Protein-losing enteropathy in patients with fontan operations.
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Child↗
Biomedical subjects
Brigitte Stiller
Publications and source records attributed to Brigitte Stiller.
Safe minimization of cardiopulmonary bypass circuit volume for complex cardiac surgery in a 3.7 kg neonate.
Over recent years, increasing awareness has been aroused to the hazards of the utilization of donor blood products. Particularly in neonate cardiac surgery employing cardiopulmonary bypass (CPB), the relative high priming volume of the CPB system and its adjunctive components, such as hemofilters, causes severe hemodilution and, therefore, particularly during extended perfusions, customarily requires priming of the system with autologous blood components. We report on our efforts to minimize the CPB system and adjust the perfusion strategy to the goal of transfusion-free CPB in a 3.7 kg neonate scheduled for repair of transposition of the great arteries.
Blood Transfusion↗
Pulsatile mechanical cardiac assistance in pediatric patients with the Berlin heart ventricular assist device.
Mechanical cardiac assistance for neonates, infants, children and adolescents may be accomplished with pulsatile ventricular assist devices (VAD) instead of extracorporeal membrane oxygenation or centrifugal pumps. The Berlin Heart VAD consists of extracorporeal, pneumatically driven blood pumps for pulsatile univentricular or biventricular assistance for patients of all age groups. The blood pumps are heparin-coated. The stationary driving unit (IKUS) has the required enhanced compressor performance for pediatric pump sizes. The Berlin Heart VAD was used in a total number of 424 patients from 1987 to November 2001 at our institution. In 45 pediatric patients aged 2 days-17 years the Berlin Heart VAD was applied for long-term support (1-111 days, mean 20 days). There were three patient groups: Group I: "Bridge to transplantation" with various forms of cardiomyopathy (N = 21) or chronic stages of congenital heart disease (N = 9); Group II: "Rescue" in intractable heart failure after corrective surgery for congenital disease (N = 7) or in early graft failure after heart transplantation (N = 1); and Group III: "Acute myocarditis" (N = 7) as either bridge to transplantation or bridge to recovery. Seventeen patients were transplanted after support periods of between 4 and 111 days with 12 long-term survivors, having now survived for up to 10 years. Five patients (Groups I and III) were weaned from the system with four long-term survivors. In Group II only one patient survived after successful transplantation. Prolonged circulatory support with the Berlin Heart VAD is an effective method for bridging until cardiac recovery or transplantation in the pediatric age group. Extubation, mobilization, and enteral nutrition are possible. For long-term use, the Berlin Heart VAD offers advantages over centrifugal pumps and ECMO in respect to patient mobility and safety.
Adolescent↗
Left ventricular assist device.
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Adolescent↗
Giant fibroma in the left ventricle of an infant: imaging findings in magnetic resonance imaging, echocardiography and angiography.
The diagnosis of a rare case of giant intraventricular fibroma in an infant by MRI in comparison with other imaging modalities, such as echocardiography and angiography, is discussed. For preoperative planning only MRI showed the myocardial infiltration. The myocardial blood supply of the tumor could be evaluated qualitatively by contrast-enhanced MRI, but the direct visualization of the distally located branches could only be assessed by coronary angiography. Magnetic resonance coronary angiography using the navigator technique failed to depict the distal part of the coronaries. The obstruction of the left ventricle outflow tract could be assessed and quantified by all imaging modalities.
Coronary Angiography↗
[Heart assist systems--current status].
BACKGROUND: Heart failure is the leading cause of death in the developed countries. Organ-preserving operative procedures on the failing heart like coronary artery bypass procedures or resection of left ventricular aneurysms are part of the routine care in cardiac surgery today. Terminal heart failure refractory to optimized drug therapy, however, will require a heart transplantation or the implantation of artificial blood pumps. While heart transplantation has proven to provide excellent long-term results with 10-year survival rates at 50%, it will remain a casuistic therapy, limited by the comparatively small number of procedures which can be performed. ARTIFICIAL BLOOD PUMPS: The current status of development of artificial blood pumps is represented by the broad availability of partially-implantable electric motor-driven left ventricular assist devices (LVAD), which still require a percutaneous cable for energy supply and device control from external sources while the blood pump itself resides inside the body. The recently introduced axial-flow devices (DeBakey, Jarvik 2000 and HeartMate II LVADs) deliver a continuous blood flow and obviously provide distinct advantages with regard to a reduction in size, weight and energy demands, simplified implantation technique and device control when compared to the previously available partially-implantable electric motor-driven pulsatile blood pumps (Novacor N100, TCl Heart Mate LVADs). The first systems designed for long-term or permanent use (Lion Heart LVAD, AbioCor replacement heart) are completely implantable devices featuring percutaneous energy transmission and device control. However, the latter are more complex devices and their clinical application is still limited to a small number of cases, which precludes a judgment about their potential at this time. INDICATIONS: The use of paracorporeal pneumatically accentuated blood pump systems is still indicated in cases of most severe biventricular heart failure and multiorgan failure or if only short- to mid-term circulatory support is anticipated. Well established indications for utilization of artificial blood pumps are the bridge-to-transplant procedure, which yields results comparable to primary heart transplantation, and acute cardiac failure following myocardial infarction or cardiac surgical procedures. In newborns and children, encouraging results were obtained when miniaturized blood pumps of the Berlin Heart System were utilized for heart failure in myocarditis or dilative cardiomyopathy. With advanced reliability of artificial blood pumps and in face of the high incidence of heart failure, especially in the older age group, the long-term application of artificial bloods pump appears to be justified.
Equipment Design↗
Pulsatile pediatric ventricular assist devices: Current results for bridge to transplantation.
While pulsatile ventricular assist devices have gained widespread use in adult patients awaiting heart transplantation, only very limited experience with these devices exists in the pediatric population. In the past mostly non-pulsatile systems such as ECMO have been used to support pediatric patients with heart failure for very limited periods of time. Only recently have miniaturized pulsatile devices became available. This article describes the technical characteristics of these devices which have been implanted at the Deutsches Herzzentrum Berlin since 1992. The Berlin Heart assist device has since been used in 34 children between the ages of 6 days and 16 years. We report our strategy in patient selection, perioperative care and subsequent heart transplantation. The current literature on mechanical circulatory support in children is reviewed. Copyright 1999 by W.B. Saunders Company
Journal Article↗
Current clinical status of pulsatile pediatric circulatory support.
Whereas circulatory support with pulsatile assist devices is an established therapy in adults today, it remains unusual and extremely challenging in children. Specifically designed smaller size pumps are, to date, only available in Europe. This review summarizes the experience with both adult size pumps in the U.S. and pediatric pumps in Europe. Thoratec ventricular assist devices (VADs) were implanted in 101 patients worldwide who were between 7 and 17 years of age. Survival in this group was 68.8%, which is comparable with that in adult patients and was independent of age or body size. Berlin Heart VADs, available in several sizes, were implanted at the Deutsches Herzzentrum Berlin in 45 patients between 2 days and 16 years old. Survival in patients with myocarditis and cardiomyopathy was 66.7% and 71.4%, respectively; overall survival was 48.9%. In a number of European centers, 64 children up to 16 years of age received Medos devices, which are also available in several sizes, with an overall survival of 36.2%, similar for all pump sizes. End-stage congenital heart defects or failure to wean off bypass carried a poorer prognosis than cardiomyopathies or myocarditis for all devices.
Body Constitution↗
Transfusion-free cardiac surgery with cardiopulmonary bypass in a 2.2-kg neonate.
Particularly in neonates, complex cardiac surgery employing cardiopulmonary bypass normally requires the transfusion of autologous blood components. This is predominately caused by the relatively high priming volume of the circuit with subsequent extreme hemodilution. We report on a synoptic approach to avoiding transfusions in a 2.2 kg neonate with scheduled for correction of an intracardiac total anomalous pulmonary venous connection to the coronary sinus and a persistent foramen ovale. In this patient with a preoperative hemoglobin value of 16.5 g/dL, minimization of the cardiopulmonary bypass circuit, adjustment of the perfusion technique and strict reduction of blood sampling resulted in complete avoidance of transfusions during the entire course of the operation while maintaining safe hemoglobin levels, which never fell below a concentration of 8 g/mL.
Blood Transfusion, Autologous↗
Repair of ALCAPA in a 4-kg patient followed by successful weaning and "off-pump" explantation of an apical venting pulsatile LVAD.
Even in infants and small children, ventricular assist devices have an emerging role in the treatment of congenital and postcardiotomy heart failure. Extracorporeal pneumatic pulsatile devices are considered the strategy of choice if long-lasting bridge to recovery or transplantation is expected. However, complete explantation of the device may be complicated by hemorrhage and subsequent transfusions due to the establishment of CPB. The present case demonstrates successful weaning and complete removal of an apical venting pulsatile LVAD in a 4-kg infant without the employment of CPB.
Abnormalities, Multiple↗
Management of pediatric patients after implantation of the Berlin Heart EXCOR ventricular assist device.
Within the last 16 years, the Berlin Heart EXCOR systems have been used for circulatory support in 73 infants and children up to 17 years of age (mean, 7 years) with severe circulatory failure resistant to pharmacological therapy at our institution. These were patients with cardiomyopathy, fulminant myocarditis, end-stage congenital cardiac defects, and acute heart failure after congenital heart surgery. Mean EXCOR support time was 36 days (range, 1 to 420 days). Forty-four patients (62%) survived up to transplantation or after weaning and 37 (51%) of them, including 9 infants, were discharged home. These results in patients with very advanced disease have improved significantly during the past few years as the result of technical developments and growing experience in the treatment of patients on the device and in postoperative care. The following article gives answers to five questions frequently asked about intensive care unit treatment while on the device, anticoagulation and additional medical support during left ventricular or biventricular support, the daily treatment of the children, and options for the weaning procedure.
Adolescent↗