RESUMEN
Background: Patients with single ventricle congenital heart disease who undergo total cavo-pulmonary anastomosis (Fontan surgery) suffer from elevated pulmonary artery pressure (PAP), which leads to multiple adverse sequelae. Traditionally, the Fontan pressures are assessed via invasive haemodynamic catheterization that exposes these medically fragile patients to the risks of vascular injury and anaesthesia. While the CardioMEM remote PAP monitor has been extensively used in adults with heart failure, the safety of this device has not been established in children. Case summary: We report safety and utility of this device in eight paediatric Fontan patients. Our patients ranged from 9 to 18 years of age. There were no reported complications related to the implantation of the CardioMEMS device in our population. Discussion: This is the first case series of safety of CardioMEMS device in paediatric Fontan Powered by Editorial Manager® and ProduXion Manager® from Aries Systems Corporation patients. Our experience indicates that the device may be safely utilized for the management of Fontan-related complications in this vulnerable population.
RESUMEN
Background: The Fontan operation is a well-described palliative procedure for functional single-ventricle patients. This population has an increased risk of thrombo-embolic events. Adequate imaging, and therefore diagnosis, requires an understanding of the unique anatomy and physiology of a Fontan. Optimal strategies for the prevention and treatment of thrombo-embolic complications in the Fontan population are poorly defined. Case summary: A 28-year-old female with a history of Ebstein anomaly of the tricuspid valve status post-Fontan presented with chest pain and acute hypoxia. Computed tomographic angiography (CTA) reported a submassive pulmonary embolism (PE). She was initiated on a heparin drip. Catheterization demonstrated elevated Fontan pressures and a large thrombus in the right lower pulmonary artery that was removed with an aspiration device. The patient was transitioned to a direct oral anticoagulant (DOAC) following the procedure. Discussion: Thrombotic complications are common in the adult Fontan population. Given the morbidity and mortality associated with this complication, the use of proper imaging techniques is imperative. Traditional CTA imaging for PE in Fontan patients often has contrast filling defects related to their anatomy and physiology. Utilization of adequate imaging techniques helps decrease cost and additional radiation exposure. In addition, it avoids inappropriate hospitalization, need for anticoagulation, and potential need for catheterization toconfirm or exclude the presence of PE. For Fontan patients with a thrombus, prior event, and no contraindications, current guidelines recommend oral anticoagulation with a vitamin K antagonist; however, there are increasing data on the use of DOACs in this population.
RESUMEN
Perforation of the atretic pulmonary valve with balloon dilation in infants with pulmonary atresia with intact ventricular septum (PA-IVS) is standard initial therapy for right-ventricular (RV) decompression. This procedure often results in adequate pulmonary blood flow, thus eliminating the need for neonatal surgery. Nonetheless, the incidence of RV outflow-tract complications and mortality for this intervention is significant. We report our experience with retrograde snare-guided radiofrequency (RF) perforation in an attempt to improve accuracy and decrease procedural complications. Medical records were reviewed for the period between March 2007 and May 2010 for all patients with PA-IVS who presented to the catheterization laboratory for attempted RF perforation in infancy. Specific details reviewed included demographics, preprocedural echocardiographic (echo) data, procedural technique and complications, pre- and post-RV pressures and pulmonary valve gradients, need for surgical intervention in the neonatal period, and short- to medium-term follow-up. Eleven neonates with PA-IVS underwent RF perforation using a retrograde snare-guided technique during the study period. The pulmonary valve was successfully perforated and the wire snared in all 11 patients. Six of 11 atretic valves were crossed on the first attempt with low energy (5 W × 2 s). No episodes of tamponade or RV/PA perforation occurred as confirmed by echocardiogram performed immediately after the procedure. There was no ductal spasm with retrograde catheter manipulations. Sequential dilation of the perforated valve was not necessary. RV pressures decreased from 169 % systemic before dilation to 93 % after dilation (p < 0.001) with a residual pulmonary valve gradient of 16 ± 6 mm Hg. Eight of 11 patients (73 %) were discharged without surgery at an average 16 days after the intervention. Three patients required Blalock-Taussig shunts to augment pulmonary blood flow. Femoral artery thrombus occurred in 4 of 11 patients treated with anticoagulation, and 2 patients had atrial arrhythmias during the procedure. No other complications occurred. No preprocedural echo parameters predicted requirement for surgical shunt before hospital discharge. Retrograde snare-guided pulmonary valve perforation in infants with PA-IVS is safe and may decrease the incidence of significant procedural complications.