodos , Gemelos Siameses , Técnicas de Imagen Sincronizada Cardíacas , Medios de Contraste , Ecocardiografía , Resultado Fatal , Humanos , Imagenología Tridimensional , Recién Nacido , Yopamidol/análogos & derivados , Imagen por Resonancia Magnética , Masculino , Interpretación de Imagen Radiográfica Asistida por Computador , Ultrasonografía Prenatal
8.
J Card Surg ; 29(4): 564-6, 2014 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-24684561

RESUMEN

A 17-year-old male presented with two episodes of syncope, cyanosis, and exercise intolerance 13 years after the Fontan operation. Echocardiography and magnetic resonance imaging showed dilated intrahepatic collaterals, which drained into the atrium through the left hepatic vein, and 24-hour Holter monitoring revealed sinus node dysfunction. We performed ligation of the left hepatic vein using intrahepatic collaterals as channels draining hepatic venous blood into the Fontan pathway, and implanted an epicardial dual chamber pacemaker. At one-year follow-up, the patient remained asymptomatic and his cardiac performance was much improved.


Asunto(s)
Circulación Colateral , Procedimiento de Fontan , Venas Hepáticas/cirugía , Ligadura/métodos , Complicaciones Posoperatorias/cirugía , Adolescente , Dilatación Patológica , Ecocardiografía , Estudios de Seguimiento , Venas Hepáticas/patología , Venas Hepáticas/fisiopatología , Humanos , Imagen por Resonancia Magnética , Masculino , Marcapaso Artificial , Complicaciones Posoperatorias/diagnóstico , Factores de Tiempo , Resultado del Tratamiento
9.
Sci Rep ; 14(1): 12797, 2024 06 04.
Artículo en Inglés | MEDLINE | ID: mdl-38834773

RESUMEN

The systemic inflammatory response syndrome can occur due to an inflammatory reaction to the release of cytokines, and it has been linked to the circulation of pro- and anti-inflammatory cytokines. The cardiopulmonary bypass (CPB) system is known to activate numerous inflammatory pathways. Applying CPB in large animals for an extended period may be useful as a controlled experimental model for systemic inflammatory responses. The authors hypothesized that 0.2 mg/kg NuSepin® would inhibit CBP-induced proinflammatory cytokine release, and attenuate CPB-induced vasoplegia. CPB was maintained for 2 h in 8 male Yorkshire pigs. Ten ml of saline was administered intravenously to the control group, while the study group received 10 ml of NuSepin® (0.2 mg/kg), before start of CPB. Blood samples were collected at four different time points to evaluating the level of cytokine (TNF-α, IL-1ß, IL-6, IL-8) release during and after CBP. All vital signals were recorded as continuous waveforms using the vital recorder®. Our study demonstrated that IL-6 increased in both groups during CPB remained unchanged. However, in the Nusepin group, IL-6 levels rapidly decreased when CPB was stopped and the proinflammatory reaction subsided. Furthermore, the dose of norepinephrine required to maintain a mean pressure of 60 mmHg was also lower in the Nusepin group.


Asunto(s)
Puente Cardiopulmonar , Citocinas , Animales , Puente Cardiopulmonar/efectos adversos , Porcinos , Citocinas/metabolismo , Citocinas/sangre , Proyectos Piloto , Masculino , Inflamasomas/metabolismo , Inflamasomas/antagonistas & inhibidores , Modelos Animales de Enfermedad , Administración Intravenosa , Vasoplejía/tratamiento farmacológico , Vasoplejía/etiología
10.
J Cardiothorac Vasc Anesth ; 27(6): 1153-7, 2013 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-23972985

RESUMEN

OBJECTIVE: Acute kidney injury is a serious postoperative complication following cardiac surgery. The aortic arch repair technique using antegrade selective cerebral perfusion has been used, but it is unclear whether subdiaphragmatic organs such as the kidneys are perfused adequately. The authors compared intraoperative renal regional oxygen saturation using near-infrared spectroscopy between infants with and without postoperative acute kidney injury after undergoing aortic arch reconstruction. DESIGN: Retrospective medical records review. SETTING: University medical center. PARTICIPANTS: Elective cardiac surgical infants. INTERVENTIONS: The author reviewed the perioperative clinical records of infants who underwent aortic arch reconstruction surgery using antegrade selective cerebral perfusion. During the operation, prestenotic (right radial or right brachial artery) and poststenotic (femoral or umbilical artery) invasive arterial blood pressure and cerebral and renal regional oxygen saturation were monitored continuously. Development of acute kidney injury within 72 hours of surgery was investigated. RESULTS: A total of 47 patients were enrolled in this study. Postoperative acute kidney injury occurred in 19 patients (40.4%). Intraoperative renal regional oxygen saturation was similar between patients with and without acute kidney injury. Intraoperative prestenotic and poststenotic mean arterial blood pressure and cerebral regional oxygen saturation also were similar between the 2 groups. CONCLUSIONS: The intraoperative renal tissue oxygen saturation was not different between the groups with or without postoperative acute kidney injury in infants who underwent aortic arch repair using antegrade selective cerebral perfusion.


Asunto(s)
Lesión Renal Aguda/etiología , Aorta Torácica/cirugía , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Consumo de Oxígeno/fisiología , Complicaciones Posoperatorias/epidemiología , Anestesia General , Puente Cardiopulmonar , Circulación Colateral , Femenino , Humanos , Lactante , Recién Nacido , Riñón/metabolismo , Masculino , Perfusión , Procedimientos de Cirugía Plástica , Estudios Retrospectivos , Resultado del Tratamiento
11.
Pediatr Cardiol ; 34(7): 1590-6, 2013 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-23494543

RESUMEN

Ebstein's anomaly is frequently detected before birth, with prenatal detection accounting for the majority of cases in the current population. This study aimed to identify the outcome variables among these infants. The medical records of 59 patients with neonatal Ebstein's anomaly managed at the Asan Medical Center between January, 2001 and June, 2012 were investigated retrospectively. In 46 cases, the diagnosis was made prenatally. Surgical/interventional procedures were performed for 27 of the analyzed patients. Biventricular repair was successful for 12 patients but not for 9 patients with pulmonary atresia. The median follow-up period was 1.96 years (range 0.0-10.4 years). The overall mortality rate was 23.7 % (14/59). Of the 14 deaths, 5 occurred within several hours after birth. The 1- and 5-year survival rates were 78.6 and 76.3 %, respectively. Univariate analysis identified several variables related to the time to death: fetal distress (p = 0.002), prematurity (p = 0.036), low birth weight (p = 0.003), diameter of the atrial septal defect (p = 0.022), and pulmonary stenosis/atresia (p = 0.001). Neither the Carpentier classification (p = 0.175) nor the Celermajer index (p = 0.958) was a significant variable. According to the multivariate analysis, fetal distress (p = 0.004) and pulmonary atresia/stenosis (p < 0.001) were significant determinants of outcome. In conclusion, fetal distress and pulmonary atresia/stenosis are significant predictors of mortality in the current population of patients with neonatal Ebstein's anomaly. A close cooperation of associated clinicians is required for an improvement in outcome. To establish a better surgical strategy for patients with Ebstein's anomaly and pulmonary atresia, studies of larger populations are required.


Asunto(s)
Anomalía de Ebstein/mortalidad , Procedimientos Quirúrgicos Cardíacos/métodos , Anomalía de Ebstein/diagnóstico , Anomalía de Ebstein/cirugía , Ecocardiografía , Femenino , Humanos , Recién Nacido , Masculino , Pronóstico , Radiografía Torácica , República de Corea/epidemiología , Estudios Retrospectivos , Tasa de Supervivencia/tendencias , Factores de Tiempo
12.
Fetal Diagn Ther ; 34(3): 152-7, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-24051687

RESUMEN

INTRODUCTION: To report our experience in fetuses prenatally diagnosed with common arterial trunk (CAT) and to evaluate the postnatal outcomes. MATERIAL AND METHODS: This was a retrospective study conducted at Asan Medical Center, Seoul, Korea, between 2003 and 2012. Maternal medical and fetal echocardiographic records regarding fetuses were prenatally diagnosed with CAT were reviewed. Postnatal outcomes of survivors were also assessed during the mean follow-up of 36 months. RESULTS: Of the 17 fetuses that were prenatally diagnosed with CAT and had a final diagnosis by postnatal echocardiography or autopsy, 12 were confirmed to have CAT and 5 were incorrectly diagnosed with CAT. Of the latter, 3 had pulmonary atresia and 2 had aortic atresia. The diagnostic accuracy was 71%. The median gestational age at prenatal diagnosis of confirmed CAT was 24.4 weeks (range 21.1-34.3). The truncal valve was prenatally thickened in 8 fetuses. All but 1 of the 12 confirmed CAT cases had associated heart and/or extracardiac anomalies. Of the 8 liveborn cases of postnatally confirmed CAT, 2 died before or after surgery respectively. The remaining 6 remained alive after successful corrective surgery. CONCLUSION: Prenatal diagnosis of CAT can be difficult in some cases and other diseases should be excluded before diagnosing CAT. Isolated CAT can be repaired by postnatal corrective surgery with a good outcome.


Asunto(s)
Cardiopatías Congénitas/diagnóstico por imagen , Ultrasonografía Prenatal , Diagnóstico Diferencial , Femenino , Humanos , Valor Predictivo de las Pruebas , Embarazo , Estudios Retrospectivos
13.
Medicine (Baltimore) ; 102(34): e35007, 2023 Aug 25.
Artículo en Inglés | MEDLINE | ID: mdl-37653750

RESUMEN

BACKGROUND: Gastric insufflation can cause gastric regurgitation, which may be exacerbated in patients who are expected to have difficult airways. The purpose of this study was to investigate the difference in respiratory parameters and the frequency of gastric insufflation according to the ventilation mode during the anesthestic induction on patients who were predicted to have difficult facemask ventilation. METHODS: A total of eighty patients with expected airway difficulties were included. Patient were allocated to 2 groups (n = 40 each). In the manual ventilation group, ventilation was performed by putting a mask on the patient's face with 1-hand and adjusting the pressure limiting valve to 15 cm H2O. In the pressure-controlled ventilation group, a mask was held in place using 2-handed jaw-thrust maneuver. The pressure-controlled ventilation was applied and peak inspiration pressure was adjusted to achieve a tidal volume of 6 to 8 mL/kg. The primary outcome was the difference of the peak airway pressure between 2 groups every 30 seconds for 120 seconds duration of mask ventilation. We also evaluated respiratory variables including peak airway pressure, End-tidal carbon dioxide and also gastric insufflation using ultrasonography. RESULTS: The pressure-controlled ventilation group demonstrated lower peak airway pressure than the manual ventilation group (P = .005). End-tidal carbon dioxide was higher in the pressure-controlled ventilation group (P = .012). The incidence of gastric insufflation assessed by real-time ultrasonography of the gastric antrum was higher in the manual ventilation group than in the pressure-controlled ventilation group [3 (7.5%) vs 17 (42.5%), risk ratio (95% confidence interval): 0.06 to 0.56, P = .003]. CONCLUSIONS: Pressure-controlled ventilation during facemask ventilation in patients who were expected to have difficult airways showed a lower gastric insufflation rate with low peak airway pressure compared to manual ventilation.


Asunto(s)
Anestésicos , Dióxido de Carbono , Humanos , Estudios Prospectivos , Respiración Artificial , Respiración , Disnea
14.
Pediatr Transplant ; 16(7): E281-5, 2012 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-22093921

RESUMEN

Massive pulmonary hemorrhage and other serious cardiopulmonary diseases in patients with fulminant hepatitis result not only in graft failure but also mortality after LT. ECMO is used to treat children with cardiorespiratory failure refractory to conventional intensive care. We describe a five-yr-old girl with genetically confirmed fulminant Wilson disease and severe pulmonary hemorrhage who underwent successful primary LT following veno-arterial ECMO. To our knowledge, this is the first report of successful primary LT in a patient using veno-arterial ECMO. The present case demonstrates that ECMO, as a bridging modality to LT, may be necessary to manage both massive pulmonary hemorrhage and possible graft loss because of hypoxemia.


Asunto(s)
Oxigenación por Membrana Extracorpórea/métodos , Degeneración Hepatolenticular/terapia , Trasplante de Hígado/métodos , Pulmón/patología , Preescolar , Femenino , Supervivencia de Injerto , Hemorragia/terapia , Humanos , Hipoxia , Oxígeno/metabolismo , Radiografía Torácica/métodos , Insuficiencia Respiratoria/complicaciones
15.
Pediatr Cardiol ; 33(4): 639-45, 2012 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-22349667

RESUMEN

Compared with mitral repair, mitral valve replacement is an uncommon procedure in children due to associated high mortality and morbidity rates. The present study investigated early and late outcomes after MVR with mechanical prostheses in children at our institution. Between January 1994 and December 2009, 19 children underwent MVR. Mean patient age was 7.6 ± 5.5 years (range 3 months-16 years), and mean body weight was 23.7 ± 15.1 kg (range 5.0-58.1 kg). Mean prosthesis size was 25.8 ± 4.2 mm (range 19-31 mm). There were no operative or late mortalities. Three patients showed decreased left-ventricular function before surgery, and one of them underwent successful heart transplantation due to progressive LV dysfunction at 10 months after MVR. The proportion of patients with freedom from reoperation at 10 years was 94.7 ± 5%. There were no major thromboembolic or bleeding episodes. Although the small number of patients in our study was a limitation, MVR in children was found to result in excellent early and long-term outcomes. It appears that MVR could be considered in children before LV dysfunction develops.


Asunto(s)
Enfermedades de las Válvulas Cardíacas/cirugía , Prótesis Valvulares Cardíacas , Función Ventricular Izquierda , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Enfermedades de las Válvulas Cardíacas/fisiopatología , Humanos , Incidencia , Lactante , Masculino , Complicaciones Posoperatorias , Diseño de Prótesis , República de Corea/epidemiología , Estudios Retrospectivos , Tasa de Supervivencia/tendencias , Factores de Tiempo , Resultado del Tratamiento , Disfunción Ventricular Izquierda/epidemiología , Disfunción Ventricular Izquierda/fisiopatología
16.
Transplantation ; 106(11): 2256-2262, 2022 11 01.
Artículo en Inglés | MEDLINE | ID: mdl-35706094

RESUMEN

BACKGROUND: In systemic light-chain (AL) amyloidosis, cardiac involvement is a major determinant of survival; however, cardiac response is limited even after systemic treatment in a majority of patients, and some require heart transplantation. Additionally, limited information is available on specific indications for heart transplantation. We aimed to explore clinical outcomes of cardiac amyloidosis and its association with heart transplantation, including identifying factors favoring heart transplantation amenability. METHODS: We retrospectively analyzed data from patients diagnosed with AL amyloidosis with cardiac involvement between January 2007 and December 2020 at a tertiary referral center. RESULTS: Among 73 patients, 72 (99%) received systemic treatment, and 12 (16%) underwent heart transplantation. Characteristics at diagnosis were similar between heart transplant recipients and nonrecipients, although left ventricular ejection fraction tended to be lower in recipients (median 48% versus 57%, P = 0.085). Eight weeks after systemic treatment, 67% and 12% of patients achieved hematologic and brain natriuretic peptide responses. Overall survival was longer among heart transplantation recipients than nonrecipients, with 5-y survival rates of 61.1% (95% confidence interval, 25.5%-83.8%) versus 32.0% (95% confidence interval, 20.3%-44.4%; P = 0.022), respectively. Among the 34 with identifiable causes of death out of 51 deaths, 21 nonrecipients (62%) died of cardiac problems compared with none in the heart transplant recipients. Additionally, survival outcomes favored heart transplant recipients in most subgroups, including patients with higher Mayo 2004 European stage at diagnosis and with extracardiac involvement of amyloidosis. CONCLUSIONS: Heart transplantation can achieve long-term survival in appropriately selected patients with AL cardiac amyloidosis.


Asunto(s)
Amiloidosis , Trasplante de Corazón , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas , Humanos , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/complicaciones , Volumen Sistólico , Péptido Natriurético Encefálico , Estudios Retrospectivos , Función Ventricular Izquierda , Amiloidosis/diagnóstico , Amiloidosis/cirugía , Amiloidosis/complicaciones , Trasplante de Corazón/efectos adversos
17.
J Korean Med Sci ; 26(5): 593-8, 2011 May.
Artículo en Inglés | MEDLINE | ID: mdl-21532847

RESUMEN

Heart transplantation is a standard treatment for end-stage heart disease. Pediatric heart transplantation, however, is not frequently performed due to the shortage of pediatric heart donors. This is the first report of pediatric heart transplantation in Korea. Our retrospective study included 37 patients younger than 18 yr of age who underwent heart transplantation at Asan Medical Center between August 1997 and April 2009. Preoperative diagnosis was either cardiomyopathy (n = 29, 78.3%) or congenital heart disease (n = 8, 22.7%). Mean follow up period was 56.9 ± 44.6 months. There were no early death, but 7 late deaths (7/37, 18.9%) due to rejection after 11, 15, 41 months (n = 3), infection after 5, 8, 10 months (n = 3), suspicious ventricular arrhythmia after 50 months (n = 1). There was no significant risk factor for survival. There were 25 rejections (25/37, 67.6%); less than grade II occurred in 17 patients (17/25, 68%) and more than grade II occurred in 8 patients (8/25, 32%). Actuarial 1, 5, and 10 yr survival was 88.6%, 76.8%, and 76.8%. Our midterm survival of pediatric heart transplantation showed excellent results. We hope this result could be an encouraging message to do more pediatric heart transplantation in Korean society.


Asunto(s)
Trasplante de Corazón , Adolescente , Arritmias Cardíacas/mortalidad , Cardiomiopatías/cirugía , Niño , Preescolar , Femenino , Rechazo de Injerto/mortalidad , Cardiopatías Congénitas/cirugía , Humanos , Terapia de Inmunosupresión/métodos , Lactante , Infecciones/mortalidad , Masculino , Complicaciones Posoperatorias , República de Corea , Estudios Retrospectivos , Donantes de Tejidos , Resultado del Tratamiento
18.
Pediatr Cardiol ; 32(5): 578-84, 2011 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-21347835

RESUMEN

Although the outcome of neonatal cardiac surgery has dramatically improved, low body weight (LBW) is still considered an important risk for open heart surgery. The factors contributing to poor outcomes in LBW infants, however, are still unclear. We investigated risk factors for poor outcomes in infants weighing <2500 g who underwent surgical correction with cardiopulmonary bypass (CPB). From January 1995 to December 2009, 102 consecutive patients were included in this study. Median age and body weight at the time of surgery was 19 (range 1 to 365) days and 2.23 kg (range 1.3 to 2.5), respectively. Corrective surgery was performed on 75 infants. The median follow-up duration was 45.03 months (range 0.33 to 155.23). There were 23 (22.5%) hospital mortalities. Emergency surgery and low cardiac output (LCO) were associated with early mortality; however, body weight, Aristotle basic complex score, and type of surgery was not. Early morbidities, including delayed sterna closure, arrhythmia, and chylothorax, occurred in 39 (38.2%) infants. The overall actuarial survival rate at 10 years was 74.95% ± 4.37%. In conclusion, among infants weighing <2500 g who underwent open heart surgery with CPB, perioperative hemodynamic status, such as emergency surgery and LCO, strongly influenced early mortality. In contrast, LBW itself was not associated with patient morbidity or mortality.


Asunto(s)
Cardiopatías Congénitas/cirugía , Recién Nacido de Bajo Peso , Enfermedades del Prematuro/cirugía , Recién Nacido de muy Bajo Peso , Anomalías Múltiples/mortalidad , Anomalías Múltiples/cirugía , Análisis Actuarial , Gasto Cardíaco Bajo/mortalidad , Gasto Cardíaco Bajo/cirugía , Puente Cardiopulmonar/mortalidad , Causas de Muerte , Niño , Preescolar , Estudios de Cohortes , Urgencias Médicas , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/mortalidad , Mortalidad Hospitalaria , Humanos , Lactante , Recién Nacido , Enfermedades del Prematuro/mortalidad , Masculino , Complicaciones Posoperatorias/mortalidad , Factores de Riesgo , Tasa de Supervivencia
19.
Healthcare (Basel) ; 9(8)2021 Aug 23.
Artículo en Inglés | MEDLINE | ID: mdl-34442220

RESUMEN

BACKGROUND: Ultrasound-guided regional anesthesia has gained popularity over the last decade. This study aimed to assess whether YouTube videos sufficiently serve as an adjunctive tool for learning how to perform an ultrasound-guided brachial plexus block (BPB). METHODS: All YouTube videos were classified, based on their sources, as either academic, manufacturer, educational, or individual videos. The metrics, accuracy, utility, reliability (using the Journal of American Medical Association Score benchmark criteria (JAMAS)), and educational quality (using the Global Quality Score (GQS) and Brachial Plexus Block Specific Quality Score (BSQS)) were validated. RESULTS: Here, 175 videos were included. Academic (1.19 ± 0.62, mean ± standard deviation), manufacturer (1.17 ± 0.71), and educational videos (1.15 ± 0.76) had better JAMAS accuracy and reliability than individual videos (0.26 ± 0.67) (p < 0.001). Manufacturer (11.22 ± 1.63) and educational videos (10.33 ± 3.34) had a higher BSQS than individual videos (7.32 ± 4.20) (p < 0.001). All sources weakly addressed the equipment preparation and post-procedure questions after BSQS analysis. CONCLUSIONS: The reliability and quality of ultrasound-guided BPB videos differ depending on their source. As YouTube is a useful educational platform for learners and teachers, global societies of regional anesthesiologists should set a standard for videos.

20.
Biosci Rep ; 41(1)2021 01 29.
Artículo en Inglés | MEDLINE | ID: mdl-33245093

RESUMEN

Abdominal aortic aneurysm (AAA), when ruptured, results in high mortality. The identification of molecular pathways involved in AAA progression is required to improve AAA prognosis. The aim of the present study was to assess the key genes for the progression of AAA and their functional role. Genomic and clinical data of three independent cohorts were downloaded from the National Center for Biotechnology Information (NCBI) Gene Expression Omnibus (GEO) (GSE57691, GSE7084, and GSE98278). To develop AAA diagnosis and progression-related differentially expressed genes (DEGs), we used a significance analysis of microarray (SAM). Spearman correlation test and gene set analysis were performed to identify potential enriched pathways for DEGs. Only the Frizzled-related protein (FRZB) gene and chromosome 1 open reading frame 24 (C1orf24) exhibited significant down-regulation in all analyses. With FRZB, the pathways were associated with RHO GTPase and elastin fiber formation. With C1orf24, the pathways were elastic fiber formation, extracellular matrix organization, and cell-cell communication. Since only FRZB was evolutionally conserved in the vertebrates, function of FRZB was validated using zebrafish embryos. Knockdown of frzb remarkably reduced vascular integrity in zebrafish embryos. We believe that FRZB is a key gene involved in AAA initiation and progression affecting vascular integrity.


Asunto(s)
Aneurisma de la Aorta Abdominal/genética , Vasos Sanguíneos/patología , Péptidos y Proteínas de Señalización Intracelular/genética , Anciano , Animales , Aneurisma de la Aorta Abdominal/patología , Estudios de Cohortes , Progresión de la Enfermedad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Análisis de Secuencia por Matrices de Oligonucleótidos , Sistemas de Lectura Abierta , Pez Cebra/embriología
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