RESUMEN
Coronary sinus ostial atresia (CSOA) with persistent left superior vena cava (LSVC) in the absence of an unroofed coronary sinus is a benign and rare anomaly that may be taken lightly in most instances. However, if overlooked in patients undergoing univentricular heart repair such as bidirectional Glenn or Fontan-type surgery, fatal surgical outcomes may occur due to coronary venous drainage failure. We report a case of CSOA with a persistent LSVC that was managed through coronary sinus rerouting during a total cavopulmonary connection, and provide a review of the literature regarding this rare anomaly.
Asunto(s)
Humanos , Seno Coronario , Drenaje , Procedimiento de Fontan , Corazón , Puente Cardíaco Derecho , Vena Cava SuperiorRESUMEN
Coronary arteriovenous fistula (CAVF) is a rare condition defined as an anomalous termination of the coronary arteries. The etiology of CAVF is either congenital or acquired, and iatrogenic CAVF is most commonly caused by cardiovascular surgery or percutaneous intervention. Most of the prenatally diagnosed CAVFs were related to complex heart disease, and only few cases of an isolated CAVF have been reported to date. We successfully diagnosed an isolated CAVF by fetal echocardiography at 25.3 weeks of gestation. Accurate prenatal diagnosis resulted in the prompt decision for postnatal surgical correction, and the neonate thrived well without any complications.
Asunto(s)
Humanos , Recién Nacido , Embarazo , Fístula Arteriovenosa , Anomalías de los Vasos Coronarios , Vasos Coronarios , Ecocardiografía , Cardiopatías Congénitas , Cardiopatías , Diagnóstico PrenatalRESUMEN
Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic autoimmune liver disease characterized by progressive bile duct injury. The most common symptoms of this disease include fatigue and pruritus. The diagnosis of PBC is based on cholestatic biochemical liver tests, presence of antimitochondrial antibodies, and characteristic histological biopsy findings. We report a case of a patient with PBS, who was initially suspected to be in the window period of hepatitis B by a private doctor in a local clinic based on the detection of isolated immunoglobulin M antibody against hepatitis B core antigen. The presence of this antibody is the most useful index in diagnosing acute hepatitis B (+) by immunoserological test. The final diagnosis of the patient in Good Gang-An Hospital was PBC through additional tests. The patient is receiving outpatient treatment.
Asunto(s)
Humanos , Anticuerpos , Conductos Biliares , Biopsia , Colestasis , Diagnóstico , Fatiga , Antígenos del Núcleo de la Hepatitis B , Hepatitis B , Hepatitis , Inmunoglobulina M , Inmunoglobulinas , Hígado , Cirrosis Hepática Biliar , Hepatopatías , Pacientes Ambulatorios , Prurito , República de CoreaRESUMEN
The extracardiac conduit Fontan procedure is the last surgical step in the treatment of patients with a functional single ventricle. An acquired pulmonary arteriovenous malformation may appear perioperatively or postoperatively due to an uneven hepatic flow distribution. Here we report a case of a bifurcated Y-graft Fontan operation in a 15-year-old male patient with a unilateral pulmonary arteriovenous malformation after an extracardiac conduit Fontan operation.
Asunto(s)
Adolescente , Humanos , Masculino , Malformaciones Arteriovenosas , Procedimiento de Fontan , Cardiopatías Congénitas , Enfermedades VascularesRESUMEN
No abstract available.
Asunto(s)
Urgencias Médicas , Colgajos Tisulares Libres , Gemelos SiamesesRESUMEN
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)
Preescolar , Femenino , Humanos , Desinfectantes/toxicidad , Oxigenación por Membrana Extracorpórea , Humidificadores , Pulmón/efectos de los fármacos , Enfermedades Pulmonares Intersticiales/inducido químicamente , Trasplante de Pulmón , República de Corea , Frecuencia Respiratoria , Estudios Retrospectivos , Tórax/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
Double outlet right ventricle (DORV) and transposition of the great arteries (TGA) with ventricular septal defect (VSD) and pulmonary stenosis (PS) are complex heart diseases, the treatment of which remains a surgical challenge. The Rastelli procedure is still the most commonly performed treatment. Aortic root translocation including an arterial switch operation is advantageous anatomically since it has a lower possibility of conduit blockage and the left ventricle outflow tract remains straight. This study reports successful aortic root transpositions in two patients, one with DORV with VSD and PS and one with TGA with VSD and PS. Both patients were discharged without postoperative complications.
Asunto(s)
Humanos , Operación de Switch Arterial , Arterias , Ventrículo Derecho con Doble Salida , Cardiopatías , Defectos del Tabique Interventricular , Ventrículos Cardíacos , Complicaciones Posoperatorias , Estenosis de la Válvula PulmonarRESUMEN
Hypoplastic left heart complex (HLHC) consists of less severe underdevelopment of the left ventricle without intrinsic left valvular stenosis, i.e., a subset of hypoplastic left heart syndrome (HLHS). HLHC patients may be able to undergo biventricular repair, while HLHS requires single ventricle palliation (or transplant). However, there is no consensus regarding the likelihood of favorable outcomes in neonates with HLHC selected to undergo this surgical approach. This case report describes a neonate with HLHC, co-arctation of the aorta (CoA), and patent ductus arteriosus (PDA) who was initially palliated using bilateral pulmonary artery banding due to unstable ductus-dependent circulation. A postoperative echocardiogram showed newly appearing CoA and progressively narrowing PDA, which resulted in the need for biventricular repair 21 days following the palliation surgery. The patient was discharged on postoperative day 13 without complications and is doing clinically well seven months after surgery.
Asunto(s)
Humanos , Recién Nacido , Aorta , Consenso , Constricción Patológica , Conducto Arterioso Permeable , Cardiopatías Congénitas , Ventrículos Cardíacos , Corazón , Síndrome del Corazón Izquierdo Hipoplásico , Arteria PulmonarRESUMEN
We report the case of a 37-year-old man who suffered from biventricular failure due to left isomerism, inferior vena cava interruption with azygos vein continuation, bilateral superior vena cava, double outlet of right ventricle, complete atrioventricular septal defect, pulmonary stenosis, and isolated dextrocardia. Heart transplantation in patients with systemic venous anomalies often requires the correction and reconstruction of the upper & lower venous drainage. We present a case of heart transplantation in a patient with left isomerism, highlighting technical modifications to the procedure, including the unifocalization of the caval veins and reconstruction with patch augmentation.
Asunto(s)
Adulto , Humanos , Vena Ácigos , Dextrocardia , Drenaje , Cardiopatías Congénitas , Trasplante de Corazón , Ventrículos Cardíacos , Corazón , Isomerismo , Estenosis de la Válvula Pulmonar , Venas , Vena Cava Inferior , Vena Cava SuperiorRESUMEN
Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder that is characterized by aggressive arterial and aortic disease, often involving the formation of aortic aneurysms. We describe the cases of two children with LDS who were diagnosed with aortic root aneurysms and successfully treated by valve-sparing aortic root replacement (VSRR) with a Valsalva graft. VSRR is a safe and suitable operation for children that avoids prosthetic valve replacement.
Asunto(s)
Niño , Humanos , Aneurisma , Aorta , Aneurisma de la Aorta , Enfermedades de la Aorta , Tejido Conectivo , Síndrome de Loeys-Dietz , TrasplantesRESUMEN
OBJECTIVE: To report our experiences in the prenatal diagnosis of atrial isomerism and postnatal outcomes. METHODS: A total of 80 fetuses prenatally diagnosed with atrial isomerism were retrospectively analyzed between 1999 and 2011 at a single institution. RESULTS: Of 43 fetuses with prenatally diagnosed right atrial isomerism (RAI), 40 cases were analyzed. The diagnostic accuracy was 93%. The main intracardiac anomalies in RAI were atrioventricular septal defect (AVSD), abnormal pulmonary venous connection, bilateral superior vena cava (BSVC), and pulmonary atresia. Among 28 live births, three infants were lost to follow up, and the overall survival rate was 60%. Of 37 fetuses with prenatally diagnosed left atrial isomerism (LAI), 35 were evaluated. The diagnostic accuracy was 97%. The main intracardiac anomalies in LAI were ventricular septal defect, BSVC, AVSD, double outlet right ventricle, and bradyarrhythmia. Among seven patients with bradyarrhythmia, only one showed a complete atrioventricular block. All fetuses had an interrupted inferior vena cava with azygous continuation. The overall survival rate was 90%. CONCLUSION: Our study confirms the previous findings of fetal atrial isomerism. We also demonstrates a much lower prevalence of AVSD and complete heart block in LAI and a better survival rate in RAI. Although the postnatal outcomes for RAI were worse than those for LAI, successful postnatal surgery with active management improved the survival rate.
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Humanos , Lactante , Bloqueo Atrioventricular , Bradicardia , Ventrículo Derecho con Doble Salida , Ecocardiografía , Feto , Bloqueo Cardíaco , Cardiopatías Congénitas , Defectos del Tabique Interventricular , Síndrome de Heterotaxia , Isomerismo , Nacimiento Vivo , Perdida de Seguimiento , Diagnóstico Prenatal , Prevalencia , Atresia Pulmonar , Estudios Retrospectivos , Tasa de Supervivencia , Vena Cava Inferior , Vena Cava SuperiorRESUMEN
Retroesophageal aortic arch, in which the aortic arch crosses the midline behind the esophagus to the contralateral side, is a rare form of vascular anomaly. The complete form may cause symptoms by compressing the esophagus or the trachea and need a surgical intervention. We report a rare case of a hypoplastic left heart syndrome variant with the left retroesophageal circumflex aortic arch in which the left aortic arch, retroesophageal circumflex aorta, and the right descending aorta with the aberrant right subclavian artery encircle the esophagus completely, thus causing central bronchial compression. Bilateral pulmonary artery banding and subsequent modified Norwood procedure with extensive mobilization and creation of the neo-aorta were performed. As a result of the successful translocation of the aorta, the airway compression was relieved. The patient underwent the second-stage operation and is doing well currently.
Asunto(s)
Humanos , Aorta , Aorta Torácica , Esófago , Cardiopatías Congénitas , Síndrome del Corazón Izquierdo Hipoplásico , Procedimientos de Norwood , Arteria Pulmonar , Arteria Subclavia , TráqueaRESUMEN
PURPOSE: Common conditions, such as dehydration or respiratory infection can aggravate hypoxia and are associated with interstage mortality in infants who have undergone palliative surgery for congenital heart diseases. This study was done to evaluate the efficacy of a home monitoring program (HMP) in decreasing infant mortality. METHODS: Since its inception in May 2010, all infants who have undergone palliative surgery have been enrolled in HMP. This study was a prospective observational study and infant outcomes during HMP were compared with those of previous comparison groups. Parents were trained to measure oxygen saturation, body weight and feeding volume and to contact the hospital through the hotline for emergency situations. Telephone counseling was conducted by clinical nurse specialists every week post discharge. RESULTS: Forty-one infants were enrolled in HMP. Nine hundred telephone counseling sessions were conducted. Seventy-three infants required telephone triage with the most common conditions being gastrointestinal (50.7%) and respiratory symptoms (32.9%). With HMP intervention, interstage mortality decreased from 18.6% (8/43) to 9.8% (4/41) (chi2=1.15, p=.283). CONCLUSION: Results indicate that active measures and treatments using the HMP decrease mortality rates, however further investigation is required to identify various factors that contribute to hemodynamic complications during the interstage period.
Asunto(s)
Femenino , Humanos , Lactante , Recién Nacido , Masculino , Temperatura Corporal , Peso Corporal , Cuidadores/educación , Consejo , Cardiopatías Congénitas/mortalidad , Frecuencia Cardíaca , Hospitalización , Líneas Directas , Monitoreo Fisiológico/métodos , Consumo de Oxígeno , Cuidados Paliativos , Evaluación de Programas y Proyectos de Salud , Estudios Prospectivos , Factores de TiempoRESUMEN
Various surgical techniques have been developed for the repair of an interrupted aortic arch. However, tension and Gothic arch formation at the anastomotic site have remained major problems for these techniques: Excessive tension causes arch stenosis and left main bronchus compression, and Gothic arch configuration is related to cardiovascular complications. To resolve these problems, we adopted a modified surgical technique of distal aortic arch augmentation using an autologous main pulmonary artery patch. The descending aorta was then anastomosed to the augmented aortic arch in an end-to-side manner. Here, we report two cases of interrupted aortic arch that were repaired using this technique.
Asunto(s)
Aorta Torácica , Bronquios , Constricción Patológica , Arteria PulmonarRESUMEN
BACKGROUND AND OBJECTIVES: Pulmonary arterial stenosis is a relatively common complication after corrective operation of congenital heart disease. Unilateral stenosis of pulmonary arteries could result in decrease perfusion of affected lung, pulmonary regurgitation, or elevation of right ventricular pressure. Eventually there are increasing risks of right ventricular failure, arrhythmia, or sudden death. However we have limited data of pulmonary arterial stent in paediatric population as the treatment of branch pulmonary stenosis. This study aimed at validating the effectiveness and investigating complications of pulmonary arterial stent implantation in a single institution during mid-term follow up period. SUBJECTS AND METHODS: A total of 42 patients (50 stents) were implanted for treating branch pulmonary arterial stenosis. We used cardiac catheterization for comparing diameter after stent implantation directly and lung perfusion scan indirectly. We also investigated any adverse effect relating the procedure. RESULTS: Percent stenosis of stenotic lesions were decreased from 54.1+/-10.7% to 22.8+/-12.5% (p<0.001) and degree of decrement in affected lung perfusion was declined from 22.7+/-8.0% to 10.3+/-9.0% (p<0.001) immediately and lasts during mid-term follow up period. Complication rate relating the procedure was 12% (6 out of 12) and there was no mortality case. CONCLUSION: This series showed immediate and short term effectiveness of pulmonary arterial stent in congenital heart defects. We concluded that percutaneous transcatheter implantation of pulmonary arterial stent was safe and effective during short and mid-term follow up period.
Asunto(s)
Humanos , Arritmias Cardíacas , Cateterismo Cardíaco , Catéteres Cardíacos , Cateterismo , Constricción Patológica , Muerte Súbita , Estudios de Seguimiento , Corazón , Cardiopatías Congénitas , Cardiopatías , Pulmón , Perfusión , Arteria Pulmonar , Insuficiencia de la Válvula Pulmonar , Estenosis de la Válvula Pulmonar , Stents , Presión VentricularRESUMEN
Status asthmaticus is a rare, fatal condition, especially in children. Sometimes respiratory support is insufficient with a mechanical ventilator or medical therapy for patients with status asthmaticus. In such situations, early extracorporeal membrane oxygenation application is a useful method for treating refractory respiratory failure. We report on a case of a six-year-old, male child who underwent venovenous extracorporeal membrane oxygenation support for refractory status asthmaticus.
Asunto(s)
Niño , Humanos , Masculino , Asma , Circulación Extracorporea , Oxigenación por Membrana Extracorpórea , Insuficiencia Respiratoria , Estado Asmático , Ventiladores MecánicosRESUMEN
BACKGROUND: Homograft cardiac valves and valved-conduits have been available in our institute since 1992. We sought to determine the long-term outcome after right ventricular outflow tract (RVOT) reconstruction using homografts, and risk factors for reoperation were analyzed. MATERIALS AND METHODS: We retrospectively reviewed 112 patients who had undergone repair using 116 homografts between 1992 and 2008. Median age and body weight at operation were 31.2 months and 12.2 kg, respectively. The diagnoses were pulmonary atresia or stenosis with ventricular septal defect (n=93), congenital aortic valve diseases (n=15), and truncus arteriosus (N=8). Mean follow-up duration was 79.2+/-14.8 months. RESULTS: There were 10 early and 4 late deaths. Overall survival rate was 89.6%, 88.7%, 86.1% at postoperative 1 year, 5 years and 10 years, respectively. Body weight at operation, cardiopulmonary bypass (CPB) time and aortic cross-clamping (ACC) time were identified as risk factors for death. Forty-three reoperations were performed in thirty-nine patients. Freedom from reoperation was 97.0%, 77.8%, 35.0% at postoperative 1 year, 5 years and 10 years respectively. Small-sized graft was identified as a risk factor for reoperation. CONCLUSION: Although long-term survival after RVOT reconstruction with homografts was excellent, freedom from reoperation was unsatisfactory, especially in patients who had small grafts upon initial repair. Thus, alternative surgical strategies not using small grafts may need to be considered in this subset.
Asunto(s)
Humanos , Válvula Aórtica , Peso Corporal , Puente Cardiopulmonar , Constricción Patológica , Estudios de Seguimiento , Libertad , Defectos del Tabique Interventricular , Válvulas Cardíacas , Atresia Pulmonar , Reoperación , Estudios Retrospectivos , Factores de Riesgo , Tasa de Supervivencia , Trasplante Homólogo , Trasplantes , Tronco ArterialRESUMEN
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)
Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Arritmias Cardíacas/mortalidad , Cardiomiopatías/cirugía , Rechazo de Injerto/mortalidad , Cardiopatías Congénitas/cirugía , Trasplante de Corazón , Terapia de Inmunosupresión/métodos , Infecciones/mortalidad , Complicaciones Posoperatorias , República de Corea , Estudios Retrospectivos , Donantes de Tejidos , Resultado del TratamientoRESUMEN
Extracorporeal membrane oxygenation (ECMO) has been utilized in congenital diaphragmatic hernia (CDH) patients with severe respiratory failure unresponsive to conventional medical treatment. We retrospectively reviewed 12 CDH patients who were treated using ECMO in our center between April 2008 and February 2011. The pre ECMO and on ECMO variables analyzed included gestational age, sex, birth weight, age at the time of ECMO cannulation, arterial blood gas analysis results, CDH location, timing of CDH repair operation, complications and survival. There were 9 boys and 3 girls. All patients were prenatally diagnosed. Mean gestational age was 38.8 +/- 1.7 weeks and mean birth weight was 3031 +/- 499 gram. Mean age at the time of ECMO cannulation was 29.9 +/- 28.9 hours. There were 4 patients who survived. Survivors showed higher 5 min Apgar scores (8.25 +/- 0.96 vs. 7.00 +/- 1.20, p=0.109), higher pre ECMO mean pH (7.258 +/- 0.830 vs. 7.159 +/- 0.986, p=0.073) and lower pre ECMO PaCO2 (48.2 +/- 7.9 vs. 64.8 +/- 16.1, p=0.109) without statistical significance. The hernia was located on the left side in 10 patients and the right side in 2 patients. The time interval from ECMO placement to operative repair was about 3~4 days in 5 early cases and around 24 in the remaining cases. There were 3 cases of post operative bleeding requiring re operation and 2 cases of abdominal compartment syndrome requiring abdominal fascia reopening. ECMO catheter reposition was required in 4 cases. Three cases of arterial or venous thrombosis were detected and improved with follow up. Our data suggests that ECMO therapy could save the lives of some neonates with CDH who can not be maintained on other treatment modalities. Protocolized management and accumulation of case experience might be valuable in improving outcomes for neonates with CDH treated with ECMO.
Asunto(s)
Humanos , Recién Nacido , Peso al Nacer , Análisis de los Gases de la Sangre , Cateterismo , Catéteres , Oxigenación por Membrana Extracorpórea , Fascia , Estudios de Seguimiento , Edad Gestacional , Hemorragia , Hernia , Hernia Diafragmática , Concentración de Iones de Hidrógeno , Hipertensión Intraabdominal , Insuficiencia Respiratoria , Estudios Retrospectivos , Sobrevivientes , Trombosis de la VenaRESUMEN
Postoperative intractable arrhythmia can result in high morbidity and mortality. This report describes our experiences using mechanical circulatory support (MCS) to control medically intractable arrhythmias in three pediatric patients with congenital heart disease (CHD), after palliative or total corrective open-heart surgery.