RESUMEN
OBJECTIVE: Fontan-associated liver disease (FALD) is widely recognised as a common complication in patients long after the Fontan operation. However, data on the predictors of FALD that can guide its screening and management are lacking. The present study aimed to identify the predictors of liver cirrhosis (LC) and hepatocellular carcinoma (HCC) in post-Fontan patients. METHODS: This was a multi-institutional retrospective cohort study. Clinical data of all perioperative survivors of Fontan operation before 2011 who underwent postoperative catheterisation were collected through a retrospective chart review. RESULTS: A total of 1117 patients (538 women, 48.2%) underwent their first Fontan operation at a median age of 3.4 years. Postoperative cardiac catheterisation was conducted at a median of 1.0 year. During a median follow-up period of 10.3 years, 67 patients (6.0%) died; 181 (16.2%) were diagnosed with liver fibrosis, 67 (6.0%) with LC, 54 (4.8%) with focal nodular hyperplasia and 7 (0.6%) with HCC. On multivariable analysis, high central venous pressure (CVP) (HR, 1.28 (95% CI 1.01 to 1.63) per 3 mm Hg; p=0.042) and severe atrioventricular valve regurgitation (HR, 6.02 (95% CI 1.53 to 23.77); p=0.010) at the postoperative catheterisation were identified as independent predictors of LC/HCC. CONCLUSIONS: Patients with high CVP and/or severe atrioventricular valve regurgitation approximately 1 year after the Fontan operation are at increased risk of developing advanced liver disease in the long term. Whether therapeutic interventions to reduce CVP and atrioventricular valve regurgitation decrease the incidence of advanced liver disease requires further elucidation.
Asunto(s)
Carcinoma Hepatocelular , Procedimiento de Fontan , Cardiopatías Congénitas , Neoplasias Hepáticas , Humanos , Femenino , Preescolar , Carcinoma Hepatocelular/diagnóstico , Carcinoma Hepatocelular/epidemiología , Carcinoma Hepatocelular/etiología , Estudios Retrospectivos , Procedimiento de Fontan/efectos adversos , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/cirugía , Neoplasias Hepáticas/complicaciones , Cirrosis Hepática/complicaciones , Cirrosis Hepática/diagnóstico , Cirrosis Hepática/epidemiología , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/cirugía , Factores de Riesgo , Resultado del TratamientoAsunto(s)
Anomalías Múltiples/cirugía , Defectos del Tabique Interatrial/cirugía , Válvula Mitral/anomalías , Estenosis de la Válvula Pulmonar/cirugía , Estenosis de la Válvula Tricúspide/cirugía , Válvula Tricúspide/cirugía , Anomalías Múltiples/diagnóstico por imagen , Ventrículos Cardíacos/anomalías , Ventrículos Cardíacos/diagnóstico por imagen , Humanos , Lactante , Recién Nacido , Masculino , Válvula Mitral/diagnóstico por imagen , Válvula Mitral/cirugía , Válvula Tricúspide/anomalías , Válvula Tricúspide/diagnóstico por imagen , Estenosis de la Válvula Tricúspide/diagnóstico por imagenRESUMEN
BACKGROUND: The choice of appropriate treatment in children with aortic valvular lesions remains controversial. The purpose of this study is to assess early and late outcomes of aortic valve replacement with annular enlargement in children. METHODS: A retrospective study was conducted in 16 consecutive patients aged 0.26 to 15.9 years operated on between 1993 and 2008. Thirteen children underwent aortic valve replacement with Konno procedure (mechanical valve: 12, homograft: 1), 2 children underwent Ross procedure, and the last child underwent Nicks procedure with mechanical valve replacement. All patients undergoing mechanical valve replacement were given warfarin with a monthly international normalized ratio (INR) control. RESULTS: Overall early mortality was 12.5% (2 cases). Emergency operation was performed in these cases because of infectious endocarditis and acute cardiac failure. However, cardiopulmonary bypass (CPB) weaning could not be obtained. There was 1 late death 5 months after mechanical valve replacement. The patient developed methicillin-resistant Staphylococcus aureus (MRSA) sepsis after cleft palate repair. Reoperation was needed in 1 case. CONCLUSION: We conclude that mechanical valve replacement with aortic annular enlargement is an acceptable treatment in children. It is associated with acceptable mortality and low incidence of late events, and provides long-term survival.
Asunto(s)
Válvula Aórtica/cirugía , Prótesis Valvulares Cardíacas , Adolescente , Procedimientos Quirúrgicos Cardíacos/métodos , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos , Trasplante Homólogo , Resultado del TratamientoRESUMEN
BACKGROUND: Interrupted aortic arch (IAA) is associated with a multitude of lesions ranging from isolated ventricular septal defect to hypoplastic left ventricle or severe subaortic stenosis (SAS). Left ventricular outflow obstruction such as SAS continues to be an important factor for deciding the surgical procedure between univentricular and biventricular repairs. METHODS: A retrospective study was conducted in 8 consecutive infants aged 14 to 117 days and operated on between 2004 and 2009. Seven patients had undergone bilateral pulmonary artery banding for pulmonary high flow regulation. All patients underwent Norwood-type operation (4 with systemic to pulmonary artery shunt, 3 with right ventricle to pulmonary artery shunt, and 1 with bidirectional Glenn shunt). RESULTS: One patient died 2 months after surgery due to respiratory failure. The others were discharged in a good condition. One patient underwent Rastelli-type operation and biventricular circulation was achieved. The other 6 patients were all Fontan candidates. CONCLUSION: Satisfactory initial palliation can be achieved by Norwood-type operation for IAA with severe SAS or hypoplastic left ventricular-aortic complex.
Asunto(s)
Aorta Torácica/anomalías , Procedimientos de Norwood , Aorta Torácica/cirugía , Estenosis de la Válvula Aórtica/complicaciones , Toma de Decisiones , Femenino , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/complicaciones , Lactante , Recién Nacido , Masculino , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
We report a female infant with acute coronary syndrome after Aubert-Imai modification for transposition of the great arteries. Two months postoperatively, she visited the emergency room because of perspiration and tachypnea. She was diagnosed with acute coronary syndrome by blood chemistry examination. Cardiac catheterization revealed Aubert route stenosis, and emergency operation was performed. The lumen around the aortopulmonary window was almost totally occluded by fibrous tissue extending from the equine pericardial patch. This fibrous tissue was completely resected and the aortopulmonary window was deepened to enlarge the coronary ostium. We recommend a close follow-up including angiography after these procedures.
Asunto(s)
Síndrome Coronario Agudo/cirugía , Transposición de los Grandes Vasos/cirugía , Síndrome Coronario Agudo/etiología , Vasos Coronarios/cirugía , Femenino , Humanos , Lactante , Complicaciones Posoperatorias , ReoperaciónRESUMEN
Operative correction of tetralogy of Fallot has been performed for more than 50 years and well established. To date, the literature on reoperation has focused and indications and the long-term outcome have not been well defined. We herein report a case of pulmonary valve replacement due to ectopic ossification on expanded polytetrafluoroethylene (ePTFE) cusp in a female child who had undergone right ventricular outflow tract reconstruction for tetralogy of Fallot with pulmonary atresia. Because of severe right ventricular dilatation with end diastolic volume of 186 ml/m2 on cardiac catheterization, redo operation was planned for functional recovery of the right ventricle. Previous transannlar patch was removed and pulmonary valve replacement was performed using CEP 23 mm. A hard calcification was observed around the cusp of transannular patch. Pathological findings revealed ectopic ossification with osteoblast colonization around the ePTFE cusp. This is the 1st report of ectopic ossification on ePTFE.