RESUMEN
The Fontan operation and its modifications are currently the standard palliation in patients with single ventricle physiology. The advantages of extracardiac conduit Fontan operation (ECF) have previously been described. We evaluated the outcome of patients who underwent ECF operation at our center since the year 2000 to assess morbidity, mortality and New York Heart Association (NYHA) functional class in the current era. Medical records of 97 patients who underwent ECF operation from November 2000 till October 2013 were reviewed to determine the diagnosis, age at surgery, surgical technique, postoperative complications, NYHA class at last follow-up, echocardiographic parameters, and most recent Holter monitor results. The median age at surgery was 3.4 years with 4 total deaths until last follow-up (hospital mortality was 1 %, and mortality after hospital discharge was 3 %). Seventy-eight of the 97 patients (80 %) underwent fenestrated Fontan surgery. Median duration of follow-up was 3.9 years (IQR 0.2-12.2 years). At the latest follow-up, 62 patients (67 %) were determined to be in NYHA class I, 25 patients (27 %) in NYHA class II, and 6 (6 %) patients in NYHA class III, and the median oxygen saturation was 92 % (IQR 86-96). There were 15 patients with history of preoperative arrhythmias and 6 of them required pacemaker placement at the time of surgery. One patient developed ectopic atrial tachycardia, and 1 patient had clinical thromboembolism on follow-up. Systolic function of the single ventricle by transthoracic echocardiogram at the latest follow-up was described as normal in 77 patients (83 %), fair in 15 patients (16 %), and poor in 1 patient (1 %). The results of our study show that patients who undergo extracardiac conduit Fontan operation in the modern era may have significantly less morbidity and mortality when compared to earlier years.
Asunto(s)
Ventrículos Cardíacos , Adolescente , Arritmias Cardíacas , Niño , Preescolar , Estudios de Seguimiento , Procedimiento de Fontan , Cardiopatías Congénitas , Humanos , Resultado del Tratamiento , Adulto JovenRESUMEN
We report a venous bullet embolus into the left pulmonary artery following a gunshot wound to the right chest. The diagnosis and surgical management of migrating venous bullet emboli are reviewed.
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Migración de Cuerpo Extraño/complicaciones , Arteria Pulmonar , Embolia Pulmonar/etiología , Heridas por Arma de Fuego/diagnóstico , Adolescente , Migración de Cuerpo Extraño/diagnóstico , Migración de Cuerpo Extraño/cirugía , Humanos , Imagenología Tridimensional , Masculino , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/cirugía , Radiografía Torácica , Tomografía Computarizada por Rayos X , Procedimientos Quirúrgicos Vasculares/métodos , Heridas por Arma de Fuego/complicaciones , Heridas por Arma de Fuego/cirugíaRESUMEN
An 11-year-old boy, who underwent bicaval orthotopic heart transplantation for idiopathic dilated cardiomyopathy, had a focal atrial tachycardia originating from the donor superior vena cava. The pathogenesis of this tachycardia may be related to transplant rejection or transplant vasculopathy. Radiofrequency catheter ablation can eliminate this unique tachycardia and result in hemodynamic improvement.
Asunto(s)
Electrocardiografía , Trasplante de Corazón/efectos adversos , Taquicardia Supraventricular/fisiopatología , Vena Cava Superior/fisiopatología , Cardiomiopatía Dilatada/cirugía , Ablación por Catéter , Niño , Técnicas Electrofisiológicas Cardíacas , Humanos , Masculino , Taquicardia Supraventricular/etiología , Taquicardia Supraventricular/cirugía , Vena Cava Superior/trasplanteRESUMEN
Ectopia cordis is defined as a congenital malposition of the heart outside of the thoracic cavity. It is a rare condition, and complete ectopia cordis can be a fatal condition. Successful surgical reconstruction of this defect has been reported but is uncommon. The general approach to reconstructing the chest wall involves repositioning the heart and providing adequate coverage of the chest wall defect. We describe our experience with a patient who had complete thoracic ectopia cordis treated with staged chest wall reconstruction. The first stage involved temporary closure with synthetic material, and the second stage involved definitive reconstruction with autologous bone and cartilage grafts supported with plates. The patient has been active and without complaints since the second stage and is awaiting tracheal decannulation. There have been a few descriptions of how to approach chest wall reconstruction in patients with ectopia cordis. The 2 stage method described can be considered to repair the chest wall defect in complete thoracic ectopia cordis.
Asunto(s)
Ectopía Cordis/cirugía , Procedimientos de Cirugía Plástica/métodos , Procedimientos Quirúrgicos Torácicos/métodos , Pared Torácica/cirugía , Humanos , Recién Nacido , Masculino , Pared Torácica/anomalíasRESUMEN
Tetralogy of Fallot with absent pulmonary valve syndrome is a rare form of congenital heart disease. Among the different variations with this rare anomaly is nonconfluent pulmonary artery branches with anomalous origin of the left pulmonary artery from the ductus arteriosus. The authors present one such case which was diagnosed prenatally to have tetralogy of Fallot with absent pulmonary valve and identified postnatally to have nonconfluent pulmonary artery branches in addition. We discuss the conundrum of respiratory management in this patient pre- and postoperatively due to a unique ventilation perfusion mismatch problem, which varies between the two lungs.
Asunto(s)
Conducto Arterioso Permeable/diagnóstico , Ecocardiografía/métodos , Diagnóstico Prenatal , Arteria Pulmonar/anomalías , Atresia Pulmonar/diagnóstico , Válvula Pulmonar/anomalías , Tetralogía de Fallot/diagnóstico , Conducto Arterioso Permeable/embriología , Resultado Fatal , Femenino , Humanos , Recién Nacido , Masculino , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/embriología , Atresia Pulmonar/embriología , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/embriología , Tetralogía de Fallot/embriología , Tomografía Computarizada por Rayos X , Adulto JovenRESUMEN
BACKGROUND: Florida is the fourth largest state in the United States of America. In 2004, 218,045 live babies were born in Florida, accounting for approximately 1744 new cases of congenital heart disease. We review the initial experience of The Society of Thoracic Surgeons Congenital Heart Surgery Database with a regional outcomes report, namely the Society of Thoracic Surgeons Florida Regional Report. METHODS: Eight centres in Florida provide services for congenital cardiac surgery. The Children's Medical Services of Florida provide a framework for quality improvement collaboration between centres. All congenital cardiac surgical centres in Florida have voluntarily agreed to submit data to the Society of Thoracic Surgeons Database. The Society of Thoracic Surgeons and Duke Clinical Research Institute prepared a Florida Regional Report to allow detailed regional analysis of outcomes for congenital cardiac surgery. RESULTS: The report of 2007 from the Society of Thoracic Surgeons Congenital Heart Surgery Database includes details of 61,014 operations performed during the 4 year data harvest window, which extended from 2003 through 2006. Of these operations, 6,385 (10.5%) were performed in Florida. Discharge mortality in the data from Florida overall, and from each Florida site, with 95% confidence intervals, is not different from cumulative data from the entire Society of Thoracic Surgeons Database, both for all patients and for patients stratified by complexity. CONCLUSIONS: A regional consortium of congenital heart surgery centres in Florida under the framework of the Children's Medical Services has allowed for inter-institutional collaboration with the goal of quality improvement. This experience demonstrates, first, that the database maintained by the Society of Thoracic Surgeons can provide the framework for regional analysis of outcomes, and second, that voluntary regional collaborative efforts permit the pooling of data for such analysis.
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Procedimientos Quirúrgicos Cardíacos/estadística & datos numéricos , Cardiopatías Congénitas/cirugía , Sistema de Registros , Sociedades Médicas/estadística & datos numéricos , Cirugía Torácica , Florida/epidemiología , Cardiopatías Congénitas/mortalidad , Humanos , Recién Nacido , Evaluación de Resultado en la Atención de Salud , Estudios Retrospectivos , Tasa de Supervivencia/tendenciasRESUMEN
BACKGROUND: Large mediastinal teratomas in the fetus are rare and can present with direct compression of intrathoracic structures as well as pericardial and pleural effusions. Mediastinal fetal teratoma may be detected as a mass in the chest during a routine prenatal ultrasound. Because of the pressure on mediastinal structures it may result in non-immune fetal hydrops (NIFH) and polyhydramnios. The development of hydrops may lead to fetal demise. Timely obstetric and/or surgical intervention is important to improve survival in this patient population. Case review: We report a case of a large mediastinal teratoma in a fetus who presented with a large pericardial effusion at 28 weeks gestation. The fetus developed NIFH at 31 weeks gestation. The fetus was successfully managed with an ex utero intrapartum therapy (EXIT) procedure which involved pericardiocentesis and surgical resection of the large teratoma. Histological examination revealed an encapsulated immature teratoma. DISCUSSION: Mediastinal teratomas are usually detected by routine second- and third-trimester ultrasound. Large teratomas have been described to present with airway compromise and NIFH. Some cases have been managed by postnatal surgery and rare cases by EXIT procedure. CONCLUSIONS: This is the first reported case of a fetal mediastinal teratoma and severe pericardial effusion who developed NIFH, in whom EXIT procedure was successfully employed to simultaneously drain the effusion and resect the tumor.
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Hidropesía Fetal/cirugía , Neoplasias del Mediastino/cirugía , Derrame Pericárdico/cirugía , Teratoma/cirugía , Adulto , Femenino , Humanos , Hidropesía Fetal/etiología , Recién Nacido , Neoplasias del Mediastino/complicaciones , Derrame Pericárdico/etiología , Embarazo , Teratoma/complicacionesRESUMEN
Pulmonary interstitial emphysema (PIE) is a common problem in premature neonates with respiratory distress syndrome. This condition is often related to barotrauma caused by mechanical ventilation or continuous positive airway pressure applied to low birth weight neonates. The clinical diagnosis can be challenging. However, after proper diagnosis, several interventions are available for successful management. We describe an infant who developed severe PIE with recurrent pneumothoraces and development of a persistent bronchopleural fistula shortly after repair of a hypoplastic aortic arch and description of successful lobectomy with the assistance of extracorporeal support (ECMO).
Asunto(s)
Aorta Torácica/cirugía , Enfermedades de la Aorta/cirugía , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Enfisema Pulmonar/etiología , Aorta Torácica/anomalías , Aorta Torácica/diagnóstico por imagen , Enfermedades de la Aorta/congénito , Enfermedades de la Aorta/diagnóstico por imagen , Oxigenación por Membrana Extracorpórea , Humanos , Recién Nacido de Bajo Peso , Recién Nacido , Recien Nacido Prematuro , Masculino , Persona de Mediana Edad , Enfisema Pulmonar/diagnóstico por imagenRESUMEN
INTRODUCTION: The Fontan operation is the final stage of single ventricle palliation in patients with complex congenital heart disease. Fenestration in the Fontan conduit, providing an atrial level right to left shunt, has been shown to reduce early postoperative morbidity. However, there is limited data on the long-term fate of this fenestration. The aim of this study is to define the rate of spontaneous closure of the fenestration in the Fontan conduit and factors predictive of the fate of the fenestration. METHODS: This was a retrospective study reviewing the medical records of the patients who underwent fenestrated Fontan operation at our center. Preoperative, intraoperative and postoperative variables including the status of the Fontan fenestration were extracted and analyzed. RESULTS: Of 67 patients included in the study, 15 (22%) had spontaneous closure of the fenestration. Of the remaining 52 patients, 11 (20%) had procedural closure of this fenestration (10 via cardiac catheterization and 1 via surgery) at a median duration of 3 months after the Fontan operation. Patients with higher preoperative pulmonary vascular resistance and a history of postoperative systemic venous thromboembolism had higher likelihood of having persistence of the fenestration with P value of .045 and .037, respectively. CONCLUSIONS: The rate of spontaneous closure of the Fontan fenestration was 22% in our study. Elevated preoperative pulmonary vascular resistance and history of systemic venous thromboembolism are predictive of persistent Fontan fenestration.
Asunto(s)
Procedimiento de Fontan/métodos , Cardiopatías Congénitas/cirugía , Ventrículos Cardíacos/anomalías , Complicaciones Posoperatorias/epidemiología , Adolescente , Adulto , Cateterismo Cardíaco/métodos , Niño , Preescolar , Femenino , Florida/epidemiología , Estudios de Seguimiento , Cardiopatías Congénitas/diagnóstico , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/cirugía , Humanos , Incidencia , Masculino , Estudios Retrospectivos , Factores de Riesgo , Adulto JovenAsunto(s)
Ecocardiografía Transesofágica/métodos , Transposición de los Grandes Vasos/cirugía , Obstrucción del Flujo Ventricular Externo/diagnóstico por imagen , Adulto , Desfibriladores Implantables , Electrodos , Cuerpos Extraños/cirugía , Humanos , Terapia por Láser , Masculino , Monitoreo Intraoperatorio , Derrame Pericárdico/diagnóstico por imagen , Transposición de los Grandes Vasos/diagnóstico por imagenAsunto(s)
Ecocardiografía Transesofágica , Derrame Pericárdico/cirugía , Técnicas de Ventana Pericárdica , Transposición de los Grandes Vasos/diagnóstico por imagen , Adulto , Desfibriladores Implantables/efectos adversos , Válvulas Cardíacas/diagnóstico por imagen , Ventrículos Cardíacos/diagnóstico por imagen , Hemodinámica , Humanos , Marcapaso Artificial/efectos adversos , Derrame Pericárdico/diagnóstico por imagen , Derrame Pericárdico/etiología , Transposición de los Grandes Vasos/complicaciones , Transposición de los Grandes Vasos/fisiopatología , Resultado del TratamientoRESUMEN
Transmyocardial migration of a retained temporary epicardial pacing wire has been rarely reported in adult patients after heart surgery. We present the case of a child in whom a temporary epicardial pacing wire was discovered incidentally in the right ventricular outflow tract one year after surgical repair of congenital heart disease. The pacing wire was subsequently extracted using the snare method during cardiac catheterization. Clinicians caring for patients after congenital heart surgery should be aware of this uncommon though potentially life-threatening complication.
Asunto(s)
Estimulación Cardíaca Artificial , Electrodos Implantados/efectos adversos , Migración de Cuerpo Extraño/terapia , Cardiopatías Congénitas/cirugía , Cateterismo Cardíaco , Femenino , Humanos , Lactante , Cuidados Posoperatorios , Complicaciones Posoperatorias , Tomografía Computarizada por Rayos XRESUMEN
Abstract Pulmonary interstitial emphysema (PIE) is a common problem in premature neonates with respiratory distress syndrome. This condition is often related to barotrauma caused by mechanical ventilation or continuous positive airway pressure applied to low birth weight neonates. The clinical diagnosis can be challenging. However, after proper diagnosis, several interventions are available for successful management. We describe an infant who developed severe PIE with recurrent pneumothoraces and development of a persistent bronchopleural fistula shortly after repair of a hypoplastic aortic arch and description of successful lobectomy with the assistance of extracorporeal support (ECMO).
Asunto(s)
Humanos , Masculino , Recién Nacido , Persona de Mediana Edad , Aorta Torácica/cirugía , Enfermedades de la Aorta/cirugía , Enfisema Pulmonar/etiología , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Aorta Torácica/anomalías , Aorta Torácica/diagnóstico por imagen , Enfermedades de la Aorta/congénito , Enfermedades de la Aorta/diagnóstico por imagen , Enfisema Pulmonar/diagnóstico por imagen , Recién Nacido de Bajo Peso , Recien Nacido Prematuro , Oxigenación por Membrana ExtracorpóreaRESUMEN
Fontan and Baudet described in 1971 the separation of the pulmonary and systemic circulations resulting in univentricular physiology. The evolution of the Fontan procedure, most notably the substitution of right atrial-to-pulmonary artery anastomosis with cavopulmonary connections, resulted in significantly improved late outcomes. Many patients survive well into adulthood and are able to lead productive lives. While ideally under medical care at specialized centers for adult congenital cardiac pathology, these patients may present to the outside hospitals for emergency surgery, electrophysiologic interventions, and pregnancy. This presentation presents a "train of thought," linking the TEE images to the perioperative physiologic considerations faced by an anesthesiologist caring for a patient with Fontan circulation in the perioperative settings. Relevant effects of mechanical ventilation on pulmonary vascular resistance, pulmonary blood flow and cardiac preload, presence of coagulopathy and thromboembolic potential, danger of abrupt changes of systemic vascular resistance and systemic venous return are discussed.
RESUMEN
A case of a doubly committed ventricular septal defect is presented that highlights the value of 3-dimensional echocardiography in the preoperative planning of surgical closure.
RESUMEN
Supravalvar mitral ring (SVMR) is a rare cause of congenital mitral stenosis. It can occur in isolation but often coexists with other cardiac anomalies, such as a ventricular septal defect or left-sided obstructive lesions. Conversely, a complete atrioventricular septal defect (AVSD) is a much more common anomaly. An AVSD may be associated with other major cardiac defects, such as tetralogy of Fallot, transposition of the great arteries, or double-outlet right ventricle. The authors describe what they believe is the first case of SVMR and complete AVSD occurring together; the SVMR was diagnosed by two-dimensional echocardiography, and its morphology could be more accurately delineate using three-dimensional echocardiography.
Asunto(s)
Anomalías Múltiples/diagnóstico por imagen , Ecocardiografía Tridimensional/métodos , Cardiopatías Congénitas/diagnóstico por imagen , Defectos del Tabique Interatrial/diagnóstico por imagen , Defectos del Tabique Interventricular/diagnóstico por imagen , Estenosis de la Válvula Mitral/diagnóstico por imagen , Válvula Mitral/diagnóstico por imagen , Diagnóstico Diferencial , Humanos , Lactante , Masculino , Válvula Mitral/anomalíasRESUMEN
OBJECTIVES: Delayed sternal closure after pediatric cardiac surgery can temporarily impair cardiac output. Cerebral and somatic regional oxygen saturation measured by using near-infrared spectroscopy (NIRS) have been used as potential surrogates of cerebral and somatic mixed venous oxygen saturation. We hypothesized that cerebral and somatic regional oxygen saturation correlate with indicators of hemodynamic compromise after delayed sternal closure in children undergoing cardiac surgery. METHODS: We studied 36 postoperative children (median age, 10 days; range, 1-510 days) undergoing delayed sternal closure 3.7 +/- 2 days after cardiac surgery. Twenty-five had biventricular physiology, whereas 11 had single-ventricle physiology. Cerebral regional oxygen saturation, somatic regional oxygen saturation, and other physiologic parameters (hemodynamic data, respiratory data, blood gas analysis, lactate levels, and inotrope scores) were analyzed at 16 different time points 24 hours before and after sternal closure. One-way analysis of variance and the paired t test were used for statistical comparisons. RESULTS: Cerebral and somatic regional oxygen saturation decreased after delayed sternal closure compared with preclosure levels (P = .02 and P = .01, respectively). Higher heart rate (P = .03), lactate levels (P = .02), and left atrial pressure (P = .001) were also noted, suggesting mild hemodynamic compromise. Arterial pressure and inotrope score were unchanged. Somatic regional oxygen saturation returned to preclosure levels earlier in the biventricular group than in the single-ventricle group, whereas cerebral regional oxygen saturation remained decreased after sternal closure with no evidence of return to preclosure levels during the observation period. Oxygen saturation, Pao(2), and Paco(2) levels were unaffected by sternal closure, although greater positive-pressure ventilation was required (P < .01), suggesting reduced lung compliance. CONCLUSION: Cerebral and somatic regional oxygen saturation decrease after delayed sternal closure in children recovering from congenital cardiac surgery. These indices are in agreement with other physiologic indicators of cardiac performance, suggesting mild and transient hemodynamic compromise after sternal closure. Cerebral and somatic regional oxygen saturation monitoring might be a useful adjunct during delayed sternal closure.
Asunto(s)
Cardiopatías Congénitas/fisiopatología , Cardiopatías Congénitas/cirugía , Oxígeno/análisis , Esternón/cirugía , Abdomen , Análisis de los Gases de la Sangre , Química Encefálica , Gasto Cardíaco , Procedimientos Quirúrgicos Cardíacos , Hemodinámica , Humanos , Recién Nacido , Oximetría/métodos , Oxígeno/sangre , Periodo Posoperatorio , Espectroscopía Infrarroja Corta , Toracotomía , Factores de TiempoRESUMEN
Because the currently available total artificial hearts are rather bulky, the use of a custom-made paracorporeal total artificial heart constructed with 2 ventricular assist devices is an alternative for children and adults with small stature. This article reports our experience using this system in an adult and a pediatric patient. The advantages and disadvantages of this technique are discussed.
Asunto(s)
Insuficiencia Cardíaca/cirugía , Trasplante de Corazón , Corazón Artificial , Corazón Auxiliar , Adolescente , Adulto , Femenino , Insuficiencia Cardíaca/etiología , Humanos , ReoperaciónRESUMEN
Options for mechanical ventricular assistance in pediatric patients are limited. Extracorporeal membrane oxygenation is used in most cases for short-term support. The TandemHeart circulatory support system is an established device that is used in adult patients to provide short-term ventricular support. In this article, we report three children in whom a TandemHeart ventricular assist device was used for right ventricular support, two after heart transplantation and another for failed Fontan physiology. Herein, we report the novel application of this technology to pediatric patients, and we discuss the lessons learned from its utilization.
Asunto(s)
Procedimiento de Fontan/efectos adversos , Corazón Auxiliar , Disfunción Ventricular Derecha/cirugía , Adolescente , Anticoagulantes/administración & dosificación , Niño , Presión de las Vías Aéreas Positiva Contínua/métodos , Resultado Fatal , Femenino , Heparina/administración & dosificación , Humanos , Infusiones Intravenosas , Contrapulsador Intraaórtico/métodos , Masculino , Modelos Cardiovasculares , Insuficiencia Multiorgánica/fisiopatología , Tiempo de Tromboplastina Parcial/métodos , Resultado del Tratamiento , Insuficiencia de la Válvula Tricúspide/diagnóstico por imagen , Insuficiencia de la Válvula Tricúspide/fisiopatología , Ultrasonografía , Disfunción Ventricular Derecha/diagnóstico por imagenRESUMEN
OBJECTIVES: In single-ventricle physiology with aortopulmonary connection, diastolic hypotension could alter regional myocardial blood flow. Also, afterload increases could impair myocardial blood flow by increased wall tension and relative subendocardial malperfusion. This study explores the effects of acute single-ventricle physiology on regional myocardial blood flow distribution and investigates the consequences of moderate afterload augmentation on myocardial blood flow. METHODS: Single-ventricle physiology was created in 8 piglets without using bypass, and 8 animals served as a sham control group. Aortopulmonary shunt, echo-guided atrial septostomy, tricuspid valve avulsion, and pulmonary artery occlusion allowed the left ventricle to support systemic and pulmonary circulations. Afterload augmentation was produced by aortic balloon inflation. Physiologic recordings and stable-isotope microsphere determination of myocardial blood flow to the subepicardium and subendocardium were obtained at baseline and during single-ventricle physiology (at 30 minutes, 120 minutes, and afterload increase). RESULTS: Arterial oxygen content, diastolic pressure, and coronary perfusion pressure declined after creation of single-ventricle physiology (P < .05). Acute single-ventricle physiology resulted in higher myocardial blood flow (P < .05), unchanged subendocardial/subepicardial flow ratio and oxygen delivery, and lower coronary resistance (P < .01) as compared with biventricular physiology. Afterload augmentation increased coronary perfusion pressure, causing a trend for higher myocardial blood flow and oxygen delivery (P = NS), without affecting subendocardial/subepicardial flow distribution. Myocardial oxygen supply/demand balance fell in single-ventricle physiology, remaining unchanged during afterload augmentation. CONCLUSIONS: Our study demonstrates that, in acute single-ventricle physiology with aortopulmonary shunt, myocardial blood flow is maintained by lower coronary perfusion pressure. Further, single-ventricle physiology results in less favorable myocardial oxygen supply/demand balance, although normal transmural myocardial blood flow distribution is maintained. Avoidance of diastolic runoff (ventricle-pulmonary conduit) could improve coronary reserve. In our study, moderate afterload augmentation did not induce relative subendocardial malperfusion, nor did it worsen oxygen supply/demand balance.