R. Kozlik-Feldmann

Efficacy of extracorporeal membrane oxygenation in a congenital heart surgery program

SummaryBackgroundTo report our experience with extracorporeal membrane oxygenation (ECMO) in a congenital heart surgery program.MethodsSince 12/1996, 24 patients (8 newborns, 9 infants, 3 children, 4 adolescents/ adults 17–23 years), mean age 4.0±7.4 years (2 days–23 years), body weight 2.7–87 kg had ECMO as circulatory support. Indication was failure to wean from cardiopulmonary bypass in the majority of cases.ResultsMean duration of support was 3.8±2.9 d (12 h–13 d). Fourteen patients were weaned from ECMO (9 discharged), three successfully transplanted (one after switching to a pulsatile assist device). One patient on left-ventricular support required ECMO for sudden rightventricular failure (decreased). There were six deaths on ECMO due to multiorgan failure (MOV) (3) or no myocardial recovery (3). Six patients died after weaning (3 MOV, 2 myocardial failure, 1 fungal sepsis). Overall, twelve patients (50%) were discharged and are clinically well after 3.4±2.4 years (0.8–7.2 years).ConclusionIn our series, ECMO markedly reduces mortality in patients who would otherwise not survive either open heart surgery or myocardial failure of any origin and was not associated with discernible morbidity in the midterm.

Interventional embolization of a giant pulmonary arteriovenous malformation with right-left-shunt associated with hereditary hemorrhagic telangiectasia

SummaryA 46–year old woman experienced an episode of arterial desaturation despite administration of 100% oxygen during anesthetization for an elective laparoscopy. Further evaluation revealed a giant pulmonary arteriovenous malformation (PAVM) with right–left shunt associated with previously undiagnosed hereditary hemorrhagic telangiectasia (HHT, Morbus Osler– Weber–Rendu). The PAVM was treated interventionally with an Amplatzer® duct occluder. Transcatheter embolization of the PAVM was well tolerated with symptomatic and hemodynamic improvement. CT scan after six months demonstrated correct position of the duct occluder in the left pulmonary artery with nearly complete occlusion of the feeding vessel.PAVMs are rare direct communications between pulmonary arteries and veins, associated with HHT in the majority of cases and often presenting with dyspnea or major neurological complications due to paradoxic embolism. In this case report, we present a rational and stepwise diagnostic workup for this rare medical condition and show that transcatheter embolization is an appropriate treatment for larger malformations.

Normative angiographic data relating to the dimensions of the aorta and pulmonary trunk in children and adolescents.

BACKGROUND Definition of normative data of the great arteries from neonatal to adult ages may aid in assessment of the growth of cardiovascular structures, thus guiding the timing and type of intervention in patients with congenital cardiac disease. METHODS We calculated the cross-sectional areas of the arterial roots at the basal attachment of the valvar leaflets, the sinuses, and standardized distal sites using cineangiograms of 59 normal children and adolescents with mean age of 5.4 plus or minus 4.7 years and a range from 0.1 to 16 years, the children having a mean weight of 21.2 plus or minus 15.7 kilograms, with a range from 2.2 to 68 kilograms, and mean height of 108 plus or minus 35 centimetres, with a range from 43 to 184 centimetres. Values at each site were calculated averaging end-diastolic and end-systolic measurements, and indexed to body surface area. Results are expressed as the mean plus or minus the standard deviation. RESULTS The diameter of the aortic root at the basal attachment of the leaflets was 249 plus or minus 26, the midpoint of the sinuses 379 plus or minus 59, the sinutubular junction 290 plus or minus 58, the isthmus 158 plus or minus 36, the postisthmic region 152 plus or minus 33, and the descending aorta at the level of diaphragm 130 plus or minus 18 millimetres squared per metre squared. The pulmonary root measured at the basal attachment of the leaflets was 253 plus or minus 28, the midpoint of the sinuses 352 plus or minus 58, the sinutubular junction 293 plus or minus 58, the right pulmonary artery 176 plus or minus 25, the left pulmonary artery 153 plus or minus 20, and sum of right and left pulmonary arteries 330 plus or minus 37 millimetres squared per metre squared. All indexes were consistent over a wide range for body surface areas. CONCLUSIONS Definition of normative data of the great vessels may aid in the evaluation of congenital or acquired abnormalities, serving as guidelines for intervention during medical or surgical management and follow-up.

Echocardiographic assessment of left ventricular size and function in normal children from infancy to adolescence: Acoustic quantification in comparison with traditional echocardiographic techniques

AbstractThe purpose of this study was to establish normal left ventricular acoustic quantification (AQ) reference values for children from infancy to adolescence and to compare AQ-derived parameters with traditional M-mode and Doppler indices. We studied 150 normal, healthy children aged 2 days to 14 1/2 years. Left ventricular parasternal end diastolic area and left ventricular end diastolic volume calculated by AQ were linearly related to the body surface area to the 1.1 and 1.4 powers. AQ parasternal peak filling rate and atrial fractional area change in the neonatal period were 140% and 142% of the adolescents values, respectively, and decreased to 110% and 112% by 36 months of age. The duration of the rapid filling phase and the rapid filling contribution, as identified by AQ, was shorter or lower than that measured by the Doppler method. Interobserver variability of AQ parameters ranged from 8.1% for the ejection fraction to 18.2% for the peak filling rate. Manual biplane determinations of volumes were slightly higher than AQ calculations with highly significant correlations (p <0.001). Our data permit the determination of normal ranges of AQ parameters in relation to body surface area or age.

Interventional closure of two fistulas after aortic valve surgery

IntroductionParavalvular fistulas may complicate the clinical course after heart valve surgery. Medical treatment may be ineffective, repeated surgical revisions may be associated with increased morbidity and mortality.Case reportAfter valve sparing surgery in bicuspid aortic valve, a significant aorto-left atrial fistula was diagnosed in a 72-year-old patient causing heart failure and catecholamine-dependency. Due to the critical hemodynamic state, percutaneous closure was performed with an AGA Amplatzer duct occluder. Secondary to this closure, a second fistula between the aortic root and the right atrium appeared which was closed during the same procedure implanting a second duct occluder beneath the first device.ConclusionPercutaneous closure of paravalvular fistulas is feasible and a safe method for the treatment of significant shunts after valve surgery. Even in multiple fistulas, the implantation of small devices allows for a focussed interventional closure of such leaks. This procedure should be considered for such defects as it represents a safe method for the causative treatment in paravalvular lesions after valve surgery especially in patients with critical hemodynamic conditions.

ABO-Compatible Retransplantation After ABO-Incompatible Infant Heart Transplantation: Absence of Donor Specific Isohemagglutinins

Implementation of ABO‐incompatible (ABOi) pediatric heart transplantation has contributed to significant reduction in the mortality of infants on the waiting list, without increasing the risk of rejection. This has been attributed to the immature and therefore not fully competent immune system in this population group, which results in lower production of isohemagglutinins compared to older children and adults. Serial evaluations of isohemagglutinin titers in infants revealed cases with absence of donor specific anti‐blood group antibodies. However, it is currently unknown whether continuous exposure to donor antigens is necessary to prevent formation of donor specific isohemagglutinins (DSI) in recipients. We are reporting a case of an infant who underwent ABOi heart transplantation, with no evidence of DSI even 4 years after ABO‐compatible retransplantation. Hence, temporary exposure to donor antigens in infants may contribute to permanent absence of donor specific anti‐blood group antibodies, suggesting the possibility of induced permanent B cell tolerance.

Herz- und Lungentransplantation im Kindes- und Jugendalter

ZusammenfassungDie thorakale Organtransplantation im Kindes- und Jugendalter kann ein strategisches Therapieziel bei der Behandlung chronischer Krankheiten sein. Die Prognose der Herztransplantation ist günstiger als die der Lungentransplantation. Die kombinierte Herz-Lungen-Transplantation bleibt Spezialindikationen von Lungenerkrankungen mit terminalem Rechtsherzversagen vorbehalten. Vor einer Listung müssen alle konservativen und invasiven modernen Therapieoptionen für die Grunderkrankungen ausgenutzt worden sein. Aufgrund des limitierten Spenderaufkommens muss die mögliche Wartezeit in die Therapiestrategie mit einbezogen werden. Durch die Transplantation wird die Lebensqualität in der Regel erheblich verbessert. Die meisten Kinder kehren zu einem normalen Lebensrhythmus mit keiner oder nur einer geringen Limitierung ihrer körperlichen und geistigen Aktivitäten zurück. Im Normalfall ist eine volle körperliche Rehabilitation möglich. Eine sorgfältige Indikationsstellung und adäquate Auswahl von Spender und Empfänger sind Voraussetzung für den erfolgreichen Verlauf einer Transplantation. Eine lebenslange Immunsuppression mit ihren möglichen Nebenwirkungen ist notwendig.AbstractThoracic organ transplantation in childhood and adolescence may be a strategic goal of therapy in the treatment of chronic diseases. After heart transplantation the survival rates are more favorable than those of lung transplantation. Combined heart and lung transplantation is only reserved for special indications of lung disease in combination with end-stage right heart failure. All modern conservative and invasive treatment options for the underlying disease must be used before a listing. Due to the limited availability of donor organs the possible waiting time has to be included into the therapeutic strategy for the patients. After a successful transplantation a significant improvement in quality of life is usually achieved. Most children return to a normal rhythm of life with little or no limitation of their physical and mental capabilities. Normally, a full physical rehabilitation is possible. A thorough patient checkup for the indication and an adequate selection of donors and recipients is the cornerstone for a successful transplantation. Lifelong immunosuppression is required with possible side effects.

Kreislaufunterstützungssysteme bei Kindern und Jugendlichen

ZusammenfassungDie Implantation eines mechanischen Kreislaufunterstützungssystems als Überbrückung bis zu einer Herztransplantation (Bridge-to-transplant-Konzept) oder bis zur Erholung des nativen Myokards (Bridge-to-recovery-Konzept) ist bei Kindern mit Herzinsuffizienz im Endstadium die Therapie der Wahl. Dieser Beitrag soll praktisch tätigen Kinderärztinnen und Kinderärzten einen Überblick über die derzeit in der Pädiatrie gebräuchlichsten mechanischen Kreislaufunterstützungssysteme (Assist devices) geben. Die in der Pädiatrie am gebräuchlichsten Assist devices, wie die extrakorporale Membranoxygenierung (ECMO), die Zentrifugalpumpen-, das Berlin Heart-, das Medos- und das Thoratec-Unterstützungssystem, werden in ihrem technischen Aufbau und klinischen Einsatz vorgestellt.AbstractThe implantation of a mechanical circulatory support system as a stopgap measure until heart transplantation (bridge-to-transplant concept) or until recovery of the native myocardium (bridge-to-recovery concept) is the therapy of choice for children with end-stage cardiac insufficiency. This report is intended to furnish practicing pediatricians with an overview of the mechanical assist devices currently employed most commonly in pediatrics. A description is provided for the technical assembly and clinical use of the assist devices routinely applied in pediatrics such as extracorporeal membrane oxygenation (ECMO), centrifugal pump systems, and the Berlin Heart, Medos, and Thoratec assist devices.

Wolff-Parkinson-White-Syndrom Medikamentöse und interventionelle Therapiestrategien

ZusammenfassungDefinition. Atrioventrikuläre Reentry-Tachykardien im Kindesalter werden zumeist durch akzessorische Leitungsbahnen (Kent-Bündel) verursacht. Das Wolff-Parkinson-White(WPW)-Syndrom tritt im Kindesalter mit einer Häufigkeit von 0,4–1,0/1000 Kinder auf. Die betroffenen Patienten zeigen oft keine Symptome, es kann jedoch durchaus zu Herzinsuffizienz, Synkopen oder ventrikulären Herzrhythmusstörungen und kardialem Arrest kommen. Das Auftreten der Tachykardien bei Kindern mit WPW-Syndrom ist durch 2 Altersgipfel (erste 2 Lebensmonate und 5.–10. Lebensjahr) charakterisiert. Diagnose. Das klassische WPW-Syndrom zeigt im EKG eine ventrikuläre Präexzitation mit Verkürzung der PQ-Zeit und eine Verbreiterung des QRS-Komplexes mit initialer Delta-Welle. Die Herzfrequenz liegt während Tachykardien zwischen 180 und 280 Schlägen/min. Beim klassischen WPW-Syndrom finden sich bei antegrader Leitung über den AV-Knoten und retrograder Leitung über die akzessorische Bahn schmale Kammerkomplexe, während bei der selteneren antidromen Tachykardie die Erregungsleitung umgekehrt erfolgt, woraus breite Kammerkomplexe während der Tachykardie resultieren (Abb. 1). Diskussion. Die Therapiestrategien beim WPW-Syndrom werden exemplarisch anhand von 3 Kasuistiken und der Literatur diskutiert.AbstractDefinition. WPW syndrome is an ECG pattern associated with an accessory connection (Kent bundle). WPW occurs in 0,4–1,0 in 1000 children. Although most children are asymptomatic, when it is asociated with SVT, cardiac heart failure, syncope, and, rarely, ventricular fibrillation and cardiac arrest may occur. Most cases of tachycardia in children with WPW occur within the first 2 months of age and between 5 and 10 years of age. Diagnosis. The ECG has a short PQ intervall, a slurred upstroke of the QRS complex (delta wave), and prolongation of the QRS. The heart rate varies between 180 and 280 beats per minute. The classic form of SVT in WPW syndrome is a reentrant tachycardia that travels antegrade through the AV node and retrograde through the accessory pathway resulting in a narrow complex tachycardia. A wide complex tachycardia is seen when the reentry occurs with antegrade conduction through the accessory pathway and retrograde conduction through the AV node (antidromic tachycardia). Discussion. We report on the clinical course and management of three patients and also review the relevant literature.

Kurz- und mittelfristige Resultate der Herz-Lungen-Transplantation bei Kindern Erfahrungen eines einzelnen Zentrums

ZusammenfassungHintergrund: Im Endstadium kardiopulmonaler Erkrankungen ist die Herz- oder Lungen- oder Herz-Lungen-Transplantation die einzige therapeutische Option. Patienten: 7 Kinder mit kardiopulmonalen Erkrankungen im Endstadium und schwerer rechtsventrikulärer Insuffizienz (5 primäre, 1 sekundäre pulmonalarterielle Hypertension, 1 Pulmonalatresie mit VSD) erhielten in den Jahren von 1992–1998 eine Herz-Lungen-Transplantation. Nach einer mittleren Wartezeit von 7,1 Monaten (1–16 Monaten) betrug das mittlere Empfängeralter 97/12 Jahre (21/4–145/12 Jahre). Vor der Herz-Lungen-Transplantation litten alle Patienten an Dyspnoe, Synkopen oder Sauerstoffabhängigkeit. Ergebnisse: Alle Kinder überlebten die Herz-Lungen-Transplantation. Nach einer mittleren Beatmungsdauer von 23 Tagen (1–76 Tage) und einer mittleren Krankenhausaufenthaltsdauer von 64 Tagen (21–95 Tage) wurden die Patienten nach Hause entlassen. Während einer Beobachtungszeit (Stand Februar 1999) von 8–56 Monaten (im Mittel 35,7 Monate) starb 1 Kind 48 Monate nach der Herz-Lungen-Transplantation an einer Aspergillose. Zur Immunsuppression wuren Cyclosporin A oder Tacrolimus, Azathioprin oder Mycofenolatmofetil und Glukosteroide verwendet. Akute Lungenabstoßungen wurden bei 4 und akute Herzabstoßungen bei 2 Patienten beobachtet. Verdacht auf Bronchiolitis obliterans bestand bei 2 Patienten und war bei 1 Patienten nachgewiesen. Dies machte eine Einzellungenretransplantation 42 Monate nach der Herz-Lungen-Transplantation erforderlich. Die aufgetretenen Komplikationen nach Herz-Lungen-Transplantation lassen sich in unmittelbar transplantationschirurgische, infektiöse, Medikamenten-assozierte und übrige Komplikationen unterteilen. Nach der Entlassung aus dem stationären Aufenthalt waren 6 der 7 Patienten körperlich normal belastbar. Dies ermöglichte eine zuvor nicht realisierbare soziale Integration. Schlußfolgerung: Die Herz-Lungen-Transplantation ist eine therapeutische Option bei Kindern mit einer chronischen kardiopulmonalen Erkrankung im Endstadium. Sie führt zu einer Verbesserung der Lebensqualität. Akute Abstoßungen sowie Früh- und Spätkomplikationen sind erfolgreich zu behandeln. Langfristig bestimmt die Bronchiolitis obliterans als Form der chronischen Abstoßung die Überlebensrate.SummaryIntroduction: For end-stage cardiopulmonary disease in childhood combined heart-lung transplantation may be the only therapeutic option. Patients: Seven children with end-stage cardiac pulmonary disease (5 primary and 1 secondary pulmonary hypertension, 1 pulmonary atresia with VSD) underwent heart-lung transplantation between 1992 and 1998. Average recipient age was 97/12 years (23/12–145/12 years). All children were orthopnoec, syncopal, oxygen- or ventilator-dependent prior to transplantation. Results: All children survived transplantation. Average ventilation period was 23 days (1–76 days). Follow-up period was 8 to 56 months (35,7 months). One child died 48 months after transplantation due to Aspergillus infection. Cyclosporine or Tracrolimus, and Azathioprine or Mycofenolatmofetil and steroids were used for immunosuppression. Acute lung-rejection was observed in 4, and acute heart-rejection in 2 patients. Three children suffered from bronchiolitis obliterans syndrome and one underwent a single lung retransplantation 42 months after transplantation. Complications had to be divided in surgical, infectious, drug-related and other complications. After their discharge from the hospital all the patients resumed to almost normal physical activity and life style. Conclusion: Heart-lung transplantation is a therapeutic option in end-stage cardiac pulmonary disease leading to almost normal life. Rejections and complications can be managed successfully. Nevertheless carefull follow-up monitoring is necessary. Limiting factor for long-term survival are still bronchiolitis obliterans and infections.