RESUMEN
We report on the case of a 13-year-old female presenting with dizziness and nausea related to high blood pressure. A complete medical evaluation revealed that the patient had middle-aortic syndrome (MAS), in which there was a severe stenosis of the abdominal aorta that affected her renal and visceral arteries. If left untreated, this syndrome may lead to serious complications such as renal insufficiency or congestive heart failure. Therefore, the decision was made to perform a thoracoabdominal surgery with surgical patch augmentation. The patient had successful postoperative recovery. We have reported this case to draw attention to the coarctation of abdominal aorta, a condition which should be considered in pediatric patients with hypertension, and to emphasize a successful treatment approach for MAS.
Asunto(s)
Aorta Abdominal , Enfermedades de la Aorta/diagnóstico , Adolescente , Enfermedades de la Aorta/complicaciones , Enfermedades de la Aorta/diagnóstico por imagen , Enfermedades de la Aorta/cirugía , Constricción Patológica , Humanos , Hipertensión/complicaciones , Masculino , Radiografía , Arteria RenalRESUMEN
Current techniques to resolve heart valve defects involve the use of prosthetic and bioprosthetic materials. These materials lack the potential to grow and are not ideal, especially not for pediatric patients. Novel techniques like tissue engineering involve the use of biodegradable polymers coated with autologous myofibroblast and endothelial cells. We inspected morphological and marker gene expression differences between cells harvested from the saphenous vein, or from veins and arteries of the umbilical cord, and the cells they are designed to replace: the interstitial and endothelial cells of the pulmonary heart valve. We assessed the extent to which the endothelial cells from the inspected sources in vitro resemble endothelial cells of human pulmonary heart valves, and we found that myofibroblast cells, respective of their source, in vitro differ from the interstitial cells from human pulmonary heart valves regarding collagen and smooth muscle alpha-actin. Therefore we conclude that the cells isolated from the saphenous veins, or from veins and arteries of the umbilical cord might be feasible cell sources for tissue engineering of heart valve for the pulmonary position.
Asunto(s)
Células Endoteliales/citología , Válvulas Cardíacas/citología , Válvula Pulmonar/citología , Ingeniería de Tejidos/métodos , Biomarcadores/análisis , Expresión Génica , Enfermedades de las Válvulas Cardíacas/terapia , Humanos , Inmunohistoquímica , Inmunofenotipificación , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Vena Safena/citología , Venas Umbilicales/citologíaRESUMEN
The number of grown-up patients with congenital heart disease (GUCH) is constantly increasing and will equal the number of children requiring surgery for congenital heart disease (CHD). Specialized centers dealing with the medical and paramedical problems of these patients are required. GUCH patients can be divided into the following groups: (1) patients with minor cardiac malformations presenting at adult age for first treatment; (2) patients presenting for correction as adults because they are either naturally balanced or were surgically palliated; (3) patients presenting for expected reoperations after correction in childhood; (4) patients requiring repair of residual defects after correction; (5) patients developing heart failure after correction or palliation of CHD requiring thoracic transplantation; and (6) patients developing acquired heart disease in addition to CHD. Special aspects of malformations frequently occurring in GUCH patients are discussed in detail. Acquired heart disease in this patient population is expected to increase in the coming decades as survivors of CHD grow old.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas/cirugía , Adulto , Procedimientos Quirúrgicos Cardíacos/métodos , Procedimientos Quirúrgicos Cardíacos/tendencias , Atención a la Salud , Cardiopatías Congénitas/patología , Trasplante de Corazón , Humanos , Admisión del Paciente , ReoperaciónRESUMEN
Chronic rejection is still the major limitation of long-term outcome of heart transplant recipients. Several recent studies demonstrated that a not negligible proportion of chronic allograft rejection episodes are not only mediated by T-cell response but also triggered by pre-transplant and de novo post-transplant donor-specific alloantibodies. This points at a fundamental role of humoral immune response mechanisms that contribute to early and late graft failure. This type of rejection is an unsolved problem solid organ transplantation because current immunosuppressive regimens are generally intended to interfere in T-cell signalling pathways. Various options for the removal of circulating alloantibodies and the prevention of alloantibody formation by B-cell depletion have been described. Nevertheless, effective standardized schemes for the treatment of antibody-mediated graft rejection have to be defined. We present a heart transplant recipient with sustained antibody-mediated graft rejection who was successfully managed with a combination treatment consisting of 3 cycles of immunoadsorption and a single-dose administration of the anti-CD20 monoclonal antibody rituximab.
Asunto(s)
Anticuerpos Monoclonales/uso terapéutico , Rechazo de Injerto/terapia , Trasplante de Corazón/efectos adversos , Inmunoterapia/métodos , Adulto , Anticuerpos Monoclonales de Origen Murino , Antígenos CD20/inmunología , Terapia Combinada , Progresión de la Enfermedad , Estudios de Seguimiento , Rechazo de Injerto/inmunología , Trasplante de Corazón/inmunología , Humanos , Técnicas de Inmunoadsorción , Masculino , Infarto del Miocardio/complicaciones , Infarto del Miocardio/diagnóstico , Infarto del Miocardio/cirugía , Medición de Riesgo , Rituximab , Índice de Severidad de la Enfermedad , Inmunología del Trasplante , Trasplante Homólogo/efectos adversos , Resultado del TratamientoRESUMEN
OBJECTIVE: Donor organ shortage in pediatric heart transplantation (HTx) is causing mortality rates of 30-50% on the waiting list. Due to immaturity of the immune system of newborns and infants, ABO-incompatible HTx may be an option to increase donor availability. We present our experience with ABO-incompatible HTx. METHODS: Three infants were transplanted ABO-incompatible since 12/2004: (1) hypoplastic left heart complex, (2) restrictive hypertrophic cardiomyopathy, (3) dilative cardiomyopathy. Age at HTx was 7, 5, and 3.5 months. All recipients had blood type O, donors were A, A, and B. Informed consent was given by parents, the ethics committee, and Eurotransplant. RESULTS: Preoperative isohemagglutinin titers were low (Patient 1: 1:4 for anti-A1, A2, B, Patient 2: 1:4, 1:1, 1:4 for anti-A1, A2, B, respectively, and Patient 3: 0 for all, but quick spin 1+ for all). Intraoperatively, plasma was separated from red blood cells and discarded up to six times until antibodies were eliminated. Immunosuppressive induction with ATG was started for 5 days. Basic immunosuppression consisted of tacrolimus, mycophenolate mofetil, and prednisone. Extubation was performed on days 15, 2, and 1, respectively. After a follow-up of 17, 16, and 12 months all patients are well, ventricular function is excellent without any acute rejection periods; Patient 1 is still on dialysis. Isohemagglutinin titers against donor blood type have disappeared in follow-up. CONCLUSIONS: ABO-incompatible cardiac transplantation shows good short-term results in young infants and seems to be a safe procedure to lower the mortality on the waiting list.
Asunto(s)
Incompatibilidad de Grupos Sanguíneos , Cardiopatías Congénitas/cirugía , Trasplante de Corazón/métodos , Sistema del Grupo Sanguíneo ABO , Tipificación y Pruebas Cruzadas Sanguíneas/métodos , Femenino , Estudios de Seguimiento , Hemaglutininas/sangre , Humanos , Inmunosupresores/uso terapéutico , Lactante , Masculino , Cuidados Posoperatorios/métodos , Periodo Posoperatorio , Cuidados Preoperatorios/métodos , Resultado del TratamientoRESUMEN
In children, systemic heart valve replacement with bioprostheses is associated with accelerated valve degeneration, and mechanical prostheses require permanent anticoagulation. Novel "biomechanical" polymeric valve prostheses ("bio" = flexible, "mechanical" = synthetic), solely made of polycarbonate urethane (PCU), were tested in vitro and in a growing animal (calf) model with the aim of improved durability without permanent anticoagulation. The trileaflet aortic prosthesis has diminished pressure loss and reduced stress and strain peaks. The asymmetric bileaflet mitral valve mimics natural nonaxial inflow. The valves underwent long-term in vitro testing and in vivo testing in growing calves for 20 weeks [mitral (7), aortic (7)] with comparison to different commercial bioprostheses [mitral (7), aortic (2)]. In vitro durability of PCU valves was proved up to 20 years. Survival of PCU valves versus bioprostheses was 7 versus 2 mitral and 5 versus 0 aortic valves, respectively. Two animals with PCU aortic valves died of pannus overgrowth causing left ventricular outflow tract obstruction. Degeneration and calcification were mild (mitral) and moderate (aortic) in PCU valves but were severe in biological valves. There was no increased thrombogenicity of the PCU valves compared to bioprostheses. The novel polymeric valve prostheses revealed superior durability compared to current bioprostheses in growing animal model without permanent anticoagulation and thus, may be a future option for pediatric patients.
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Válvula Aórtica/cirugía , Prótesis Valvulares Cardíacas/tendencias , Válvula Mitral/cirugía , Animales , Fenómenos Biomecánicos , Bovinos , Niño , Humanos , Polímeros , Diseño de PrótesisRESUMEN
Truncus arteriosus communis is a congenital heart malformation, which is usually repaired in the neonatal period or early infancy. Although results of repair are good, there is long-term morbidity caused by reoperations mainly owing to right ventricle to pulmonary artery conduit exchange or stenosis at the pulmonary artery bifurcation as recently reported for Contegra conduits. We present a new technique for complete autologous reconstruction of the aorta and the pulmonary bifurcation in truncus arteriosus type I and II.
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Aorta/cirugía , Arteria Pulmonar/cirugía , Tronco Arterial Persistente/cirugía , Anastomosis Quirúrgica , Humanos , Recién Nacido , Tronco Arterial/cirugíaRESUMEN
BACKGROUND: The development of hypertensive pulmonary vascular disease (HPVD) is considered a risk factor in the long-term course of patients with secundum atrial septal defects (ASD). The aim of this study was to assess the prevalence and histologic degree of HPVD and pulmonary hypertension in relation to preoperative clinical and hemodynamic data, intraoperative findings, and operative outcome in adults. METHODS: Lung biopsies of 75 patients, mean age 44 +/- 14 years (18-71 years), with secundum ASD or sinus venosus defect including ten patients with partial anomalous pulmonary venous return were analyzed in accordance with preoperative and intraoperative findings as well as operative outcome. Lung biopsy was performed at the time of defect closure and was classified according to Heath and Edwards. RESULTS: Structural changes of the pulmonary vasculature were found in 59% of patients; grade 3 and higher changes were present in 19%. There were no statistically significant relations between histologic findings and preoperative clinical and hemodynamic data, intraoperative findings, and operative outcome. The prevalence of moderate (32-50 mm Hg) and severe (> 50 mm Hg) systolic pulmonary hypertension was 27% and 17%, respectively. Increased systolic pulmonary arterial pressure was associated with increased pulmonary vascular resistance (p < 0.000) and patients' age (p = 0.001). Patients with a lower functional capacity had a higher prevalence of pulmonary hypertension (p = 0.011). CONCLUSIONS: The prevalence of HPVD and pulmonary hypertension in adult patients with secundum ASD or sinus venosus defect is considerable. Preoperative hemodynamic data do not predict the degree of HPVD in lung biopsy. Closure is generally advised to prevent increasing pulmonary arterial pressure and decreasing functional capacity over time.
Asunto(s)
Defectos del Tabique Interatrial/complicaciones , Hipertensión Pulmonar/etiología , Pulmón/irrigación sanguínea , Adolescente , Adulto , Anciano , Arteritis/etiología , Biopsia , Presión Sanguínea , Cateterismo Cardíaco , Progresión de la Enfermedad , Femenino , Fibrosis , Defectos del Tabique Interatrial/cirugía , Humanos , Hipertrofia , Pulmón/patología , Enfermedades Pulmonares/complicaciones , Enfermedades Pulmonares/patología , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/fisiopatología , Arteria Pulmonar/patología , Venas Pulmonares/anomalías , Venas Pulmonares/patología , Estudios Retrospectivos , Factores de Riesgo , Resultado del Tratamiento , Túnica Íntima/patología , Resistencia VascularRESUMEN
BACKGROUND: Since introduction in 1999, pulmonary valve replacement in pediatric patients with the Contegra conduit (Medtronic Inc, Minneapolis, MN) has gained widespread application with increasing enthusiasm. However, unexpected graft related adverse effects may occur. METHODS: Between April 2001 and December 2002, 29 patients (20 male; mean age, 3.39 +/- 3.66 years; range, 0.01 to 13.0 years; mean weight, 11.62 +/- 8.73 kg) underwent right ventricular outflow tract reconstruction with the Contegra conduit. Seventeen patients underwent primary repair, 8 had prior homografts, and 4 had other previous operations. RESULTS: There were no deaths. Three early graft related complications were observed. In two infants (age, 1.8 and 3.5 months; weight, 3.6 and 3.8 kg, respectively) thrombus formation at the conduit valve was detected 2 weeks postoperatively. Under anticoagulation with low-molecular-weight heparin, thrombi resolved completely in both patients. One patient (4.5 months, 4.43 kg) developed severe regurgitation due to a fibrous layer covering the inner conduit wall and required conduit exchange 3 weeks postoperatively. After a mean follow-up time of 10.2 +/- 6.4 months all patients are in good clinical condition. However, one patient with systemic right ventricular pressure developed pseudoaneurysm at both graft insertion sites and is scheduled for reoperation. Two other patients underwent balloon dilation. Freedom from reoperation and intervention at 1 year is 89.4%. With regard to regurgitation and conduit stenosis all other conduits perform well. CONCLUSIONS: Contegra conduits are an alternative to homografts for right ventricular outflow tract reconstruction. However, there is a risk of thrombus formation in small infants so that prophylactic anticoagulation may be necessary. Patients with systemic right ventricular pressure require close observation as pseudoaneurysm formation has been observed.
Asunto(s)
Bioprótesis , Prótesis Vascular , Prótesis Valvulares Cardíacas , Ventrículos Cardíacos/cirugía , Arteria Pulmonar/cirugía , Adolescente , Bioprótesis/efectos adversos , Prótesis Vascular/efectos adversos , Implantación de Prótesis Vascular/efectos adversos , Niño , Preescolar , Femenino , Cardiopatías Congénitas/cirugía , Humanos , Lactante , Masculino , Complicaciones Posoperatorias , Trombosis/etiologíaRESUMEN
OBJECTIVE: Current prosthetic heart valves necessitate permanent anticoagulation or have limited durability and impaired hemodynamic performance compared to natural valves. Recently a polymeric valve prostheses with special design for mitral position demonstrated excellent in vitro and in vivo results with improved durability and no need for permanent anticoagulation. In this study, a respective flexible polymeric aortic valve is presented and in vitro and in vivo results are reported. METHODS: The aortic prosthesis (ADIAM lifescience AG, Erkelenz, Germany) is entirely made of polycarbonaturethane. The tri-leaflet flexible prosthesis mimicks the natural aortic valve and has a diminished pressure loss and reduced stress and strain peaks at the commissures. The valve underwent long-term in vitro testing and in vivo-testing in a growing calve animal model (20 weeks, 7 aortic valves) and was compared to two different commercial bioprostheses. RESULTS: The polymeric aortic heart valve substitute demonstrated excellent in vitro and in vivo hemodynamics. Five/seven animals with aortic PCU-prostheses had an excellent clinical long-term course. The explanted valves showed a variable degree of calcification. Two of the seven animals died at 27 and 77 days due to pannus overgrowth causing severe LVOTO without degeneration of the valve itself. Both animals with commercial bioprostheses had to be sacrificed because of congestive heart failure related to structural degeneration of the bioprosthesis after 10 and 30 days of implantation. There was no increased thrombogenity of the PCU valves compared to bioprostheses. CONCLUSION: The new flexible polymeric aortic valve prosthesis is superior to current bioprostheses in animal testing.
Asunto(s)
Válvula Aórtica/cirugía , Prótesis Valvulares Cardíacas , Animales , Bioprótesis , Bovinos , Femenino , Implantación de Prótesis de Válvulas Cardíacas , Ensayo de Materiales/métodos , Poliuretanos , Diseño de Prótesis , Falla de Prótesis , Resultado del TratamientoRESUMEN
OBJECTIVE: Current prosthetic heart valves necessitate permanent anticoagulation or have limited durability and impaired hemodynamic performance compared with natural valves. We report in vivo and in vitro results with new polymeric valve prostheses that have a special design for the mitral and aortic positions. The aims are improved durability and elimination of the need for permanent anticoagulation. METHODS: The mitral and aortic prostheses (Adiam Life Science, Erkelenz, Germany) are made entirely of polycarbonate urethane (PCU). The bileaflet asymmetric mitral valve mimics natural, nonaxial inflow, which creates a left ventricular vortex, saving energy for systolic ejection of blood. The trileaflet aortic prosthesis has diminished pressure loss and reduced stress and strain peaks at the commissures. The valves were subjected to long-term in vitro testing and in vivo testing in a growing calf model (20 weeks; 7 mitral and 7 aortic valves) with comparison with 2 commercial bioprostheses (7 mitral, 2 aortic). Two-dimensional echocardiography was performed after implantation and prior to sacrifice with autopsy and valve examination. RESULTS: In vitro durability of the PCU valves was proved up to 20 years. In vivo durability and hemodynamics were superior to those of all bioprostheses. Survival of PCU valves versus bioprostheses was 7 versus 2 mitral valves and 5 versus 0 aortic valves, respectively. Two animals with PCU aortic valves died of pannus overgrowth that caused severe left ventricular outflow tract obstruction without changes in the valves. Degeneration and calcification were mild (mitral) and moderate (aortic) in PCU valves but were severe in biological valves. There was no increased thrombogenicity of the PCU valves compared with bioprostheses. CONCLUSION: The new flexible polymeric aortic and mitral valve prostheses were superior to current bioprostheses in animal testing.
Asunto(s)
Válvula Aórtica/cirugía , Prótesis Valvulares Cardíacas , Válvula Mitral/cirugía , Diseño de Equipo , Análisis de Falla de Equipo , PolímerosRESUMEN
BACKGROUND: Current heart valve prostheses are constructed mimicking the native aortic valve. Special hemodynamic characteristics of the mitral valve such as a nonaxial central inflow with creation of a left ventricular vortex have so far not been taken into account. A new polycarbonaturethane (PCU) bileaflet heart valve prosthesis with special design for the mitral position is introduced, and results of animal testing are presented. METHODS AND RESULTS: After in vitro testing, 7 PCU-prostheses and 7 commercial bioprostheses (Perimount, n=4; Mosaic, n=3) were implanted in mitral position into growing Jersey calves (age 3-5 months, weight 60-97 kg) for 20 weeks. 2-Dimensional echocardiography was performed after implantation and before sacrification. Autopsy included histologic, radiographic, and electron microscopic examination of the valves. In vitro durability was proven for >15 years. After implantation 2-dimensional-echocardiography showed no relevant gradient or regurgitation of any prosthesis. Clinical course of the animals with PCU valves was excellent. In contrast, 5 of 7 calves with bioprostheses were sacrificed after 1-9 weeks because of congestive heart failure. 2-Dimensional echocardiography of the PCU valves after 20 weeks showed mild leaflet thickening with trivial regurgitation; mean gradient was 8.1+/-5.0 mm Hg (weight: 160-170 kg). The explanted PCU prostheses revealed mild calcification and no structural degeneration. All of the Perimount bioprostheses were severely calcified and degenerated after 11+/-7 weeks. One Mosaic bioprosthesis was thrombosed after 1 week, and 2 showed severe and mild-to-moderate degeneration after 4 and 22 weeks, respectively. CONCLUSIONS: Polycarbonaturethane valve prostheses with special design for mitral position show excellent hemodynamic performance and durability in vivo. Calcification and structural changes are mild compared with bioprostheses. Controlled clinical studies are planned.
Asunto(s)
Prótesis Valvulares Cardíacas , Válvula Mitral/cirugía , Poliuretanos , Animales , Bioprótesis , Bovinos , Femenino , Válvula Mitral/diagnóstico por imagen , Válvula Mitral/patología , Radiografía , UltrasonografíaRESUMEN
BACKGROUND: Patients with absent pulmonary valve syndrome (APVS) with respiratory distress (RD) have previously had a high mortality. In 1990 we adopted a strategy of primary repair including total replacement of the aneurysmal central pulmonary arteries (PAs) for patients with RD. METHODS: Retrospective review was made of 54 consecutive patients with APVS between 1960 and 1998. Median age and weight were 4 months and 4.8 kg. RD was present in 23 patients (10 neonates, 16 required ventilation). Fifteen patients had repair with homograft replacement of the PAs and VSD closure (group 1). Twenty-seven patients had transannular patch with VSD closure with PA-plasty (group 2, n = 21) or without PA plasty (group 3, n = 6). Twelve had miscellaneous procedures (group 4); in 6 the VSD was left open. RESULTS: Operative, 1-, 5-, and 10-year survivals were 83%, 80%, 78%, and 78%, respectively. Risk factors for operative mortality in multivariate analysis were RD (p = 0.04), neonates (p = 0.02), weight less than 3 kg (p = 0.02), open VSD (p = 0.02) and surgery before 1990 (p = 0.04). Since 1990 operative mortality has decreased to 11% (p = 0.04). RD was the only time-related predictor of survival in multivariate analysis (p = 0.004). In patients with RD, survival with homograft was 73% versus 41% with other techniques (p = 0.2). Mean follow-up was 72 +/- 50 months. There were no significant differences in freedom from reintervention rates among the surgical groups (p = 0.08). CONCLUSIONS: Aggressive homograft replacement of the central pulmonary arteries has been associated with improved survival in patients with APVS especially in neonates with severe RD.