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1.
Artículo en Inglés | MEDLINE | ID: mdl-31027558

RESUMEN

The established techniques of deep hypothermia with circulatory arrest and regional cerebral perfusion expose infants and children to additional physiologic stress and deleterious effects which may adversely affect the outcome of operations involving reconstruction of the aortic arch. Alternative techniques to supplement perfusion support are an area of innovation today. The most effective adjunct for somatic perfusion during arch reconstruction is direct cannulation of the innominate artery and the descending aorta, with full flow at mild hypothermia distributed throughout the entire body just as it is during routine, single cannulation surgery with an intact aorta. Detailed facilitating techniques for descending aortic cannulation are discussed.


Asunto(s)
Aorta Torácica/cirugía , Tronco Braquiocefálico/cirugía , Puente Cardiopulmonar/métodos , Cateterismo/métodos , Cardiopatías Congénitas/cirugía , Hipotermia Inducida/métodos , Humanos , Recién Nacido
2.
Am J Med Genet A ; 167A(12): 2893-901, 2015 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-26420300

RESUMEN

Myhre syndrome, a connective tissue disorder characterized by deafness, restricted joint movement, compact body habitus, and distinctive craniofacial and skeletal features, is caused by heterozygous mutations in SMAD4. Cardiac manifestations reported to date have included patent ductus arteriosus, septal defects, aortic coarctation and pericarditis. We present five previously unreported patients with Myhre syndrome. Despite varied clinical phenotypes all had significant cardiac and/or pulmonary pathology and abnormal wound healing. Included herein is the first report of cardiac transplantation in patients with Myhre syndrome. A progressive and markedly abnormal fibroproliferative response to surgical intervention is a newly delineated complication that occurred in all patients and contributes to our understanding of the natural history of this disorder. We recommend routine cardiopulmonary surveillance for patients with Myhre syndrome. Surgical intervention should be approached with extreme caution and with as little invasion as possible as the propensity to develop fibrosis/scar tissue is dramatic and can cause significant morbidity and mortality.


Asunto(s)
Criptorquidismo/etiología , Criptorquidismo/terapia , Trastornos del Crecimiento/etiología , Trastornos del Crecimiento/terapia , Deformidades Congénitas de la Mano/etiología , Deformidades Congénitas de la Mano/terapia , Cardiopatías/cirugía , Discapacidad Intelectual/etiología , Discapacidad Intelectual/terapia , Niño , Criptorquidismo/complicaciones , Electrocardiografía , Facies , Femenino , Trastornos del Crecimiento/complicaciones , Deformidades Congénitas de la Mano/complicaciones , Trasplante de Corazón , Humanos , Discapacidad Intelectual/complicaciones , Masculino , Mutación , Embarazo , Proteína Smad4/genética , Adulto Joven
3.
Circ Res ; 112(5): 802-15, 2013 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-23334860

RESUMEN

RATIONALE: Closure of the ductus arteriosus (DA) is essential for the transition from fetal to neonatal patterns of circulation. Initial PO2-dependent vasoconstriction causes functional DA closure within minutes. Within days a fibrogenic, proliferative mechanism causes anatomic closure. Though modulated by endothelial-derived vasodilators and constrictors, O2 sensing is intrinsic to ductal smooth muscle cells and oxygen-induced DA constriction persists in the absence of endothelium, endothelin, and cyclooxygenase mediators. O2 increases mitochondrial-derived H2O2, which constricts ductal smooth muscle cells by raising intracellular calcium and activating rho kinase. However, the mechanism by which oxygen changes mitochondrial function is unknown. OBJECTIVE: The purpose of this study was to determine whether mitochondrial fission is crucial for O2-induced DA constriction and closure. METHODS AND RESULTS: Using DA harvested from 30 term infants during correction of congenital heart disease, as well as DA from term rabbits, we demonstrate that mitochondrial fission is crucial for O2-induced constriction and closure. O2 rapidly (<5 minutes) causes mitochondrial fission by a cyclin-dependent kinase- mediated phosphorylation of dynamin-related protein 1 (Drp1) at serine 616. Fission triggers a metabolic shift in the ductal smooth muscle cells that activates pyruvate dehydrogenase and increases mitochondrial H2O2 production. Subsequently, fission increases complex I activity. Mitochondrial-targeted catalase overexpression eliminates PO2-induced increases in mitochondrial-derived H2O2 and cytosolic calcium. The small molecule Drp1 inhibitor, Mdivi-1, and siDRP1 yield concordant results, inhibiting O2-induced constriction (without altering the response to phenylephrine or KCl) and preventing O2-induced increases in oxidative metabolism, cytosolic calcium, and ductal smooth muscle cells proliferation. Prolonged Drp1 inhibition reduces DA closure in a tissue culture model. CONCLUSIONS: Mitochondrial fission is an obligatory, early step in mammalian O2 sensing and offers a promising target for modulating DA patency.


Asunto(s)
Conducto Arterial/fisiología , GTP Fosfohidrolasas/fisiología , Proteínas Asociadas a Microtúbulos/fisiología , Dinámicas Mitocondriales/fisiología , Proteínas Mitocondriales/fisiología , Músculo Liso Vascular/fisiología , Oxígeno/fisiología , Vasoconstricción/fisiología , Animales , Animales Recién Nacidos , Calcio/metabolismo , Proliferación Celular , Células Cultivadas , Conducto Arterial/citología , Dinaminas , Femenino , Humanos , Peróxido de Hidrógeno/metabolismo , Recién Nacido , Masculino , Mitocondrias/metabolismo , Modelos Animales , Músculo Liso Vascular/citología , Consumo de Oxígeno/fisiología , Conejos , Técnicas de Cultivo de Tejidos , Quinasas Asociadas a rho/metabolismo
4.
Echocardiography ; 32(3): 598-601, 2015 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-25327996

RESUMEN

We present the anatomic constellation of mitral stenosis/aortic atresia variant of hypoplastic left heart syndrome, Ebstein's anomaly, and partial anomalous pulmonary venous return, an exceeding rare congenital heart defect. Prenatal echocardiography led to concern about the capacity of the right ventricle to increase cardiac output with lung expansion and pulmonary arterial runoff at birth, prompting the precaution of extracorporeal membrane oxygenator standby at delivery. Stage I palliation was not attempted, and control of pulmonary arterial blood flow was achieved with pulmonary artery banding, allowing sufficient ongoing hemodynamic stability. Orthotopic cardiac transplantation, repair of hypoplastic aortic arch, and primary sutureless repair of left pulmonary veins was performed, using dual-site arterial cannulation and continuous mild hypothermic cardiopulmonary bypass. We discuss how this unique echocardiographic anatomy influenced the surgical decision and point out how it guided therapy toward a strategy of primary transplantation rather than standard staged surgical palliation.


Asunto(s)
Anomalía de Ebstein/diagnóstico por imagen , Anomalía de Ebstein/cirugía , Trasplante de Corazón , Síndrome del Corazón Izquierdo Hipoplásico/diagnóstico por imagen , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Ultrasonografía Prenatal/métodos , Ecocardiografía/métodos , Femenino , Humanos , Recién Nacido , Tamizaje Neonatal/métodos , Embarazo , Cirugía Asistida por Computador/métodos , Resultado del Tratamiento , Adulto Joven
5.
Pediatr Crit Care Med ; 15(4): 329-35, 2014 May.
Artículo en Inglés | MEDLINE | ID: mdl-24614607

RESUMEN

OBJECTIVES: Although transthoracic echocardiography is commonly performed in the PICU, its utility is not specifically known. The purpose of this investigation was to evaluate the clinical impact of echocardiography in the PICU in terms of frequency of unanticipated findings and the frequency and nature of clinical management changes attributed to the results of echocardiography. DESIGN: Prospective cohort study. SETTING: Nineteen-bed combined medical-surgical-cardiac PICU at a tertiary care children's hospital. PATIENTS: All patients in PICU undergoing transthoracic echocardiography. INTERVENTIONS, MEASUREMENTS, AND MAIN RESULTS: Data collected included echocardiography indications, pre-echocardiography clinical assessment of anticipated echocardiography findings, height, weight, primary diagnosis, age, and urgency (stat vs routine) of echocardiography. Input of the attending care team (intensivist, cardiologist, and/or cardiovascular surgeon) allowed classification of echocardiography results as either confirming the pre-echocardiography impression, altering the pre-echocardiography clinical impression regarding the indication for which the test was performed, or altering the impression by virtue of new findings unrelated to the specific indication. The nature of the new findings were recorded and categorized. The team recorded clinical management changes made in response to the echocardiography results; the nature of these were listed and categorized. Echocardiograms (n = 416) were performed in 132 patients. Of these, 244 echocardiograms (59%) were ordered on male patients, 31% were under 30 days old, median age was 103 days, 379 (91%) had a primary cardiac diagnosis, and 92 (22%) were ordered stat. Sixty-three percent of echocardiograms confirmed and 24% altered the pre-echocardiography impression regarding the indication for the echocardiography; 13% introduced new findings unrelated to the indication. Cardiac surgical revision was the management change required in 26 patients (6.3%). Stat echocardiography was more likely to alter the pre-echocardiography assessment than routine echocardiography (p < 0.001). Management changes were more commonly associated with stat echocardiograms (p = 0.002) and those with new unexpected findings (p < 0.001) but had no demonstrable association with age less than 30 days (p = 0.332). CONCLUSIONS: Unanticipated echocardiography results are common in the PICU, and they often alter the clinical impressions that prompted the echocardiogram or introduce new findings unrelated to the reason for which the echocardiogram was recorded. Clinical management changes attributable to echocardiography findings are frequent in the PICU, including occasional surgical intervention. Echocardiography adds diagnostic value and contributes to the management approach in the PICU, accounting for its frequent use.


Asunto(s)
Ecocardiografía , Cardiopatías/diagnóstico por imagen , Cardiopatías/terapia , Unidades de Cuidado Intensivo Pediátrico , Enfermedad Crítica , Femenino , Cardiopatías/cirugía , Humanos , Hallazgos Incidentales , Lactante , Recién Nacido , Enfermedades Pulmonares/diagnóstico por imagen , Masculino , Estudios Prospectivos , Factores de Tiempo
6.
Ann Thorac Surg ; 115(3): 649-654, 2023 03.
Artículo en Inglés | MEDLINE | ID: mdl-35863395

RESUMEN

BACKGROUND: The Norwood operation is a complex neonatal surgery. There are limited data to inform the timing of sternal closure. After the Norwood operation, delayed sternal closure (DSC) is frequent. We aimed to examine the association of DSC with outcomes, with a particular interest in how sternal closure at the time of surgery compared with the timing of DSC. Our outcomes included mortality, length of ventilation, length of stay, and postoperative complications. METHODS: This retrospective study included neonates who underwent a Norwood operation reported in the Pediatric Cardiac Critical Care Consortium registry from February 2019 through April 2021. Outcomes of patients with closed sternum were compared to those with sternal closure prior to postoperative day 3 (early closure) and prior to postoperative day 6 (intermediate closure). RESULTS: The incidence of DSC was 74% (500 of 674). The median duration of open sternum was 4 days (interquartile range 3-5 days). Comparing patients with closed sternum to patients with early sternal closure, there was no statistical difference in mortality rate (1.1% vs 0%) and the median hospital postoperative stay (30 days vs 31 days). Compared with closed sternum, patients with intermediate sternal closure required longer mechanical ventilation (5.9 days vs 3.9 days) and fewer subsequent sternotomies (3% vs 7.5%). CONCLUSIONS: For important outcomes following the Norwood operation there is no advantage to chest closure at the time of surgery if the chest can be closed prior to postoperative day 3.


Asunto(s)
Procedimientos Quirúrgicos Cardíacos , Procedimientos de Norwood , Recién Nacido , Humanos , Niño , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Estudios Retrospectivos , Esternón/cirugía , Complicaciones Posoperatorias/etiología , Procedimientos de Norwood/efectos adversos , Infección de la Herida Quirúrgica/epidemiología
7.
Pediatr Radiol ; 42(11): 1339-46, 2012 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-22885603

RESUMEN

BACKGROUND: With increasing applications of cardiac magnetic resonance (CMR) and magnetic resonance angiography (MRA) for evaluation of congenital heart disease (CHD), safety of this technology in the very young is of particular interest. OBJECTIVE: We report our 10-year experience with CMR in neonates and small infants with particular focus on the safety profile and incidence of adverse events (AEs). MATERIALS AND METHODS: We reviewed clinical, anesthesia and nursing records of all children ≤120 days of age who underwent CMR. We recorded variables including cardiac diagnosis, study duration, anesthesia type and agents, prostaglandin E1 (PGE1) dependence and gadolinium (Gd) use. Serially recorded temperature, systemic saturation (SpO(2)) and cardiac rhythm were analyzed. Primary outcome measure was any AE during or <24 h after the procedure, including minor AEs such as hypothermia (axillary temperature ≤95 °F), desaturation (SpO(2) drop ≥10% below baseline) and bradycardia (heart rate ≤100 bpm). Secondary outcome measure was unplanned overnight hospitalization of outpatients. RESULTS: Children (n = 143; 74 boys, 69 girls) had a median age of 6 days (1-117), and 98 were ≤30 days at the time of CMR. The median weight was 3.4 kg (1.4-6 kg) and body surface area 0.22 m(2) (0.13-0.32 m(2)). There were 118 (83%) inpatients (108 receiving intensive care) and 25 (17%) outpatients. Indications for CMR were assessment of aortic arch (n = 57), complex CHD (n = 41), pulmonary veins (n = 15), vascular ring (n = 8), intracardiac mass (n = 8), pulmonary artery (n = 7), ventricular volume (n = 4), and systemic veins (n = 3). CMR was performed using a 1.5-T scanner and a commercially available coil. CMR utilized general anesthesia (GA) in 86 children, deep sedation (DS) in 50 and comforting methods in seven. MRA was performed in 136 children. Fifty-nine children were PGE1-dependent and 39 had single-ventricle circulation. Among children on PGE1, 43 (73%) had GA and 10 (17%) had DS. Twelve children (9%) had adverse events (AEs)-one major and 11 minor. Of those 12, nine children had GA (10%) and three had DS (6%). The single major AE was respiratory arrest after DS in a neonate (resuscitated without sequelae). Minor AEs included desaturations (n = 2), hypothermia (n = 5), bradycardia (n = 2), and bradycardia with hypoxemia (n = 2). Incidence of minor AEs was 9% for inpatients (vs. 4% for outpatients), and 8% for neonates (vs. 9% for age ≥30 days). Incidence of minor AEs was similar between PGE1-dependent infants and the non-PGE1 group. There were no adverse events related to MRA. Of 25 outpatients, 5 (20%) were admitted for overnight observation due to desaturations. CONCLUSION: CMR and MRA can be accomplished safely in neonates and infants ≤120 days old for a wide range of pre-surgical cardiac indications. Adverse events were unrelated to patient age, complexity of heart disease, type of anesthesia or PGE1 dependence.


Asunto(s)
Anestésicos Generales/uso terapéutico , Bradicardia/epidemiología , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/epidemiología , Gadolinio , Hipotermia/epidemiología , Angiografía por Resonancia Magnética/estadística & datos numéricos , Imagen por Resonancia Cinemagnética/estadística & datos numéricos , Comorbilidad , Medios de Contraste , Femenino , Humanos , Incidencia , Lactante , Recién Nacido , Estudios Longitudinales , Masculino , Nebraska/epidemiología , Estudios Retrospectivos , Medición de Riesgo
8.
Catheter Cardiovasc Interv ; 78(7): 1045-50, 2011 Dec 01.
Artículo en Inglés | MEDLINE | ID: mdl-21542115

RESUMEN

Vein of Galen aneurysmal malformation is the most common cerebral arteriovenous malformation in children. Neurointerventional embolization has improved outcomes with this lesion. An association with cardiac defects has been recognized and can increase the risk of systemic embolic events during treatment. Cardiopulmonary bypass (CPB) to correct the cardiac defect before repair of the malformation may be associated with compromised cerebral perfusion and possible neurologic injury. We describe a successful hybrid peratrial closure of two intracardiac shunts (a patent foramen ovale and a sinus venosus ASD) before neurointervention, avoiding CPB, and eliminating the intracardiac embolic risk during treatment of the vein of Galen malformation.


Asunto(s)
Anomalías Múltiples , Cateterismo Cardíaco/instrumentación , Foramen Oval Permeable/terapia , Defectos del Tabique Interatrial/terapia , Dispositivo Oclusor Septal , Malformaciones de la Vena de Galeno/terapia , Ecocardiografía Transesofágica , Femenino , Foramen Oval Permeable/complicaciones , Foramen Oval Permeable/diagnóstico , Defectos del Tabique Interatrial/complicaciones , Defectos del Tabique Interatrial/diagnóstico , Humanos , Lactante , Imagen por Resonancia Magnética , Diseño de Prótesis , Síndrome de Cimitarra/complicaciones , Factores de Tiempo , Resultado del Tratamiento , Malformaciones de la Vena de Galeno/complicaciones , Malformaciones de la Vena de Galeno/diagnóstico
9.
World J Pediatr Congenit Heart Surg ; 12(2): 293-296, 2021 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-33684003

RESUMEN

Interruption of the ascending aorta is an extremely rare anomaly defined by a point of interruption between the intrapericardial and extrapericardial aorta and can be explained by developmental errors proximal to the embryologic right aortic sac. Herein, we present a case of interruption of the ascending aorta and describe a successful biventricular surgical repair of this unique anomaly.


Asunto(s)
Aorta Torácica/cirugía , Tronco Braquiocefálico/cirugía , Procedimientos Quirúrgicos Cardíacos/métodos , Malformaciones Vasculares/cirugía , Aorta Torácica/diagnóstico por imagen , Tronco Braquiocefálico/diagnóstico por imagen , Ecocardiografía/métodos , Femenino , Humanos , Lactante , Malformaciones Vasculares/diagnóstico
10.
JACC Case Rep ; 2(11): 1716-1719, 2020 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-34317042

RESUMEN

Williams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis and branch pulmonary stenosis. We describe double-outlet right ventricle with mitral atresia and aortic arch hypoplasia in an infant with WS. This case demonstrates the difficulty in managing patients with WS with complex cardiac defects. To our knowledge, this is the first reported single-ventricle physiology in a patient with WS. (Level of Difficulty: Advanced.).

11.
World J Pediatr Congenit Heart Surg ; 11(4): 401-408, 2020 07.
Artículo en Inglés | MEDLINE | ID: mdl-32645775

RESUMEN

BACKGROUND: Mortality after congenital heart defect surgery has dropped dramatically in the last few decades. Current research on long-term outcomes has focused on preventing secondary neurological sequelae, for which embolic burden is suspected. In children, little is known of the correlation between specific surgical maneuvers and embolic burden. Transcranial Doppler ultrasound is highly useful for detecting emboli but has not been widely used with infants and children. METHODS: Bilateral middle cerebral artery blood flow was continuously monitored from sternal incision to chest closure in 20 infants undergoing congenital heart defect repair or palliative surgery. Embolus counts for specific maneuvers were recorded using widely accepted criteria for identifying emboli via high-intensity transient signals (HITS). RESULTS: An average of only 13% of all HITS detected during an operation were correlated with any of the surgical maneuvers of interest. The highest mean number of HITS associated with a specific maneuver occurred during cross-clamp removal. Cross-clamp placement also had elevated HITS counts that significantly differed from other maneuvers. CONCLUSIONS: In this study of infants undergoing cardiac surgery with cardiopulmonary bypass, the great majority of HITS detected are not definitively associated with a specific subset of surgical maneuvers. Among the measured maneuvers, removal of the aortic cross-clamp was associated with the greatest occurrence of HITS. Future recommended research efforts include identifying and confirming other sources for emboli and longitudinal outcome studies to determine if limiting embolic burden affects long-term neurological outcomes.


Asunto(s)
Cardiopatías Congénitas/cirugía , Embolia Intracraneal/diagnóstico , Ultrasonografía Doppler Transcraneal/métodos , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Embolia Intracraneal/etiología , Embolia Intracraneal/fisiopatología , Masculino
12.
Cardiovasc Pathol ; 39: 54-60, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-30660869

RESUMEN

The search for an ideal material for cardiac tissue repair has led to utilization of porcine small intestinal submucosa extracellular matrix (CorMatrix). Here, we examine the histologic features of CorMatrix and the associated cellular growth at a variety of time intervals. Tissues with CorMatrix from ten patients (4 male, 6 female) with ages ranging from 2 weeks to 2 years, and implant duration ranging from 1 week to 2 years were included in this study. Samples for analysis were collected at autopsy. Surgical repair sites included great vessel repair (n=9), atrial septum defect (n=1), coronary vessels (n=1), as well as aortic (n=1) and mitral valve (n=2) leaflets. In all specimens, CorMatrix was composed of dense laminated regions of collagen, without appreciable elastin staining. In most grafts, especially those implanted for extended periods of time, tissue with luminal CD31 positivity covered the intimal surface of the CorMatrix graft. This tissue (neo-intima) consisted of spindled myofibroblasts (SMA) and small CD31 positive vessels with occasional mononuclear cells in a matrix composed of collagen, glycosaminoglycans, and rarely elastin, after extended periods of implantation. These features were readily identified in patients as early as 1 month after CorMatrix implantation. The matrix comprising the CorMatrix itself remained largely acellular, despite implantation times up to 2 years, with degradation of the graft material. We provide a framework for histologic expectations when evaluating explanted CorMatrix grafts. In this regard, the CorMatrix matrix is likely to remain acellular without significant elastin deposition, whereas the intimal and adventitial surfaces become coated by proliferating cells in a novel matrix of collagen and glycosaminoglycans.


Asunto(s)
Procedimientos Quirúrgicos Cardíacos , Proliferación Celular , Matriz Extracelular/trasplante , Cardiopatías Congénitas/cirugía , Intestino Delgado/trasplante , Animales , Autopsia , Biopsia , Preescolar , Colágeno/metabolismo , Matriz Extracelular/metabolismo , Matriz Extracelular/patología , Femenino , Glicosaminoglicanos/metabolismo , Cardiopatías Congénitas/metabolismo , Cardiopatías Congénitas/patología , Xenoinjertos , Humanos , Lactante , Intestino Delgado/metabolismo , Intestino Delgado/patología , Masculino , Propiedades de Superficie , Sus scrofa , Factores de Tiempo , Resultado del Tratamiento
13.
J Thorac Cardiovasc Surg ; 157(4): 1591-1598, 2019 04.
Artículo en Inglés | MEDLINE | ID: mdl-30665762

RESUMEN

OBJECTIVE: Immediate extubation may have outcome benefits when judiciously instituted after neonatal congenital cardiac surgery. We sought to evaluate the outcomes of immediate extubation specifically in neonates undergoing stage 1 Norwood palliation of hypoplastic left heart syndrome. METHODS: Consecutive neonates undergoing stage 1 Norwood (January 2010 to December 2016) for hypoplastic left heart syndrome were retrospectively studied. Immediate extubation was defined as successful extubation before termination of anesthetic care. Preoperative and intraoperative variables were compared between immediate extubation and nonimmediate extubation groups, and bivariate analyses and descriptive methods were used to express the association of outcome variables with immediate extubation. Data were expressed as number and percent for categoric variables, and median and interquartile range for continuous variables. RESULTS: Of 23 patients who underwent stage 1 palliation, 5 had immediate extubation (22%). There were no differences in preoperative or intraoperative factors between patients who did and did not undergo immediate extubation. There were no deaths in the immediate extubation group. In the nonimmediate extubation group, 3 patients died before hospital discharge. One patient who had immediate extubation and 4 patients among those who did not have immediate extubation had to be reintubated in the 96 hours that followed extubation (P = 1). Intensive care unit length of stay was 8 (3-17) and 8 (5-18) (days) for the immediate extubation group and nonimmediate extubation groups, respectively (P = .71). CONCLUSIONS: Immediate extubation strategy was safely accomplished in one-fifth of this cohort of hypoplastic left heart syndrome. A larger cohort may delineate the determinants of immediate extubation and its benefits in infants undergoing stage 1 single ventricle palliation.


Asunto(s)
Extubación Traqueal , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Procedimientos de Norwood , Cuidados Paliativos , Tiempo de Tratamiento , Extubación Traqueal/efectos adversos , Extubación Traqueal/mortalidad , Femenino , Mortalidad Hospitalaria , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/diagnóstico , Síndrome del Corazón Izquierdo Hipoplásico/mortalidad , Síndrome del Corazón Izquierdo Hipoplásico/fisiopatología , Recién Nacido , Intubación Intratraqueal , Masculino , Procedimientos de Norwood/efectos adversos , Procedimientos de Norwood/mortalidad , Complicaciones Posoperatorias/mortalidad , Complicaciones Posoperatorias/terapia , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento
14.
Congenit Heart Dis ; 14(6): 1149-1156, 2019 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-31917528

RESUMEN

INTRODUCTION: The right ventricular infundibular sparing approach (RVIS) to the repair of tetralogy of Fallot (TOF) avoids a full-thickness ventricular incision, typically utilized in the transinfundibular (TI) method. METHODS: We performed a retrospective, age-matched cohort study of patients who underwent RVIS at Texas Children's Hospital or TI at Children's Hospital Medical Center in Nebraska and subsequently underwent cardiac magnetic resonance imaging (CMR). We compared right ventricular end-diastolic and systolic volumes indexed to body surface area (RVEDVi and RVESVi) and right ventricular ejection fraction (RVEF) as primary endpoints. Secondary endpoints were indexed left ventricular diastolic and systolic volume (LVEDVi and LVESVi), left ventricular ejection fraction (LVEF), right ventricular (RV) sinus ejection fraction (EF) and RV outflow tract EF (RVOT EF). RESULTS: Seventy-nine patients were included in the analysis; 40 underwent RVIS and 39 underwent TI repair. None of the patients in the TI repair group had an initial palliation with a systemic to pulmonary arterial shunt compared to seven (18%) in the RVIS group (P < .01). There was no appreciable difference in RVEDVi (122 ± 29 cc/m2 vs 130 ± 29 cc/m2 , P = .59) or pulmonary regurgitant fraction (40 ± 13 vs 37 ± 18, P = .29) between the RVIS and TI groups. Compared to the TI group, the RVIS group had higher RVEF (54 ± 6% vs 44 ± 9%, P < .01), lower RVESV (57 ± 17 cc/m2 vs 67 ± 25 cc/m2 , P = .03), higher LVEF (61 ± 11% vs 54 ± 8%, P < .01), higher RVOT EF (47 ± 12% vs 41 ± 11%, P = .03), and higher RV sinus EF (56 ± 5% vs 49 ± 6%, P < .01) CONCLUSIONS: In this selected cohort, patients who underwent RVIS repair for TOF had higher right and left ventricular ejection fraction compared to those who underwent TI repair.


Asunto(s)
Procedimientos Quirúrgicos Cardíacos , Tetralogía de Fallot/cirugía , Adolescente , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Niño , Preescolar , Femenino , Humanos , Imagen por Resonancia Cinemagnética , Masculino , Nebraska , Recuperación de la Función , Estudios Retrospectivos , Factores de Riesgo , Volumen Sistólico , Tetralogía de Fallot/diagnóstico por imagen , Tetralogía de Fallot/fisiopatología , Texas , Resultado del Tratamiento , Función Ventricular Izquierda , Función Ventricular Derecha
16.
Int J Cardiol ; 263: 165-170, 2018 07 15.
Artículo en Inglés | MEDLINE | ID: mdl-29657080

RESUMEN

OBJECTIVE: We investigated a full energetic profile of pressure and volume loaded right ventricle (RV) in porcine models by evaluating kinetic energy (KE), stroke power, power output and power loss across pulmonary valves with stenosis (PS) or with regurgitation (PR). METHODS: Fifteen pigs (6 PS and 6 PR, 3 unoperated controls) were studied. Phase-contrast 4D-flow MRI was performed in models of PS and PR at baseline and at 10-12 weeks, in conjunction with cardiac catheterization. Phase contrast velocities over 1 cardiac cycle were registered with a dynamic mask of the RV segmented from cine images. Mean KE and KE curve profiles were measured, normalized for RV volumes and compared between groups. Right heart catheterization pressures were used to calculate RV stroke power and power output, from which pulmonary valve power loss and RV power output ratio were calculated, and compared between groups. RESULTS: PS and PR groups had similar KE pre procedure but significant changes in KE post procedure. The PR group had higher RV power output ratio and KE (72.1% ±â€¯11.4%; 20.6 ±â€¯6.1) than PS group (25.6% ±â€¯4.7%; 13.8 ±â€¯5.0) post procedure. Volume loaded RV from PR had higher KE and power output ratio compared to pressure load from PS. CONCLUSIONS: In porcine models of PS and PR, the RV presents altered systolic and diastolic energetic profiles. Pulmonary valve efficiency appeared to decrease in the medium term with somatic growth, with increased power loss in all groups studied, and greatly within the PS group.


Asunto(s)
Imagen por Resonancia Cinemagnética/métodos , Insuficiencia de la Válvula Pulmonar/diagnóstico por imagen , Insuficiencia de la Válvula Pulmonar/fisiopatología , Estenosis de la Válvula Pulmonar/diagnóstico por imagen , Estenosis de la Válvula Pulmonar/fisiopatología , Función Ventricular Derecha/fisiología , Animales , Medios de Contraste , Ecocardiografía/métodos , Porcinos
17.
Circ Cardiovasc Interv ; 11(11): e007145, 2018 11.
Artículo en Inglés | MEDLINE | ID: mdl-30571200

RESUMEN

Background Pediatric patients with atrioventricular valve disease have limited options for prosthetic valve replacement in sizes <15 mm. Based on successful experience with the stented bovine jugular vein graft (Melody valve) in the right ventricular outflow tract, the prosthesis has been modified for surgical valve replacement in pediatric patients with atrioventricular dysfunction with the intention of subsequent valve expansion in the catheterization laboratory as the child grows. Methods and Results A multicenter, retrospective cohort study was performed among patients who underwent atrioventricular valve replacement with Melody valve at 17 participating sites from North America and Europe, including 68 patients with either mitral (n=59) or tricuspid (n=9) replacement at a median age of 8 months (range, 3 days to 13 years). The median size at implantation was 14 mm (range, 9-24 mm). Immediately postoperatively, the valve was competent with low gradients in all patients. Fifteen patients died; 3 patients underwent transplantation. Nineteen patients required reoperation for adverse outcomes, including valve explantation (n=16), left ventricular outflow tract obstruction (n=1), permanent pacemaker implantation (n=1), and paravalvular leak repair (n=1). Twenty-five patients underwent 41 episodes of catheter-based balloon expansion, exhibiting a significant decrease in median gradient ( P<0.001) with no significant increase in grade of regurgitation. Twelve months after implantation, cumulative incidence analysis indicated that 55% of the patients would be expected to be free from death, heart transplantation, structural valve deterioration, or valve replacement. Conclusions The Melody valve is a feasible option for surgical atrioventricular valve replacement in patients with hypoplastic annuli. The prosthesis shows acceptable short-term function and is amenable to catheter-based enlargement as the child grows. However, patients remain at risk for mortality and structural valve deterioration, despite adequate early valvular function. Device design and implantation techniques must be refined to reduce complications and extend durability. Clinical Trial Registration URL: https://www.clinicaltrials.gov. Unique identifier: NCT02505074.


Asunto(s)
Bioprótesis , Implantación de Prótesis de Válvulas Cardíacas/instrumentación , Prótesis Valvulares Cardíacas , Prolapso de la Válvula Mitral/cirugía , Estenosis de la Válvula Mitral/cirugía , Válvula Mitral/cirugía , Adolescente , Factores de Edad , Niño , Preescolar , Ecocardiografía Doppler en Color , Europa (Continente) , Femenino , Implantación de Prótesis de Válvulas Cardíacas/efectos adversos , Implantación de Prótesis de Válvulas Cardíacas/mortalidad , Hemodinámica , Humanos , Lactante , Recién Nacido , Masculino , Válvula Mitral/diagnóstico por imagen , Válvula Mitral/fisiopatología , Prolapso de la Válvula Mitral/diagnóstico por imagen , Prolapso de la Válvula Mitral/mortalidad , Prolapso de la Válvula Mitral/fisiopatología , Estenosis de la Válvula Mitral/diagnóstico por imagen , Estenosis de la Válvula Mitral/mortalidad , Estenosis de la Válvula Mitral/fisiopatología , América del Norte , Complicaciones Posoperatorias/fisiopatología , Complicaciones Posoperatorias/terapia , Diseño de Prótesis , Recuperación de la Función , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento
18.
J Thorac Cardiovasc Surg ; 163(5): 1626, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-34922753
19.
Eur J Cardiothorac Surg ; 51(4): 728-734, 2017 04 01.
Artículo en Inglés | MEDLINE | ID: mdl-28199509

RESUMEN

Objectives: We sought to identify preoperative, intraoperative and anatomical factors associated with immediate extubation (IE) after arterial switch operation for d-transposition of great arteries (dTGA). Methods: This was a single-centre retrospective study performed from 1 January 2010 to 30 June 2015. IE was defined as successful extubation in the operating room (OR). Univariate/bivariate regression of preoperative, intraoperative and anatomical variables was used to determine associations with IE. Results: Of 32 patients in the dTGA spectrum (age at operation 6 days), 18 (56%) underwent IE. Twelve (71%) of the 17 patients with an intact ventricular septum and 6 (43%) of the 14 patients with ventricular septal defect (VSD) underwent IE, whereas none of the patients with double outlet right ventricle or aortic arch obstruction ( n = 4) did. Patients who had cardiopulmonary bypass time (CPB) >173 min ( P = 0.01), lowest temperature on CPB (T min) ≤ 30.4°C ( P = 0.04) and aortic cross-clamp time >86 min ( P = 0.04) were more likely to be left intubated at the end of the procedure. There was no significant difference in patient's chronological age, gestational age, post-conceptual age, weight, coronary anatomy or prevalence of VSD between those who did and did not undergo IE. There was a median increase in intensive care unit (ICU) length of stay (LOS) by 1 day (33%, P = 0.03) and ICU costs by $12 338 (15%, P = 0.06) in non-IE patients. The OR turnover time ( P = 0.09) and reintubation rate ( P = 1) at 24 h post-extubation did not differ between those who did and did not have IE. There was no myocardial dysfunction evident on predismissal echocardiography in either group. Conclusions: In this cohort of infants, post repair for TGA, 56% were extubated immediately in the OR. Greater CPB and cross-clamp times and T min ≤ 30.4°C were associated with a lesser likelihood of IE. IE was associated with shorter ICU length of stay.


Asunto(s)
Extubación Traqueal/métodos , Operación de Switch Arterial/métodos , Transposición de los Grandes Vasos/cirugía , Anestesia General/métodos , Cuidados Críticos , Defectos del Tabique Interventricular/patología , Humanos , Recién Nacido , Tiempo de Internación/estadística & datos numéricos , Cuidados Posoperatorios/métodos , Periodo Posoperatorio , Estudios Retrospectivos , Transposición de los Grandes Vasos/patología , Resultado del Tratamiento
20.
Ann Thorac Surg ; 102(5): 1588-1595, 2016 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-27324528

RESUMEN

BACKGROUND: We sought to identify preoperative and intraoperative predictors of immediate extubation (IE) after open heart surgery in neonates. The effect of IE on the postoperative intensive care unit (ICU) length of stay (LOS), cost of postoperative ICU care, operating room turnover, and reintubation rates was assessed. METHODS: Patients younger than 31 days who underwent cardiac surgery with cardiopulmonary bypass (January 2010 to December 2013) at a tertiary-care children's hospital were studied. Immediate extubation was defined as successful extubation before termination of anesthetic care. Data on preoperative and intraoperative variables were compared using descriptive, bivariate, and multivariate statistics to identify the predictors of IE. Propensity scores were used to assess effects of IE on ICU LOS, the cost of ICU care, reintubation rates, and operating room turnover time. RESULTS: One hundred forty-eight procedures done at a median age of 7 days resulted in 45 IEs (30.4%). The IE rate was 22.2% with single-ventricle heart disease. Independent predictors of IE were the absence of the need for preoperative ventilatory assistance, higher gestational age, anesthesiologist, and shorter cardiopulmonary bypass. Immediate extubation was associated with shorter ICU LOS (8.3 versus 12.7 days; p < 0.0001) and lower cost of ICU care (mean postoperative ICU charges, $157,449 versus $198,197; p < 0.0001) with no significant difference in the probability of reintubation (p = 0.7). Immediate extubation was associated with longer operating room turnover time (38.4 versus 46.7 minutes; p = 0.009). CONCLUSIONS: Immediate extubation was accomplished in 30.4% of neonates undergoing open heart surgery involving cardiopulmonary bypass. Immediate extubation was associated with lesser ICU LOS, postoperative ICU costs, and minimal increase in operating room turnover time, but without an increase in reintubation rates. Low gestational age, preoperative ventilatory support requirement, and prolonged cardiopulmonary bypass time were inversely associated with the ability to accomplish IE.


Asunto(s)
Extubación Traqueal , Procedimientos Quirúrgicos Cardíacos , Cuidados Posoperatorios/estadística & datos numéricos , Extubación Traqueal/economía , Extubación Traqueal/estadística & datos numéricos , Anestesia/economía , Anestesia/métodos , Anestesia/estadística & datos numéricos , Procedimientos Quirúrgicos Cardíacos/economía , Puente Cardiopulmonar , Femenino , Edad Gestacional , Costos de Hospital , Humanos , Recién Nacido , Unidades de Cuidado Intensivo Pediátrico/economía , Unidades de Cuidado Intensivo Pediátrico/estadística & datos numéricos , Intubación Intratraqueal/economía , Intubación Intratraqueal/estadística & datos numéricos , Tiempo de Internación/economía , Tiempo de Internación/estadística & datos numéricos , Masculino , Quirófanos/economía , Tempo Operativo , Cuidados Posoperatorios/economía , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/terapia , Curva ROC , Sala de Recuperación/economía , Sala de Recuperación/estadística & datos numéricos , Reoperación/estadística & datos numéricos , Respiración Artificial/estadística & datos numéricos , Estudios Retrospectivos
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