Mid-aortic syndrome presenting with unusual stenoses.

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.

Long-term self-management of anticoagulation therapy after mechanical heart valve replacement in outside trial conditions.

In this investigation, we hypothesize that quality of oral anticoagulation (OA) and long-term outcome after mechanical heart valve (MHV) replacement with self-management (Self-M) of OA is superior to conventional anticoagulation treatment (Conv-T), even in outside trial conditions. One hundred sixty patients (78.8% aortic valve replacements) were trained in international normalized ratio Self-M and 260 patients (86.

Use of human umbilical cord blood-derived progenitor cells for tissue-engineered heart valves.

Background: Tissue engineering of autologous heart valves with the potential to grow and to remodel represents a promising concept. Here we describe the use of cryopreserved umbilical cord blood-derived CD133(+) cells as a single cell source for the tissue engineering of heart valves.

Methods: After expansion and differentiation of CD133(+) cells, phenotypes were analyzed by immunohistochemistry and cryopreserved.

Mycophenolate and sirolimus as calcineurin inhibitor-free immunosuppression improves renal function better than calcineurin inhibitor-reduction in late cardiac transplant recipients with chronic renal failure.

Background: Calcineurin-inhibitor-(CNI)-induced renal failure is one major cause of morbidity in cardiac transplantation (HTx). In this prospective, randomized, multicenter trial, the impact of immunosuppressive conversion toward CNI-free (mycophenolate mofetil [MMF] and sirolimus) or a CNI-reduced immunosuppressive regimen on renal function, efficacy, and safety was evaluated.

Methods: Since 2004, 63 HTx-patients (0.

Potential cell sources for tissue engineering of heart valves in comparison with human pulmonary valve cells.

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.

Adeno-associated virus-mediated gene transfer in a rabbit vein graft model.

Background: Stenoses of venous grafts represent a major limitation in coronary artery bypass surgery. The use of viral vectors to facilitate over-expression of factors within the graft to promote long-term patency is a promising new therapeutic concept. One of the viral vector systems is the adeno-associated virus (AAV); a non-pathogenic single stranded DNA virus, which elicits only low immunological responses.

Calcineurin inhibitor withdrawal and conversion to mycophenolate mofetil and steroids in cardiac transplant recipients with chronic renal failure: a word of caution.

Background: Chronic renal failure (CRF) is a common complication of calcineurin inhibitor (CNI)-based immunosuppression following cardiac transplantation (HTx). The aim of this prospective study was to evaluate the impact of an immunosuppressive conversion from CNIs to mycophenolate mofetil (MMF) and steroids in cardiac transplant recipients with CRF on renal and cardiac graft function.

Methods: Since 1999, 12 HTx recipients (10 men; 58 +/- 3.

ALCAPA with the ectopic orifice at the non-facing sinus: successful anatomic repair by creation of an autologous extrapulmonary tunnel.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital defect. This anomaly leads to a coronary hypoperfusion phenomenon and to substantial left ventricular dysfunction caused by abnormal perfusion of the left ventricle. The optimal surgical management of such cases is not clearly established.

Caspofungin as first-line therapy for the treatment of invasive aspergillosis after thoracic organ transplantation.

Introduction: Although amphotericin was the gold standard in the treatment of invasive aspergillosis in transplant recipients, nephrotoxicity and lack of efficacy often limits its use. Itraconazole is better tolerated but less efficacious and influences immunosuppressant trough levels significantly. We report our first clinical experience with the use of caspofungin as first-line therapy in heart and lung transplant recipients with invasive aspergillosis.

Update in adult congenital cardiac surgery.

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.

Stereolithographic models for surgical planning in congenital heart surgery.

Purpose: Currently we are exploring the impact of using rapid prototyping techniques for surgical planning and intraoperative orientation during the correction of complex congenital malformation.

Description: We studied a patient with a left abnormal subclavian artery and right descending aorta as a rare cause of dyspnea and dysphagia. The patient was examined by magnetic resonance imaging angiography.

Successful management of antibody-mediated cardiac allograft rejection with combined immunoadsorption and anti-CD20 monoclonal antibody treatment: case report and literature review.

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.

Blood type incompatible cardiac transplantation in young infants.

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.

Novel "biomechanical" polymeric valve prostheses with special design for aortic and mitral position: a future option for pediatric patients?

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.

HLA-DR matching improves survival after heart transplantation: is it time to change allocation policies?

Background: HLA matching has improved outcome in kidney transplantation but is not considered in current allocation policies in heart transplantation. The aim of this single-center study was to assess the impact of HLA matching on long- term outcome after heart transplantation.

Methods: The records of 240 consecutive heart transplant recipients (time period 1995 to 2002; mean age 51.

Complete autologous reconstruction of the aorta and the pulmonary bifurcation in truncus arteriosus communis.

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.

Tissue engineering of autologous human heart valves using cryopreserved vascular umbilical cord cells.

Background: Tissue engineering of autologous heart valves with the potential to grow and to remodel represents a promising concept in pediatric cardiovascular surgery. Currently we are exploring the impact of cryopreserved human umbilical cord cells (CHUCCs) for the fabrication of tissue-engineered heart valves for patients diagnosed prenatally with congenital heart lesions, potentially enabling heart valve replacement in the early years of life.

Methods: Human umbilical cord cells were isolated from vascular segments of umbilical cords and cryopreserved in a cell bank.

Preventing cardiac allograft vasculopathy: long-term beneficial effects of mycophenolate mofetil.

Background: The impact of long-term mycophenolate mofetil (MMF) treatment on the development of cardiac allograft vasculopathy (CAV) after heart transplantation is an area of much recent interest. This study analyzed the effects of various immunosuppressive combinations, including cyclosporine (CsA), azathioprine (Aza), tacrolimus (Tac) and MMF, on the time of onset, extent and progression of CAV.

Methods: Two hundred seventy-three consecutive heart transplant recipients (mean age: 51.

Successful endothelialization of porcine glutaraldehyde-fixed aortic valves in a heterotopic sheep model.

Purpose: The purpose of our study was to evaluate the stability of an artificially seeded endothelial cell layer on porcine aortic prostheses under in vivo conditions in the arterial system.

Description: Ten female sheep were divided into two groups. Animals of the study group (n = 7) had dissection of their right external jugular vein for cell harvesting.

Hypertensive pulmonary vascular disease in adults with secundum or sinus venosus atrial septal defect.

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.